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Sample records for type 1a critical

  1. Quantitative fluorescence-polymerase chain reaction assay for the detection of the duplication of the Charcot Marie Tooth disease type 1A critical region.

    Science.gov (United States)

    De Toffol, Simona; Bellone, Emilia; Dulcetti, Francesca; Ruggeri, Anna Maria; Maggio, Pietro Paolo; Pulimeno, Maria Rosaria; Mandich, Paola; Maggi, Federico; Simoni, Giuseppe; Grati, Francesca Romana

    2010-04-01

    Charcot Marie Tooth (CMT) syndrome is the most common hereditary peripheral neuropathy, with an incidence of about 1 in 2500. The subtype 1A (CMT1A) is caused by a tandem duplication of a 1.5-Mb region encompassing the PMP22 gene. Conventional short tandem repeat (STR) analysis can reveal this imbalance if a triallelic pattern, defining with certainty the presence of duplication, is present. In case of duplication with a biallelic pattern, it can only indicate a semiquantitative dosage of the fluorescence intensity ratio of the two fragments. In this study we developed a quantitative fluorescence-PCR using seven highly informative STRs within the CMT1A critical region that successfully disclosed or excluded the presence of the pathogenic imbalance in a cohort of 60 samples including 40 DNAs from samples with the CMT1A duplication previously characterized with two different molecular approaches, and 20 diagnostic samples from 10 members of a five-generation pedigree segregating CMT1A, 8 unrelated cases and 2 prenatal samples. The application of the quantitative fluorescence-PCR using STRs located in the critical region could be a reliable method to evaluate the presence of the PMP22 duplication for the diagnosis and classification of hereditary neuropathies in asymptomatic subjects with a family history of inherited neuropathy, in prenatal samples in cases with one affected parent, and in unrelated patients with a sporadic demyelinating neuropathy with clinical features resembling CMT (i.e., pes cavus with hammer toes) or with conduction velocities in the range of CMT1A.

  2. DMPD: IRAK1: a critical signaling mediator of innate immunity. [Dynamic Macrophage Pathway CSML Database

    Lifescience Database Archive (English)

    Full Text Available 17890055 IRAK1: a critical signaling mediator of innate immunity. Gottipati S, Rao ...IRAK1: a critical signaling mediator of innate immunity. PubmedID 17890055 Title IRAK1: a critical signaling mediator

  3. Critical Wave Forms in Dry Type Transformers

    DEFF Research Database (Denmark)

    Pedersen, Kenneth; Holbøll, Joachim; Henriksen, Mogens

    2005-01-01

    This paper concerns critical wave forms in dry type transformers under transient voltage application. A very general approach has been applied, meaning that many of the results will be applicable to various types of power transformers. The results can be very useful if they are combined...... with knowledge of the ageing effects of the insulation. Especially fast transients are likely to become a major issue due to fast breakers and power electronics. In order to perform relevant research in transformer insulation with respect to this subject, fundamental knowledge about the transmission...... and distortion of incoming transients is required. Thus, in this paper it is illustrated how the resulting internal wave forms are affected by different transformer characteristics....

  4. Charcot-Marie-Tooth disease type 1A

    DEFF Research Database (Denmark)

    Hertz, Jens Michael; Børglum, A D; Brandt, C A

    1994-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) is an autosomal dominant peripheral neuropathy associated with a DNA duplication on chromosome 17p11.2-p12 in the majority of cases. Most of the sporadic cases are due to a de novo duplication. We have screened for this duplication in 11 Danish patients...

  5. Critical solution for a Hill's type problem

    International Nuclear Information System (INIS)

    Cabral, Hildeberto; Castilho, Cesar

    2001-08-01

    We studied the problem of two satellites attracted by a center of force. Assuming the motion of the center of mass of the two satellites describes a keplerian circular motion around the center of force we regularized the collision between them using the Levi-Civita procedure. The existence of a constant of motion in the extended phase space allowed us to study the stability of the solution where the two satellites are tied together in their circular motion around the center of force. We call this solution the critical solution. A theorem of M Kummer is applied to prove, in specific conditions, the existence of two one-parametric families of almost periodic orbits for the satellites motion that bifurcates from the critical solution. (author)

  6. New Type of Quantum Criticality in the Pyrochlore Iridates

    Directory of Open Access Journals (Sweden)

    Lucile Savary

    2014-11-01

    Full Text Available Magnetic fluctuations and electrons couple in intriguing ways in the vicinity of zero-temperature phase transitions—quantum critical points—in conducting materials. Quantum criticality is implicated in non-Fermi liquid behavior of diverse materials and in the formation of unconventional superconductors. Here, we uncover an entirely new type of quantum critical point describing the onset of antiferromagnetism in a nodal semimetal engendered by the combination of strong spin-orbit coupling and electron correlations, and which is predicted to occur in the iridium oxide pyrochlores. We formulate and solve a field theory for this quantum critical point by renormalization group techniques and show that electrons and antiferromagnetic fluctuations are strongly coupled and that both these excitations are modified in an essential way. This quantum critical point has many novel features, including strong emergent spatial anisotropy, a vital role for Coulomb interactions, and highly unconventional critical exponents. Our theory motivates and informs experiments on pyrochlore iridates and constitutes a singular realistic example of a nontrivial quantum critical point with gapless fermions in three dimensions.

  7. Serotonin type-1A receptor imaging in depression

    International Nuclear Information System (INIS)

    Drevets, Wayne C.; Frank, Ellen; Price, Julie C.; Kupfer, David J.; Greer, Phil J.; Mathis, Chester

    2000-01-01

    Regional 5-hydroxytryptamine 1A (5-HT 1A ) receptor binding potential (BP) of depressed subjects with primary, recurrent, familial mood disorders was compared to that of healthy controls by using positron emission tomography and [carbonyl- 11 C]WAY-100635 {[ 11 C]N-(2-(4-(2-methoxyphenyl)-1-piperazin-1-yl)ethyl)-N-(2-pyridyl) cyclohexanecarboxamide}. The mean 5-HT 1A receptor BP was reduced 42% in the midbrain raphe and 25-33% in limbic and neocortical areas in the mesiotemporal, occipital, and parietal cortex. The magnitude of these abnormalities was most prominent in bipolar depressives and unipolar depressives who had bipolar relatives. These abnormal reductions in 5-HT 1A receptor BP are consistent with in vivo evidence that 5-HT 1A receptor sensitivity is reduced in major depressive disorder and postmortem data showing a widespread deficit of 5-HT 1A receptor expression in primary mood disorders

  8. Serotonin type-1A receptor imaging in depression

    Energy Technology Data Exchange (ETDEWEB)

    Drevets, Wayne C. E-mail: drevets@pet.upmc.edu; Frank, Ellen; Price, Julie C.; Kupfer, David J.; Greer, Phil J.; Mathis, Chester

    2000-07-01

    Regional 5-hydroxytryptamine{sub 1A} (5-HT{sub 1A}) receptor binding potential (BP) of depressed subjects with primary, recurrent, familial mood disorders was compared to that of healthy controls by using positron emission tomography and [carbonyl-{sup 11}C]WAY-100635 {l_brace}[{sup 11}C]N-(2-(4-(2-methoxyphenyl)-1-piperazin-1-yl)ethyl)-N-(2-pyridyl) cyclohexanecarboxamide{r_brace}. The mean 5-HT{sub 1A} receptor BP was reduced 42% in the midbrain raphe and 25-33% in limbic and neocortical areas in the mesiotemporal, occipital, and parietal cortex. The magnitude of these abnormalities was most prominent in bipolar depressives and unipolar depressives who had bipolar relatives. These abnormal reductions in 5-HT{sub 1A} receptor BP are consistent with in vivo evidence that 5-HT{sub 1A} receptor sensitivity is reduced in major depressive disorder and postmortem data showing a widespread deficit of 5-HT{sub 1A} receptor expression in primary mood disorders.

  9. Oro-facial-digital syndrome Type 1: A case report

    Directory of Open Access Journals (Sweden)

    Kanika Singh Dhull

    2014-01-01

    Full Text Available Oro-Facial Digital Syndrome (OFDS is a generic term for group of apparently distinctive genetic diseases that affect the development of the oral cavity, facial features, and digits. One of these is OFDS type I (OFDS-I which has rarely been reported in Asian countries. This is the case report of a 13 year old patient with OFDS type I who reported to the Department of Pedodontics and Preventive Dentistry, with the complaint of discolored upper front teeth.

  10. Type II critical phenomena of neutron star collapse

    International Nuclear Information System (INIS)

    Noble, Scott C.; Choptuik, Matthew W.

    2008-01-01

    We investigate spherically symmetric, general relativistic systems of collapsing perfect fluid distributions. We consider neutron star models that are driven to collapse by the addition of an initially 'ingoing' velocity profile to the nominally static star solution. The neutron star models we use are Tolman-Oppenheimer-Volkoff solutions with an initially isentropic, gamma law equation of state. The initial values of (1) the amplitude of the velocity profile, and (2) the central density of the star, span a parameter space, and we focus only on that region that gives rise to type II critical behavior, wherein black holes of arbitrarily small mass can be formed. In contrast to previously published work, we find that--for a specific value of the adiabatic index (Γ=2)--the observed type II critical solution has approximately the same scaling exponent as that calculated for an ultrarelativistic fluid of the same index. Further, we find that the critical solution computed using the ideal-gas equations of state asymptotes to the ultrarelativistic critical solution.

  11. ASIC1A in neurons is critical for fear-related behaviors.

    Science.gov (United States)

    Taugher, R J; Lu, Y; Fan, R; Ghobbeh, A; Kreple, C J; Faraci, F M; Wemmie, J A

    2017-11-01

    Acid-sensing ion channels (ASICs) have been implicated in fear-, addiction- and depression-related behaviors in mice. While these effects have been attributed to ASIC1A in neurons, it has been reported that ASICs may also function in nonneuronal cells. To determine if ASIC1A in neurons is indeed required, we generated neuron-specific knockout (KO) mice with floxed Asic1a alleles disrupted by Cre recombinase driven by the neuron-specific synapsin I promoter (SynAsic1a KO mice). We confirmed that Cre expression occurred in neurons, but not all neurons, and not in nonneuronal cells including astrocytes. Consequent loss of ASIC1A in some but not all neurons was verified by western blotting, immunohistochemistry and electrophysiology. We found ASIC1A was disrupted in fear circuit neurons, and SynAsic1a KO mice exhibited prominent deficits in multiple fear-related behaviors including Pavlovian fear conditioning to cue and context, predator odor-evoked freezing and freezing responses to carbon dioxide inhalation. In contrast, in the nucleus accumbens ASIC1A expression was relatively normal in SynAsic1a KO mice, and consistent with this observation, cocaine conditioned place preference (CPP) was normal. Interestingly, depression-related behavior in the forced swim test, which has been previously linked to ASIC1A in the amygdala, was also normal. Together, these data suggest neurons are an important site of ASIC1A action in fear-related behaviors, whereas other behaviors likely depend on ASIC1A in other neurons or cell types not targeted in SynAsic1a KO mice. These findings highlight the need for further work to discern the roles of ASICs in specific cell types and brain sites. © 2017 John Wiley & Sons Ltd and International Behavioural and Neural Genetics Society.

  12. The vaccines for Bovine Herpesvirus Type 1: A review | Zhao ...

    African Journals Online (AJOL)

    Bovine herpesvirus type 1 (BoHV-1) is the pathogen of Infectious Bovine Rhinothracheitis (IBR) disease, causing great economic losses in the livestock industry. Vaccine is a powerful means to control the virus. Here, the review described the currently available knowledge regarding to the advance in the field of BoHV-1 ...

  13. Speech Disorders in Neurofibromatosis Type 1: A Sample Survey

    Science.gov (United States)

    Cosyns, Marjan; Vandeweghe, Lies; Mortier, Geert; Janssens, Sandra; Van Borsel, John

    2010-01-01

    Background: Neurofibromatosis type 1 (NF1) is an autosomal-dominant neurocutaneous disorder with an estimated prevalence of two to three cases per 10 000 population. While the physical characteristics have been well documented, speech disorders have not been fully characterized in NF1 patients. Aims: This study serves as a pilot to identify key…

  14. Lipoprotein composition in HNF1A-MODY: differentiating between HNF1A-MODY and type 2 diabetes.

    Science.gov (United States)

    McDonald, Tim J; McEneny, Jane; Pearson, Ewan R; Thanabalasingham, Gaya; Szopa, Magdalena; Shields, Beverley M; Ellard, Sian; Owen, Katharine R; Malecki, Maciej T; Hattersley, Andrew T; Young, Ian S

    2012-05-18

    The young-onset diabetes seen in HNF1A-MODY is often misdiagnosed as Type 2 diabetes. Type 2 diabetes, unlike HNF1A-MODY, is associated with insulin resistance and a characteristic dyslipidaemia. We aimed to compare the lipid profiles in HNF1A-MODY, Type 2 diabetes and control subjects and to determine if lipids can be used to aid the differential diagnosis of diabetes sub-type. 1) 14 subjects in each group (HNF1A-MODY, Type 2 diabetes and controls) were matched for gender and BMI. Fasting lipid profiles and HDL lipid constituents were compared in the 3 groups. 2) HDL-cholesterol was assessed in a further 267 patients with HNF1A-MODY and 297 patients with a diagnosis of Type 2 diabetes to determine its discriminative value. 1) In HNF1A-MODY subjects, plasma-triglycerides were lower (1.36 vs. 1.93 mmol/l, p = 0.07) and plasma-HDL-cholesterol was higher than in subjects with Type 2 diabetes (1.47 vs. 1.15 mmol/l, p = 0.0008), but was similar to controls. Furthermore, in the isolated HDL; HDL-phospholipid and HDL-cholesterol ester content were higher in HNF1A-MODY, than in Type 2 diabetes (1.59 vs. 1.33 mmol/L, p = 0.04 and 1.10 vs. 0.83 mmol/L, p = 0.019, respectively), but were similar to controls (1.59 vs. 1.45 mmol/L, p = 0.35 and 1.10 vs. 1.21 mmol/L, p = 0.19, respectively). 2) A plasma-HDL-cholesterol > 1.12 mmol/L was 75% sensitive and 64% specific (ROC AUC = 0.76) at discriminating HNF1A-MODY from Type 2 diabetes. The plasma-lipid profiles of HNF1A-MODY and the lipid constituents of HDL are similar to non-diabetic controls. However, HDL-cholesterol was higher in HNF1A-MODY than in Type 2 diabetes and could be used as a biomarker to aid in the identification of patients with HNF1A-MODY. Copyright © 2012 Elsevier B.V. All rights reserved.

  15. Critical temperature gradient and critical current density in thin films of a type I superconductor

    Energy Technology Data Exchange (ETDEWEB)

    Heubener, R P

    1968-12-16

    Measurements of the critical temperature gradient and the critical current density in superconducting lead films in a transverse magnetic field indicate that the critical current flows predominantly along the surface of the films and that the critical surface currents contribute only very little to the Lorentz force on a fluxoid.

  16. Coexistent Charcot-Marie-Tooth type 1A and type 2 diabetes mellitus neuropathies in a Chinese family

    Directory of Open Access Journals (Sweden)

    A-ping Sun

    2015-01-01

    Full Text Available Charcot-Marie-Tooth disease type 1A (CMT1A is caused by duplication of the peripheral myelin protein 22 (PMP22 gene on chromosome 17. It is the most common inherited demyelinating neuropathy. Type 2 diabetes mellitus is a common metabolic disorder that frequently causes predominantly sensory neuropathy. In this study, we report the occurrence of CMT1A in a Chinese family affected by type 2 diabetes mellitus. In this family, seven individuals had duplication of the PMP22 gene, although only four had clinical features of polyneuropathy. All CMT1A patients with a clinical phenotype also presented with type 2 diabetes mellitus. The other three individuals had no signs of CMT1A or type 2 diabetes mellitus. We believe that there may be a genetic link between these two diseases.

  17. Ectopic Calcification as Discernible Manifestation in Neonates with Pseudohypoparathyroidism Type 1a

    Directory of Open Access Journals (Sweden)

    Masanori Adachi

    2009-01-01

    Full Text Available The diagnosis of pseudohypoparathyroidism type 1a (PHP1a is challenging, because both the osteodystrophy, such as brachydactyly and round face, and the symptomatic hypocalcemia usually develop beyond infancy. Although ectopic calcification may be an early sign of PHP1a, there are no systematic reviews regarding the time of its appearance. We here report on two PHP1a patients who presented with subcutaneous calcification in neonatal period.

  18. Evaluation of central nervous system in patients with glycogen storage disease type 1a.

    Science.gov (United States)

    Aydemir, Yusuf; Gürakan, Figen; Saltık Temizel, İnci Nur; Demir, Hülya; Oğuz, Kader Karlı; Yalnızoğlu, Dilek; Topçu, Meral; Özen, Hasan; Yüce, Aysel

    2016-01-01

    We aimed to evaluate structure and functions of central nervous system (CNS) in children with glycogen storage disease (GSD) type 1a. Neurological examination, psychometric tests, electroencephalography (EEG), magnetic resonance imaging (MRI), visual evoked potentials (VEP) and brainstem auditory evoked potentials (BAEP) were performed. The results were compared between patients with good and poor metabolic control and healthy children. Twenty-three patients with GSD type 1a were studied. Twelve patients were in poor metabolic control group and 11 patients in good metabolic control group. Five patients had intellectual disability, 10 had EEG abnormalities, seven had abnormal VEP and two had abnormal BAEP results. MRI was abnormal in five patients. There was significant correlation between the number of hypoglycemic attacks and MRI abnormalities. Central nervous system may be affected in GSD type 1a even in patients with normal neurologic examination. Accumulation of abnormal results in patients with poor metabolic control supports the importance of metabolic control in GSD type 1a.

  19. Case report of novel CACNA1A gene mutation causing episodic ataxia type 2

    Directory of Open Access Journals (Sweden)

    David Alan Isaacs

    2017-05-01

    Full Text Available Background: Episodic ataxia type 2 (OMIM 108500 is an autosomal dominant channelopathy characterized by paroxysms of ataxia, vertigo, nausea, and other neurologic symptoms. More than 50 mutations of the CACNA1A gene have been discovered in families with episodic ataxia type 2, although 30%–50% of all patients with typical episodic ataxia type 2 phenotype have no detectable mutation of the CACNA1A gene. Case: A 46-year-old Caucasian man, with a long history of bouts of imbalance, vertigo, and nausea, presented to our hospital with 2 weeks of ataxia and headache. Subsequent evaluation revealed a novel mutation in the CACNA1A gene: c.1364 G > A Arg455Gln. Acetazolamide was initiated with symptomatic improvement. Conclusion: This case report expands the list of known CACNA1A mutations associated with episodic ataxia type 2.

  20. Hand involvement in children with Charcot-Marie-Tooth disease type 1A

    NARCIS (Netherlands)

    Burns, Joshua; Bray, Paula; Cross, Lauren A.; North, Kathryn N.; Ryan, Monique M.; Ouvrier, Robert A.

    2008-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A), a demyelinating neuropathy characterised by progressive length-dependent muscle weakness and atrophy, is thought to affect the foot and leg first followed some time later by hand weakness and dysfunction. We aimed to characterise hand Strength, function

  1. Apolipoprotein M can discriminate HNF1A-MODY from Type 1 diabetes.

    Science.gov (United States)

    Mughal, S A; Park, R; Nowak, N; Gloyn, A L; Karpe, F; Matile, H; Malecki, M T; McCarthy, M I; Stoffel, M; Owen, K R

    2013-02-01

    Missed diagnosis of maturity-onset diabetes of the young (MODY) has led to an interest in biomarkers that enable efficient prioritization of patients for definitive molecular testing. Apolipoprotein M (apoM) was suggested as a biomarker for hepatocyte nuclear factor 1 alpha (HNF1A)-MODY because of its reduced expression in Hnf1a(-/-) mice. However, subsequent human studies examining apoM as a biomarker have yielded conflicting results. We aimed to evaluate apoM as a biomarker for HNF1A-MODY using a highly specific and sensitive ELISA. ApoM concentration was measured in subjects with HNF1A-MODY (n = 69), Type 1 diabetes (n = 50), Type 2 diabetes (n = 120) and healthy control subjects (n = 100). The discriminative accuracy of apoM and of the apoM/HDL ratio for diabetes aetiology was evaluated. Mean (standard deviation) serum apoM concentration (μmol/l) was significantly lower for subjects with HNF1A-MODY [0.86 (0.29)], than for those with Type 1 diabetes [1.37 (0.26), P = 3.1 × 10(-18) ) and control subjects [1.34 (0.22), P = 7.2 × 10(-19) ). There was no significant difference in apoM concentration between subjects with HNF1A-MODY and Type 2 diabetes [0.89 (0.28), P = 0.13]. The C-statistic measure of discriminative accuracy for apoM was 0.91 for HNF1A-MODY vs. Type 1 diabetes, indicating high discriminative accuracy. The apoM/HDL ratio was significantly lower in HNF1A-MODY than other study groups. However, this ratio did not perform well in discriminating HNF1A-MODY from either Type 1 diabetes (C-statistic = 0.79) or Type 2 diabetes (C-statistic = 0.68). We confirm an earlier report that serum apoM levels are lower in HNF1A-MODY than in controls. Serum apoM provides good discrimination between HNF1A-MODY and Type 1 diabetes and warrants further investigation for clinical utility in diabetes diagnostics. © 2012 The Authors. Diabetic Medicine © 2012 Diabetes UK.

  2. Lower critical field of an anisotropic type-II superconductor

    International Nuclear Information System (INIS)

    Klemm, R.A.; Clem, J.R.

    1980-01-01

    We consider the Ginzburg-Landau free energy of the anisotropic mass form in the presence of a magnetic field of arbitrary fixed direction. It is shown that the free energy may be transformed into the isotropic Ginsburg-Landau form with a kappa that depends upon the direction of the magnetic induction B relative to the crystal lattice. The lower critical field H/sub c/1 is then found for arbitrary direction of B. For highly anisotropic crystals the angular dependence of H/sub c/1 can exhibit a discontinuity or a cusp. The special case of a crystal with uniaxial symmetry is considered in detail

  3. Synoptic weather types associated with critical fire weather

    Science.gov (United States)

    Mark J. Schroeder; Monte Glovinsky; Virgil F. Hendricks; Frank C. Hood; Melvin K. Hull; Henry L. Jacobson; Robert Kirkpatrick; Daniel W. Krueger; Lester P. Mallory; Albert G. Oeztel; Robert H. Reese; Leo A. Sergius; Charles E. Syverson

    1964-01-01

    Recognizing that weather is an important factor in the spread of both urban and wildland fires, a study was made of the synoptic weather patterns and types which produce strong winds, low relative humidities, high temperatures, and lack of rainfall--the conditions conducive to rapid fire spread. Such historic fires as the San Francisco fire of 1906, the Berkeley fire...

  4. Kawasaki dynamics with two types of particles : critical droplets

    NARCIS (Netherlands)

    Hollander, den W.Th.F.; Nardi, F.R.; Troiani, A.

    2012-01-01

    This is the third in a series of three papers in which we study a two-dimensional lattice gas consisting of two types of particles subject to Kawasaki dynamics at low temperature in a large finite box with an open boundary. Each pair of particles occupying neighboring sites has a negative binding

  5. Kawasaki dynamics with two types of particles : critical droplets

    NARCIS (Netherlands)

    Hollander, den W.Th.F.; Nardi, F.R.; Troiani, A.

    2012-01-01

    This is the third in a series of three papers in which we study a two-dimensional lattice gas consisting of two types of particles subject to Kawasaki dynamics at low temperature in a large ¿nite box with an open boundary. Each pair of particles occupying neighboring sites has a negative binding

  6. Critical behavior of the Lyapunov exponent in type-III intermittency

    Energy Technology Data Exchange (ETDEWEB)

    Alvarez-Llamoza, O. [Departamento de Fisica, FACYT, Universidad de Carabobo, Valencia (Venezuela); Centro de Fisica Fundamental, Grupo de Caos y Sistemas Complejos, Universidad de Los Andes, Merida 5251, Merida (Venezuela)], E-mail: llamoza@ula.ve; Cosenza, M.G. [Centro de Fisica Fundamental, Grupo de Caos y Sistemas Complejos, Universidad de Los Andes, Merida 5251, Merida (Venezuela); Ponce, G.A. [Departamento de Fisica, Universidad Nacional Autonoma de Honduras (Honduras); Departamento de Ciencias Naturales, Universidad Pedagogica Nacional Francisco Morazan, Tegucigalpa (Honduras)

    2008-04-15

    The critical behavior of the Lyapunov exponent near the transition to robust chaos via type-III intermittency is determined for a family of one-dimensional singular maps. Critical boundaries separating the region of robust chaos from the region where stable fixed points exist are calculated on the parameter space of the system. A critical exponent {beta} expressing the scaling of the Lyapunov exponent is calculated along the critical curve corresponding to the type-III intermittent transition to chaos. It is found that {beta} varies on the interval 0 {<=} {beta} < 1/2 as a function of the order of the singularity of the map. This contrasts with earlier predictions for the scaling behavior of the Lyapunov exponent in type-III intermittency. The variation of the critical exponent {beta} implies a continuous change in the nature of the transition to chaos via type-III intermittency, from a second-order, continuous transition to a first-order, discontinuous transition.

  7. Transcriptional activation by the E1A regions of adenovirus types 40 and 41

    NARCIS (Netherlands)

    Loon, A.E. van; Gilardi, P.; Perricaudet, M.; Rozijn, Th. H.; Sussenbach, J.S.

    In order to establish whether the poor growth of the two fastidious adenoviruses types 40 and 41 (Ad40 and Ad41) in HeLa cells is due to a reduced trans-activation by the early region to (E1A), we have determined the trans-activating effect of this region on the expression of the chloramphenicol

  8. Reduced post-synaptic serotonin type 1A receptor binding in bipolar depression

    Science.gov (United States)

    Nugent, Allison C.; Bain, Earle E.; Carlson, Paul J.; Neumeister, Alexander; Bonne, Omer; Carson, Richard E.; Eckelman, William; Herscovitch, Peter; Zarate, Carlos A.; Charney, Dennis S.; Drevets, Wayne C.

    2013-01-01

    Multiple lines of evidence suggest that serotonin type 1A (5-HT1A) receptor dysfunction is involved in the pathophysiology of mood disorders, and that alterations in 5-HT1A receptor function play a role in the mechanisms of antidepressant and mood stabilizer treatment. The literature is in disagreement, however, as to whether 5-HT1A receptor binding abnormalities exist in bipolar disorder (BD). We acquired PET images of 5-HT1A receptor binding in 26 unmedicated BD subjects and 37 healthy controls using [18F]FCWAY, a highly selective 5-HT1A receptor radio-ligand. The mean 5-HT1A receptor binding potential (BPP) was significantly lower in BD subjects compared to controls in cortical regions where 5-HT1A receptors are expressed post-synaptically, most prominently in the mesiotemporal cortex. Post-hoc assessments involving other receptor specific binding parameters suggested that this difference particularly affected the females with BD. The mean BPP did not differ between groups in the raphe nucleus, however, where 5-HT1A receptors are predominantly expressed pre-synaptically. Across subjects the BPP in the mesiotemporal cortex was inversely correlated with trough plasma cortisol levels, consistent with preclinical literature indicating that hippocampal 5-HT1A receptor expression is inhibited by glucocorticoid receptor stimulation. These findings suggest that 5-HT1A receptor binding is abnormally reduced in BD, and this abnormality may particularly involve the postsynaptic 5-HT1A receptor system of individuals with a tendency toward cortisol hypersecretion. PMID:23434290

  9. The skeletal phenotype of achondrogenesis type 1A is caused exclusively by cartilage defects.

    Science.gov (United States)

    Bird, Ian M; Kim, Susie H; Schweppe, Devin K; Caetano-Lopes, Joana; Robling, Alexander G; Charles, Julia F; Gygi, Steven P; Warman, Matthew L; Smits, Patrick J

    2018-01-08

    Inactivating mutations in the ubiquitously expressed membrane trafficking component GMAP-210 (encoded by Trip11 ) cause achondrogenesis type 1A (ACG1A). ACG1A is surprisingly tissue specific, mainly affecting cartilage development. Bone development is also abnormal, but as chondrogenesis and osteogenesis are closely coupled, this could be a secondary consequence of the cartilage defect. A possible explanation for the tissue specificity of ACG1A is that cartilage and bone are highly secretory tissues with a high use of the membrane trafficking machinery. The perinatal lethality of ACG1A prevents investigating this hypothesis. We therefore generated mice with conditional Trip11 knockout alleles and inactivated Trip11 in chondrocytes, osteoblasts, osteoclasts and pancreas acinar cells, all highly secretory cell types. We discovered that the ACG1A skeletal phenotype is solely due to absence of GMAP-210 in chondrocytes. Mice lacking GMAP-210 in osteoblasts, osteoclasts and acinar cells were normal. When we inactivated Trip11 in primary chondrocyte cultures, GMAP-210 deficiency affected trafficking of a subset of chondrocyte-expressed proteins rather than globally impairing membrane trafficking. Thus, GMAP-210 is essential for trafficking specific cargoes in chondrocytes but is dispensable in other highly secretory cells. © 2018. Published by The Company of Biologists Ltd.

  10. Phosphodiesterase type 5 inhibitor abuse: a critical review.

    Science.gov (United States)

    Lowe, Gregory; Costabile, Raymond

    2011-06-01

    Abuse of sildenafil has been reported since its introduction in 1999 and commonly documented in combination with illicit drugs among men and women of all ages. Increased risks of sexually transmissible diseases including HIV have been associated with sildenafil use in men who have sex with men. Recognizing the abuse potential of phosphodiesterase type 5 inhibitors (PDE5), we aim to summarize the current knowledge of this abuse. An investigation of EMBASE, PubMed, the Food and Drug Administration (FDA) website, MedWatch, and search engines was performed to evaluate information regarding sildenafil, tadalafil, and vardenafil abuse. The EMBASE search provided 46 articles fitting the search criteria and evaluation led to 21 separate publications with specific information regarding PDE5 abuse. A PubMed search found 10 additional publications. MedWatch reported 44 separate warnings since 2000, most of which reported contamination of herbal products with active drug components. Few reports of abuse were among the 14,818 reports in the FDA AERS for sildenafil. A search for "internet drug store" revealed 6.4 million hits and of 7000 internet pharmacies identified by the Verified Internet Pharmacy Practice Sites Program (VIPPS) only 4% were in proper compliance. The role internet pharmacies play in counterfeit PDE5 or abuse is not well documented; however based on easy access, direct patient marketing, and low advertised cost it is likely this role is underreported. Currently the best recommendation for providers is to recognize the possibility of abuse and to educate patients on risks of this behavior.

  11. Mood stabilizer treatment increases serotonin type 1A receptor binding in bipolar depression

    Science.gov (United States)

    Nugent, Allison C; Carlson, Paul J; Bain, Earle E; Eckelman, William; Herscovitch, Peter; Manji, Husseini; Zarate, Carlos A; Drevets, Wayne C

    2013-01-01

    Abnormal serotonin type 1A (5-HT1A) receptor function and binding have been implicated in the pathophysiology of mood disorders. Preclinical studies have consistently shown that stress decreases the gene expression of 5-HT1A receptors in experimental animals, and that the associated increase in hormone secretion plays a crucial role in mediating this effect. Chronic administration of the mood stabilizers lithium and divalproex (valproate semisodium) reduces glucocorticoid signaling and function in the hippocampus. Lithium has further been shown to enhance 5-HT1A receptor function. To assess whether these effects translate to human subject with bipolar disorder (BD), positron emission tomography (PET) and [18F]trans-4-fluoro-N-(2-[4-(2-methoxyphenyl) piperazino]-ethyl)-N-(2-pyridyl) cyclohexanecarboxamide ([18F]FCWAY) were used to acquire PET images of 5-HT1A receptor binding in 10 subjects with BD, before and after treatment with lithium or divalproex. Mean 5-HT1A binding potential (BPP) significantly increased following mood stabilizer treatment, most prominently in the mesiotemporal cortex (hippocampus plus amygdala). When mood state was also controlled for, treatment was associated with increases in BPP in widespread cortical areas. These preliminary findings are consistent with the hypothesis that these mood stabilizers enhance 5-HT1A receptor expression in BD, which may underscore an important component of these agents' mechanism of action. PMID:23926239

  12. Eating behaviors in obese children with pseudohypoparathyroidism type 1a: a cross-sectional study.

    Science.gov (United States)

    Wang, Lulu; Shoemaker, Ashley H

    2014-01-01

    Children with pseudohypoparathyroidism type 1a (PHP-1a) develop early-onset obesity. These children have decreased resting energy expenditure but it is unknown if hyperphagia contributes to their obesity. We conducted a survey assessment of patients 2 to 12 years old with PHP-1a and matched controls using the Hyperphagia Questionnaire (HQ) and Children's Eating Behavior Questionnaire (CEBQ). Results of the PHP-1a group were also compared with an obese control group and normal weight sibling group. We enrolled 10 patients with PHP-1a and 9 matched controls. There was not a significant difference between the PHP-1a group and matched controls for total HQ score (p = 0.72), Behavior (p = 0.91), Drive (p = 0.48) or Severity (p = 0.73) subset scores. There was also no difference between the PHP-1a group and matched controls on the CEBQ. In a secondary analysis, the PHP-1a group was compared with obese controls (n = 30) and normal weight siblings (n = 6). Caregivers reported an increased interest in food before age 2 years in 6 of 10 PHP-1a patients (60%), 9 of 30 obese controls (30%) and none of the siblings (p = 0.04). The sibling group had a significantly lower Positive Eating Behavior score than the PHP-1a group (2.6 [2.4, 2.9] vs. 3.5 [3.1, 4.0], p PHP-1a and obese controls (p = 0.35). The sibling group had a lower Desire to Drink score than both the PHP-1a group (1.8 [1.6, 2.7] vs. 4.3 [3.3, 5.0], p PHP-1a and obese control Desire to Drink scores (p = 0.11). Patients with PHP-1a demonstrate hyperphagic symptoms similar to matched obese controls.

  13. Troglitazone stimulates β-arrestin-dependent cardiomyocyte contractility via the angiotensin II type 1A receptor

    International Nuclear Information System (INIS)

    Tilley, Douglas G.; Nguyen, Anny D.; Rockman, Howard A.

    2010-01-01

    Peroxisome proliferator-activated receptor γ (PPARγ) agonists are commonly used to treat cardiovascular diseases, and are reported to have several effects on cardiovascular function that may be due to PPARγ-independent signaling events. Select angiotensin receptor blockers (ARBs) interact with and modulate PPARγ activity, thus we hypothesized that a PPARγ agonist may exert physiologic effects via the angiotensin II type 1 A receptor (AT1 A R). In AT1 A R-overexpressing HEK 293 cells, both angiotensin II (Ang II) and the PPARγ agonist troglitazone (Trog) enhanced AT1 A R internalization and recruitment of endogenous β-arrestin1/2 (βarr1/2) to the AT1 A R. A fluorescence assay to measure diacylglycerol (DAG) accumulation showed that although Ang II induced AT1 A R-G q protein-mediated DAG accumulation, Trog had no impact on DAG generation. Trog-mediated recruitment of βarr1/2 was selective to AT1 A R as the response was prevented by an ARB- and Trog-mediated βarr1/2 recruitment to β1-adrenergic receptor (β1AR) was not observed. In isolated mouse cardiomyocytes, Trog increased both % and rate of cell shortening to a similar extent as Ang II, effects which were blocked with an ARB. Additionally, these effects were found to be βarr2-dependent, as cardiomyocytes isolated from βarr2-KO mice showed blunted contractile responses to Trog. These findings show for the first time that the PPARγ agonist Trog acts at the AT1 A R to simultaneously block G q protein activation and induce the recruitment of βarr1/2, which leads to an increase in cardiomyocyte contractility.

  14. Environment-assisted Quantum Critical Effect for Excitation Energy Transfer in a LH2-type Trimer

    Science.gov (United States)

    Xu, Lan; Xu, Bo

    2015-10-01

    In this article, we are investigating excitation energy transfer (EET) in a basic unit cell of light-harvesting complex II (LH2), named a LH2-type trimer. Calculation of energy transfer efficiency (ETE) in the framework of non-Markovian environment is also implemented. With these achievements, we theoretically predict the environment-assisted quantum critical effect, where ETE exhibits a sudden change at the critical point of quantum phase transition (QPT) for the LH2-type trimer. It is found that highly efficient EET with nearly unit efficiency may occur in the vicinity of the critical point of QPT.

  15. Word type effects in false recall: concrete, abstract, and emotion word critical lures.

    Science.gov (United States)

    Bauer, Lisa M; Olheiser, Erik L; Altarriba, Jeanette; Landi, Nicole

    2009-01-01

    Previous research has demonstrated that definable qualities of verbal stimuli have implications for memory. For example, the distinction between concrete and abstract words has led to the finding that concrete words have an advantage in memory tasks (i.e., the concreteness effect). However, other word types, such as words that label specific human emotions, may also affect memory processes. This study examined the effects of word type on the production of false memories by using a list-learning false memory paradigm. Participants heard lists of words that were highly associated to nonpresented concrete, abstract, or emotion words (i.e., the critical lures) and then engaged in list recall. Emotion word critical lures were falsely recalled at a significantly higher rate (with the effect carried by the positively valenced critical lures) than concrete and abstract critical lures. These findings suggest that the word type variable has implications for our understanding of the mechanisms that underlie recall and false recall.

  16. Targeting the CACNA1A IRES as a Treatment for Spinocerebellar Ataxia Type 6.

    Science.gov (United States)

    Pastor, Parviz Daniel Hejazi; Du, Xiaofei; Fazal, Sarah; Davies, Andre N; Gomez, Christopher M

    2018-02-01

    We have discovered that the P/Q-type voltage-gated Ca 2+ channel (VGCC) gene, CACNA1A, encodes both the α1A (Cav2.1) subunit and a newly recognized transcription factor, α1ACT, by means of a novel internal ribosomal entry site (IRES) within the α1A C-terminal coding region. α1ACT, when mutated with an expansion of the polyglutamine tract in the C-terminus, gives rise to spinocerebellar ataxia type 6 (SCA6). Because silencing of the entire CACNA1A gene would result in the loss of the essential Cav2.1 channel, the IRES controlling α1ACT expression is an excellent target for selective silencing of α1ACT as a therapeutic intervention for SCA6. We performed a high-throughput screen of FDA-approved small molecules using a dual luciferase reporter system and identified ten hits able to selectively inhibit the IRES. We identified four main candidates that showed selective suppression of α1ACT relative to α1A in HEK cells expressing a native CACNA1A vector. We previously pursued another avenue of molecular intervention through miRNA silencing. We studied three human miRNAs (miRNA-711, -3191-5p, -4786) that would potentially bind to sequences within the CACNA1A IRES region, based on an miRNA prediction program. Only miRNA-3191-5p was found to selectively inhibit the translation of α1ACT in cells. We developed a hyperacute model of SCA6 in mice by injecting a pathogenic form of the IRES-mediated α1ACT (AAV9-α1ACTQ33). Finally, we tested the effectiveness of the miRNA therapy by co-expressing either control miRNA or miRNA-3191-5p and found that miRNA-3191-5p decreased the levels of α1ACTQ33 and prevented the hyperacute disease in mice. These studies provide the proof of principle that a therapy directed at selectively preventing α1ACT expression could be used to treat SCA6.

  17. Roles of Type 1A Topoisomerases in Genome Maintenance in Escherichia coli

    Science.gov (United States)

    Usongo, Valentine; Drolet, Marc

    2014-01-01

    In eukaryotes, type 1A topoisomerases (topos) act with RecQ-like helicases to maintain the stability of the genome. Despite having been the first type 1A enzymes to be discovered, much less is known about the involvement of the E. coli topo I (topA) and III (topB) enzymes in genome maintenance. These enzymes are thought to have distinct cellular functions: topo I regulates supercoiling and R-loop formation, and topo III is involved in chromosome segregation. To better characterize their roles in genome maintenance, we have used genetic approaches including suppressor screens, combined with microscopy for the examination of cell morphology and nucleoid shape. We show that topA mutants can suffer from growth-inhibitory and supercoiling-dependent chromosome segregation defects. These problems are corrected by deleting recA or recQ but not by deleting recJ or recO, indicating that the RecF pathway is not involved. Rather, our data suggest that RecQ acts with a type 1A topo on RecA-generated recombination intermediates because: 1-topo III overproduction corrects the defects and 2-recQ deletion and topo IIII overproduction are epistatic to recA deletion. The segregation defects are also linked to over-replication, as they are significantly alleviated by an oriC::aph suppressor mutation which is oriC-competent in topA null but not in isogenic topA+ cells. When both topo I and topo III are missing, excess supercoiling triggers growth inhibition that correlates with the formation of extremely long filaments fully packed with unsegregated and diffuse DNA. These phenotypes are likely related to replication from R-loops as they are corrected by overproducing RNase HI or by genetic suppressors of double topA rnhA mutants affecting constitutive stable DNA replication, dnaT::aph and rne::aph, which initiates from R-loops. Thus, bacterial type 1A topos maintain the stability of the genome (i) by preventing over-replication originating from oriC (topo I alone) and R-loops and (ii

  18. Charcot-Marie-Tooth disease type 1A: morphological phenotype of the 17p duplication versus PMP22 point mutations

    NARCIS (Netherlands)

    Gabreëls-Festen, A. A.; Bolhuis, P. A.; Hoogendijk, J. E.; Valentijn, L. J.; Eshuis, E. J.; Gabreëls, F. J.

    1995-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A) or hereditary motor and sensory neuropathy type Ia (HMSN type Ia) is an autosomal dominant demyelinating polyneuropathy, which may result from duplications as large as 1.5 Mb on chromosome 17p 11.2-p12 encompassing the gene for the peripheral myelin

  19. Large Genomic Deletions in CACNA1A Cause Episodic Ataxia Type 2

    Directory of Open Access Journals (Sweden)

    Jijun eWan

    2011-09-01

    Full Text Available Episodic ataxia (EA syndromes are heritable diseases characterized by dramatic episodes of imbalance and incoordination. Episodic ataxia type 2 (EA2, the most common and the best characterized subtype, is caused by mostly nonsense, splice site, small indel and sometimes missense mutations in CACNA1A. Direct sequencing of CACNA1A fails to identify mutations in some patients with EA2-like features, possibly due to incomplete interrogation of CACNA1A or defects in other EA genes not yet defined. Previous reports described genomic deletions between 4-40kb in EA2. In 47 subjects with EA (26 with EA2-like features who tested negative for mutations in the known EA genes, we used Multiplex Ligation-dependent Probe Amplification (MLPA to analyze CACNA1A for exonic copy number variations. Breakpoints were further defined by long-range PCR. We identified distinct multi-exonic deletions in three probands with classic EA2-like features: episodes of prolonged vertigo and ataxia triggered by stress and fatigue, interictal nystagmus, with onset during infancy or early childhood. The breakpoints in all three probands are located in Alu sequences, indicating errors in homologous recombination of Alu sequences as the underlying mechanism. The smallest deletion spanned exons 39 and 40, while the largest deletion spanned 200kb, missing all but the first three exons. One deletion involving exons 39 through 47 arose spontaneously. The search for mutations in CACNA1A appears most fruitful in EA patients with interictal nystagmus and onset early in life. The finding of large heterozygous deletions suggests haploinsufficiency as a possible pathomechanism of EA2.

  20. Novel mutations in the SCNN1A gene causing Pseudohypoaldosteronism type 1.

    Directory of Open Access Journals (Sweden)

    Jian Wang

    Full Text Available Pseudohypoaldosteronism type 1 (PHA1 is a rare inherited disease characterized by resistance to the actions of aldosterone. Mutations in the subunit genes (SCNN1A, SCNN1B, SCNN1G of the epithelial sodium channel (ENaC and the NR3C2 gene encoding the mineralocorticoid receptor, result in systemic PHA1 and renal PHA1 respectively. Common clinical manifestations of PHA1 include salt wasting, hyperkalaemia, metabolic acidosis and elevated plasma aldosterone levels in the neonatal period. In this study, we describe the clinical and biochemical manifestations in two Chinese patients with systemic PHA1. Sequence analysis of the SCNN1A gene revealed a compound heterozygous mutation (c.1311delG and c.1439+1G>C in one patient and a homozygous mutation (c.814_815insG in another patient, all three variants are novel. Further analysis of the splicing pattern in a minigene construct showed that the c.1439+1G>C mutation can lead to the retainment of intron 9 as the 5'-donor splice site disappears during post-transcriptional processing of mRNA. In conclusion, our study identified three novel SCNN1A gene mutations in two Chinese patients with systemic PHA1.

  1. Critical behavior of the Lyapunov exponent in type-III intermittency

    International Nuclear Information System (INIS)

    Alvarez-Llamoza, O.; Cosenza, M.G.; Ponce, G.A.

    2008-01-01

    The critical behavior of the Lyapunov exponent near the transition to robust chaos via type-III intermittency is determined for a family of one-dimensional singular maps. Critical boundaries separating the region of robust chaos from the region where stable fixed points exist are calculated on the parameter space of the system. A critical exponent β expressing the scaling of the Lyapunov exponent is calculated along the critical curve corresponding to the type-III intermittent transition to chaos. It is found that β varies on the interval 0 ≤ β < 1/2 as a function of the order of the singularity of the map. This contrasts with earlier predictions for the scaling behavior of the Lyapunov exponent in type-III intermittency. The variation of the critical exponent β implies a continuous change in the nature of the transition to chaos via type-III intermittency, from a second-order, continuous transition to a first-order, discontinuous transition

  2. The Occurrence of Type S1A Serine Proteases in Sponge and Jellyfish

    Science.gov (United States)

    Rojas, Ana; Doolittle, Russell F.

    2003-01-01

    Although serine proteases are found in all kinds of cellular organisms and many viruses, the classic "chymotrypsin family" (Group S1A by th e 1998 Barrett nomenclature) has an unusual phylogenetic distribution , being especially common in animals, entirely absent from plants and protists, and rare among fungi. The distribution in Bacteria is larg ely restricted to the genus Streptomyces, although a few isolated occ urrences in other bacteria have been reported. The family may be enti rely absent from Archaea. Although more than a thousand sequences have been reported for enzymes of this type from animals, none of them ha ve been from early diverging phyla like Porifera or Cnidaria, We now report the existence of Group SlA serine proteases in a sponge (phylu m Porifera) and a jellyfish (phylum Cnidaria), making it safe to conc lude that all animal groups possess these enzymes.

  3. Electric field obtained from an elliptic critical-state model for anisotropic type-II superconductors

    Energy Technology Data Exchange (ETDEWEB)

    Romero-Salazar, C., E-mail: cromeros@ifuap.buap.mx; Hernández-Flores, O.A.

    2016-02-15

    Highlights: • An anisotropic critical state model that incorporates a non-zero electric field is proposed. • The critical current density is driven by the electric field. • To determinate the magnetic properties is not required a material law for the electric field magnitude. - Abstract: The conventional elliptic critical-state models (ECSM) establish that the electric field vector is zero when it flows a critical current density in a type-II superconductor. This proposal incorporates a finite electric field on the ECSM to study samples with anisotropic-current-carrying capacity. Our theoretical scheme has the advantage of being able to dispense of a material law which drives the electric field magnitude, however, it does not consider the magnetic history of the superconductor.

  4. Combined effects of changing-sign potential and critical nonlinearities in Kirchhoff type problems

    Directory of Open Access Journals (Sweden)

    Gao-Sheng Liu

    2016-08-01

    Full Text Available In this article, we study the existence and multiplicity of positive solutions for a class of Kirchhoff type problems involving changing-sign potential and critical growth terms. Using the concentration compactness principle and Nehari manifold, we obtain the existence and multiplicity of nonzero non-negative solutions.

  5. A Criticality Safety Study on Storing Unirradiated Cintichem-Type Targets at Sandia National Laboratories

    International Nuclear Information System (INIS)

    Romero, D.J.; Parma, E.J.; Busch, R.D.

    1999-01-01

    This criticality safety analysis is performed to determine the effective multiplication factor (k eff ) for a storage cabinet filled with unirradiated Cintichem-type targets. These targets will be used to produce 99 Mo at Sandia National Laboratories and will be stored on-site prior to irradiation in the Annular Core Research Reactor. The analysis consisted of using the Monte Carlo code MCNP (Version 4A) to model and predict the k eff for the proposed dry storage configuration under credible loss of geometry and moderator control. Effects of target pitch, non-uniform loading, and target internal/external flooding are evaluated. Further studies were done with deterministic methods to verify the results obtained from MCNP and to obtain a clearer understanding of the parameters affecting system criticality. The diffusion accelerated neutral particle transport code ONEDANT was used to model the target in a one-dimensional, infinite half-slab geometry and determine the critical slab thickness. Hand calculations were also completed to determine the critical slab thickness with modified one-group, and one-group, two region approximations. Results obtained from ONEDANT and the hand calculations were compared to applicable cases in a commonly used criticality safety analysis handbook. Overall, the critical slab thicknesses obtained in the deterministic analysis were much larger than the dimensions of the cabinet and further support the predictions by MCNP that a critical system cannot be attained for the base case or in conditions where loss of geometry and moderation control occur

  6. Craniofacial and dental characteristics of patients with vitamin-D-dependent rickets type 1A compared to controls and patients with X-linked hypophosphatemia

    DEFF Research Database (Denmark)

    Gjørup, Hans; Beck-Nielsen, Signe Sparre; Haubek, Dorte

    2017-01-01

    ᅟOBJECTIVES: Vitamin-D-dependent rickets type 1A (VDDR1A) is a rare inherited disease caused by defective activation of vitamin D. The aim of the study was to describe the craniofacial characteristics and the dental phenotype of patients with genetically confirmed VDDR1A. The VDDR1A findings were...... of dental findings was 30 adults with XLH. Clinical examination, clinical photos, and radiographs were obtained. Cephalometric analysis was performed. Photos and radiographs were visually evaluated. RESULTS: The depth of the posterior cranial fossa (d-p and d-s-iop) in VDDR1A adults was reduced compared...... of both calcium and phosphate to be critical in periods of enamel formation. CLINICAL RELEVANCE: Knowledge on craniofacial and dental aberration in patients with rare diseases, e.g., inherited rickets, is of importance to the dental practitioner, especially during diagnostics and treatment in special care...

  7. Mutation Analysis of COL1A1 and COL1A2 in Fetuses with Osteogenesis Imperfecta Type II/III.

    Science.gov (United States)

    Wang, Wenbo; Wu, Qichang; Cao, Lin; Sun, Li; Xu, Yasong; Guo, Qiwei

    2015-01-27

    Aim: To analyze COL1A1/2 mutations in prenatal-onset OI for determine the proportion of mutations in type I collagen genes among prenatal onset OI and to provide additional data for genotype-phenotype analyses. Material and Methods: Ten cases of severe fetal short-limb dwarfism detected by antenatal ultrasonography were referred to our center. Before the termination of pregnancy, cordocentesis was performed for fetal karyotype and COL1A1/2 gene sequencing analysis. Postmortem radiographic examination was performed at all instances for definitive diagnosis. Results: COL1A1 and COL1A2 SNP and mutations were identified in all the cases. Among these, one synonymous SNP and four synonymous SNPs were recognized in COL1A1/2, respectively, seven cases have distinct heterozygous mutations and six new COL1A1/2 gene mutations were identified. Conclusion: There has been substantial progress in the identification of the molecular defects responsible for skeletal dysplasias. With the constant increase in the number of identified mutations in COL1A1 and COL1A2, genotype-phenotype correlation is becoming increasingly pertinent. © 2015 S. Karger AG, Basel.

  8. CYP1A1 and CYP1A2 expression: Comparing 'humanized' mouse lines and wild-type mice; comparing human and mouse hepatoma-derived cell lines

    International Nuclear Information System (INIS)

    Uno, Shigeyuki; Endo, Kaori; Ishida, Yuji; Tateno, Chise; Makishima, Makoto; Yoshizato, Katsutoshi; Nebert, Daniel W.

    2009-01-01

    Human and rodent cytochrome P450 (CYP) enzymes sometimes exhibit striking species-specific differences in substrate preference and rate of metabolism. Human risk assessment of CYP substrates might therefore best be evaluated in the intact mouse by replacing mouse Cyp genes with human CYP orthologs; however, how 'human-like' can human gene expression be expected in mouse tissues? Previously a bacterial-artificial-chromosome-transgenic mouse, carrying the human CYP1A1 C YP1A2 locus and lacking the mouse Cyp1a1 and Cyp1a2 orthologs, was shown to express robustly human dioxin-inducible CYP1A1 and basal versus inducible CYP1A2 (mRNAs, proteins, enzyme activities) in each of nine mouse tissues examined. Chimeric mice carrying humanized liver have also been generated, by transplanting human hepatocytes into a urokinase-type plasminogen activator(+/+) s evere-combined-immunodeficiency (uPA/SCID) line with most of its mouse hepatocytes ablated. Herein we compare basal and dioxin-induced CYP1A mRNA copy numbers, protein levels, and four enzymes (benzo[a]pyrene hydroxylase, ethoxyresorufin O-deethylase, acetanilide 4-hydroxylase, methoxyresorufin O-demethylase) in liver of these two humanized mouse lines versus wild-type mice; we also compare these same parameters in mouse Hepa-1c1c7 and human HepG2 hepatoma-derived established cell lines. Most strikingly, mouse liver CYP1A1-specific enzyme activities are between 38- and 170-fold higher than human CYP1A1-specific enzyme activities (per unit of mRNA), whereas mouse versus human CYP1A2 enzyme activities (per unit of mRNA) are within 2.5-fold of one another. Moreover, both the mouse and human hepatoma cell lines exhibit striking differences in CYP1A mRNA levels and enzyme activities. These findings are relevant to risk assessment involving human CYP1A1 and CYP1A2 substrates, when administered to mice as environmental toxicants or drugs.

  9. A formulation for the critical temperature T/sub c/ of Ll2-type superconductors

    International Nuclear Information System (INIS)

    Wang Rong-Yao; Zhang Xiao

    1985-01-01

    From the examination of Ll 2 type superconductors, the superconducting critical temperature T/sub b/ of Ll 2 -type superconductors is obtained by: T/sub c/ = 15.9T/sub B/V(B)G/sub A//(√M/sub m/) V(Ll 2 )/sub m/ G/sub B/ where T/sub B/ is the superconducting critical temperature of pure B, V(B) the atomic volume in pure B, V(Ll 2 )/sub m/ the average atomic volume in the Ll 2 type compound, M/sub m/ the average atomic weight of the compound, and G/sub A/, G/sub B/ are the Gordy electronegative values. (author)

  10. Postoperative shoulder imbalance in Lenke Type 1A adolescent idiopathic scoliosis and related factors.

    Science.gov (United States)

    Matsumoto, Morio; Watanabe, Kota; Kawakami, Noriaki; Tsuji, Taichi; Uno, Koki; Suzuki, Teppei; Ito, Manabu; Yanagida, Haruhisa; Minami, Shohei; Akazawa, Tsutomu

    2014-11-05

    The purpose of this study was to investigate the occurrence and factors associated with postoperative shoulder imbalance (PSI) in Lenke type 1A curve. This study included 106 patients with Lenke Type 1A curve who were followed up more than two years after posterior correction surgery. Pedicle screw (PS) constructs were used in 84 patients, and hybrid constructs in 22. The upper instrumented vertebra was rostral to the upper-end vertebra (UEV) in 70 patients, at UEV in 26, and below UEV in 10. The clavicle angle and T1 tilt angle were measured as PSI indicators, and correlations between radiographic parameters of shoulder balance and other radiographic parameters and associations between PSI and clinical parameters were investigated. For statistical analyses, paired and unpaired t-tests were used. The mean Cobb angles of the main and proximal thoracic curves were 54.6 ± 9.5 and 26.7 ± 7.9 degrees before surgery, 14.5 ± 7.5, and 14.9 ± 7.1 at follow-up. Clavicle angle and T1 tilt angle were -2.9 ± 2.8 and -2.6 ± 6.3 before surgery, 2.4 ± 2.8 and 4.4 ± 4.3 immediately after surgery, and 1.8 ± 2.1 and 3.4 ± 5.5 at follow-up. Twenty patients developed distal adding-on. Clavicle angle at follow-up correlated weakly but significantly with preoperative clavicle angle (r = 0.34, p = 0.001) and with the correction rates of the main thoracic curve (r = 0.34, p = 0.001); it correlated negatively with the proximal curve spontaneous correction rate (r=-0.21, p = 0.034). The clavicle angle at follow-up was significantly larger in patients with PS-only constructs (PS 2.1 degrees vs. hybrid 0.9, p = 0.02), and tended to be smaller in patients with distal adding-on (adding-on 1.1 vs. non adding-on 2.0, p = 0.09). PSI was more common with better correction of the main curve (using PS constructs), in patients with a larger preoperative clavicle angle, and with a larger and more rigid proximal curve. Distal adding-on may compensate for PSI.

  11. Student’s Critical Thinking in Solving Open-Ended Problems Based on Their Personality Type

    Science.gov (United States)

    Fitriana, L. D.; Fuad, Y.; Ekawati, R.

    2018-01-01

    Critical thinking plays an important role for students in solving open-ended problems. This research aims at describing student’s critical thinking in solving open-ended problems based on Keirsey’s personality types, namely rational, idealist, guardian, and artisan. Four students, with the higher rank in the mathematics’ test and representing each type of Keirsey personality, were selected as the research subjects. The data were collected from the geometry problem and interviews. The student’s critical thinking is described based on the FRISCO criteria. The result underlines that rational and idealist students fulfilled all FRISCO criteria, and but not for guardian and artisan students. Related to the inference criteria, guardian and artisan students could not make reasonable conclusions and connect the concepts. Related to the reason of criteria, rational student performed critical thinking by providing logical reason that supported his strategy to solve the problem. In contrast, the idealist student provided subjective reason. This results suggest that teachers should frequently train the students’ logical thinkingin every lesson and activity to develop student’s critical thinking and take the student’s personality character into account, especially for guardian and artisan students.

  12. Participation restriction in childhood phenotype of myotonic dystrophy type 1: a systematic retrospective chart review.

    Science.gov (United States)

    Gagnon, Cynthia; Kierkegaard, Marie; Blackburn, Catherine; Chrestian, Nicolas; Lavoie, Mélissa; Bouchard, Marie-Frédéric; Mathieu, Jean

    2017-03-01

    Myotonic dystrophy type 1 (DM1), a neuromuscular disorder, is divided into four clinical phenotypes: congenital; childhood; adult-onset, and late-onset. Publications about the childhood phenotype, especially the long-term outcome, are scarce. The aims of this study were to assess and describe participation outcomes in adults with the childhood phenotype. A retrospective chart methodology. Data were extracted from health records for 63 adults with childhood DM1 (32 males, 31 females; mean age 34y, standard deviation [SD] 11y 6mo; range 18-54y) who had attended the Saguenay Neuromuscular Clinic, Canada. Thirty-four adults (54%) lived with their parents or in foster homes, and most patients needed services or help to live independently. A significant proportion (22%) were isolated in regard to friendship. Very few adults had children, although 33% lived with a spouse. The majority of patients (86%) relied on social security and only one person was currently working. Financial responsibilities were often an issue and 13 (21%) were under legal guardianship. This study showed that patients with the childhood phenotype present a guarded prognosis regarding long-term social participation. These participation restrictions could be related to behavioural, cognitive, and social stigma problems in childhood. This study illustrates the absolute necessity to pursue an interdisciplinary follow-up of these patients when they are reaching adulthood. © 2016 Mac Keith Press.

  13. Clinical presentation and biochemical findings children with glycogen storage disease type 1A

    International Nuclear Information System (INIS)

    Saeed, A.; Suleman, H.; Arshad, H.

    2015-01-01

    To determine the clinical pattern of presentation and biochemical characteristics of glycogen storage disease (GSD) type 1a in children at a tertiary referral centre. Study Design: Descriptive/ cross sectional study. Place and Duration of Study: Department of Pediatric, division of Gastroenterology and Hepatology of the Children's hospital, Lahore over a period of 11 years. Patients and Methods: Confirmed cases of glycogen storage disease (clinical plus biochemical findings consistent with GSD 1a and proven on liver biopsy) were enrolled in this study from neonatal age till 18 years. Data was retrieved from files and electronic record for these cases. Diagnosis was made on the basis of history, clinical findings including hepatomegaly, hypertriglyceridemia, hypercholesterolemia, hypoglycemia and hyperuricemia (if present). Diagnosis was confirmed on liver biopsy. Patients with other storage disorders and benign and malignant tumours were excluded from the study. Results: Total patients included in the study were 360 with male to female ratio of 1.25:1. Median age at the time of diagnosis was 25.6 months (age range from one month to 18 years). Most common presentation was abdominal distension (83%) followed by failure to thrive (69%) and recurrent wheezing and diarrhoea (44%) each. Seizures were present in only 1/3rd of children. Other presentations included vomiting, respiratory distress, altered sensorium, nephrocalcinosis, epistaxis and hypothyroidism. Few patients around 11% presented with acute hepatitis and later were diagnosed as GSD. Significant hepatomegaly was evident in almost all patients but nephromegaly was present in only 5.5% patients. All children had marked hypertriglyceridemia but cholesterol levels were raised in 1/3rd of children. A large majority of children had deranged ALT more than 2 times of normal and around 38% children had marked anemia. Significant hypoglycemia and metabolic acidosis was documented in around 1/3rd of children

  14. Forces of vortice trapping and critical current in type II superconductors

    International Nuclear Information System (INIS)

    Bormio, C.

    1985-12-01

    The vortice-centers interactions of trapping in type II superconductor materials were studied by two theories: thermodynamic (Hampshire-Taylor) and microscopic (Larkin - Ovchinnikov). The study was applied to NbTi with composition of 50% weight of Ti. They are commercial cables containing 361 filaments with final diameter of 0.35 mm for the wire and 9.2 μm foi filaments. The material presents high deformation rate in area and high density of dislocations. These defects actuate as centers of trapping. Variations in themomechanical treatments of superconductor cables modify the interaction mechanisms. The specific mechanism for each treatment type was identified. Measurements of critical current density in function of magnetic field in the range from 1 to 7 Tesla were done, which the usual superconductor parameters as upper critical field and Ginzburg - Landau (Kappa-k) parameter are estimated from literature data. (M.C.K.) [pt

  15. Experimental infection of serotine bats (Eptesicus serotinus) with European bat lyssavirus type 1a.

    Science.gov (United States)

    Freuling, C; Vos, A; Johnson, N; Kaipf, I; Denzinger, A; Neubert, L; Mansfield, K; Hicks, D; Nuñez, A; Tordo, N; Rupprecht, C E; Fooks, A R; Müller, T

    2009-10-01

    The serotine bat (Eptesicus serotinus) accounts for the vast majority of bat rabies cases in Europe and is considered the main reservoir for European bat lyssavirus type 1 (EBLV-1, genotype 5). However, so far the disease has not been investigated in its native host under experimental conditions. To assess viral virulence, dissemination and probable means of transmission, captive bats were infected experimentally with an EBLV-1a virus isolated from a naturally infected conspecific from Germany. Twenty-nine wild caught bats were divided into five groups and inoculated by intracranial (i.c.), intramuscular (i.m.) or subcutaneous (s.c.) injection or by intranasal (i.n.) inoculation to mimic the various potential routes of infection. One group of bats was maintained as uninfected controls. Mortality was highest in the i.c.-infected animals, followed by the s.c. and i.m. groups. Incubation periods varied from 7 to 26 days depending on the route of infection. Rabies did not develop in the i.n. group or in the negative-control group. None of the infected bats seroconverted. Viral antigen was detected in more than 50% of the taste buds of an i.c.-infected animal. Shedding of viable virus was measured by virus isolation in cell culture for one bat from the s.c. group at 13 and 14 days post-inoculation, i.e. 7 days before death. In conclusion, it is postulated that s.c. inoculation, in nature caused by bites, may be an efficient way of transmitting EBLV-1 among free-living serotine bats.

  16. Critical level of radionuclides pollution estimation for different soil type of Ukrainian Polessye

    International Nuclear Information System (INIS)

    Kravets, A.; Pavlenko, Y.

    1996-01-01

    The successive development and adaptation of general algorithm of calculation of doses from intake 137 Cs and 90 Sr as a function of pollution level and a type of soil as a source of the human trophycal chains and its use in solution of reverse problem, namely- estimation of the critical level of radionuclides pollution for the main type of soil of Ukrainian Polessye has been proposed. Calculation was realized as a combination of dynamic model of migration of radionuclides in soil and spreadsheet form with Quattro Pro, version 4.0. (author)

  17. Critical region of a type II superconducting film near Hsub(c2): rational approximants

    International Nuclear Information System (INIS)

    Ruggeri, G.J.

    1979-01-01

    The high-temperature perturbative expansions for the thermal quantities of a type II superconducting film are extrapolated to the critical region near Hsub(c2) by means of new rational approximants of the Pade type. The new approximants are forced to reproduce the leading correction to the flux lattice contribution on the low-temperature side of the transition. Compared to those previously considered in the literature: (i) the mutual consistency of the approximants is improved; and (ii) they are nearer to the exact solution of the zero-dimensional Landau-Ginsburg model. (author)

  18. Recurrent spontaneous hip dislocation in a patient with neurofibromatosis type 1: a case report.

    LENUS (Irish Health Repository)

    Galbraith, John G

    2011-01-01

    Neurofibromatosis type-1 is a common genetic disorder which often affects the skeleton. Skeletal manifestations of neurofibromatosis type-1 include scoliosis, congenital pseudarthrosis of the tibia and intraosseous cystic lesions. Dislocation of the hip associated with neurofibromatosis type-1 is a rare occurrence and is underreported in the literature.

  19. Unintended targeting of Dmp1-Cre reveals a critical role for Bmpr1a signaling in the gastrointestinal mesenchyme of adult mice.

    Science.gov (United States)

    Lim, Joohyun; Burclaff, Joseph; He, Guangxu; Mills, Jason C; Long, Fanxin

    2017-01-01

    Cre/loxP technology has been widely used to study cell type-specific functions of genes. Proper interpretation of such data critically depends on a clear understanding of the tissue specificity of Cre expression. The Dmp1-Cre mouse, expressing Cre from a 14-kb DNA fragment of the mouse Dmp1 gene, has become a common tool for studying gene function in osteocytes, but the presumed cell specificity is yet to be fully established. By using the Ai9 reporter line that expresses a red fluorescent protein upon Cre recombination, we find that in 2-month-old mice, Dmp1-Cre targets not only osteocytes within the bone matrix but also osteoblasts on the bone surface and preosteoblasts at the metaphyseal chondro-osseous junction. In the bone marrow, Cre activity is evident in certain stromal cells adjacent to the blood vessels, but not in adipocytes. Outside the skeleton, Dmp1-Cre marks not only the skeletal muscle fibers, certain cells in the cerebellum and the hindbrain but also gastric and intestinal mesenchymal cells that express Pdgfra . Confirming the utility of Dmp1-Cre in the gastrointestinal mesenchyme, deletion of Bmpr1a with Dmp1-Cre causes numerous large polyps along the gastrointestinal tract, consistent with prior work involving inhibition of BMP signaling. Thus, caution needs to be exercised when using Dmp1-Cre because it targets not only the osteoblast lineage at an earlier stage than previously appreciated, but also a number of non-skeletal cell types.

  20. Quantitative measurement of duplicated DNA as a diagnostic test for Charcot-Marie-Tooth disease type 1a

    NARCIS (Netherlands)

    Hensels, G. W.; Janssen, E. A.; Hoogendijk, J. E.; Valentijn, L. J.; Baas, F.; Bolhuis, P. A.

    1993-01-01

    Charcot-Marie-Tooth disease type 1 (CMT1) is a hereditary motor and sensory neuropathy. The autosomal dominant subtype is often linked with a large duplication on chromosome 17p11.2. The gene encoding the peripheral myelin protein PMP 22 (the critical gene in this subtype of CMT1) is located within

  1. PMP22 related neuropathies: Charcot-Marie-Tooth disease type 1A and Hereditary Neuropathy with liability to Pressure Palsies

    NARCIS (Netherlands)

    van Paassen, Barbara W.; van der Kooi, Anneke J.; van Spaendonck-Zwarts, Karin Y.; Verhamme, Camiel; Baas, Frank; de Visser, Marianne

    2014-01-01

    PMP22 related neuropathies comprise (1) PMP22 duplications leading to Charcot-Marie-Tooth disease type 1A (CMT1A), (2) PMP22 deletions, leading to Hereditary Neuropathy with liability to Pressure Palsies (HNPP), and (3) PMP22 point mutations, causing both phenotypes. Overall prevalence of CMT is

  2. BmpR1A is a major type 1 BMP receptor for BMP-Smad signaling during skull development.

    Science.gov (United States)

    Pan, Haichun; Zhang, Honghao; Abraham, Ponnu; Komatsu, Yoshihiro; Lyons, Karen; Kaartinen, Vesa; Mishina, Yuji

    2017-09-01

    Craniosynostosis is caused by premature fusion of one or more sutures in an infant skull, resulting in abnormal facial features. The molecular and cellular mechanisms by which genetic mutations cause craniosynostosis are incompletely characterized, and many of the causative genes for diverse types of syndromic craniosynostosis have not yet been identified. We previously demonstrated that augmentation of BMP signaling mediated by a constitutively active BMP type IA receptor (ca-BmpR1A) in neural crest cells (ca1A hereafter) causes craniosynostosis and superimposition of heterozygous null mutation of Bmpr1a rescues premature suture fusion (ca1A;1aH hereafter). In this study, we superimposed heterozygous null mutations of the other two BMP type I receptors, Bmpr1b and Acvr1 (ca1A;1bH and ca1A;AcH respectively hereafter) to further dissect involvement of BMP-Smad signaling. Unlike caA1;1aH, ca1A;1bH and ca1A;AcH did not restore the craniosynostosis phenotypes. In our in vivo study, Smad-dependent BMP signaling was decreased to normal levels in mut;1aH mice. However, BMP receptor-regulated Smads (R-Smads; pSmad1/5/9 hereafter) levels were comparable between ca1A, ca1A;1bH and ca1A;AcH mice, and elevated compared to control mice. Bmpr1a, Bmpr1b and Acvr1 null cells were used to examine potential mechanisms underlying the differences in ability of heterozygosity for Bmpr1a vs. Bmpr1b or Acvr1 to rescue the mut phenotype. pSmad1/5/9 level was undetectable in Bmpr1a homozygous null cells while pSmad1/5/9 levels did not decrease in Bmpr1b or Acvr1 homozygous null cells. Taken together, our study indicates that different levels of expression and subsequent activation of Smad signaling differentially contribute each BMP type I receptor to BMP-Smad signaling and craniofacial development. These results also suggest differential involvement of each type 1 receptor in pathogenesis of syndromic craniosynostoses. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Allelic heterogeneity in hereditary motor and sensory neuropathy type Ia (Charcot-Marie-Tooth disease type 1a)

    NARCIS (Netherlands)

    Hoogendijk, J. E.; Janssen, E. A.; Gabreëls-Festen, A. A.; Hensels, G. W.; Joosten, E. M.; Gabreëls, F. J.; Zorn, I.; Valentijn, L. J.; Baas, F.; Ongerboer de Visser, B. W.

    1993-01-01

    The most frequently found mutation in autosomal dominant hereditary motor and sensory neuropathy type I (HMSN I) is a large duplication on chromosome 17p11.2 containing probes VAW409R3, VAW412R3, and EW401. We investigated a family with severe features of HMSN I, and demonstrated the absence of this

  4. Critical pitting temperature for Type 254 SMO stainless steel in chloride solutions

    International Nuclear Information System (INIS)

    Abd El Meguid, E.A.; Abd El Latif, A.A.

    2007-01-01

    The variation with time of the open circuit potential of high molybdenum containing stainless steel (Type 254 SMO) was measured in 4% sodium chloride solution in the temperatures range 30-100 deg. C. The plot of steady state potentials as function of temperature showed an inflection at 50 deg. C, attributed to the decrease of oxygen solubility in test solution above 50 deg. C. Potentiodynamic cycling anodic polarization technique was used to determine the critical pitting potential (E pit ) and the critical protection potential (E prot ) of the steel in 4-30% NaCl solutions at temperatures between 30 and 100 deg. C. By plotting the two values versus solution temperature, the corresponding critical pitting (CPT) and the critical protection (CPrT) temperatures were determined. Both parameters decreased with increasing chloride content. Above the CPT, E pit and E prot decreased linearly with log[Cl - ]. The addition of bromide ions to the solution shifted both E pit and E prot towards positive values. In 4% NaCl, E pit increased linearly with pH in the range 1-10. The combined effect of chloride ion concentration and pH on the morphology of the pits was examined by scanning electron microscopy (SEM) following potentiodynamic cycling anodic polarization

  5. Regeneration of alveolar type I and II cells from Scgb1a1-expressing cells following severe pulmonary damage induced by bleomycin and influenza.

    Directory of Open Access Journals (Sweden)

    Dahai Zheng

    Full Text Available The lung comprises an extensive surface of epithelia constantly exposed to environmental insults. Maintaining the integrity of the alveolar epithelia is critical for lung function and gaseous exchange. However, following severe pulmonary damage, what progenitor cells give rise to alveolar type I and II cells during the regeneration of alveolar epithelia has not been fully determined. In this study, we have investigated this issue by using transgenic mice in which Scgb1a1-expressing cells and their progeny can be genetically labeled with EGFP. We show that following severe alveolar damage induced either by bleomycin or by infection with influenza virus, the majority of the newly generated alveolar type II cells in the damaged parenchyma were labeled with EGFP. A large proportion of EGFP-expressing type I cells were also observed among the type II cells. These findings strongly suggest that Scgb1a1-expressing cells, most likely Clara cells, are a major cell type that gives rise to alveolar type I and II cells during the regeneration of alveolar epithelia in response to severe pulmonary damage in mice.

  6. Critical phenomena in Ising-type thin films by Monte Carlo study

    International Nuclear Information System (INIS)

    Masrour, R.; Jabar, A.; Benyoussef, A.; Hamedoun, M.

    2016-01-01

    The magnetic properties of ferrimagnetic spin-2 and 3/2 Ising-typed thin films are studied by Monte Carlo simulation. The critical temperature is obtained for different values of thickness of the thin film and for different exchange interactions. The total magnetization has been determined for different values of exchange interactions in surface and in bulk and different temperatures. The magnetic hysteresis cycle is obtained for different values of exchange interactions ferro and antiferromagnetic in the surface and in the bulk and for different values of temperatures for a fixed size of the film thickness. The coercive field increase with increasing the film thickness. - Highlights: • The magnetic properties of thin films are studied by Monte Carlo simulation. • The critical temperature is obtained for different values of thickness of thin film. • The magnetic hysteresis cycle is obtained in the surface and in the bulk. • The coercive field increase with increasing the thin film thickness.

  7. Critical phenomena in Ising-type thin films by Monte Carlo study

    Energy Technology Data Exchange (ETDEWEB)

    Masrour, R., E-mail: rachidmasrour@hotmail.com [Laboratory of Materials, Processes, Environment and Quality, Cady Ayyed University, National School of Applied Sciences, 63, 46000 Safi (Morocco); Laboratoire de Magnétisme et Physique des Hautes Energies L.M.P.H.E.URAC 12, Université Mohammed V, Faculté des Sciences, B.P. 1014, Rabat (Morocco); Jabar, A. [Laboratoire de Magnétisme et Physique des Hautes Energies L.M.P.H.E.URAC 12, Université Mohammed V, Faculté des Sciences, B.P. 1014, Rabat (Morocco); Benyoussef, A. [Laboratoire de Magnétisme et Physique des Hautes Energies L.M.P.H.E.URAC 12, Université Mohammed V, Faculté des Sciences, B.P. 1014, Rabat (Morocco); Institute of Nanomaterials and Nanotechnologies, MAScIR, Rabat (Morocco); Hassan II Academy of Science and Technology, Rabat (Morocco); Hamedoun, M. [Institute of Nanomaterials and Nanotechnologies, MAScIR, Rabat (Morocco)

    2016-04-01

    The magnetic properties of ferrimagnetic spin-2 and 3/2 Ising-typed thin films are studied by Monte Carlo simulation. The critical temperature is obtained for different values of thickness of the thin film and for different exchange interactions. The total magnetization has been determined for different values of exchange interactions in surface and in bulk and different temperatures. The magnetic hysteresis cycle is obtained for different values of exchange interactions ferro and antiferromagnetic in the surface and in the bulk and for different values of temperatures for a fixed size of the film thickness. The coercive field increase with increasing the film thickness. - Highlights: • The magnetic properties of thin films are studied by Monte Carlo simulation. • The critical temperature is obtained for different values of thickness of thin film. • The magnetic hysteresis cycle is obtained in the surface and in the bulk. • The coercive field increase with increasing the thin film thickness.

  8. Cdkal1, a type 2 diabetes susceptibility gene, regulates mitochondrial function in adipose tissue

    Directory of Open Access Journals (Sweden)

    Colin J. Palmer

    2017-10-01

    Conclusions: Cdkal1 is necessary for normal mitochondrial morphology and function in adipose tissue. These results suggest that the type 2 diabetes susceptibility gene CDKAL1 has novel functions in regulating mitochondrial activity.

  9. Facial Plexiform neurofibromatosis in a patient with neurofibromatosis type1: a case report

    Directory of Open Access Journals (Sweden)

    Iffat Hassan

    2012-01-01

    Full Text Available Plexiform neurofibroma is a poorly circumscribed, diffuse enlargement of neural sheets that typically involves major nerve trunks of the head and neck region because of the rich innervations of this area. It is a benign tumor and is a virtually pathognomonic and often disabling feature of neurofibromatosis type 1 (NF-1 or Von Recklinghausen’s disease. We hereby report a case of facial neurofibroma in an adult female with neurofibromatosis type 1 (NF-1.

  10. The criticality problem in reflected slab type reactor in the two-group transport theory

    International Nuclear Information System (INIS)

    Garcia, R.D.M.

    1978-01-01

    The criticality problem in reflected slab type reactor is solved for the first time in the two group neutron transport theory, by singular eingenfunctions expansion, the singular integrals obtained through continuity conditions of angular distributions at the interface are regularized by a recently proposed method. The result is a coupled system of regular integral equations for the expansion coefficients, this system is solved by an ordinary interactive method. Numerical results that can be utilized as a comparative standard for aproximation methods, are presented [pt

  11. Recurrent spontaneous hip dislocation in a patient with neurofibromatosis type 1: a case report

    Directory of Open Access Journals (Sweden)

    Harty James A

    2011-03-01

    Full Text Available Abstract Introduction Neurofibromatosis type-1 is a common genetic disorder which often affects the skeleton. Skeletal manifestations of neurofibromatosis type-1 include scoliosis, congenital pseudarthrosis of the tibia and intraosseous cystic lesions. Dislocation of the hip associated with neurofibromatosis type-1 is a rare occurrence and is underreported in the literature. Case presentation We report a case of hip dislocation resulting from an intra-articular neurofibroma in an 18-year-old Caucasian woman following minor trauma. This was originally suggested by the abnormalities on early radiographs of her pelvis and later confirmed with computed tomography and magnetic resonance imaging. Treatment was successful with skeletal traction for six weeks with no further hip dislocations at a 12-year follow-up. Conclusion This case illustrates the radiological features of this rare complication of neurofibromatosis type-1 using the modalities of plain radiograph, magnetic resonance imaging and computed tomography reconstruction. The radiological images give a clear insight into the mechanism by which neurofibromatosis type-1 leads to hip dislocation. It also demonstrates one treatment option with excellent results on long-term follow-up.

  12. [Ideal type and history--a critical review of applied criminology].

    Science.gov (United States)

    Köchel, Stefan

    2013-01-01

    Applied Criminology describes an established criminological school in the German-speaking area, which was founded by Hans Göppinger and Michael Bock, criminologists at Tübingen, in the 1980s and has meanwhile published a number of comprehensive basic methodological papers. The conceptual centrepiece with interdisciplinary approach is the formation and application of concepts referring to the so-called ideal type, which has been essentially inspired by the epistemology of Max Weber. However, the result of a critical reconstruction of these fundamentals is that the claimed interdisciplinary approach comes into conflict with a second much more phenomenological approach of Applied Criminology which is unable to comply with the political implications of criminological research and thus disavows the necessary historical relationality of the ideal type concepts.

  13. Well-posedness for Semi-relativistic Hartree Equations of Critical Type

    International Nuclear Information System (INIS)

    Lenzmann, Enno

    2007-01-01

    We prove local and global well-posedness for semi-relativistic, nonlinear Schroedinger equations with initial data in H s (R 3 ). Here F(u) is a critical Hartree nonlinearity that corresponds to Coulomb or Yukawa type self-interactions. For focusing F(u), which arise in the quantum theory of boson stars, we derive global-in-time existence for small initial data, where the smallness condition is expressed in terms of the L 2 -norm of solitary wave ground states. Our proof of well-posedness does not rely on Strichartz type estimates. As a major benefit from this, our method enables us to consider external potentials of a quite general class

  14. Increase in VVER type reactor critical heat fluxes due to placing the mixing grids

    International Nuclear Information System (INIS)

    Bezrukov, Y.; Lisenkov, E.; Vasilchenko, I.

    2011-01-01

    The report deals with the results of studies of critical heat fluxes (CHF) on the models of VVER type reactor fuel assembly models equipped with the 'Vihr' intensifiers-grids. The models are the seven-rod bundles with the uniform and non-uniform axial power that correspond to two periods of FA operation i.e. beginning of cycle and end of cycle. The experiments performed showed that the mixing grids of this type are capable of increasing the FA burnout power. The power ascension rate depends on both coolant pressure and steam quality value in the CHF point. Placing the mixing grids in the bundle upper spans results in shifting the point of DNB occurrence downward along the FA height. The experimental data obtained will be used to develop the correlations for determining the CHF in the FA equipped with the mixing grids. (authors)

  15. Temperature dependence of the upper critical field of type II superconductors with fluctuation effects

    International Nuclear Information System (INIS)

    Mikitik, G.P.

    1992-01-01

    Fluctuations of the order parameter are taken into consideration in an analysis of the temperature dependence of the upper critical field of a type II superconductor with a three-dimensional superconductivity. This temperature dependence is of universal applicability, to all type II superconductors, if the magnetic fields and temperatures are expressed in appropriate units. This dependence is derived explicitly for the regions of strong and weak magnetic fields. The results are applied to high T c superconductors, for which fluctuation effects are important. For these superconductors, the H c2 (T) dependence is quite different from the linear dependence characteristic of the mean-field theory, over a broad range of magnetic fields

  16. Effect of simvastatin on cognitive functioning in children with neurofibromatosis type 1: A randomized controlled trial

    NARCIS (Netherlands)

    L.C. Krab (Lianne); A. de Goede-Bolder (Arja); F.K. Aarsen (Femke); S. Pluijm (Saskia); M.J. Bouman (Marlies); J.N. van der Geest (Jos); M. Leguin (Maarten); C. Catsman (Coriene); W.F.M. Arts (Willem Frans); S.A. Kushner (Steven); A.J. Silva (Alcino); C.I. de Zeeuw (Chris); H.A. Moll (Henriëtte); Y. Elgersma (Ype)

    2008-01-01

    textabstractContext: Neurofibromatosis type 1 (NF1) is among the most common genetic disorders that cause learning disabilities. Recently, it was shown that statin-mediated inhibition of 3-hydroxy-3-methylglutaryl coenzyme A reductase restores the cognitive deficits in an NF1 mouse model. Objective:

  17. MR imaging of abdominopelvic involvement in neurofibromatosis type 1: a review of 43 patients

    Energy Technology Data Exchange (ETDEWEB)

    Zacharia, T. Thomas [Massachusetts General Hospital, Department of Radiology, Boston, MA (United States); Jaramillo, Diego [Children' s Hospital of Philadelphia, Department of Radiology, Philadelphia, PA (United States); Poussaint, Tina Young [Children' s Hospital Boston, Department of Radiology, Boston, MA (United States); Korf, Bruce [University of Alabama at Birmingham Hospital, Department of Genetics, Birmingham, AL (United States)

    2005-03-01

    Plexiform neurofibromas are a frequent complication of neurofibromatosis type 1. This article discusses MR imaging findings and distribution of plexiform neurofibromas in the abdomen and pelvis. To define the most prevalent patterns of involvement and MR imaging findings in abdominopelvic neurofibromatosis type 1. We reviewed the MR appearance of abdominopelvic lesions in 23 male and 20 female patients (median age: 16 years) with type 1 neurofibromatosis. The patients were part of a multi-institutional study of 300 patients. Imaging included coronal or sagittal, and axial short tau inversion recovery images. The most common abdominopelvic involvement was in the abdominopelvic wall (n=28, 65%) and lumbosacral plexus (n=27, 63%). Retroperitoneal involvement was frequent (n=15, 35%). Lesions were less often intraperitoneal (21%) (P=0.001). Pelvic disease (n=27, 63%), neural canal involvement (n=18, 42%), and hydronephrosis (n=4, 9%) were also noted. Target-like appearance of plexiform lesions was noted in more than half the patients. Abdominopelvic involvement in neurofibromatosis type 1 is primarily extraperitoneal. Although lesions are most prevalent in the abdominopelvic wall and lumbosacral plexus, retroperitoneal and pelvic involvement is common and usually affects important organs. MR imaging added information in the initial and follow-up clinical evaluation of these patients. (orig.)

  18. Stress Induced Hyperglycemia and the Subsequent Risk of Type 2 Diabetes in Survivors of Critical Illness

    Science.gov (United States)

    Plummer, Mark P.; Finnis, Mark E.; Phillips, Liza K.; Kar, Palash; Bihari, Shailesh; Biradar, Vishwanath; Moodie, Stewart; Horowitz, Michael; Shaw, Jonathan E.; Deane, Adam M.

    2016-01-01

    Objective Stress induced hyperglycemia occurs in critically ill patients who have normal glucose tolerance following resolution of their acute illness. The objective was to evaluate the association between stress induced hyperglycemia and incident diabetes in survivors of critical illness. Design Retrospective cohort study. Setting All adult patients surviving admission to a public hospital intensive care unit (ICU) in South Australia between 2004 and 2011. Patients Stress induced hyperglycemia was defined as a blood glucose ≥ 11.1 mmol/L (200 mg/dL) within 24 hours of ICU admission. Prevalent diabetes was identified through ICD-10 coding or prior registration with the Australian National Diabetes Service Scheme (NDSS). Incident diabetes was identified as NDSS registration beyond 30 days after hospital discharge until July 2015. The predicted risk of developing diabetes was described as sub-hazard ratios using competing risk regression. Survival was assessed using Cox proportional hazards regression. Main Results Stress induced hyperglycemia was identified in 2,883 (17%) of 17,074 patients without diabetes. The incidence of type 2 diabetes following critical illness was 4.8% (821 of 17,074). The risk of diabetes in patients with stress induced hyperglycemia was approximately double that of those without (HR 1.91 (95% CI 1.62, 2.26), phyperglycemia identifies patients at subsequent risk of incident diabetes. PMID:27824898

  19. Ethical conflict in critical care nursing: Correlation between exposure and types.

    Science.gov (United States)

    Falcó-Pegueroles, Anna; Lluch-Canut, Teresa; Roldan-Merino, Juan; Goberna-Tricas, Josefina; Guàrdia-Olmos, Joan

    2015-08-01

    Ethical conflicts in nursing have generally been studied in terms of temporal frequency and the degree of conflict. This study presents a new perspective for examining ethical conflict in terms of the degree of exposure to conflict and its typology. The aim was to examine the level of exposure to ethical conflict for professional nurses in critical care units and to analyze the relation between this level and the types of ethical conflict and moral states. This was a descriptive correlational study. Central and dispersion, normality tests, and analysis of variance were carried out. A total of 203 nurses were from two third-level teaching hospitals in Spain. Both centers are part of the University of Barcelona Health Network. Participants filled out the Ethical Conflict in Nursing Questionnaire-Critical Care Version. This investigation received the approval of the ethical committees for clinical investigation of the two participating hospitals. Participants were informed of the authorship and aims of the study. The index of exposure to ethical conflict was [Formula: see text]. The situations involving analgesic treatment and end-of-life care were shown to be frequent sources of conflict. The types of ethical conflict and moral states generally arranged themselves from lesser to greater levels of index of exposure to ethical conflict. The moderate level of exposure to ethical conflict was consistent with other international studies. However, the situations related with family are infrequent, and this presents differences with previous research. The results suggest that there is a logical relationship between types of conflict and levels of exposure to ethical conflict. The types of ethical conflict and moral states were related with the levels of exposure to ethical conflict. The new perspective was shown to be useful for analyzing the phenomenon of ethical conflict in the nurse. © The Author(s) 2014.

  20. Critical role of constitutive type I interferon response in bronchial epithelial cell to influenza infection.

    Directory of Open Access Journals (Sweden)

    Alan C-Y Hsu

    Full Text Available Innate antiviral responses in bronchial epithelial cells (BECs provide the first line of defense against respiratory viral infection and the effectiveness of this response is critically dependent on the type I interferons (IFNs. However the importance of the antiviral responses in BECs during influenza infection is not well understood. We profiled the innate immune response to infection with H3N2 and H5N1 virus using Calu-3 cells and primary BECs to model proximal airway cells. The susceptibility of BECs to influenza infection was not solely dependent on the sialic acid-bearing glycoprotein, and antiviral responses that occurred after viral endocytosis was more important in limiting viral replication. The early antiviral response and apoptosis correlated with the ability to limit viral replication. Both viruses reduced RIG-I associated antiviral responses and subsequent induction of IFN-β. However it was found that there was constitutive release of IFN-β by BECs and this was critical in inducing late antiviral signaling via type I IFN receptors, and was crucial in limiting viral infection. This study characterizes anti-influenza virus responses in airway epithelial cells and shows that constitutive IFN-β release plays a more important role in initiating protective late IFN-stimulated responses during human influenza infection in bronchial epithelial cells.

  1. Radiologic investigation of apert syndrome (acrocephalosyndactyly type 1) -a case report-

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Yeon Hee; Cho, Whi Youl; Kim, Myung Soon; Hong, In Soo; Sung, Ki Joon; Yang, Jae Seung [Wonju College of Medicine, Yonsei University, Wonju (Korea, Republic of)

    1991-03-15

    Apert syndrome (Acrocephalosyndactyly type 1) is a rare congenital anomaly characterized by craniosynosis and symmetric-syndactyly of both extremities. Radiological examination of the skull shows hydrocephalus due to aqueductal stenosis. In the facial bones, the hypoplastic maxilla and relatively prominent mandible are observed associated with other anomalies such as cleft palate. Radiologic examination of both hands and feet show bony or subcutaneous syndactylism and typical mitten hands and webbed toes.

  2. Radiologic investigation of apert syndrome (acrocephalosyndactyly type 1) -a case report-

    International Nuclear Information System (INIS)

    Lee, Yeon Hee; Cho, Whi Youl; Kim, Myung Soon; Hong, In Soo; Sung, Ki Joon; Yang, Jae Seung

    1991-01-01

    Apert syndrome (Acrocephalosyndactyly type 1) is a rare congenital anomaly characterized by craniosynosis and symmetric-syndactyly of both extremities. Radiological examination of the skull shows hydrocephalus due to aqueductal stenosis. In the facial bones, the hypoplastic maxilla and relatively prominent mandible are observed associated with other anomalies such as cleft palate. Radiologic examination of both hands and feet show bony or subcutaneous syndactylism and typical mitten hands and webbed toes

  3. Angiotensin type 1a receptors in the paraventricular nucleus of the hypothalamus protect against diet-induced obesity

    Science.gov (United States)

    de Kloet, Annette D.; Pati, Dipanwita; Wang, Lei; Hiller, Helmut; Sumners, Colin; Frazier, Charles J.; Seeley, Randy J.; Herman, James P.; Woods, Stephen C.; Krause, Eric G.

    2013-01-01

    Obesity is associated with increased levels of angiotensin-II (Ang-II), which activates angiotensin type-1a receptors (AT1a) to influence cardiovascular function and energy homeostasis. To test the hypothesis that specific AT1a within the brain control these processes, we utilized the Cre/lox system to delete AT1a from the paraventricular nucleus of the hypothalamus (PVN) of mice. PVN AT1a deletion did not affect body mass or adiposity when mice were maintained on standard chow. However, maintenance on a high-fat diet revealed a gene by environment interaction whereby mice lacking AT1a in the PVN had increased food intake and decreased energy expenditure that augmented body mass and adiposity relative to controls. Despite this increased adiposity, PVN AT1a deletion reduced systolic blood pressure, suggesting that this receptor population mediates the positive correlation between adiposity and blood pressure. Gene expression studies revealed that PVN AT1a deletion decreased hypothalamic expression of corticotrophin-releasing hormone and oxytocin, neuropeptides known to control food intake and sympathetic nervous system activity. Whole cell patch clamp recordings confirmed that PVN AT1a deletion eliminates responsiveness of PVN parvocellular neurons to Ang-II, and suggest that Ang-II responsiveness is increased in obese wild-type mice. Central inflammation is associated with metabolic and cardiovascular disorders and PVN AT1a deletion reduced indices of hypothalamic inflammation. Collectively, these studies demonstrate that PVN AT1a regulate energy balance during environmental challenges that promote metabolic and cardiovascular pathologies. The implication is that the elevated Ang-II that accompanies obesity serves as a negative feedback signal that activates PVN neurons to alleviate weight gain. PMID:23486953

  4. Type 1a endoleak following Zone 1 and Zone 2 thoracic endovascular aortic repair: effect of bird-beak configuration.

    Science.gov (United States)

    Kudo, Tomoaki; Kuratani, Toru; Shimamura, Kazuo; Sakamoto, Tomohiko; Kin, Keiwa; Masada, Kenta; Shijo, Takayuki; Torikai, Kei; Maeda, Koichi; Sawa, Yoshiki

    2017-10-01

    Type 1a endoleak is one of the most severe complications after thoracic endovascular aortic repair (TEVAR), because it carries the risk of aortic rupture. The association between bird-beak configuration and Type 1a endoleak remains unclear. The purpose of this study was to analyse the predictors of Type 1a endoleak following Zone 1 and Zone 2 TEVAR, with a particular focus on the effect of bird-beak configuration. From April 2008 to July 2015, 105 patients (mean age 68.6 years) who underwent Zone 1 and 2 landing TEVAR were enrolled, with a mean follow-up period of 4.3 years. The patients were categorized into 2 groups, according to the presence (Group B, n = 32) or the absence (Group N, n = 73) of bird-beak configuration on the first postoperative multidetector computed tomography. The Kaplan-Meier event-free rate curve showed that Type 1a endoleak and bird-beak progression occurred less frequently in Group N than in Group B. Five-year freedom from Type 1a endoleak rates were 79.7% and 100% for Groups B and N, respectively (P = 0.007). Multivariable logistic regression analysis showed that dissecting aortic aneurysm (odds ratio 3.72, 95% confidence interval 1.30-11.0; P = 0.014) and shorter radius of inner curvature (odds ratio 1.09, 95% confidence interval 0.85-0.99; P = 0.025) were significant risk factors for bird-beak configuration. Multivariable Cox proportional hazard regression showed that Z-type stent graft (hazard ratio 2.69, 95% confidence interval 1.11-6.51; P = 0.030) was a significant risk factor for bird-beak progression. Appropriate stent grafts need to be chosen carefully to prevent Type 1a endoleak and bird-beak configuration after landing Zone 1 and 2 TEVAR. Patients with bird-beak configuration on early postoperative multidetector computed tomography require closer follow-up to screen for Type 1a endoleak. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio

  5. Banting Lecture 2009: An Unfinished Journey: Molecular Pathogenesis to Prevention of Type 1A Diabetes

    Science.gov (United States)

    Eisenbarth, George S.

    2010-01-01

    The Banting Medal for Scientific Achievement Award is the American Diabetes Association's highest scientific award and honors an individual who has made significant, long-term contributions to the understanding of diabetes, its treatment, and/or prevention. The award is named after Nobel Prize winner Sir Frederick Banting, who codiscovered insulin treatment for diabetes. Dr. Eisenbarth received the American Diabetes Association's Banting Medal for Scientific Achievement at the Association's 69th Scientific Sessions, June 5–9, 2009, in New Orleans, Louisiana. He presented the Banting Lecture, An Unfinished Journey—Type 1 Diabetes—Molecular Pathogenesis to Prevention, on Sunday, June 7, 2009. PMID:20350969

  6. Modeling the Pathogenesis of Charcot-Marie-Tooth Disease Type 1A Using Patient-Specific iPSCs

    Directory of Open Access Journals (Sweden)

    Lei Shi

    2018-01-01

    Full Text Available Charcot-Marie-Tooth disease type 1A (CMT1A, one of the most frequent inherited peripheral neuropathies, is associated with PMP22 gene duplication. Previous studies of CMT1A mainly relied on rodent models, and it is not yet clear how PMP22 overexpression leads to the phenotype in patients. Here, we generated the human induced pluripotent stem cell (hiPSC lines from two CMT1A patients as an in vitro cell model. We found that, unlike the normal control cells, CMT1A hiPSCs rarely generated Schwann cells through neural crest stem cells (NCSCs. Instead, CMT1A NCSCs produced numerous endoneurial fibroblast-like cells in the Schwann cell differentiation system, and similar results were obtained in a PMP22-overexpressing iPSC model. Therefore, despite the demyelination-remyelination and/or dysmyelination theory for CMT1A pathogenesis, developmental disabilities of Schwann cells may be considered as an underlying cause of CMT1A. Our results may have important implications for the uncovering of the underlying mechanism and the development of a promising therapeutic strategy for CMT1A neuropathy.

  7. Psychiatric comorbidity in diabetes type 1: a cross-sectional observational study.

    Science.gov (United States)

    Maia, Ana Claudia C de Ornelas; Braga, Arthur de Azevedo; Paes, Flávia; Machado, Sérgio; Nardi, Antonio Egidio; Silva, Adriana Cardoso da

    2014-01-01

    This study aims to investigate the prevalence of psychiatric disorders, i.e., the presence of signs and symptoms of anxiety and depression in type 1 diabetic patients, as well as to investigate the prevalence of psychiatric disorders in insulin dependent patients. A cross-sectional observational study of 110 diabetic outpatients (mean = 58.3, SD = 14.5; 50 male and 60 female) was conducted in a public health clinic with patients diagnosed with diabetes mellitus who were under the medical supervision of an endocrinologist. The patients were evaluated through the Mini International Neuropsychiatric Interview and the Hospital Anxiety and Depression Scale(HADS). With respect to anxiety symptoms, we found a prevalence of 60% (n = 66) among patients, while in depression symptoms we found a prevalence of 53.6% (n = 59) concerning the 110 patients evaluated. More specifically, we found 28.2% (n = 31) of patients without depression or anxiety, 13.6% (n = 15) of patients with depression, 16.4% (n = 18) of patients with anxiety and 41.8% (n = 46) of patients with depression combined with anxiety. The most remarkable data were generalized anxiety disorder (22.7%), dysthymia (18.2%), panic disorder (8.2%) and social phobia (5.5%). The need for accurate assessments about the presence of symptoms related to psychopathology in patients with type 1 diabetes is evident.

  8. Psychiatric comorbidity in diabetes type 1: a cross-sectional observational study

    Directory of Open Access Journals (Sweden)

    Ana Claudia C. de Ornelas Maia

    2014-02-01

    Full Text Available Objective This study aims to investigate the prevalence of psychiatric disorders, i.e., the presence of signs and symptoms of anxiety and depression in type 1 diabetic patients, as well as to investigate the prevalence of psychiatric disorders in insulin dependent patients. Methods A cross-sectional observational study of 110 diabetic outpatients (mean = 58.3, SD = 14.5; 50 male and 60 female was conducted in a public health clinic with patients diagnosed with diabetes mellitus who were under the medical supervision of an endocrinologist. The patients were evaluated through the Mini International Neuropsychiatric Interview and the Hospital Anxiety and Depression Scale(HADS. Results With respect to anxiety symptoms, we found a prevalence of 60% (n = 66 among patients, while in depression symptoms we found a prevalence of 53.6% (n = 59 concerning the 110 patients evaluated. More specifically, we found 28.2% (n = 31 of patients without depression or anxiety, 13.6% (n = 15 of patients with depression, 16.4% (n = 18 of patients with anxiety and 41.8% (n = 46 of patients with depression combined with anxiety. The most remarkable data were generalized anxiety disorder (22.7%, dysthymia (18.2%, panic disorder (8.2% and social phobia (5.5%. Conclusion The need for accurate assessments about the presence of symptoms related to psychopathology in patients with type 1 diabetes is evident.

  9. The natural history of Charcot-Marie-Tooth type 1A in adults: a 5-year follow-up study

    NARCIS (Netherlands)

    Verhamme, Camiel; van Schaik, Ivo N.; Koelman, Johannes H. T. M.; de Haan, Rob J.; de Visser, Marianne

    2009-01-01

    Charcot-Marie-Tooth type 1A is the most prevalent hereditary demyelinating polyneuropathy. The aim of this study was to investigate the natural history of the disease in adults during a 5-year follow-up and to compare the changes over time with those found in normal ageing. In a cohort of 46 adult

  10. The interaction between the first transmembrane domain and the thumb of ASIC1a is critical for its N-glycosylation and trafficking.

    Directory of Open Access Journals (Sweden)

    Lan Jing

    Full Text Available Acid-sensing ion channel-1a (ASIC1a, the primary proton receptor in the brain, contributes to multiple diseases including stroke, epilepsy and multiple sclerosis. Thus, a better understanding of its biogenesis will provide important insights into the regulation of ASIC1a in diseases. Interestingly, ASIC1a contains a large, yet well organized ectodomain, which suggests the hypothesis that correct formation of domain-domain interactions at the extracellular side is a key regulatory step for ASIC1a maturation and trafficking. We tested this hypothesis here by focusing on the interaction between the first transmembrane domain (TM1 and the thumb of ASIC1a, an interaction known to be critical in channel gating. We mutated Tyr71 and Trp287, two key residues involved in the TM1-thumb interaction in mouse ASIC1a, and found that both Y71G and W287G decreased synaptic targeting and surface expression of ASIC1a. These defects were likely due to altered folding; both mutants showed increased resistance to tryptic cleavage, suggesting a change in conformation. Moreover, both mutants lacked the maturation of N-linked glycans through mid to late Golgi. These data suggest that disrupting the interaction between TM1 and thumb alters ASIC1a folding, impedes its glycosylation and reduces its trafficking. Moreover, reducing the culture temperature, an approach commonly used to facilitate protein folding, increased ASIC1a glycosylation, surface expression, current density and slowed the rate of desensitization. These results suggest that correct folding of extracellular ectodomain plays a critical role in ASIC1a biogenesis and function.

  11. A critical role of glutamate transporter type 3 in the learning and memory of mice.

    Science.gov (United States)

    Wang, Zhi; Park, Sang-Hon; Zhao, Huijuan; Peng, Shuling; Zuo, Zhiyi

    2014-10-01

    Hippocampus-dependent learning and memory are associated with trafficking of excitatory amino acid transporter type 3 (EAAT3) to the plasma membrane. To assess whether this trafficking is an intrinsic component of the biochemical responses underlying learning and memory, 7- to 9-week old male EAAT3 knockout mice and CD-1 wild-type mice were subjected to fear conditioning. Their hippocampal CA1 regions, amygdalae and entorhinal cortices were harvested before, or 30 min or 3 h after the fear conditioning stimulation. We found that EAAT3 knockout mice had worse contextual and tone-related learning and memory than did the wild-type mice. The expression of EAAT3, glutamate receptor (GluR)1 and GluR2 in the plasma membrane and of phospho-GluR1 (at Ser 831) and phospho-CaMKII in the hippocampus of the wild-type mice was increased at 30 min after the fear conditioning stimulation. Similar biochemical changes occurred in the amygdala. Fear conditioning also increased the expression of c-Fos and activity-regulated cytoskeleton-associated protein (Arc) in the CA1 regions and of Arc in the entorhinal cortices of the wild-type mice. These biochemical responses were attenuated in the EAAT3 knockout mice. These results suggest that EAAT3 plays a critical role in learning and memory. Our results also provide initial evidence that EAAT3 may have receptor-like functions to participate in the biochemical reactions underlying learning and memory. Copyright © 2014 Elsevier Inc. All rights reserved.

  12. Patient considerations in the management of type 2 diabetes – critical appraisal of dapagliflozin

    Directory of Open Access Journals (Sweden)

    Salvo MC

    2014-04-01

    Full Text Available Marissa C Salvo,1 Amie D Brooks,2 Stacey M Thacker3 1Department of Pharmacy Practice, University of Connecticut School of Pharmacy, Storrs, CT, 2Department of Pharmacy Practice, St Louis College of Pharmacy, St Louis, MO, 3Department of Pharmacy Practice, Southern Illinois University Edwardsville, Edwardsville, IL, USA Abstract: Type 2 diabetes affects more than 350 million people worldwide, and its prevalence is increasing. Many patients with diabetes do not achieve and/or maintain glycemic targets, despite therapy implementation and escalation. Multiple therapeutic classes of agents are available for the treatment of type 2 diabetes, and the armamentarium has expanded significantly in the past decade. Selective sodium glucose co-transporter 2 inhibitors, including dapagliflozin, represent the latest development in pharmacologic treatment options for type 2 diabetes. This class has a unique mechanism of action, working by increasing glucose excretion in the urine. The insulin-independent mechanism results in decreased serum glucose, without hypoglycemia or weight gain. Dapagliflozin is a once-daily oral therapy. Expanding therapy options for a complex patient population is critical, and dapagliflozin has a distinct niche that can be a viable option for select patients with diabetes. Keywords: SGLT2 inhibitor, selective sodium glucose co-transporter 2 inhibitors, pharmacological treatment

  13. A different role of angiotensin II type 1a receptor in the development and hypertrophy of plantaris muscle in mice.

    Science.gov (United States)

    Zempo, Hirofumi; Suzuki, Jun-Ichi; Ogawa, Masahito; Watanabe, Ryo; Isobe, Mitsuaki

    2016-02-01

    The role of angiotensin II type 1 (AT1) receptors in muscle development and hypertrophy remains unclear. This study was designed to reveal the effects that a loss of AT1 receptors has on skeletal muscle development and hypertrophy in mice. Eight-week-old male AT1a receptor knockout (AT1a(-/-)) mice were used for this experiment. The plantaris muscle to body weight ratio, muscle fiber cross-sectional area, and number of muscle fibers of AT1a(-/-) mice was significantly greater than wild type (WT) mice in the non-intervention condition. Next, the functional overload (OL) model was used to induce plantaris muscle hypertrophy by surgically removing the two triceps muscles consisting of the calf, soleus, and gastrocnemius muscles in mice. After 14 days of OL intervention, the plantaris muscle weight, the amount of fiber, and the fiber area increased. However, the magnitude of the increment of plantaris weight was not different between the two strains. Agtr1a mRNA expression did not change after OL in WT muscle. Actually, the Agt mRNA expression level of WT-OL was lower than WT-Control (C) muscle. An atrophy-related gene, atrogin-1 mRNA expression levels of AT1a(-/-)-C, WT-OL, and AT1a(-/-)-OL muscle were lower than that of WT-C muscle. Our findings suggest that AT1 receptor contributes to plantaris muscle development via atrogin-1 in mice.

  14. Cognitive decline over time in adults with myotonic dystrophy type 1: A 9-year longitudinal study.

    Science.gov (United States)

    Gallais, Benjamin; Gagnon, Cynthia; Mathieu, Jean; Richer, Louis

    2017-01-01

    Myotonic dystrophy type 1 (DM1) is an inherited neuromuscular disease with multisystemic involvement including the central nervous system. The evolution of the cognitive profile is a matter of debate, whether an eventual decline could be global or process-specific. Study aims are to describe, compare and document the clinical relevance of the progression of cognitive abilities in DM1 patients with adult and late-onset phenotypes. A total of 115 DM1 patients (90 adult; 25 late-onset) were assessed twice within a 9-year period on cognitive abilities (language, memory, visual attention, processing speed, visuoconstructive abilities and executive functions) and intellectual functioning (WAIS-R 7). A significant worsening over time was observed for verbal memory, visual attention, and processing speed. The progression in cognitive scores correlated with age and disease duration, but not with nCTG, muscular impairment nor education at baseline. Intellectual functioning remained stable. The rate of decline was higher among the late-onset phenotype than in the adult phenotype. Results showed that executive functions, language, and visual memory are impaired earlier in adult life, while verbal memory, visual attention, and processing speed decline later. Globally, results suggest an early and accelerated normal ageing process. This longitudinal study was based on the largest sample and the longest time period studied to date. These findings are highly relevant for clinical practice and genetic counselling. Copyright © 2016 Elsevier B.V. All rights reserved.

  15. Giant elephantiasis neuromatosa in the setting of neurofibromatosis type 1: A case report

    Science.gov (United States)

    PONTI, GIOVANNI; PELLACANI, GIOVANNI; MARTORANA, DAVIDE; MANDEL, VICTOR DESMOND; LOSCHI, PIETRO; POLLIO, ANNAMARIA; PECCHI, ANNARITA; DEALIS, CRISTINA; SEIDENARI, STEFANIA; TOMASI, ALDO

    2016-01-01

    Elephantiasis neuromatosa (EN) can arise from a plexiform neurofibroma of the superficial and deep nerves developing from a hyperproliferation of the perineural connective tissue infiltrating adjacent fat and muscles. To date, the clinical association between EN and neurofibromatosis type 1 (NF1) has been poorly defined, particularly with regard to the role of lymphatic alterations and the consequent lymphedema. The present study reports the clinical and biomolecular features of EN in a NF1 patient with the clear clinical diagnostic criteria of multiple cafè-au-lait macules, neurofibromas, EN, a positive family history and a novel NF1 germline c.1541_1542del mutation. Lymphoscintigraphy (LS) highlighted marked dermal backflow in the affected limb, hypertrophy of the ipsilateral inguinal and external iliac lymph nodes, and a bilateral lower limb lymph flow delay. These data support the hypothesis that an extensive hyperproliferative process involving perineural connective, limb soft tissues, bones and the lymphatic system can be responsible for EN in NF1 patients, on the basis of adipocyte metaplasia triggered by lymphostasis and lymphedema, and bone overgrowth and gigantism caused by chronic hyperemia. LS and magnetic resonance imaging can be efficacious tools in the diagnosis and clinical characterization of the early onset of the disease. PMID:27284375

  16. Structural and functional cardiac changes in myotonic dystrophy type 1: a cardiovascular magnetic resonance study

    Directory of Open Access Journals (Sweden)

    Hermans Mieke CE

    2012-07-01

    Full Text Available Abstract Background Myotonic dystrophy type 1 (MD1 is a neuromuscular disorder with potential involvement of the heart and increased risk of sudden death. Considering the importance of cardiomyopathy as a predictor of prognosis, we aimed to systematically evaluate and describe structural and functional cardiac alterations in patients with MD1. Methods Eighty MD1 patients underwent physical examination, electrocardiography (ECG, echocardiography and cardiovascular magnetic resonance (CMR. Blood samples were taken for determination of NT-proBNP plasma levels and CTG repeat length. Results Functional and structural abnormalities were detected in 35 patients (44%. Left ventricular systolic dysfunction was found in 20 cases, left ventricular dilatation in 7 patients, and left ventricular hypertrophy in 6 patients. Myocardial fibrosis was seen in 10 patients (12.5%. In general, patients had low left ventricular mass indexes. Right ventricular involvement was uncommon and only seen together with left ventricular abnormalities. Functional or structural cardiac involvement was associated with age (p = 0.04, male gender (p Conclusions CMR can be useful to detect early structural and functional myocardial abnormalities in patients with MD1. Myocardial involvement is strongly associated with conduction abnormalities, but a normal ECG does not exclude myocardial alterations. These findings lend support to the hypothesis that MD1 patients have a complex cardiac phenotype, including both myocardial and conduction system alteration.

  17. Lifestyle of patients with diabetes mellitus type 1: a systematic review.

    Science.gov (United States)

    Sales-Peres, Silvia Helena de Carvalho; Guedes, Maria de Fatima Santos; Sá, Letícia Marques; Negrato, Carlos Antonio; Lauris, José Roberto Pereira

    2016-04-01

    The aim of this review was to verify data concerning the relationship between the existent lifestyle and glycemic control in patients with Diabetes Mellitus Type 1 (DM1). The methods applied included the literature search strategy, selection of studies by means of inclusion and exclusion strategies, according to the characteristics of the studies. The search was conducted in the Lilacs, Medline, PubMed, Cochrame, SciELO and IBECS databases between in the period between 2005 and 2014. The articles selected were studies in humans, investing lifestyle, physical activities and glycemic levels. Of the 1798 studies initially identified, 11 met the eligibility criteria. Among the studies analyzed, 1 cohort; 1 longitudinal prospective, 1 case control and 8 transversal studies that approached the proposed theme were related. Regular physical activity was the variable that presented greatest relationship with the improvement in glycemic levels. Healthy active life, balanced diet, physical activities and education in diabetes improved the glycemic control of the DM1 patient. The results allowed the authors to conclude that a lifestyle based on physical activities interfered directly in the health of patients with DM1, in addition to contributing the glycemic control.

  18. Sphenoid dysplasia in neurofibromatosis type 1: a new technique for repair.

    Science.gov (United States)

    Concezio, Di Rocco; Amir, Samii; Gianpiero, Tamburrini; Luca, Massimi; Mario, Giordano

    2017-06-01

    Sphenoid bone dysplasia in neurofibromatosis type 1 is characterized by progressive exophthalmos and facial disfiguration secondary to herniation of meningeal and cerebral structures. We describe a technique for reconstruction of the sphenoid defect apt at preventing or correcting the ocular globe dislocation. After placement of spinal cerebrospinal fluid drainage to reduce intracranial pressure, the temporal pole is posteriorly dislocated extradurally. The greater sphenoid wing defect is identified. A titanium mesh covered by lyophilized dura, modeled in a curved fashion, is interposed between the bone defect and the cerebro-meningeal structures with its convex surface over the retracted temporal pole. The particular configuration of the titanium mesh allows a self-maintaining position due to the pressure exerted by the brain over its convex central part with its lateral margins consequently pushed and self-anchored against the medial and lateral walls of the temporal fossa. Screw fixation is not needed. The technique utilized in four cases proved to be reliable at the long-term clinical and neuroradiological controls (6 to 19 years). Sphenoid bone dysplasia in NF1, resulting in proptosis and exophthalmos, is usually progressive. It can be surgically repaired using a curved titanium mesh with the convexity faced to the temporal pole that is in the opposite fashion from all the techniques previously introduced. When utilized early in life, the technique can prevent the occurrence of the orbital and facial disfiguration.

  19. Safeguarding information intensive critical infrastructures against novel types of emerging failures

    Energy Technology Data Exchange (ETDEWEB)

    Balducelli, C. [ENEA-Italian National Agency for new Technology, Energy and the Environment Via Anguillasere 301, 00060 Rome (Italy)]. E-mail: claudio.balducelli@casaccia.enea.it; Bologna, S. [ENEA-Italian National Agency for new Technology, Energy and the Environment Via Anguillasere 301, 00060 Rome (Italy); Lavalle, L. [ENEA-Italian National Agency for new Technology, Energy and the Environment Via Anguillasere 301, 00060 Rome (Italy); Vicoli, G. [ENEA-Italian National Agency for new Technology, Energy and the Environment Via Anguillasere 301, 00060 Rome (Italy)

    2007-09-15

    The complexity of information intensive critical infrastructures, like electricity networks, telecommunication networks and public transportation networks is today augmented much more than in the past: such complexity augments the number of possible failures and anomalous working conditions and consequently decreases the survivability of the infrastructures. In this paper, the possibility is investigated to detect early anomalies and failures inside information intensive critical infrastructures by the introduction of anomaly detectors being 'self-aware' about the normal working conditions of the infrastructure itself. This approach has the objective to improve the performance of the most popular signature-based algorithms for intrusion detection, and makes use of different classes of time-oriented algorithms based on artificial intelligence paradigm. It has the advantage to work also in presence of unknown and unexpected types of attacks or failures. The tests, to evaluate the performance of the utilised detectors, are executed inside an emulated supervisory control and data acquisition (SCADA) system of an electrical power transmission grid, and a proposal for the future integration inside real SCADA systems is also reported.

  20. Safeguarding information intensive critical infrastructures against novel types of emerging failures

    International Nuclear Information System (INIS)

    Balducelli, C.; Bologna, S.; Lavalle, L.; Vicoli, G.

    2007-01-01

    The complexity of information intensive critical infrastructures, like electricity networks, telecommunication networks and public transportation networks is today augmented much more than in the past: such complexity augments the number of possible failures and anomalous working conditions and consequently decreases the survivability of the infrastructures. In this paper, the possibility is investigated to detect early anomalies and failures inside information intensive critical infrastructures by the introduction of anomaly detectors being 'self-aware' about the normal working conditions of the infrastructure itself. This approach has the objective to improve the performance of the most popular signature-based algorithms for intrusion detection, and makes use of different classes of time-oriented algorithms based on artificial intelligence paradigm. It has the advantage to work also in presence of unknown and unexpected types of attacks or failures. The tests, to evaluate the performance of the utilised detectors, are executed inside an emulated supervisory control and data acquisition (SCADA) system of an electrical power transmission grid, and a proposal for the future integration inside real SCADA systems is also reported

  1. Skeletal improvement in patients with Gaucher disease type 1: a phase 2 trial of oral eliglustat

    International Nuclear Information System (INIS)

    Kamath, Ravi S.; Lukina, Elena; Watman, Nora; Dragosky, Marta; Pastores, Gregory M.; Arreguin, Elsa Avila; Rosenbaum, Hanna; Zimran, Ari; Aguzzi, Rasha; Puga, Ana Cristina; Norfleet, Andrea M.; Peterschmitt, M.J.; Rosenthal, Daniel I.

    2014-01-01

    Eliglustat is an investigational oral substrate reduction therapy for Gaucher disease type 1 (GD1). Its skeletal effects were evaluated by prospective monitoring of bone mineral density (BMD), fractures, marrow infiltration by Gaucher cells, focal bone lesions, and infarcts during an open-label, multi-site, single-arm phase 2 trial (NCT00358150). Institutional review board approval and patient informed consent were obtained. Eliglustat (50 or 100 mg) was self-administered by mouth twice daily; 19 patients completed 4 years of treatment. All were skeletally mature (age range, 18-55 years). DXA and MRI assessments were conducted at baseline and annually thereafter. X-rays were obtained annually until month 24, and then every other year. Lumbar spine BMD increased significantly (p = 0.02; n = 15) by a mean (SD) of 9.9 % (14.2 %) from baseline to year 4; corresponding T-scores increased significantly (p = 0.01) from a mean (SD) of -1.6 (1.1) to -0.9 (1.3). Mean femur T-score remained normal through 4 years. Femur MRI showed that 10/18 (56 %) patients had decreased Gaucher cell infiltration compared to baseline; one patient with early improvement had transient worsening at year 4. There were no lumbar spine or femoral fractures and no reported bone crises during the study. At baseline, 8/19 (42 %) patients had focal bone lesions, which remained stable, and 7/19 (37 %) patients had bone infarctions, which improved in one patient by year 2. At year 4, one new asymptomatic, indeterminate bone lesion was discovered that subsequently resolved. Eliglustat may be a therapeutic option for treating the skeletal manifestations of GD1. (orig.)

  2. Radiological surveillance in multiple endocrine neoplasia type 1: a double-edged sword?

    Directory of Open Access Journals (Sweden)

    Ruth Therese Casey

    2017-03-01

    Full Text Available Context: Multiple endocrine neoplasia type 1 (MEN1 is a hereditary condition characterised by the predisposition to hyperplasia/tumours of endocrine glands. MEN1-related disease, moreover, malignancy related to MEN1, is increasingly responsible for death in up to two-thirds of patients. Although patients undergo radiological and biochemical surveillance, current recommendations for radiological monitoring are based on non-prospective data with little consensus or evidence demonstrating improved outcome from this approach. Here, we sought to determine whether cumulative radiation exposure as part of the recommended radiological screening programme posed a distinct risk in a cohort of patients with MEN1.Patients and study design: A retrospective review of 43 patients with MEN1 attending our institution between 2007 and 2015 was performed. Demographic and clinical information including phenotype was obtained for all patients. We also obtained details regarding all radiological procedures performed as part of MEN1 surveillance or disease localisation. An estimated effective radiation dose (ED for each individual patient was calculated.Results: The mean ED for the total patient cohort was 121 mSv, and the estimated mean lifetime risk of cancer secondary to radiation exposure was 0.49%. Patients with malignant neuroendocrine tumours (NETS had significantly higher ED levels compared to patients without metastatic disease (P < 0.0022.Conclusions: In MEN1, radiological surveillance is associated with clinically significant exposure to ionising radiation. In patients with MEN1, multi-modality imaging strategies designed to minimise this exposure should be considered.

  3. Skeletal improvement in patients with Gaucher disease type 1: a phase 2 trial of oral eliglustat

    Energy Technology Data Exchange (ETDEWEB)

    Kamath, Ravi S. [Massachusetts General Hospital and Harvard Medical School, Boston, MA (United States); Fairfax Radiological Consultants, Fairfax, VA (United States); Lukina, Elena [Russian Academy of Medical Sciences, Moscow (Russian Federation); Watman, Nora [Hospital Ramos Mejia, Buenos Aires (Argentina); Dragosky, Marta [Instituto Mexicano del Seguro Social Hospital de Especialidades, Col. La Raza (Mexico); Pastores, Gregory M. [New York University, New York (United States); Yale University School of Medicine, New Haven, CT (United States); Arreguin, Elsa Avila [Instituto Argentino de Diagnostico y Tratamiento, Buenos Aires (Argentina); Rosenbaum, Hanna [Rambam Medical Center, Haifa (Israel); Zimran, Ari [Sha' are Zedek Hebrew University and Hadassah Medical School, Jerusalem (Israel); Aguzzi, Rasha [Genzyme, a Sanofi company, Cambridge, MA (United States); Alexion Pharmaceuticals, Cambridge, MA (United States); Puga, Ana Cristina; Norfleet, Andrea M.; Peterschmitt, M.J. [Genzyme, a Sanofi company, Cambridge, MA (United States); Rosenthal, Daniel I. [Massachusetts General Hospital and Harvard Medical School, Boston, MA (United States); Massachusetts General Hospital, Department of Radiology, Boston, MA (United States)

    2014-10-15

    Eliglustat is an investigational oral substrate reduction therapy for Gaucher disease type 1 (GD1). Its skeletal effects were evaluated by prospective monitoring of bone mineral density (BMD), fractures, marrow infiltration by Gaucher cells, focal bone lesions, and infarcts during an open-label, multi-site, single-arm phase 2 trial (NCT00358150). Institutional review board approval and patient informed consent were obtained. Eliglustat (50 or 100 mg) was self-administered by mouth twice daily; 19 patients completed 4 years of treatment. All were skeletally mature (age range, 18-55 years). DXA and MRI assessments were conducted at baseline and annually thereafter. X-rays were obtained annually until month 24, and then every other year. Lumbar spine BMD increased significantly (p = 0.02; n = 15) by a mean (SD) of 9.9 % (14.2 %) from baseline to year 4; corresponding T-scores increased significantly (p = 0.01) from a mean (SD) of -1.6 (1.1) to -0.9 (1.3). Mean femur T-score remained normal through 4 years. Femur MRI showed that 10/18 (56 %) patients had decreased Gaucher cell infiltration compared to baseline; one patient with early improvement had transient worsening at year 4. There were no lumbar spine or femoral fractures and no reported bone crises during the study. At baseline, 8/19 (42 %) patients had focal bone lesions, which remained stable, and 7/19 (37 %) patients had bone infarctions, which improved in one patient by year 2. At year 4, one new asymptomatic, indeterminate bone lesion was discovered that subsequently resolved. Eliglustat may be a therapeutic option for treating the skeletal manifestations of GD1. (orig.)

  4. Sleep-Related Breathing Disorders in Chiari Malformation Type 1: A Prospective Study of 90 Patients.

    Science.gov (United States)

    Ferré, Álex; Poca, Maria A; de la Calzada, Maria D; Moncho, Dulce; Romero, Odile; Sampol, Gabriel; Sahuquillo, Juan

    2017-06-01

    The aim of the present study is to describe the prevalence of sleep disorders in a large group of patients with Chiari malformation type 1 (CM-1) and determine the presence of risk factors associated with these abnormalities. Prospective study with consecutive patient selection. We included 90 adult patients with CM-1, defined by the presence of a cerebellar tonsillar descent (TD) ≥3 mm. Clinical, neuroradiological studies, and nocturnal polysomnography (PSG) was carried out. In addition, patients were also subclassified into 2 CM subtypes: CM-1, with the obex above the foramen magnum (FM) and CM-1.5, in which along with a TD ≥3 mm, the obex was located below the FM. We observed a high prevalence (50%) of sleep-related breathing disorders (SRBDs) with predominant hypopnea. Only six patients showed a central apnea index of ≥5. Hypoventilation was observed in only three patients. SRBD severity was associated with male sex, older age, excess weight, and the presence of hydrocephalus. No differences in clinical or PSG parameters were found when comparing CM subtypes (CM-1 and CM-1.5). Sleep architecture study showed decreased sleep efficiency with an increase in arousal and waking after sleep onset. The presence of SRBDs was found to be associated with poorer sleep architecture parameters. This study confirms a high prevalence of SRBDs in patients with CM-1 and CM-1.5, with a predominant obstructive component. Nocturnal PSG recordings should be systematically conducted in these patients, especially those who are male, older, or overweight or those who present hydrocephalus. © Sleep Research Society 2017. Published by Oxford University Press on behalf of the Sleep Research Society. All rights reserved. For permissions, please e-mail journals.permissions@oup.com.

  5. Brain Abnormalities in Congenital Fibrosis of the Extraocular Muscles Type 1: A Multimodal MRI Imaging Study.

    Directory of Open Access Journals (Sweden)

    Wen Miao

    Full Text Available To explore the possible brain structural and functional alterations in congenital fibrosis of extraocular muscles type 1 (CFEOM1 patients using multimodal MRI imaging.T1-weighted, diffusion tensor images and functional MRI data were obtained from 9 KIF21A positive patients and 19 age- and gender-matched healthy controls. Voxel based morphometry and tract based spatial statistics were applied to the T1-weighted and diffusion tensor images, respectively. Amplitude of low frequency fluctuations and regional homogeneity were used to process the functional MRI data. We then compared these multimodal characteristics between CFEOM1 patients and healthy controls.Compared with healthy controls, CFEOM1 patients demonstrated increased grey matter volume in bilateral frontal orbital cortex and in the right temporal pole. No diffusion indices changes were detected, indicating unaffected white matter microstructure. In addition, from resting state functional MRI data, trend of amplitude of low-frequency fluctuations increases were noted in the right inferior parietal lobe and in the right frontal cortex, and a trend of ReHo increase (p<0.001 uncorrected in the left precentral gyrus, left orbital frontal cortex, temporal pole and cingulate gyrus.CFEOM1 patients had structural and functional changes in grey matter, but the white matter was unaffected. These alterations in the brain may be due to the abnormality of extraocular muscles and their innervating nerves. Future studies should consider the possible correlations between brain morphological/functional findings and clinical data, especially pertaining to eye movements, to obtain more precise answers about the role of brain area changes and their functional consequence in CFEOM1.

  6. Brain Abnormalities in Congenital Fibrosis of the Extraocular Muscles Type 1: A Multimodal MRI Imaging Study.

    Science.gov (United States)

    Miao, Wen; Man, Fengyuan; Wu, Shaoqin; Lv, Bin; Wang, Zhenchang; Xian, Junfang; Sabel, Bernhard A; He, Huiguang; Jiao, Yonghong

    2015-01-01

    To explore the possible brain structural and functional alterations in congenital fibrosis of extraocular muscles type 1 (CFEOM1) patients using multimodal MRI imaging. T1-weighted, diffusion tensor images and functional MRI data were obtained from 9 KIF21A positive patients and 19 age- and gender-matched healthy controls. Voxel based morphometry and tract based spatial statistics were applied to the T1-weighted and diffusion tensor images, respectively. Amplitude of low frequency fluctuations and regional homogeneity were used to process the functional MRI data. We then compared these multimodal characteristics between CFEOM1 patients and healthy controls. Compared with healthy controls, CFEOM1 patients demonstrated increased grey matter volume in bilateral frontal orbital cortex and in the right temporal pole. No diffusion indices changes were detected, indicating unaffected white matter microstructure. In addition, from resting state functional MRI data, trend of amplitude of low-frequency fluctuations increases were noted in the right inferior parietal lobe and in the right frontal cortex, and a trend of ReHo increase (pleft precentral gyrus, left orbital frontal cortex, temporal pole and cingulate gyrus. CFEOM1 patients had structural and functional changes in grey matter, but the white matter was unaffected. These alterations in the brain may be due to the abnormality of extraocular muscles and their innervating nerves. Future studies should consider the possible correlations between brain morphological/functional findings and clinical data, especially pertaining to eye movements, to obtain more precise answers about the role of brain area changes and their functional consequence in CFEOM1.

  7. Brain Abnormalities in Congenital Fibrosis of the Extraocular Muscles Type 1: A Multimodal MRI Imaging Study

    Science.gov (United States)

    Wu, Shaoqin; Lv, Bin; Wang, Zhenchang; Xian, Junfang; Sabel, Bernhard A.; He, Huiguang; Jiao, Yonghong

    2015-01-01

    Purpose To explore the possible brain structural and functional alterations in congenital fibrosis of extraocular muscles type 1 (CFEOM1) patients using multimodal MRI imaging. Methods T1-weighted, diffusion tensor images and functional MRI data were obtained from 9 KIF21A positive patients and 19 age- and gender- matched healthy controls. Voxel based morphometry and tract based spatial statistics were applied to the T1-weighted and diffusion tensor images, respectively. Amplitude of low frequency fluctuations and regional homogeneity were used to process the functional MRI data. We then compared these multimodal characteristics between CFEOM1 patients and healthy controls. Results Compared with healthy controls, CFEOM1 patients demonstrated increased grey matter volume in bilateral frontal orbital cortex and in the right temporal pole. No diffusion indices changes were detected, indicating unaffected white matter microstructure. In addition, from resting state functional MRI data, trend of amplitude of low-frequency fluctuations increases were noted in the right inferior parietal lobe and in the right frontal cortex, and a trend of ReHo increase (pleft precentral gyrus, left orbital frontal cortex, temporal pole and cingulate gyrus. Conclusions CFEOM1 patients had structural and functional changes in grey matter, but the white matter was unaffected. These alterations in the brain may be due to the abnormality of extraocular muscles and their innervating nerves. Future studies should consider the possible correlations between brain morphological/functional findings and clinical data, especially pertaining to eye movements, to obtain more precise answers about the role of brain area changes and their functional consequence in CFEOM1. PMID:26186732

  8. Interactions between environmental factors and melatonin receptor type 1A polymorphism in relation to oral cancer susceptibility and clinicopathologic development.

    Directory of Open Access Journals (Sweden)

    Feng-Yan Lin

    Full Text Available The purpose of this study was to explore the combined effect of melatonin receptor type 1A (MTNR1A gene polymorphisms and exposure to environmental carcinogens on the susceptibility and clinicopathological characteristics of oral cancer.Three polymorphisms of the MTNR1A gene from 618 patients with oral cancer and 560 non-cancer controls were analyzed by real-time polymerase chain reaction (PCR. The CTA haplotype of the studied MTNR1A polymorphisms (rs2119882, rs13140012, rs6553010 was related to a higher risk of oral cancer. Moreover, MTNR1A gene polymorphisms exhibited synergistic effects of environmental factors (betel quid and tobacco use on the susceptibility of oral cancer. Finally, oral-cancer patients with betel quid-chewing habit who had T/T allele of MTNR1A rs13140012 were at higher risk for developing an advanced clinical stage and lymph node metastasis.These results support gene-environment interactions of MTNR1A polymorphisms with smoking and betel quid-chewing habits possibly altering oral-cancer susceptibility and metastasis.

  9. Complete loss of insulin secretion capacity in type 1A diabetes patients during long-term follow up.

    Science.gov (United States)

    Uno, Sae; Imagawa, Akihisa; Kozawa, Junji; Fukui, Kenji; Iwahashi, Hiromi; Shimomura, Iichiro

    2017-10-16

    Patients with type 1 diabetes are classified into three subtypes in Japan: acute onset, fulminant and slowly progressive. Acute-onset type 1 diabetes would be equivalent to type 1A diabetes, the typical type 1 diabetes in Western countries. The insulin secretion capacity in Japanese patients with long-standing type 1A diabetes is unclear. The aim of the present study was to clarify the course of endogenous insulin secretion during long-term follow up and the factors associated with residual insulin secretion in patients with acute-onset type 1 diabetes (autoimmune). We retrospectively investigated endogenous insulin secretion capacity in 71 patients who fulfilled the diagnostic criteria for acute-onset type 1 diabetes (autoimmune) in Japan. To assess the residual insulin secretion capacity, we evaluated randomly measured C-peptide levels and the results of glucagon stimulation test in 71 patients. In the first year of disease, the child- and adolescent-onset patients had significantly more in residual insulin secretion than the adult-onset patients (34 patients in total). C-peptide levels declined more rapidly in patients whose age of onset was ≤18 years than in patients whose age of onset was ≥19 years. Endogenous insulin secretion capacity stimulated by glucagon was completely lost in almost all patients at >15 years after onset (61 patients in total). Most patients with acute-onset type 1 diabetes (autoimmune) completely lose their endogenous insulin secretion capacity during the disease duration in Japan. Age of onset might affect the course of insulin secretion. © 2017 The Authors. Journal of Diabetes Investigation published by Asian Association for the Study of Diabetes (AASD) and John Wiley & Sons Australia, Ltd.

  10. The role of telenursing in the management of Diabetes Type 1: A randomized controlled trial.

    Science.gov (United States)

    Kotsani, Konstantia; Antonopoulou, Vasiliki; Kountouri, Aikaterini; Grammatiki, Maria; Rapti, Eleni; Karras, Spyridon; Trakatelli, Christina; Tsaklis, Panagiotis; Kazakos, Kiriakos; Kotsa, Kalliopi

    2018-04-01

    Diabetes Mellitus type 1 (T1DM) is a chronic disease that requires patients' self-monitoring and self-management to achieve glucose targets and prevent complications. Telenursing implicates technology in the interaction of a specialized nurse with patients with chronic diseases in order to provide personalized care and support. To evaluate the effect of telenursing on T1DM patients' compliance with glucose self-monitoring and glycemic control. Randomized controlled study. Outpatient Department of Diabetes, Endocrinology and Metabolism of a University Hospital in Northern Greece. Ninety-four T1DM patients were recruited and randomized in two groups by a random number generator. The intervention group (N = 48) was provided with telenursing services. A specialized nurse made a weekly contact via telephone motivating patients to frequently measure blood glucose and adopt a healthy lifestyle. The control group (N = 46) received standard diabetes advice and care in the clinic. The primary outcome was the effect of the intervention in glucose control and glucose variability. The secondary outcome was the effect on frequency of self-monitoring. SPSS 20.0 was used for data analysis. The two groups did not differ in age, sex, physical activity or initial HbA1c. In the intervention group, blood glucose significantly decreased at the end of the study in all predefined measurements, compared to control group: morning (93.18 ± 13.30 mg/dl vs. 105.17 ± 13.74 mg/dl, p < 0.005), pre-prandial (114.76 ± 9.54 mg/dl vs. 120.84 ± 4.05 mg/dl, p < 0.005), post-prandial (193.35 ± 25.36 mg/dl vs. 207.84 ± 18.80 mg/dl, p < 0.005), and HbA1c decreased significantly over time in the intervention group (8.3 ± 0.6% at the beginning of the study vs. 7.8 ± 1% at the end of the study, p = 0.03). In the intervention group there were also fewer omitted glucose measurements than in the control group. Patients in the

  11. Critical early roles for col27a1a and col27a1b in zebrafish notochord morphogenesis, vertebral mineralization and post-embryonic axial growth.

    Science.gov (United States)

    Christiansen, Helena E; Lang, Michael R; Pace, James M; Parichy, David M

    2009-12-29

    Fibrillar collagens are well known for their links to human diseases, with which all have been associated except for the two most recently identified fibrillar collagens, type XXIV collagen and type XXVII collagen. To assess functions and potential disease phenotypes of type XXVII collagen, we examined its roles in zebrafish embryonic and post-embryonic development. We identified two type XXVII collagen genes in zebrafish, col27a1a and col27a1b. Both col27a1a and col27a1b were expressed in notochord and cartilage in the embryo and early larva. To determine sites of type XXVII collagen function, col27a1a and col27a1b were knocked down using morpholino antisense oligonucleotides. Knockdown of col27a1a singly or in conjunction with col27a1b resulted in curvature of the notochord at early stages and formation of scoliotic curves as well as dysmorphic vertebrae at later stages. These defects were accompanied by abnormal distributions of cells and protein localization in the notochord, as visualized by transmission electron microscopy, as well as delayed vertebral mineralization as detected histologically. Together, our findings indicate a key role for type XXVII collagen in notochord morphogenesis and axial skeletogenesis and suggest a possible human disease phenotype.

  12. Assessment of Newly Proposed Clinical Criteria to Identify HNF1A MODY in Patients with an Initial Diagnosis of Type 1 or Type 2 Diabetes Mellitus

    Directory of Open Access Journals (Sweden)

    Malgorzata Grzanka

    2016-01-01

    Full Text Available The most common form of maturity-onset diabetes of the young (MODY is caused by mutations in the hepatocyte nuclear factor 1A (HNF1A gene. However, most HNF1A mutation-carriers are initially misdiagnosed with type 1 (T1DM or type 2 (T2DM diabetes mellitus; hence, they often receive nonoptimal treatment. The aim of our study was to test newly proposed clinical criteria for the identification of HNF1A MODY in patients with a diagnosis of T1DM or T2DM. To achieve this, the following criteria to preselect patients for screening were used: for T1DM: TDIR (total daily insulin requirement > 0.3 IU of insulin/kg and the percentage of basal insulin > 30% of TDIR; for T2DM: sulphonylurea- (SU- based oral treatment (monotherapy or combined with Metformin > 15 years and BMI < 30 kg/m2. We reviewed the clinical data of 140 patients with T1DM and 524 clinically diagnosed with T2DM. On the basis of these criteria, we found a HNF1A mutation in 1 out of 2 individuals with a diagnosis of T1DM and 1 out of 11 selected individuals with a diagnosis of T2DM. We believe that the simplicity of the proposed criteria might prove useful in clinical practice, as an alternative to more time-consuming classical diagnostic techniques.

  13. Assessment of Newly Proposed Clinical Criteria to Identify HNF1A MODY in Patients with an Initial Diagnosis of Type 1 or Type 2 Diabetes Mellitus.

    Science.gov (United States)

    Grzanka, Malgorzata; Matejko, Bartlomiej; Szopa, Magdalena; Kiec-Wilk, Beata; Malecki, Maciej T; Klupa, Tomasz

    2016-01-01

    The most common form of maturity-onset diabetes of the young (MODY) is caused by mutations in the hepatocyte nuclear factor 1A (HNF1A) gene. However, most HNF1A mutation-carriers are initially misdiagnosed with type 1 (T1DM) or type 2 (T2DM) diabetes mellitus; hence, they often receive nonoptimal treatment. The aim of our study was to test newly proposed clinical criteria for the identification of HNF1A MODY in patients with a diagnosis of T1DM or T2DM. To achieve this, the following criteria to preselect patients for screening were used: for T1DM: TDIR (total daily insulin requirement) > 0.3 IU of insulin/kg and the percentage of basal insulin > 30% of TDIR; for T2DM: sulphonylurea- (SU-) based oral treatment (monotherapy or combined with Metformin) > 15 years and BMI < 30 kg/m(2). We reviewed the clinical data of 140 patients with T1DM and 524 clinically diagnosed with T2DM. On the basis of these criteria, we found a HNF1A mutation in 1 out of 2 individuals with a diagnosis of T1DM and 1 out of 11 selected individuals with a diagnosis of T2DM. We believe that the simplicity of the proposed criteria might prove useful in clinical practice, as an alternative to more time-consuming classical diagnostic techniques.

  14. Functional Investigations of HNF1A Identify Rare Variants as Risk Factors for Type 2 Diabetes in the General Population

    Science.gov (United States)

    Najmi, Laeya Abdoli; Aukrust, Ingvild; Flannick, Jason; Molnes, Janne; Burtt, Noel; Molven, Anders; Groop, Leif; Altshuler, David; Johansson, Stefan; Njølstad, Pål Rasmus

    2017-01-01

    Variants in HNF1A encoding hepatocyte nuclear factor 1α (HNF-1A) are associated with maturity-onset diabetes of the young form 3 (MODY 3) and type 2 diabetes. We investigated whether functional classification of HNF1A rare coding variants can inform models of diabetes risk prediction in the general population by analyzing the effect of 27 HNF1A variants identified in well-phenotyped populations (n = 4,115). Bioinformatics tools classified 11 variants as likely pathogenic and showed no association with diabetes risk (combined minor allele frequency [MAF] 0.22%; odds ratio [OR] 2.02; 95% CI 0.73–5.60; P = 0.18). However, a different set of 11 variants that reduced HNF-1A transcriptional activity to diabetes in the general population (combined MAF 0.22%; OR 5.04; 95% CI 1.99–12.80; P = 0.0007). Our functional investigations indicate that 0.44% of the population carry HNF1A variants that result in a substantially increased risk for developing diabetes. These results suggest that functional characterization of variants within MODY genes may overcome the limitations of bioinformatics tools for the purposes of presymptomatic diabetes risk prediction in the general population. PMID:27899486

  15. Expression of the sweetpotato R2R3-type IbMYB1a gene induces anthocyanin accumulation in Arabidopsis.

    Science.gov (United States)

    Chu, Hyosub; Jeong, Jae Cheol; Kim, Wook-Jin; Chung, Dong Min; Jeon, Hyo Kon; Ahn, Young Ock; Kim, Sun Ha; Lee, Haeng-Soon; Kwak, Sang-Soo; Kim, Cha Young

    2013-06-01

    R2R3-type MYB transcription factors (TFs) play important roles in transcriptional regulation of anthocyanins. The R2R3-type IbMYB1 is known to be a key regulator of anthocyanin biosynthesis in the storage roots of sweetpotato. We previously showed that transient expression of IbMYB1a led to anthocyanin pigmentation in tobacco leaves. In this article, we generated transgenic Arabidopsis plants expressing the IbMYB1a gene under the control of CaMV 35S promoter, and the sweetpotato SPO and SWPA2 promoters. Overexpression of IbMYBa in transgenic Arabidopsis produced strong anthocyanin pigmentation in seedlings and generated a deep purple color in leaves, stems and seeds. Reverse transcription-polymerase chain reaction analysis showed that IbMYB1a expression induced upregulation of several structural genes in the anthocyanin biosynthetic pathway, including 4CL, CHI, F3'H, DFR, AGT, AAT and GST. Furthermore, overexpression of IbMYB1a led to enhanced expression of the AtTT8 (bHLH) and PAP1/AtMYB75 genes. high-performance liquid chromatography analysis revealed that IbMYB1a expression led to the production of cyanidin as a major core molecule of anthocyanidins in Arabidopsis, as occurs in the purple leaves of sweetpotato (cv. Sinzami). This result shows that the IbMYB1a TF is sufficient to induce anthocyanin accumulation in seedlings, leaves, stems and seeds of Arabidopsis plants. Copyright © Physiologia Plantarum 2012.

  16. AC losses in a type II superconductor strip with inhomogeneous critical current distribution

    International Nuclear Information System (INIS)

    Tsukamoto, Osami

    2005-01-01

    Analytical formulae derived by Brandt and Indenbom (1993 Phys. Rev. B 48 12893-906) and Norris (1970 J. Phys. D: Appl. Phys. 3 489-507) are often used to calculate the magnetization and AC transport current losses in HTS strip conductors, respectively. In these formulae, homogeneous distribution of critical sheet current density σ c in the strip is assumed. However, it is considered that σ c distributions are inhomogeneous in actual HTS strips and that the inhomogeneous σ c distributions cause deviations of the measured AC loss data of actual HTS strips from those formulae. A semi-analytical method to calculate AC transport current and magnetization losses is derived for a type II superconductor strip with inhomogeneous distribution of σ c in the direction of the strip width. The method is derived modifying the analysis of Brandt et al. The validity of the semi-analytical method is shown by comparing the results calculated by this method with those calculated by the Norris and Brandt formulae and by a different method of our previous work and also with experimental data. Moreover, it is shown that the deviation of the measured data from the Norris and Brandt models can be estimated by assuming proper σ c distributions

  17. Duplication in chromosome 17p11.2 in Charcot-Marie-Tooth neuropathy type 1a (CMT 1a). The HMSN Collaborative Research Group

    NARCIS (Netherlands)

    Raeymaekers, P.; Timmerman, V.; Nelis, E.; de Jonghe, P.; Hoogendijk, J. E.; Baas, F.; Barker, D. F.; Martin, J. J.; de Visser, M.; Bolhuis, P. A.

    1991-01-01

    Hereditary motor and sensory neuropathy type I (HMSN I) or Charcot-Marie-Tooth disease type 1 (CMT 1) is an autosomal dominant disorder of the peripheral nervous system characterized by progressive weakness and atrophy of distal limb muscles. In the majority of HMSN I families, linkage studies

  18. The mechanism of critical strain and serration type of the serrated flow in Mg–Nd–Zn alloy

    Energy Technology Data Exchange (ETDEWEB)

    Wang, W.H. [The Group of Magnesium Alloys and Their Applications, Institute of Metal Research Chinese Academy of Sciences, 62 Wencui Road, Shenyang 110016 (China); School of Materials Science and Engineering, Shenyang Ligong University, 6 Nanpingzhong Road, Shenyang 110159 (China); Wu, D., E-mail: dwu@imr.ac.cn [The Group of Magnesium Alloys and Their Applications, Institute of Metal Research Chinese Academy of Sciences, 62 Wencui Road, Shenyang 110016 (China); Shah, S.S.A. [The Group of Magnesium Alloys and Their Applications, Institute of Metal Research Chinese Academy of Sciences, 62 Wencui Road, Shenyang 110016 (China); Chen, R.S., E-mail: rschen@imr.ac.cn [The Group of Magnesium Alloys and Their Applications, Institute of Metal Research Chinese Academy of Sciences, 62 Wencui Road, Shenyang 110016 (China); Lou, C.S. [School of Materials Science and Engineering, Shenyang Ligong University, 6 Nanpingzhong Road, Shenyang 110159 (China)

    2016-01-01

    In present research the serrated flow has been observed successfully after a critical amount of strain. Two relationships between the critical strain and temperature i.e. normal and inverse, corresponding to each serration type were studied. In order to investigate systematically the onset of serrated flow and serration type in NZ31 alloy, samples in solutionized condition were tensile tested at the temperature ranging from 100 °C to 300 °C with the strain rate ranging from 1×10{sup −4} s{sup −1} to 1×10{sup −2} s{sup −1}. Results showed that normal critical strain appeared with type A and B serrated flow at temperature from 150°C to 250 °C, and inverse critical strain appeared with type C at temperature from 275 °C to 300 °C. Through analyzing the mechanism of three serration types, we found that the production of serration required improvement in diffusion for solute atoms for pinning process at low temperature, and enhance the moving ability of dislocations for unpinning process at high temperature, which need the assistance of the strain and stress respectively. So, in this work, the critical strain for pinning and the critical stress for unpinning processes were defined, which give a better explanation to the variation tendency of two definitions in accordance with temperature. Furthermore, this relationship results in the critical strain for onset of serrated flow changing from normal to inverse and corresponding different serrations.

  19. Theory of flux cutting and flux transport at the critical current of a type-II superconducting cylindrical wire

    International Nuclear Information System (INIS)

    Clem, John R.

    2011-01-01

    I introduce a critical-state theory incorporating both flux cutting and flux transport to calculate the magnetic-field and current-density distributions inside a type-II superconducting cylinder at its critical current in a longitudinal applied magnetic field. The theory is an extension of the elliptic critical-state model introduced by Romero-Salazar and Perez-Rodriguez. The vortex dynamics depend in detail on two nonlinear effective resistivities for flux cutting (ρ(parallel)) and flux flow (ρ(perpendicular)), and their ratio r = ρ(parallel)/ρ(perpendicular). When r c (φ) that makes the vortex arc unstable.

  20. Vascular Type 1A Angiotensin II Receptors Control BP by Regulating Renal Blood Flow and Urinary Sodium Excretion.

    Science.gov (United States)

    Sparks, Matthew A; Stegbauer, Johannes; Chen, Daian; Gomez, Jose A; Griffiths, Robert C; Azad, Hooman A; Herrera, Marcela; Gurley, Susan B; Coffman, Thomas M

    2015-12-01

    Inappropriate activation of the type 1A angiotensin (AT1A) receptor contributes to the pathogenesis of hypertension and its associated complications. To define the role for actions of vascular AT1A receptors in BP regulation and hypertension pathogenesis, we generated mice with cell-specific deletion of AT1A receptors in smooth muscle cells (SMKO mice) using Loxp technology and Cre transgenes with robust expression in both conductance and resistance arteries. We found that elimination of AT1A receptors from vascular smooth muscle cells (VSMCs) caused a modest (approximately 7 mmHg) yet significant reduction in baseline BP and exaggerated sodium sensitivity in mice. Additionally, the severity of angiotensin II (Ang II)-dependent hypertension was dramatically attenuated in SMKO mice, and this protection against hypertension was associated with enhanced urinary excretion of sodium. Despite the lower BP, acute vasoconstrictor responses to Ang II in the systemic vasculature were largely preserved (approximately 80% of control levels) in SMKO mice because of exaggerated activity of the sympathetic nervous system rather than residual actions of AT1B receptors. In contrast, Ang II-dependent responses in the renal circulation were almost completely eliminated in SMKO mice (approximately 5%-10% of control levels). These findings suggest that direct actions of AT1A receptors in VSMCs are essential for regulation of renal blood flow by Ang II and highlight the capacity of Ang II-dependent vascular responses in the kidney to effect natriuresis and BP control. Copyright © 2015 by the American Society of Nephrology.

  1. KIF1A, an axonal transporter of synaptic vesicles, is mutated in hereditary sensory and autonomic neuropathy type 2.

    Science.gov (United States)

    Rivière, Jean-Baptiste; Ramalingam, Siriram; Lavastre, Valérie; Shekarabi, Masoud; Holbert, Sébastien; Lafontaine, Julie; Srour, Myriam; Merner, Nancy; Rochefort, Daniel; Hince, Pascale; Gaudet, Rébecca; Mes-Masson, Anne-Marie; Baets, Jonathan; Houlden, Henry; Brais, Bernard; Nicholson, Garth A; Van Esch, Hilde; Nafissi, Shahriar; De Jonghe, Peter; Reilly, Mary M; Timmerman, Vincent; Dion, Patrick A; Rouleau, Guy A

    2011-08-12

    Hereditary sensory and autonomic neuropathy type II (HSANII) is a rare autosomal-recessive disorder characterized by peripheral nerve degeneration resulting in a severe distal sensory loss. Although mutations in FAM134B and the HSN2 exon of WNK1 were associated with HSANII, the etiology of a substantial number of cases remains unexplained. In addition, the functions of WNK1/HSN2 and FAM134B and their role in the peripheral nervous system remain poorly understood. Using a yeast two-hybrid screen, we found that KIF1A, an axonal transporter of synaptic vesicles, interacts with the domain encoded by the HSN2 exon. In parallel to this screen, we performed genome-wide homozygosity mapping in a consanguineous Afghan family affected by HSANII and identified a unique region of homozygosity located on chromosome 2q37.3 and spanning the KIF1A gene locus. Sequencing of KIF1A in this family revealed a truncating mutation segregating with the disease phenotype. Subsequent sequencing of KIF1A in a series of 112 unrelated patients with features belonging to the clinical spectrum of ulcero-mutilating sensory neuropathies revealed truncating mutations in three additional families, thus indicating that mutations in KIF1A are a rare cause of HSANII. Similarly to WNK1 mutations, pathogenic mutations in KIF1A were almost exclusively restricted to an alternatively spliced exon. This study provides additional insights into the molecular pathogenesis of HSANII and highlights the potential biological relevance of alternative splicing in the peripheral sensory nervous system. Copyright © 2011 The American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

  2. Variability and distribution of COL1A2 (type I collagen) polymorphisms in the central-eastern Mediterranean Basin.

    Science.gov (United States)

    Scorrano, Gabriele; Lelli, Roberta; Martínez-Labarga, Cristina; Scano, Giuseppina; Contini, Irene; Hafez, Hani S; Rudan, Pavao; Rickards, Olga

    2016-01-01

    The most abundant of the collagen protein family, type I collagen is encoded by the COL1A2 gene. The COL1A2 restriction fragment length polymorphisms (RFLPs) EcoRI, RsaI and MspI in samples from several different central-eastern Mediterranean populations were analysed and found to be potentially informative anthropogenetic markers. The objective was to define the genetic variability of COL1A2 in the central-eastern Mediterranean and to shed light on its genetic distribution in human groups over a wide geographic area. PCR-RFLP analysis of EcoRI, RsaI and MspI polymorphisms of the COL1A2 gene was performed on oral swab and blood samples from 308 individuals from the central-eastern Mediterranean Basin. The genetic similarities among these groups and other populations described in the literature were investigated through correspondence analysis. Single-marker data and haplotype frequencies seemed to suggest a genetic homogeneity within the European populations, whereas a certain degree of differentiation was noted for the Egyptians and the Turks. The genetic variability in the central-eastern Mediterranean area is probably a result of the geographical barrier of the Mediterranean Sea, which separated European and African populations over time.

  3. Magnetic field and temperature dependence of the critical vortex velocity in type-II superconducting films

    Energy Technology Data Exchange (ETDEWEB)

    Grimaldi, G; Leo, A; Cirillo, C; Attanasio, C; Nigro, A; Pace, S [CNR-INFM Laboratorio Regionale SuperMat, Via Salvador Allende, I-84081 Baronissi (Italy)], E-mail: grimaldi@sa.infn.it

    2009-06-24

    We study the vortex dynamics in the instability regime induced by high dissipative states well above the critical current in Nb superconducting strips. The magnetic field and temperature behavior of the critical vortex velocity corresponding to the observed dynamic instability is ascribed to intrinsic non-equilibrium phenomena. The Larkin-Ovchinnikov (LO) theory of electronic instability in high velocity vortex motion has been applied to interpret the temperature dependence of the critical vortex velocity. The magnetic field dependence of the vortex critical velocity shows new features in the low-field regime not predicted by LO.

  4. Angiotensin II type 1a receptors in subfornical organ contribute towards chronic intermittent hypoxia-associated sustained increase in mean arterial pressure.

    Science.gov (United States)

    Saxena, Ashwini; Little, Joel T; Nedungadi, T Prashant; Cunningham, J Thomas

    2015-03-01

    Sleep apnea is associated with hypertension. The mechanisms contributing to a sustained increase in mean arterial pressure (MAP) even during normoxic awake-state remain unknown. Rats exposed to chronic intermittent hypoxia for 7 days, a model of the hypoxemia associated with sleep apnea, exhibit sustained increases in MAP even during the normoxic dark phase. Activation of the renin-angiotensin system (RAS) has been implicated in chronic intermittent hypoxia (CIH) hypertension. Since the subfornical organ (SFO) serves as a primary target for the central actions of circulating ANG II, we tested the effects of ANG II type 1a receptor (AT1aR) knockdown in the SFO on the sustained increase in MAP in this CIH model. Adeno-associated virus carrying green fluorescent protein (GFP) and small-hairpin RNA against either AT1aR or a scrambled control sequence (SCM) was stereotaxically injected in the SFO of rats. After recovery, MAP, heart rate, respiratory rate, and activity were continuously recorded using radiotelemetry. In the normoxic groups, the recorded variables did not deviate from the baseline values. Both CIH groups exhibited significant increases in MAP during CIH exposures (P < 0.05). During the normoxic dark phase in the CIH groups, only the SCM-injected group exhibited a sustained increase in MAP (P < 0.05). The AT1aR-CIH group showed significant decreases in FosB/ΔFosB staining in the median preoptic nucleus and the paraventricular nuclei of the hypothalamus compared with the SCM-CIH group. Our data indicate that AT1aRs in the SFO are critical for the sustained elevation in MAP and increased FosB/ΔFosB expression in forebrain autonomic nuclei associated with CIH. Copyright © 2015 the American Physiological Society.

  5. Improvement of critical heat flux correlation for research reactors using plate-type fuel

    International Nuclear Information System (INIS)

    Kaminaga, Masanori; Yamamoto, Kazuyoshi; Sudo, Yukio

    1998-01-01

    In research reactors, plate-type fuel elements are generally adopted so as to produce high power densities and are cooled by a downward flow. A core flow reversal from a steady-state forced downward flow to an upward flow due to natural convection should occur during operational transients such as Loss of the primary coolant flow'. Therefore, in the thermal hydraulic design of research reactors, critical heat flux (CHF) under a counter-current flow limitation (CCFL) or a flooding condition are important to determine safety margins of fuel against CHF during a core flow reversal. The authors have proposed a CHF correlation scheme for the thermal hydraulic design of research reactors, based on CHF experiments for both upward and downward flows including CCFL condition. When the CHF correlation scheme was proposed, a subcooling effect for CHF correlation under CCFL condition had not been considered because of a conservative evaluation and a lack of enough CHF data to determine the subcooling effect on CHF. A too conservative evaluation is not appropriate for the design of research reactors because of construction costs etc. Also, conservativeness of the design must be determined precisely. In this study, therefore, the subcooling effect on CHF under the CCFL conditions in vertical rectangular channels heated from both sides were investigated quantitatively based on CHF experimental results obtained under uniform and non-uniform heat flux conditions. As a result, it was made clear that CHF in this region increase linearly with an increase of the channel inlet subcooling and a new CHF correlation including the effect of channel inlet subcooling was proposed. The new correlation could be adopted under the conditions of the atmospheric pressure, the inlet subcooling less than 78K, the channel gap size between 2.25 to 5.0mm, the axial peaking factor between 1.0 to 1.6 and L/De between 71 to 174 which were the ranges investigated in this study. (author)

  6. Collagen Type I alpha1 (COL1A1 Gene Polymorphism and Bone Mineral Density in Postmenopausal Kazakh Women

    Directory of Open Access Journals (Sweden)

    Akbota Aitkulova

    2014-12-01

    Full Text Available Introduction: Single nucleotide polymorphism (SNP at the collagen type I alpha 1 gene (COL1A1 rs1800012 has been widely studied and has shown an association with bone mineral density (BMD and fractures. A minor allele TT of this SNP was found to be greatly overrepresented in individuals with fractures compared to controls, thus becoming a good predictor of  increased fracture risk. The aim of this investigation was to evaluate potential association between COL1A1 gene polymorphism and osteoporosis in Kazakh postmenopausal women.Methods: The study population included 103 postmenopausal women recruited from Pavlodar and Almaty clinics. BMD was measured using DEXA. Genomic DNA was extracted from peripheral venous blood of study participants with Wizard® Genomic DNA Purification Kit (Promega, USA. Detection of COL1A1 +1245G/T (Sp1 polymorphism was done by the TaqMan® SNP Genotyping Assay of real-time PCR.Results: Densitometry results revealed 36 osteoporotic, 42 osteopenic, and 25 normal postmenopausal women. Data analysis of 1245G>T polymorphism in COL1A1 gene in the group of women with osteopenia and osteoporosis revealed deviation from Hardy-Weinberg equilibrium. The mutant TT genotype was prevalent compared to the heterozygous genotype GT in both groups. Distributions were 83% GG, 3% GT, and 14% TT in the group with osteopenia and 80% GG, 6% GT, and 14% TT in the group with osteoporosis. The distribution of genotypes frequency in the group of normal postmenopausal women was 76% GG, 16% GT, and 8% TT.Conclusion: These results suggest that TT genotype of COL1A1 +1245G/T (Sp1 polymorphism is associated with risk of postmenopausal osteoporosis in Kazakh women. Further studies involving a larger number of women are needed to clarify the relationship of this polymorphism with risk of osteoporosis. 

  7. Type I interferon is critical for the homeostasis and functional maturation of type 3 γδ T cells

    DEFF Research Database (Denmark)

    Agerholm, Rasmus; Kadekar, Darshana Dattatraya; Rizk, John

    2017-01-01

    Type I IFN (IFN-I) is highly expressed during viral infection and many autoimmune pathologies such as SLE and psoriasis. In addition, IFN-I is important to maintain the homeostasis of a number of different immune populations. Our aim was to identify whether IFN-I regulates type 3 γδ T (γδT3) cells...... behavior. Such γδT3 anergy is characterized by failure to induce skin inflammation and unresponsiveness to cytokine stimuli. Moreover, IFNAR deficient mice display deregulated γδT3homeostasis due to a neonatal maturation defect. In conclusion, our data show that tonic type I IFN signaling during neonatal...

  8. Feasibility and effects of a physical exercise programme in adults with myotonic dystrophy type 1: a randomized controlled pilot study.

    Science.gov (United States)

    Kierkegaard, Marie; Harms-Ringdahl, Karin; Edström, Lars; Widén Holmqvist, Lotta; Tollbäck, Anna

    2011-07-01

    To investigate the feasibility and effects of a physical exercise programme on functioning and health-related quality of life in adults with myotonic dystrophy type 1. A randomized controlled trial. Thirty-five adults with myotonic dystrophy type 1. After stratification for level of functioning, study participants were assigned by lot to either a training group or a control group. Training-group participants attended a 60-minute comprehensive group-training programme, Friskis&Svettis® Open Doors, twice a week for 14 weeks. The six-minute walk test was the primary outcome measure and the timed-stands test, the timed up-and-go test, the Epworth sleepiness scale and the Short Form-36 health survey were secondary outcome measures. Intention-to-treat analyses revealed no significant differences in any outcome measures, except for an increased between-group difference after intervention in the Short Form-36 mental health subscale and a decrease in the vitality subscale for the control group. The programme was well tolerated and many training-group participants perceived subjective changes for the better. No negative effects were reported. The Friskis&Svettis® Open Doors programme was feasible for adults with myotonic dystrophy type 1 who had been screened for cardiac involvement, had distal or mild-to-moderate proximal muscle impairment, and no severe cognitive impairments. No beneficial or detrimental effects were evident.

  9. PMP22 related neuropathies: Charcot-Marie-Tooth disease type 1A and Hereditary Neuropathy with liability to Pressure Palsies.

    Science.gov (United States)

    van Paassen, Barbara W; van der Kooi, Anneke J; van Spaendonck-Zwarts, Karin Y; Verhamme, Camiel; Baas, Frank; de Visser, Marianne

    2014-03-19

    PMP22 related neuropathies comprise (1) PMP22 duplications leading to Charcot-Marie-Tooth disease type 1A (CMT1A), (2) PMP22 deletions, leading to Hereditary Neuropathy with liability to Pressure Palsies (HNPP), and (3) PMP22 point mutations, causing both phenotypes. Overall prevalence of CMT is usually reported as 1:2,500, epidemiological studies show that 20-64% of CMT patients carry the PMP22 duplication. The prevalence of HNPP is not well known. CMT1A usually presents in the first two decades with difficulty walking or running. Distal symmetrical muscle weakness and wasting and sensory loss is present, legs more frequently and more severely affected than arms. HNPP typically leads to episodic, painless, recurrent, focal motor and sensory peripheral neuropathy, preceded by minor compression on the affected nerve. Electrophysiological evaluation is needed to determine whether the polyneuropathy is demyelinating. Sonography of the nerves can be useful. Diagnosis is confirmed by finding respectively a PMP22 duplication, deletion or point mutation. Differential diagnosis includes other inherited neuropathies, and acquired polyneuropathies. The mode of inheritance is autosomal dominant and de novo mutations occur. Offspring of patients have a chance of 50% to inherit the mutation from their affected parent. Prenatal testing is possible; requests for prenatal testing are not common. Treatment is currently symptomatic and may include management by a rehabilitation physician, physiotherapist, occupational therapist and orthopaedic surgeon. Adult CMT1A patients show slow clinical progression of disease, which seems to reflect a process of normal ageing. Life expectancy is normal.

  10. Coexistence of critical orbit types in sub-hyperbolic polynomial maps

    OpenAIRE

    Poirier, Alfredo

    1994-01-01

    We establish necessary and sufficient conditions for the realization of mapping schemata as post-critically finite polynomials, or more generally, as post-critically finite polynomial maps from a finite union of copies of the complex numbers {\\bf C} to itself which have degree two or more in each copy. As a consequence of these results we prove a transitivity relation between hyperbolic components in parameter space which was conjectured by Milnor.

  11. Proprotein convertase subtilisin/kexin type 7 (PCSK7) is essential for the zebrafish development and bioavailability of transforming growth factor β1a (TGFβ1a).

    Science.gov (United States)

    Turpeinen, Hannu; Oksanen, Anna; Kivinen, Virpi; Kukkurainen, Sampo; Uusimäki, Annemari; Rämet, Mika; Parikka, Mataleena; Hytönen, Vesa P; Nykter, Matti; Pesu, Marko

    2013-12-20

    Proprotein convertase subtilisin/kexin (PCSK) enzymes convert proproteins into bioactive end products. Although other PCSK enzymes are known to be essential for biological processes ranging from cholesterol metabolism to host defense, the in vivo importance of the evolutionarily ancient PCSK7 has remained enigmatic. Here, we quantified the expressions of all pcsk genes during the 1st week of fish development and in several tissues. pcsk7 expression was ubiquitous and evident already during the early development. To compare mammalian and zebrafish PCSK7, we prepared homology models, which demonstrated remarkable structural conservation. When the PCSK7 function in developing larvae was inhibited, we found that PCSK7-deficient fish have defects in various organs, including the brain, eye, and otic vesicle, and these result in mortality within 7 days postfertilization. A genome-wide analysis of PCSK7-dependent gene expression showed that, in addition to developmental processes, several immune system-related pathways are also regulated by PCSK7. Specifically, the PCSK7 contributed to the mRNA expression and proteolytic cleavage of the cytokine TGFβ1a. Consequently, tgfβ1a morphant fish displayed phenotypical similarities with pcsk7 morphants, underscoring the importance of this cytokine in the zebrafish development. Targeting PCSK activity has emerged as a strategy for treating human diseases. Our results suggest that inhibiting PCSK7 might interfere with normal vertebrate development.

  12. Membrane Estrogen Receptor-α Interacts with Metabotropic Glutamate Receptor Type 1a to Mobilize Intracellular Calcium in Hypothalamic Astrocytes

    Science.gov (United States)

    Kuo, John; Hariri, Omid R.; Bondar, Galyna; Ogi, Julie; Micevych, Paul

    2009-01-01

    Estradiol, acting on a membrane-associated estrogen receptor-α (mERα), induces an increase in free cytoplasmic calcium concentration ([Ca2+]i) needed for progesterone synthesis in hypothalamic astrocytes. To determine whether rapid estradiol signaling involves an interaction of mERα with metabotropic glutamate receptor type 1a (mGluR1a), changes in [Ca2+]i were monitored with the calcium indicator, Fluo-4 AM, in primary cultures of female postpubertal hypothalamic astrocytes. 17β-Estradiol over a range of 1 nm to 100 nm induced a maximal increase in [Ca2+]i flux measured as a change in relative fluorescence [ΔF Ca2+ = 615 ± 36 to 641 ± 47 relative fluorescent units (RFU)], whereas 0.1 nm of estradiol stimulated a moderate [Ca2+]i increase (275 ± 16 RFU). The rapid estradiol-induced [Ca2+]i flux was blocked with 1 μm of the estrogen receptor antagonist ICI 182,780 (635 ± 24 vs. 102 ± 11 RFU, P estradiol-induced membrane signaling in astrocytes. PMID:18948402

  13. Protein Kinase A (PKA) Type I Interacts with P-Rex1, a Rac Guanine Nucleotide Exchange Factor

    Science.gov (United States)

    Chávez-Vargas, Lydia; Adame-García, Sendi Rafael; Cervantes-Villagrana, Rodolfo Daniel; Castillo-Kauil, Alejandro; Bruystens, Jessica G. H.; Fukuhara, Shigetomo; Taylor, Susan S.; Mochizuki, Naoki; Reyes-Cruz, Guadalupe; Vázquez-Prado, José

    2016-01-01

    Morphology of migrating cells is regulated by Rho GTPases and fine-tuned by protein interactions and phosphorylation. PKA affects cell migration potentially through spatiotemporal interactions with regulators of Rho GTPases. Here we show that the endogenous regulatory (R) subunit of type I PKA interacts with P-Rex1, a Rac guanine nucleotide exchange factor that integrates chemotactic signals. Type I PKA holoenzyme interacts with P-Rex1 PDZ domains via the CNB B domain of RIα, which when expressed by itself facilitates endothelial cell migration. P-Rex1 activation localizes PKA to the cell periphery, whereas stimulation of PKA phosphorylates P-Rex1 and prevents its activation in cells responding to SDF-1 (stromal cell-derived factor 1). The P-Rex1 DEP1 domain is phosphorylated at Ser-436, which inhibits the DH-PH catalytic cassette by direct interaction. In addition, the P-Rex1 C terminus is indirectly targeted by PKA, promoting inhibitory interactions independently of the DEP1-PDZ2 region. A P-Rex1 S436A mutant construct shows increased RacGEF activity and prevents the inhibitory effect of forskolin on sphingosine 1-phosphate-dependent endothelial cell migration. Altogether, these results support the idea that P-Rex1 contributes to the spatiotemporal localization of type I PKA, which tightly regulates this guanine exchange factor by a multistep mechanism, initiated by interaction with the PDZ domains of P-Rex1 followed by direct phosphorylation at the first DEP domain and putatively indirect regulation of the C terminus, thus promoting inhibitory intramolecular interactions. This reciprocal regulation between PKA and P-Rex1 might represent a key node of integration by which chemotactic signaling is fine-tuned by PKA. PMID:26797121

  14. The Critical Depth of Freeze-Thaw Soil under Different Types of Snow Cover

    Directory of Open Access Journals (Sweden)

    Qiang Fu

    2017-05-01

    Full Text Available Snow cover is the most common upper boundary condition influencing the soil freeze-thaw process in the black soil farming area of northern China. Snow is a porous dielectric cover, and its unique physical properties affect the soil moisture diffusion, heat conduction, freezing rate and other variables. To understand the spatial distribution of the soil water-heat and the variable characteristics of the critical depth of the soil water and heat, we used field data to analyze the freezing rate of soil and the extent of variation in soil water-heat in a unit soil layer under bare land (BL, natural snow (NS, compacted snow (CS and thick snow (TS treatments. The critical depth of the soil water and heat activity under different snow covers were determined based on the results of the analysis, and the variation fitting curve of the difference sequences on the soil temperature and water content between different soil layers and the surface 5-cm soil layer were used to verify the critical depth. The results were as follows: snow cover slowed the rate of soil freezing, and the soil freezing rate under the NS, CS and TS treatments decreased by 0.099 cm/day, 0.147 cm/day and 0.307 cm/day, respectively, compared with that under BL. In addition, the soil thawing time was delayed, and the effect was more significant with increased snow cover. During freeze-thaw cycles, the extent of variation in the water and heat time series in the shallow soil was relatively large, while there was less variation in the deep layer. There was a critical stratum in the vertical surface during hydrothermal migration, wherein the critical depth of soil water and heat change gradually increased with increasing snow cover. The variance in differences between the surface layer and both the soil water and heat in the different layers exhibited “steady-rising-steady” behavior, and the inflection point of the curve is the critical depth of soil freezing and thawing. This critical

  15. Flux pinning and critical current in layered type-II superconductors in parallel magnetic fields

    International Nuclear Information System (INIS)

    Prokic, V.; Davidovic, D.; Dobrosavljevic-Grujic, L.

    1995-01-01

    We have shown, within the Ginzburg-Landau theory, that the interaction between vortices and normal-metal layers in high-T c superconductor--normal-metal superlattices can cause high critical-current densities j c . The interaction is primarily magnetic, except at very low temperatures T, where the core interaction is dominant. For a lattice of vortices commensurate with an array of normal-metal layers in a parallel magnetic field H, strong magnetic pinning is obtained, with a nonmonotonic critical-current dependence on H, and with j c of the order of 10 7 --10 8 A/cm 2

  16. Increased de novo lipogenesis and delayed conversion of large VLDL into intermediate density lipoprotein particles contribute to Hyperlipidemia in glycogen storage disease type 1a

    NARCIS (Netherlands)

    Bandsma, Robert H. J.; Prinsen, Berthil H.; Van Der Velden, Monique De Sain; Rake, Jan-Peter; Boer, Theo; Smit, G. Peter A.; Reijngoud, Dirk-Jan; Kuipers, Folkert

    Glycogen storage disease type 1a (GSD-1a) is a metabolic disorder characterized by fasting-induced hypoglycemia, hepatic steatosis, and hyperlipidemia. The mechanisms underlying the lipid abnormalities are largely unknown. To investigate these mechanisms seven GSD-1a patients and four healthy

  17. Clinical and virological characteristics of calves experimentally infected with a Brazilian isolate of bovine viral diarrhea virus type 1a

    Directory of Open Access Journals (Sweden)

    Luana Marchi Quadros

    Full Text Available ABSTRACT: To study the pathogenicity of the Brazilian bovine viral diarrhea virus (BVDV type 1a 241.10 isolate, four calves were intranasally inoculated with a viral suspension containing 107.2 TCID50 mL-1. One calf was left uninoculated and kept in contact with the other calves to investigate viral transmissibility. After inoculation, the animals were monitored daily for clinical signs of infection. The presence of the virus in the blood and nasal secretions was confirmed by virus isolation in cell culture. White blood cells were quantified prior to and every 3 days after infection, and the presence of antibodies was checked every 7 days, starting at day 0 until day 42 post-inoculation (pi. After infection, nasal and ocular serous secretions were observed between days 1 and 5 pi, along with a mild cough from days 2 to 4 pi; however, no severe clinical signs were present. Body temperature was slightly elevated between days 4 and 6 pi. The control calf did not develop any of the signs observed in the infected animals. Cell culture-mediated virus isolation confirmed viremia between days 4 and 8 pi and the presence of the virus in the nasal secretions between days 1 and 10 pi. All infected animals showed a decrease in white blood cell count. Antibodies could be detected from day 14 pi, and these levels remained high until day 35 pi. The control calf had no viremia, viral presence in nasal secretions, or positive serology, indicating the absence of viral transmission. Thus, isolate BVDV 1a 241.10 has low pathogenicity and transmissibility but retains immunosuppressive capacity.

  18. Functional characterization of DnSIZ1, a SIZ/PIAS-type SUMO E3 ligase from Dendrobium.

    Science.gov (United States)

    Liu, Feng; Wang, Xiao; Su, Mengying; Yu, Mengyuan; Zhang, Shengchun; Lai, Jianbin; Yang, Chengwei; Wang, Yaqin

    2015-09-17

    SUMOylation is an important post-translational modification of eukaryotic proteins that involves the reversible conjugation of a small ubiquitin-related modifier (SUMO) polypeptide to its specific protein substrates, thereby regulating numerous complex cellular processes. The PIAS (protein inhibitor of activated signal transducers and activators of transcription [STAT]) and SIZ (scaffold attachment factor A/B/acinus/PIAS [SAP] and MIZ) proteins are SUMO E3 ligases that modulate SUMO conjugation. The characteristic features and SUMOylation mechanisms of SIZ1 protein in monocotyledon are poorly understood. Here, we examined the functions of a homolog of Arabidopsis SIZ1, a functional SIZ/PIAS-type SUMO E3 ligase from Dendrobium. In Dendrobium, the predicted DnSIZ1 protein has domains that are highly conserved among SIZ/PIAS-type proteins. DnSIZ1 is widely expressed in Dendrobium organs and has a up-regulated trend by treatment with cold, high temperature and wounding. The DnSIZ1 protein localizes to the nucleus and shows SUMO E3 ligase activity when expressed in an Escherichia coli reconstitution system. Moreover, ectopic expression of DnSIZ1 in the Arabidopsis siz1-2 mutant partially complements several phenotypes and results in enhanced levels of SUMO conjugates in plants exposed to heat shock conditions. We observed that DnSIZ1 acts as a negative regulator of flowering transition which may be via a vernalization-induced pathway. In addition, ABA-hypersensitivity of siz1-2 seed germination can be partially suppressed by DnSIZ1. Our results suggest that DnSIZ1 is a functional homolog of the Arabidopsis SIZ1 with SUMO E3 ligase activity and may play an important role in the regulation of Dendrobium stress responses, flowering and development.

  19. Targeting the Immunogenetic Diseases with the Appropriate HLA Molecular Typing: Critical Appraisal on 2666 Patients Typed in One Single Centre

    Directory of Open Access Journals (Sweden)

    M. Guarene

    2013-01-01

    Full Text Available We compared the immunogenetic data from 2666 patients affected by HLA-related autoimmune diseases with those from 4389 ethnically matched controls (3157 cord blood donors CBD, 1232 adult bone marrow donors BMD, to verify the appropriateness of HLA typing requests received in the past decade. The frequency of HLA-B*27 phenotype was 10.50% in 724 ankylosing spondylitis, 16.80% in 125 uveitis (3.41% BMD, 4.24% CBD, P<0.0001; HLA-B*51 allele was 15.57% in 212 Behçet’s disease (12.91% BMD, 9.88% CBD, P<0.0001; the HLA-DRB1-rheumatoid arthritis (RA shared epitope was 13.72% in 554 RA (10.85% BMD, 13.48% CBD, P=0.016; the carriers of almost one of HLA-DQB1 susceptibility alleles were 84.91% in 795 celiac disease (CD and 59.37% in 256 insulin-dependent diabetes mellitus (IDDM (46.06% in 875 CBD, 42.75% in 662 BMD P<0.0001. Overall, our results show that the HLA marker frequencies were higher in patients than controls, but lower than expected from the literature data (excluding CD and IDDM and demonstrate that, in complex immunogenetic conditions, a substantial number of genetic analyses are redundant and inappropriate, burdening to the public health costs. For this reason, we suggest the Italian Scientific Society of Immunogenetics to establish guidelines to improve the appropriateness of typing requests.

  20. Criticality safety evaluation of a type B enriched uranium shipping container

    International Nuclear Information System (INIS)

    Hopper, C.M.

    1978-01-01

    The Oak Ridge Y-12 Plant Model DT-14 container was developed to replace and extend the enriched uranium shipping capabilities of the USA/5765/BF Vermiculite shipping container. This work was accomplished to comply with the DOE Immediate Action Directive Number 0529-02 for ''Phasing out the use of loose or bagged Vermiculite packaging material as a thermal shield and shock absorber in radioactive material packages''. The Model DT-14 is fabricated from a Specification 17H 30-gallon drum, cane fiberboard insulation, and a steel inner containment vessel (159 mm dia by 390 mm height). The single-package and array analyses are based upon results of the multigroup Monte Carlo criticality program, KENO, utilizing 16-energy-group Hansen-Roach, and Knight modified 238 U cross sections. The program and cross sections are considered well established on the basis of their success in calculating a large variety of critical experiments. Validation results show that a calculated neutron multiplication factor plus two standard deviations equal to 0.970 or greater must be considered critical, and all lower values may be considered safe

  1. Enrichment of HP1a on Drosophila chromosome 4 genes creates an alternate chromatin structure critical for regulation in this heterochromatic domain.

    Directory of Open Access Journals (Sweden)

    Nicole C Riddle

    2012-09-01

    Full Text Available Chromatin environments differ greatly within a eukaryotic genome, depending on expression state, chromosomal location, and nuclear position. In genomic regions characterized by high repeat content and high gene density, chromatin structure must silence transposable elements but permit expression of embedded genes. We have investigated one such region, chromosome 4 of Drosophila melanogaster. Using chromatin-immunoprecipitation followed by microarray (ChIP-chip analysis, we examined enrichment patterns of 20 histone modifications and 25 chromosomal proteins in S2 and BG3 cells, as well as the changes in several marks resulting from mutations in key proteins. Active genes on chromosome 4 are distinct from those in euchromatin or pericentric heterochromatin: while there is a depletion of silencing marks at the transcription start sites (TSSs, HP1a and H3K9me3, but not H3K9me2, are enriched strongly over gene bodies. Intriguingly, genes on chromosome 4 are less frequently associated with paused polymerase. However, when the chromatin is altered by depleting HP1a or POF, the RNA pol II enrichment patterns of many chromosome 4 genes shift, showing a significant decrease over gene bodies but not at TSSs, accompanied by lower expression of those genes. Chromosome 4 genes have a low incidence of TRL/GAGA factor binding sites and a low T(m downstream of the TSS, characteristics that could contribute to a low incidence of RNA polymerase pausing. Our data also indicate that EGG and POF jointly regulate H3K9 methylation and promote HP1a binding over gene bodies, while HP1a targeting and H3K9 methylation are maintained at the repeats by an independent mechanism. The HP1a-enriched, POF-associated chromatin structure over the gene bodies may represent one type of adaptation for genes embedded in repetitive DNA.

  2. Deletion of ETS-1, a gene in the Jacobsen syndrome critical region, causes ventricular septal defects and abnormal ventricular morphology in mice

    Science.gov (United States)

    Ye, Maoqing; Coldren, Chris; Liang, Xingqun; Mattina, Teresa; Goldmuntz, Elizabeth; Benson, D. Woodrow; Ivy, Dunbar; Perryman, M.B.; Garrett-Sinha, Lee Ann; Grossfeld, Paul

    2010-01-01

    Congenital heart defects comprise the most common form of major birth defects, affecting 0.7% of all newborn infants. Jacobsen syndrome (11q-) is a rare chromosomal disorder caused by deletions in distal 11q. We have previously determined that a wide spectrum of the most common congenital heart defects occur in 11q-, including an unprecedented high frequency of hypoplastic left heart syndrome (HLHS). We identified an ∼7 Mb ‘cardiac critical region’ in distal 11q that contains a putative causative gene(s) for congenital heart disease. In this study, we utilized chromosomal microarray mapping to characterize three patients with 11q- and congenital heart defects that carry interstitial deletions overlapping the 7 Mb cardiac critical region. We propose that this 1.2 Mb region of overlap harbors a gene(s) that causes at least a subset of the congenital heart defects that occur in 11q-. We demonstrate that one gene in this region, ETS-1 (a member of the ETS family of transcription factors), is expressed in the endocardium and neural crest during early mouse heart development. Gene-targeted deletion of ETS-1 in mice in a C57/B6 background causes, with high penetrance, large membranous ventricular septal defects and a bifid cardiac apex, and less frequently a non-apex-forming left ventricle (one of the hallmarks of HLHS). Our results implicate an important role for the ETS-1 transcription factor in mammalian heart development and should provide important insights into some of the most common forms of congenital heart disease. PMID:19942620

  3. Type III Interferon-Mediated Signaling Is Critical for Controlling Live Attenuated Yellow Fever Virus Infection In Vivo.

    Science.gov (United States)

    Douam, Florian; Soto Albrecht, Yentli E; Hrebikova, Gabriela; Sadimin, Evita; Davidson, Christian; Kotenko, Sergei V; Ploss, Alexander

    2017-08-15

    Yellow fever virus (YFV) is an arthropod-borne flavivirus, infecting ~200,000 people worldwide annually and causing about 30,000 deaths. The live attenuated vaccine strain, YFV-17D, has significantly contributed in controlling the global burden of yellow fever worldwide. However, the viral and host contributions to YFV-17D attenuation remain elusive. Type I interferon (IFN-α/β) signaling and type II interferon (IFN-γ) signaling have been shown to be mutually supportive in controlling YFV-17D infection despite distinct mechanisms of action in viral infection. However, it remains unclear how type III IFN (IFN-λ) integrates into this antiviral system. Here, we report that while wild-type (WT) and IFN-λ receptor knockout (λR -/- ) mice were largely resistant to YFV-17D, deficiency in type I IFN signaling resulted in robust infection. Although IFN-α/β receptor knockout (α/βR -/- ) mice survived the infection, mice with combined deficiencies in both type I signaling and type III IFN signaling were hypersusceptible to YFV-17D and succumbed to the infection. Mortality was associated with viral neuroinvasion and increased permeability of the blood-brain barrier (BBB). α/βR -/- λR -/- mice also exhibited distinct changes in the frequencies of multiple immune cell lineages, impaired T-cell activation, and severe perturbation of the proinflammatory cytokine balance. Taken together, our data highlight that type III IFN has critical immunomodulatory and neuroprotective functions that prevent viral neuroinvasion during active YFV-17D replication. Type III IFN thus likely represents a safeguard mechanism crucial for controlling YFV-17D infection and contributing to shaping vaccine immunogenicity. IMPORTANCE YFV-17D is a live attenuated flavivirus vaccine strain recognized as one of the most effective vaccines ever developed. However, the host and viral determinants governing YFV-17D attenuation and its potent immunogenicity are still unknown. Here, we analyzed the

  4. A critical re-evaluation of multilocus sequence typing (MLST) efforts in Wolbachia.

    Science.gov (United States)

    Bleidorn, Christoph; Gerth, Michael

    2018-01-01

    Wolbachia (Alphaproteobacteria, Rickettsiales) is the most common, and arguably one of the most important inherited symbionts. Molecular differentiation of Wolbachia strains is routinely performed with a set of five multilocus sequence typing (MLST) markers. However, since its inception in 2006, the performance of MLST in Wolbachia strain typing has not been assessed objectively. Here, we evaluate the properties of Wolbachia MLST markers and compare it to 252 other single copy loci present in the genome of most Wolbachia strains. Specifically, we investigated how well MLST performs at strain differentiation, at reflecting genetic diversity of strains, and as phylogenetic marker. We find that MLST loci are outperformed by other loci at all tasks they are currently employed for, and thus that they do not reflect the properties of a Wolbachia strain very well. We argue that whole genome typing approaches should be used for Wolbachia typing in the future. Alternatively, if few loci approaches are necessary, we provide a characterisation of 252 single copy loci for a number a criteria, which may assist in designing specific typing systems or phylogenetic studies. © FEMS 2017. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.

  5. Spinocerebellar ataxia type 29 due to mutations in ITPR1: a case series and review of this emerging congenital ataxia.

    Science.gov (United States)

    Zambonin, Jessica L; Bellomo, Allison; Ben-Pazi, Hilla; Everman, David B; Frazer, Lee M; Geraghty, Michael T; Harper, Amy D; Jones, Julie R; Kamien, Benjamin; Kernohan, Kristin; Koenig, Mary Kay; Lines, Matthew; Palmer, Elizabeth Emma; Richardson, Randal; Segel, Reeval; Tarnopolsky, Mark; Vanstone, Jason R; Gibbons, Melissa; Collins, Abigail; Fogel, Brent L; Dudding-Byth, Tracy; Boycott, Kym M

    2017-06-28

    Spinocerebellar ataxia type 29 (SCA29) is an autosomal dominant, non-progressive cerebellar ataxia characterized by infantile-onset hypotonia, gross motor delay and cognitive impairment. Affected individuals exhibit cerebellar dysfunction and often have cerebellar atrophy on neuroimaging. Recently, missense mutations in ITPR1 were determined to be responsible. Clinical information on 21 individuals from 15 unrelated families with ITPR1 mutations was retrospectively collected using standardized questionnaires, including 11 previously unreported singletons and 2 new patients from a previously reported family. We describe the genetic, clinical and neuroimaging features of these patients to further characterize the clinical features of this rare condition and assess for any genotype-phenotype correlation for this disorder. Our cohort consisted of 9 males and 12 females, with ages ranging from 28 months to 49 years. Disease course was non-progressive with infantile-onset hypotonia and delays in motor and speech development. Gait ataxia was present in all individuals and 10 (48%) were not ambulating independently between the ages of 3-12 years of age. Mild-to-moderate cognitive impairment was present in 17 individuals (85%). Cerebellar atrophy developed after initial symptom presentation in 13 individuals (72%) and was not associated with disease progression or worsening functional impairment. We identified 12 different mutations including 6 novel mutations; 10 mutations were missense (with 4 present in >1 individual), 1 a splice site mutation leading to an in-frame insertion and 1 an in-frame deletion. No specific genotype-phenotype correlations were observed within our cohort. Our findings document significant clinical heterogeneity between individuals with SCA29 in a large cohort of molecularly confirmed cases. Based on the retrospective observed clinical features and disease course, we provide recommendations for management. Further research into the natural

  6. Glucose effectiveness is a critical pathogenic factor leading to glucose intolerance and type 2 diabetes

    DEFF Research Database (Denmark)

    Alford, F. P.; Henriksen, J. E.; Rantzau, C.

    2018-01-01

    . A negative association between GE and insulin meditated glucose disposal (Si), is present in normal subjects without a family history of type 2 diabetes mellitus but is absent in normoglycaemic "at risk" relatives with a positive family history of diabetes mellitus. Intracellular GE disposal is mediated...

  7. Bulk superconductivity in Type II superconductors near the second critical field

    DEFF Research Database (Denmark)

    Fournais, Søren; Helffer, Bernard

    2010-01-01

    We consider superconductors of Type II near the transition from the ‘bulk superconducting’ to the ‘surface superconducting’ state. We prove a new L∞ estimate on the order parameter in the bulk, i.e. away from the boundary. This solves an open problem posed by Aftalion and Serfaty [AS]....

  8. Type III Interferon-Mediated Signaling Is Critical for Controlling Live Attenuated Yellow Fever Virus Infection In Vivo

    Directory of Open Access Journals (Sweden)

    Florian Douam

    2017-08-01

    Full Text Available Yellow fever virus (YFV is an arthropod-borne flavivirus, infecting ~200,000 people worldwide annually and causing about 30,000 deaths. The live attenuated vaccine strain, YFV-17D, has significantly contributed in controlling the global burden of yellow fever worldwide. However, the viral and host contributions to YFV-17D attenuation remain elusive. Type I interferon (IFN-α/β signaling and type II interferon (IFN-γ signaling have been shown to be mutually supportive in controlling YFV-17D infection despite distinct mechanisms of action in viral infection. However, it remains unclear how type III IFN (IFN-λ integrates into this antiviral system. Here, we report that while wild-type (WT and IFN-λ receptor knockout (λR−/− mice were largely resistant to YFV-17D, deficiency in type I IFN signaling resulted in robust infection. Although IFN-α/β receptor knockout (α/βR−/− mice survived the infection, mice with combined deficiencies in both type I signaling and type III IFN signaling were hypersusceptible to YFV-17D and succumbed to the infection. Mortality was associated with viral neuroinvasion and increased permeability of the blood-brain barrier (BBB. α/βR−/− λR−/− mice also exhibited distinct changes in the frequencies of multiple immune cell lineages, impaired T-cell activation, and severe perturbation of the proinflammatory cytokine balance. Taken together, our data highlight that type III IFN has critical immunomodulatory and neuroprotective functions that prevent viral neuroinvasion during active YFV-17D replication. Type III IFN thus likely represents a safeguard mechanism crucial for controlling YFV-17D infection and contributing to shaping vaccine immunogenicity.

  9. Manual dexterity in hereditary motor and sensory neuropathy type 1a: severity of limitations and feasibility and reliability of two assessment instruments

    NARCIS (Netherlands)

    Videler, Annemieke J.; Beelen, Anita; van Schaik, Ivo N.; de Visser, Marianne; Nollet, Frans

    2008-01-01

    OBJECTIVE: To assess the prevalence and significance of impaired manual dexterity in hereditary motor and sensory neuropathy type 1a (HMSN 1a), with the Sollerman hand function and the Functional Dexterity test, and compare the reliability and agreement of the tests. DESIGN: Descriptive

  10. A critical review of low-carbohydrate diets in people with Type 2 diabetes.

    Science.gov (United States)

    van Wyk, H J; Davis, R E; Davies, J S

    2016-02-01

    The efficacy of low-carbohydrate diets (LCD) in people with Type 2 diabetes has divided the nutrition community. This review seeks to re-examine the available data to clarify understanding. A comprehensive search of databases was used to identify meta-analyses of LCD in Type 2 diabetes. To improve the quality of the studies analysed, the following inclusion criteria were applied: randomized control trials ≥ 4 weeks in people aged > 18 years with Type 2 diabetes; a carbohydrate intake ≤ 45% of total energy intake per day; and a dietary intake assessment at the end of the study. The resulting studies were subjected to a thematic analysis. Nine meta-analyses were identified containing 153 studies. Twelve studies met our amended inclusion criteria. There were no significant differences in metabolic markers, including glycaemic control, between the two diets, although weight loss with a LCD was greater in one study. Carbohydrate intake at 1 year in very LCD (diets was as little as 8 g/day of carbohydrates. Total energy intake remains the dietary predictor of body weight. A LCD appears no different from a high-carbohydrate diet in terms of metabolic markers and glycaemic control. Very LCDs may not be sustainable over a medium to longer term as carbohydrate intake in diets within studies often converged toward a more moderate level. The variable quality of studies included in earlier meta-analyses likely explains the previous inconsistent findings between meta-analyses. © 2015 The Authors. Diabetic Medicine © 2015 Diabetes UK.

  11. The Vibrio parahaemolyticus Type III Secretion Systems manipulate host cell MAPK for critical steps in pathogenesis.

    LENUS (Irish Health Repository)

    Matlawska-Wasowska, Ksenia

    2010-12-01

    Vibrio parahaemolyticus is a food-borne pathogen causing inflammation of the gastrointestinal epithelium. Pathogenic strains of this bacterium possess two Type III Secretion Systems (TTSS) that deliver effector proteins into host cells. In order to better understand human host cell responses to V. parahaemolyticus, the modulation of Mitogen Activated Protein Kinase (MAPK) activation in epithelial cells by an O3:K6 clinical isolate, RIMD2210633, was investigated. The importance of MAPK activation for the ability of the bacterium to be cytotoxic and to induce secretion of Interleukin-8 (IL-8) was determined.

  12. Are boxer shorts really better? A critical analysis of the role of underwear type in male subfertility.

    Science.gov (United States)

    Munkelwitz, R; Gilbert, B R

    1998-10-01

    Elevation of testicular temperature may result in arrest of spermatogenesis, abnormal semen parameters and sterility. It has been proposed that brief style underwear may produce scrotal hyperthermia and lead to clinical subfertility. Although this idea is regarded as dogma by many in the lay community and the changing of underwear type is a therapy frequently recommended by medical practitioners, there is a paucity of data measuring scrotal temperature as a function of underwear type. Scrotal, core and skin temperatures were measured in 97 consecutive men presenting for evaluation of primary clinical subfertility. These cases were categorized by underwear type to boxer or brief group. Semen analyses were obtained in all patients. Individuals from each group were compared to ascertain differences in temperature when wearing and not wearing underwear. Baseline semen parameters also were compared. In 14 subjects (crossover group) underwear type was changed to the alternative type and scrotal temperature measurements were repeated. Literature regarding underwear type, testicular temperature and/or fertility was reviewed and critically analyzed. Mean scrotal temperature plus or minus standard deviation was 33.8 +/- 0.8 C and 33.6 +/- 1.1 C in the boxer and brief group, respectively. There were no significant temperature differences between the groups. Differential temperatures comparing core to scrotal temperature and semen parameters also were not significantly different. These observations remained constant in the crossover group. The hyperthermic effect of brief style underwear has been exaggerated. In our study there was no difference in scrotal temperature depending on underwear type. It is unlikely that underwear type has a significant effect on male fertility. Routinely advising infertility patients to wear boxer shorts cannot be supported by available scientific evidence.

  13. Assessing Psychological Insulin Resistance in Type 2 Diabetes: a Critical Comparison of Measures.

    Science.gov (United States)

    Holmes-Truscott, E; Pouwer, F; Speight, J

    2017-07-01

    This study aims to examine the operationalisation of 'psychological insulin resistance' (PIR) among people with type 2 diabetes and to identify and critique relevant measures. PIR has been operationalised as (1) the assessment of attitudes or beliefs about insulin therapy and (2) hypothetical or actual resistance, or unwillingness, to use to insulin. Five validated PIR questionnaires were identified. None was fully comprehensive of all aspects of PIR, and the rigour and reporting of questionnaire development and psychometric validation varied considerably between measures. Assessment of PIR should focus on the identification of negative and positive attitudes towards insulin use. Actual or hypothetical insulin refusal may be better conceptualised as a potential consequence of PIR, as its assessment overlooks the attitudes that may prevent insulin use. This paper provides guidance on the selection of questionnaires for clinical or research purpose and the development of new, or improvement of existing, questionnaires.

  14. Athletes′ criticism of coaching behavior: Differences among gender, and type of sport

    Directory of Open Access Journals (Sweden)

    Bebetsos Evangelos

    2017-03-01

    Full Text Available Most athletes are subject to intense mental and physical pressure not only during competition but also during practice. An important variable which may influence athletes′ performance is coaching behavior. The aim of the present study is to investigate if coaching behavior and its antecedents differentiate athletes according to their gender, type of sport, competition experience and weekly practice-time. The sample consisted of 367 male and female athletes who participated in both individual and team sports. They completed the Greek version of the “Coaching Behavior Questionnaire” (CBQ. Results indicated that coaching behavior differentiated athletes of individual sports, and athletes of team sports and experienced women with experienced men. Furthermore, coaches’ behavior contributed to the differentiation on athletes who practice more than those who practice less. In conclusion, these results could help athletes, coaches and sport professionals become more familiar with psychological aspects that influence athletes′ behavior.

  15. Critical analysis of the use of onabotulinumtoxinA (botulinum toxin type A) in migraine.

    Science.gov (United States)

    Robertson, Carrie E; Garza, Ivan

    2012-01-01

    OnabotulinumtoxinA, a neurotoxin, has been studied in numerous trials as a novel preventive therapy for migraine headache. The data would support that it may be effective at reducing headache days in patients suffering from chronic migraine (≥15 headache days/month, with eight or more of those migraine headache days). The mechanism by which onabotulinumtoxinA exerts its effects on migraine is not yet understood. It is known to inhibit acetylcholine release at the neuromuscular junction, but this probably does not explain the observed antinociceptive properties noted in preclinical and clinical trials. This review will discuss the known mechanisms of action of botulinum toxin type A, and will review the available randomized, placebo-controlled trials that have looked at its efficacy as a migraine preventative. We also describe the onabotulinumtoxinA injection sites used at our institution.

  16. Critical element study on autonomous position control of articulated-arm type manipulator

    International Nuclear Information System (INIS)

    Oka, Kiyoshi; Kakudate, Satoshi; Nakahira, Masataka

    1994-10-01

    An articulated-arm type manipulator can be operated effectively in a restricted space due to its flexibility and it can be attractive for a wide range of in-vessel maintenance such as viewing, inspection and limiter handling in fusion experimental reactors. In case of the in-vessel maintenance using a flexible manipulator, it is quite essential to develop an autonomous control method for compensating a deflection of manipulator so as to minimize the maintenance time with high precision. For this purpose, a new position control method using a combination of neural network predictor with a rigid inverse kinematics is being developed. The key features of this method are to simplify a kinematics modeling of flexible manipulator, to enable quick position compensation in stead of ordinary large matrix compensation, and to be applicable to a wide variety of manipulator characteristics. A sub-scaled model of flexible manipulator with 4 joints has been fabricated for a benchmark experiments of the autonomous position control. Comparing analytical simulation with experiments using the flexible manipulator, it has been demonstrated that the new position control method gives significant improvement in control performance with high precision in order of a figure. In addition, further optimization can be possible by adding other non-linear predictors such as radial basis function and fuzzy modeling. This paper describes the details of a sub-scaled flexible manipulator and a neural network position control system as well as results of analytical simulation and benchmark experiments. (author)

  17. Critical analysis of the use of onabotulinumtoxinA (botulinum toxin type A in migraine

    Directory of Open Access Journals (Sweden)

    Robertson CE

    2012-01-01

    Full Text Available Carrie E Robertson, Ivan GarzaDepartment of Neurology, Mayo Clinic, Rochester, MN, USAAbstract: OnabotulinumtoxinA, a neurotoxin, has been studied in numerous trials as a novel preventive therapy for migraine headache. The data would support that it may be effective at reducing headache days in patients suffering from chronic migraine (≥15 headache days/month, with eight or more of those migraine headache days. The mechanism by which onabotulinumtoxinA exerts its effects on migraine is not yet understood. It is known to inhibit acetylcholine release at the neuromuscular junction, but this probably does not explain the observed antinociceptive properties noted in preclinical and clinical trials. This review will discuss the known mechanisms of action of botulinum toxin type A, and will review the available randomized, placebo-controlled trials that have looked at its efficacy as a migraine preventative. We also describe the onabotulinumtoxinA injection sites used at our institution.Keywords: botulinum toxin, prophylaxis, onabotulinumtoxinA, mechanism, migraine, prevention

  18. Increased lipogenesis and resistance of lipoproteins to oxidative modification in two patients with glycogen storage disease type 1a

    NARCIS (Netherlands)

    Bandsma, RHJ; Rake, JP; Visser, G; Neese, RA; Hellerstein, MK; van Duyvenvoorde, W; Princen, HMG; Stellaard, F; Smit, GPA; Kuipers, F

    We describe 2 patients with glycogen storage disease type la and severe hyperlipidemia without premature atherosclerosis. Susceptibility of low-density lipoproteins to oxidation was decreased, possibly related to the similar to40-fold increase in palmitate synthesis altering lipoprotein saturated

  19. Cloning and characterization of SCART1, a novel scavenger receptor cysteine-rich type I transmembrane molecule

    DEFF Research Database (Denmark)

    Holm, Dorte; Fink, Dorte Rosenbek; Grønlund, Jørn

    2009-01-01

    We have cloned and characterized a novel murine transmembrane molecule, mSCART1 belonging to the scavenger receptor cysteine-rich superfamily. The cDNA encodes a polypeptide chain of 989 amino acids, organized as a type I transmembrane protein that contains eight extracellular SRCR domains followed...

  20. HTR1A a novel type 1 diabetes susceptibility gene on chromosome 5p13-q13

    DEFF Research Database (Denmark)

    Asad, Samina; Nikamo, Pernilla; Gyllenberg, Alexandra

    2012-01-01

    We have previously performed a genome-wide linkage study in Scandinavian Type 1 diabetes (T1D) families. In the Swedish families, we detected suggestive linkage (LOD≤2.2) to the chromosome 5p13-q13 region. The aim of our study was to investigate the linked region in search for possible T1D...

  1. Does DNA Methylation of PPARGC1A Influence Insulin Action in First Degree Relatives of Patients with Type 2 Diabetes?

    DEFF Research Database (Denmark)

    Gillberg, Linn; Jacobsen, Stine; Ribel-Madsen, Rasmus

    2013-01-01

    and in muscle from individuals at risk of T2D. This study aimed to investigate DNA promoter methylation and gene expression of PPARGC1A in skeletal muscle from first degree relatives (FDR) of T2D patients, and to determine the association with insulin action as well as the influence of family relation. We...... genetic regulation to play a role. No significant effect of familiality on DNA methylation was found. Taken together, increased DNA methylation of the PPARGC1A promoter is unlikely to play a major causal role for the development of insulin resistance in FDR of patients with T2D....... included 124 Danish FDR of T2D patients from 46 different families. Skeletal muscle biopsies were excised from vastus lateralis and insulin action was assessed by oral glucose tolerance tests. DNA methylation and mRNA expression levels were measured using bisulfite sequencing and quantitative real-time PCR...

  2. Combined Palmer Type 1A and 1B Traumatic Lesions of the Triangular Fibrocartilage Complex A New Category.

    Science.gov (United States)

    Nance, Erin; Ayalon, Omri; Yang, Steven

    2016-06-01

    We present a series of eight patients who underwent wrist arthroscopy for presumed solitary tears of the triangular fibrocartilage (TFC) and were, instead, found to have combined 1A (central tear) and 1B (ulnar avulsion) tears. The Palmer Classification does not currently categorize this combined pattern. All but one patient had a traumatic injury. Each subject had preoperative radiographs and MRI scans. TFC tears were evident on all MRI scans, though only one was suggestive of a combined tear pat - tern. Surgical management included arthroscopic central tear debridement and ulnar peripheral repair. Average follow-up was 22 months. Grip strength in the affected hand improved from 16% deficit as compared to the unaffected side, to 3.5% deficit postoperatively (p = 0.003), and visual analog scores (VAS) decreased from an average of 7.1/10 preoperatively to 2.3/10 postoperatively (p < 0.001). There was no statistically significant change in wrist range of motion (ROM), however. Arthroscopic debridement of the central perforation (1A lesion) with concomitant repair of the ulnar detachment (1B lesion) resulted in functional and symptomatic improvement. This combined 1A/1B TFC injury is not reliably diagnosed preoperatively and should be considered a new subset in the Palmer classification, as this will raise awareness of its presence and assist in preoperative planning of such lesions.

  3. Enhanced Wnt signaling improves bone mass and strength, but not brittleness, in the Col1a1(+/mov13) mouse model of type I Osteogenesis Imperfecta.

    Science.gov (United States)

    Jacobsen, Christina M; Schwartz, Marissa A; Roberts, Heather J; Lim, Kyung-Eun; Spevak, Lyudmila; Boskey, Adele L; Zurakowski, David; Robling, Alexander G; Warman, Matthew L

    2016-09-01

    Osteogenesis Imperfecta (OI) comprises a group of genetic skeletal fragility disorders. The mildest form of OI, Osteogenesis Imperfecta type I, is frequently caused by haploinsufficiency mutations in COL1A1, the gene encoding the α1(I) chain of type 1 collagen. Children with OI type I have a 95-fold higher fracture rate compared to unaffected children. Therapies for OI type I in the pediatric population are limited to anti-catabolic agents. In adults with osteoporosis, anabolic therapies that enhance Wnt signaling in bone improve bone mass, and ongoing clinical trials are determining if these therapies also reduce fracture risk. We performed a proof-of-principle experiment in mice to determine whether enhancing Wnt signaling in bone could benefit children with OI type I. We crossed a mouse model of OI type I (Col1a1(+/Mov13)) with a high bone mass (HBM) mouse (Lrp5(+/p.A214V)) that has increased bone strength from enhanced Wnt signaling. Offspring that inherited the OI and HBM alleles had higher bone mass and strength than mice that inherited the OI allele alone. However, OI+HBM and OI mice still had bones with lower ductility compared to wild-type mice. We conclude that enhancing Wnt signaling does not make OI bone normal, but does improve bone properties that could reduce fracture risk. Therefore, agents that enhance Wnt signaling are likely to benefit children and adults with OI type 1. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. The importance of pheochromocytoma case detection in patients with neurofibromatosis type 1: A case report and review of literature.

    Science.gov (United States)

    Tate, Joshua M; Gyorffy, Janelle B; Colburn, Jeffrey A

    2017-01-01

    Neurofibromatosis type 1 is a complex, multi-system genetic disorder that is associated with an increased prevalence of pheochromocytoma and paraganglioma compared to the general population, 1.0%-5.7% versus 0.2%-0.6%, respectively. A delay in pheochromocytoma and paraganglioma diagnosis or undiagnosed pheochromocytoma and paraganglioma, as seen in normotensive and asymptomatic patients, may portend a significant morbidity and mortality risk due to excess catecholamine secretion. Currently, there are no generally accepted guidelines of screening for pheochromocytoma and paragangliomas in asymptomatic individuals of this population with approaches and practices varying considerably between physicians. Emerging data suggest benefit in routine pheochromocytoma and paraganglioma screening of all individuals with neurofibromatosis type 1. Herein, we present a case to highlight how routine case detection screening would have identified pheochromocytoma earlier in an active duty military member.

  5. Assessing FOXO1A as a potential susceptibility locus for type 2 diabetes and obesity in American Indians.

    Science.gov (United States)

    Muller, Yunhua L; Hanson, Robert L; Wiessner, Gregory; Nieboer, Lori; Kobes, Sayuko; Piaggi, Paolo; Abdussamad, Mahdi; Okani, Chidinma; Knowler, William C; Bogardus, Clifton; Baier, Leslie J

    2015-10-01

    A prior genome-wide association study (GWAS) in Pima Indians identified variation within FOXO1A that modestly associated with early-onset (onset age obesity in a population-based sample of 7,710 American Indians. Tag SNPs in/near FOXO1A (minor allele frequency ≥ 0.05) were analyzed for association with T2D at early onset (n = 1,060) and all ages (n = 7,710) and with insulin secretion (n = 298). SNPs were also analyzed for association with maximum body mass index (BMI) in adulthood (n = 5,918), maximum BMI z-score in childhood (n = 5,350), and % body fat (n = 555). An intronic SNP rs2297627 associated with early-onset T2D [OR = 1.34 (1.13-1.58), P = 8.7 × 10(-4)] and T2D onset at any age [OR = 1.19 (1.09-1.30), P = 1 × 10(-4) ]. The T2D risk allele also associated with lower acute insulin secretion (β = 0.88, as a multiplier, P = 0.02). Another intronic SNP (rs1334241, D' = 0.99, r(2) = 0.49 with rs2297627) associated with maximum adulthood BMI (β = 1.02, as a multiplier, P = 3 × 10(-5)), maximum childhood BMI z-score (β = 0.08, P = 3 × 10(-4)), and % body fat (β = 0.83%, P = 0.04). Common variation in FOXO1A may modestly affect risk for T2D and obesity in American Indians. © 2015 The Obesity Society.

  6. Aerobic anti-gravity exercise in patients with Charcot-Marie-Tooth disease types 1A and X

    DEFF Research Database (Denmark)

    Knak, Kirsten L; Andersen, Linda K; Vissing, John

    2017-01-01

    Background: Charcot-Marie-Tooth (CMT) disease is a hereditary neuropathy associated with impaired walking capacity. Some patients are too weak in the lower extremity muscles to walk at gravity with sufficient intensity or duration to gain benefit. Aim: The aim was to investigate the effect...... of aerobic anti-gravity exercise in weak patients with CMT 1A and X. Methods: Five adult patients performed moderate-intensity aerobic anti-gravity exercise 3/week for 10 weeks. Results: There was a significant positive difference in Berg balance scale and postural stability test between test occasions...

  7. Multimodal Stepped Care Approach Involving Topical Analgesics for Severe Intractable Neuropathic Pain in CRPS Type 1: A Case Report

    Directory of Open Access Journals (Sweden)

    David J. Kopsky

    2011-01-01

    Full Text Available A multimodal stepped care approach has been successfully applied to a patient with complex regional pain syndrome type 1 and severe intractable pain, not responding to regular neuropathic pain medication. The choice to administer drugs in creams was made because of the intolerable adverse effects to oral medication. With this method, peak-dose adverse effects did not occur. The multimodal stepped care approach resulted in considerable and clinically relevant decrease in pain after every step, using topical amitriptyline, ketamine, and dimethylsulphoxide.

  8. Functional and neurochemical characterization of angiotensin type 1A receptor-expressing neurons in the nucleus of the solitary tract of the mouse.

    Science.gov (United States)

    Carter, D A; Choong, Y-T; Connelly, A A; Bassi, J K; Hunter, N O; Thongsepee, N; Llewellyn-Smith, I J; Fong, A Y; McDougall, S J; Allen, A M

    2017-10-01

    Angiotensin II acts via two main receptors within the central nervous system, with the type 1A receptor (AT 1A R) most widely expressed in adult neurons. Activation of the AT 1 R in the nucleus of the solitary tract (NTS), the principal nucleus receiving central synapses of viscerosensory afferents, modulates cardiovascular reflexes. Expression of the AT 1 R occurs in high density within the NTS of most mammals, including humans, but the fundamental electrophysiological and neurochemical characteristics of the AT 1A R-expressing NTS neurons are not known. To address this, we have used a transgenic mouse, in which the AT 1A R promoter drives expression of green fluorescent protein (GFP). Approximately one-third of AT 1A R-expressing neurons express the catecholamine-synthetic enzyme tyrosine hydroxylase (TH), and a subpopulation of these stained for the transcription factor paired-like homeobox 2b (Phox2b). A third group, comprising approximately two-thirds of the AT 1A R-expressing NTS neurons, showed Phox2b immunoreactivity alone. A fourth group in the ventral subnucleus expressed neither TH nor Phox2b. In whole cell recordings from slices in vitro, AT 1A R-GFP neurons exhibited voltage-activated potassium currents, including the transient outward current and the M-type potassium current. In two different mouse strains, both AT 1A R-GFP neurons and TH-GFP neurons showed similar AT 1A R-mediated depolarizing responses to superfusion with angiotensin II. These data provide a comprehensive description of AT 1A R-expressing neurons in the NTS and increase our understanding of the complex actions of this neuropeptide in the modulation of viscerosensory processing. Copyright © 2017 the American Physiological Society.

  9. Cytokine-like factor-1, a novel soluble protein, shares homology with members of the cytokine type I receptor family.

    Science.gov (United States)

    Elson, G C; Graber, P; Losberger, C; Herren, S; Gretener, D; Menoud, L N; Wells, T N; Kosco-Vilbois, M H; Gauchat, J F

    1998-08-01

    In this report we describe the identification, cloning, and expression pattern of human cytokine-like factor 1 (hCLF-1) and the identification and cloning of its murine homologue. They were identified from expressed sequence tags using amino acid sequences from conserved regions of the cytokine type I receptor family. Human CLF-1 and murine CLF-1 shared 96% amino acid identity and significant homology with many cytokine type I receptors. CLF-1 is a secreted protein, suggesting that it is either a soluble subunit within a cytokine receptor complex, like the soluble form of the IL-6R alpha-chain, or a subunit of a multimeric cytokine, e.g., IL-12 p40. The highest levels of hCLF-1 mRNA were observed in lymph node, spleen, thymus, appendix, placenta, stomach, bone marrow, and fetal lung, with constitutive expression of CLF-1 mRNA detected in a human kidney fibroblastic cell line. In fibroblast primary cell cultures, CLF-1 mRNA was up-regulated by TNF-alpha, IL-6, and IFN-gamma. Western blot analysis of recombinant forms of hCLF-1 showed that the protein has the tendency to form covalently linked di- and tetramers. These results suggest that CLF-1 is a novel soluble cytokine receptor subunit or part of a novel cytokine complex, possibly playing a regulatory role in the immune system and during fetal development.

  10. DMH1, a small molecule inhibitor of BMP type i receptors, suppresses growth and invasion of lung cancer.

    Directory of Open Access Journals (Sweden)

    Jijun Hao

    Full Text Available The bone morphogenetic protein (BMP signaling cascade is aberrantly activated in human non-small cell lung cancer (NSCLC but not in normal lung epithelial cells, suggesting that blocking BMP signaling may be an effective therapeutic approach for lung cancer. Previous studies demonstrated that some BMP antagonists, which bind to extracellular BMP ligands and prevent their association with BMP receptors, dramatically reduced lung tumor growth. However, clinical application of protein-based BMP antagonists is limited by short half-lives, poor intra-tumor delivery as well as resistance caused by potential gain-of-function mutations in the downstream of the BMP pathway. Small molecule BMP inhibitors which target the intracellular BMP cascades would be ideal for anticancer drug development. In a zebrafish embryo-based structure and activity study, we previously identified a group of highly selective small molecule inhibitors specifically antagonizing the intracellular kinase domain of BMP type I receptors. In the present study, we demonstrated that DMH1, one of such inhibitors, potently reduced lung cell proliferation, promoted cell death, and decreased cell migration and invasion in NSCLC cells by blocking BMP signaling, as indicated by suppression of Smad 1/5/8 phosphorylation and gene expression of Id1, Id2 and Id3. Additionally, DMH1 treatment significantly reduced the tumor growth in human lung cancer xenograft model. In conclusion, our study indicates that small molecule inhibitors of BMP type I receptors may offer a promising novel strategy for lung cancer treatment.

  11. Increased interaction with insulin receptor substrate 1, a novel abnormality in insulin resistance and type 2 diabetes

    DEFF Research Database (Denmark)

    Caruso, Michael; Ma, Danjun; Msallaty, Zaher

    2014-01-01

    Insulin receptor substrate 1 (IRS1) is a key mediator of insulin signal transduction. Perturbations involving IRS1 complexes may lead to the development of insulin resistance and type 2 diabetes (T2D). Surprisingly little is known about the proteins that interact with IRS1 in humans under health...... in obesity and T2D in humans, provides new insights into the molecular mechanism of insulin resistance and identifies new targets for T2D drug development....... and disease conditions. We used a proteomic approach to assess IRS1 interaction partners in skeletal muscle from lean healthy control subjects (LCs), obese insulin-resistant nondiabetic control subjects (OCs), and participants with T2D before and after insulin infusion. We identified 113 novel endogenous IRS1...

  12. Effects of hand-training in persons with myotonic dystrophy type 1--a randomised controlled cross-over pilot study.

    Science.gov (United States)

    Aldehag, Anna; Jonsson, Hans; Lindblad, Jan; Kottorp, Anders; Ansved, Tor; Kierkegaard, Marie

    2013-10-01

    To investigate the effects of a hand-training programme on grip, pinch and wrist force, manual dexterity and activities of daily living, in adults with myotonic dystrophy type 1 (DM1). In this randomised controlled trial with a crossover design, 35 adults with DM1 were, after stratification for grip force, assigned by lot to two groups. Group A started with 12 weeks of hand training, while group B had no intervention. After a wash-out period of 12 weeks, where none received training, the order was reversed. The Grippit® was used as primary outcome measure and the hand-held Microfet2™ myometer, the Purdue Pegboard, the Canadian Occupational Performance Measure (COPM) and the Assessment of Motor and Process Skills (AMPS) were secondary outcome measures. Assessments were performed before and after training and control periods, i.e. four times altogether. Ten persons dropped out and 13 had acceptable adherence. Intention-to-treat analyses revealed significant intervention effects for isometric wrist flexor force (p = 0.048), and for COPM performance (p = 0.047) and satisfaction (p = 0.027). On an individual level, improvements were in general showed after a training period. The hand-training programme had positive effects on wrist flexor force and self-perception of occupational performance, and of satisfaction with performance. No evident detrimental effects were shown. Myotonic dystrophy type 1 (DM1) is a slowly progressive neuromuscular disease characterised by myotonia and muscle weakness and wasting. People with DM1 are often concerned about their ability to carry out ADL and to participate in, e.g. work, sports and hobbies when they gradually become weaker. This pilot study showed that a hand-training programme improved wrist flexor force and self-perception and satisfaction of occupational performance. Resistance training of hand muscles with a silicon-based putty can be a therapy option for people with DM1 in clinical practise.

  13. NOD2, RIP2 and IRF5 Play a Critical Role in the Type I Interferon Response to Mycobacterium tuberculosis

    Science.gov (United States)

    Jiang, Zhaozhao; Fortune, Sarah M.; Coulombe, Francois; Behr, Marcel A.; Fitzgerald, Katherine A.; Sassetti, Christopher M.; Kelliher, Michelle A.

    2009-01-01

    While the recognition of microbial infection often occurs at the cell surface via Toll-like receptors, the cytosol of the cell is also under surveillance for microbial products that breach the cell membrane. An important outcome of cytosolic recognition is the induction of IFNα and IFNβ, which are critical mediators of immunity against both bacteria and viruses. Like many intracellular pathogens, a significant fraction of the transcriptional response to Mycobacterium tuberculosis infection depends on these type I interferons, but the recognition pathways responsible remain elusive. In this work, we demonstrate that intraphagosomal M. tuberculosis stimulates the cytosolic Nod2 pathway that responds to bacterial peptidoglycan, and this event requires membrane damage that is actively inflicted by the bacterium. Unexpectedly, this recognition triggers the expression of type I interferons in a Tbk1- and Irf5-dependent manner. This response is only partially impaired by the loss of Irf3 and therefore, differs fundamentally from those stimulated by bacterial DNA, which depend entirely on this transcription factor. This difference appears to result from the unusual peptidoglycan produced by mycobacteria, which we show is a uniquely potent agonist of the Nod2/Rip2/Irf5 pathway. Thus, the Nod2 system is specialized to recognize bacteria that actively perturb host membranes and is remarkably sensitive to mycobacteria, perhaps reflecting the strong evolutionary pressure exerted by these pathogens on the mammalian immune system. PMID:19578435

  14. A novel zinc finger protein 219-like (ZNF219L) is involved in the regulation of collagen type 2 alpha 1a (col2a1a) gene expression in zebrafish notochord.

    Science.gov (United States)

    Lien, Huang-Wei; Yang, Chung-Hsiang; Cheng, Chia-Hsiung; Hung, Chin-Chun; Liao, Wei-Hao; Hwang, Pung-Pung; Han, Yu-San; Huang, Chang-Jen

    2013-01-01

    The notochord is required for body plan patterning in vertebrates, and defects in notochord development during embryogenesis can lead to diseases affecting the adult. It is therefore important to elucidate the gene regulatory mechanism underlying notochord formation. In this study, we cloned the zebrafish zinc finger 219-like (ZNF219L) based on mammalian ZNF219, which contains nine C2H2-type zinc finger domains. Through whole-mount in situ hybridization, we found that znf219L mRNA is mainly expressed in the zebrafish midbrain-hindbrain boundary, hindbrain, and notochord during development. The znf219L morpholino knockdown caused partial abnormal notochord phenotype and reduced expression of endogenous col2a1a in the notochord specifically. In addition, ZNF219L could recognize binding sites with GGGGG motifs and trigger augmented activity of the col2a1a promoter in a luciferase assay. Furthermore, in vitro binding experiments revealed that ZNF219L recognizes the GGGGG motifs in the promoter region of the zebrafish col2a1a gene through its sixth and ninth zinc finger domains. Taken together, our results reveal that ZNF219L is involved in regulating the expression of col2a1a in zebrafish notochord specifically.

  15. TAIL1: an isthmin-like gene, containing type 1 thrombospondin-repeat and AMOP domain, mapped to ARVD1 critical region.

    Science.gov (United States)

    Rossi, Valeria; Beffagna, Giorgia; Rampazzo, Alessandra; Bauce, Barbara; Danieli, Gian Antonio

    2004-06-23

    Isthmins represent a novel family of vertebrate secreted proteins containing one copy of the thrombospondin type 1 repeat (TSR), which in mammals is shared by several proteins with diverse biological functions, including cell adhesion, angiogenesis, and patterning of developing nervous system. We have determined the genomic organization of human TAIL1 (thrombospondin and AMOP containing isthmin-like 1), a novel isthmin-like gene encoding a protein that contains a TSR and a C-terminal AMOP domain (adhesion-associated domain in MUC4 and other proteins), characteristic of extracellular proteins involved in adhesion processes. TAIL1 gene encompasses more than 24.4 kb. Analysis of the DNA sequence surrounding the putative transcriptional start region revealed a TATA-less promoter located in a CpG island. Several consensus binding sites for the transcription factors Sp1 and MZF-1 were identified in this promoter region. In humans, TAIL1 gene is located on chromosome 14q24.3 within ARVD1 (arrhythmogenic right ventricular dysplasia/cardiomyopathy, type 1) critical region; preliminary evidence suggests that it is expressed in several tissues, showing multiple alternative splicing.

  16. Usefulness of Whole-Body Fluorine-18-Fluorodeoxyglucose Positron Emission Tomography in Patients with Neurofibromatosis Type 1: A Systematic Review

    Directory of Open Access Journals (Sweden)

    Giorgio Treglia

    2012-01-01

    Full Text Available Aim. To systematically review the role of positron emission tomography (PET with fluorine-18-fluorodeoxyglucose (FDG in patients with neurofibromatosis type 1 (NF1. Methods. A comprehensive literature search of published studies regarding FDG-PET and PET/CT in patients with NF1 was performed. No beginning date limit and language restriction were used; the search was updated until December 2011. Only those studies or subsets in studies including whole-body FDG-PET or PET/CT scans performed in patients with NF1 were included. Results. We identified 12 studies including 352 NF1 patients. Qualitative evaluation was performed in about half of the studies and semiquantitative analysis, mainly based on different values of SUV cutoff, in the others. Most of the studies evaluated the role of FDG-PET for differentiating benign from malignant peripheral nerve sheath tumors (MPNSTs. Malignant lesions were detected with a sensitivity ranging between 100% and 89%, but with lower specificity, ranging between 100% and 72%. Moreover, FDG-PET seems to be an important imaging modality for predicting the progression to MPNST and the outcome in patients with MPNST. Two studies evaluated the role of FDG-PET in pediatric patients with NF1. Conclusions. FDG-PET and PET/CT are useful methods to identify malignant change in neurogenic tumors in NF1 and to discriminate malignant from benign neurogenic lesions.

  17. Potential Risk Factors for the Onset of Complex Regional Pain Syndrome Type 1: A Systematic Literature Review

    Directory of Open Access Journals (Sweden)

    Tracey Pons

    2015-01-01

    Full Text Available Anaesthetists in the acute and chronic pain teams are often involved in treating Complex Regional Pain Syndromes. Current literature about the risk factors for the onset of Complex Regional Pain Syndrome Type 1 (CRPS 1 remains sparse. This syndrome has a low prevalence, a highly variable presentation, and no gold standard for diagnosis. In the research setting, the pathogenesis of the syndrome continues to be elusive. There is a growing body of literature that addresses efficacy of a wide range of interventions as well as the likely mechanisms that contribute to the onset of CRPS 1. The objective for this systematic search of the literature focuses on determining the potential risk factors for the onset of CRPS 1. Eligible articles were analysed, dated 1996 to April 2014, and potential risk factors for the onset of CRPS 1 were identified from 10 prospective and 6 retrospective studies. Potential risk factors for the onset of CRPS 1 were found to include being female, particularly postmenopausal female, ankle dislocation or intra-articular fracture, immobilisation, and a report of higher than usual levels of pain in the early phases of trauma. It is not possible to draw definite conclusions as this evidence is heterogeneous and of mixed quality, relevance, and weighting strength against bias and has not been confirmed across multiple trials or in homogenous studies.

  18. Abnormal late visual responses and alpha oscillations in neurofibromatosis type 1: a link to visual and attention deficits

    Science.gov (United States)

    2014-01-01

    Background Neurofibromatosis type 1 (NF1) affects several areas of cognitive function including visual processing and attention. We investigated the neural mechanisms underlying the visual deficits of children and adolescents with NF1 by studying visual evoked potentials (VEPs) and brain oscillations during visual stimulation and rest periods. Methods Electroencephalogram/event-related potential (EEG/ERP) responses were measured during visual processing (NF1 n = 17; controls n = 19) and idle periods with eyes closed and eyes open (NF1 n = 12; controls n = 14). Visual stimulation was chosen to bias activation of the three detection mechanisms: achromatic, red-green and blue-yellow. Results We found significant differences between the groups for late chromatic VEPs and a specific enhancement in the amplitude of the parieto-occipital alpha amplitude both during visual stimulation and idle periods. Alpha modulation and the negative influence of alpha oscillations in visual performance were found in both groups. Conclusions Our findings suggest abnormal later stages of visual processing and enhanced amplitude of alpha oscillations supporting the existence of deficits in basic sensory processing in NF1. Given the link between alpha oscillations, visual perception and attention, these results indicate a neural mechanism that might underlie the visual sensitivity deficits and increased lapses of attention observed in individuals with NF1. PMID:24559228

  19. Aneurysmal rupture of the costo-cervical trunk in a patient with neurofibromatosis type 1: A case report.

    Science.gov (United States)

    Hoonjan, Bhupinder; Thayur, Nagendra; Abu-Own, Abdusalam

    2014-01-01

    Rupture of blood vessels associated with neurofibromatosis type 1 (NF-1) is a rare but life threatening complication. We report the first case of an aneurysmal rupture from the costocervical trunk in a NF-1 patient treated by endovascular embolisation. A 43 year-old gentleman with a past medical history of NF-1 presented with sudden onset left sided neck swelling. A computed tomography (CT) revealed a large cervical haematoma, which was causing airway compromise, requiring the patient to be intubated. Percutaneous embolisation of the bleeding vessel from the costo-cervical trunk was performed with successful haemostasis and no immediate complications. A repeat CT scan showed a reduction in the original cervical haematoma. However, six days post embolisation, the patient arrested with complete whiteout of the left hemithorax. CT angiography is the gold standard for diagnosis of an aneurysmal rupture in NF-1 patients, and percutaneous embolisation is the preferred modality in patients who are haemodynamically stable due to arterial fragility and high intra operative mortality rates. The increasing haemothorax could be explained by the original cervical haematoma draining down into the pleural space, or the possibility of a new second bleed. This is the first reported episode of bleeding from the costocervical trunk in NF-1 patients. Ruptured aneurysms require urgent CT angiography, if haemodynamically stable, and further input from the vascular surgeons and vascular radiologists. Copyright © 2013 The Authors. Published by Elsevier Ltd.. All rights reserved.

  20. Aneurysmal rupture of the costo-cervical trunk in a patient with neurofibromatosis type 1: A case report☆

    Science.gov (United States)

    Hoonjan, Bhupinder; Thayur, Nagendra; Abu-Own, Abdusalam

    2013-01-01

    INTRODUCTION Rupture of blood vessels associated with neurofibromatosis type 1 (NF-1) is a rare but life threatening complication. We report the first case of an aneurysmal rupture from the costocervical trunk in a NF-1 patient treated by endovascular embolisation. PRESENTATION OF CASE A 43 year-old gentleman with a past medical history of NF-1 presented with sudden onset left sided neck swelling. A computed tomography (CT) revealed a large cervical haematoma, which was causing airway compromise, requiring the patient to be intubated. Percutaneous embolisation of the bleeding vessel from the costo-cervical trunk was performed with successful haemostasis and no immediate complications. A repeat CT scan showed a reduction in the original cervical haematoma. However, six days post embolisation, the patient arrested with complete whiteout of the left hemithorax. DISCUSSION CT angiography is the gold standard for diagnosis of an aneurysmal rupture in NF-1 patients, and percutaneous embolisation is the preferred modality in patients who are haemodynamically stable due to arterial fragility and high intra operative mortality rates. The increasing haemothorax could be explained by the original cervical haematoma draining down into the pleural space, or the possibility of a new second bleed. CONCLUSION This is the first reported episode of bleeding from the costocervical trunk in NF-1 patients. Ruptured aneurysms require urgent CT angiography, if haemodynamically stable, and further input from the vascular surgeons and vascular radiologists. PMID:24463561

  1. Usefulness of Whole-Body Fluorine-18-Fluorodeoxyglucose Positron Emission Tomography in Patients with Neurofibromatosis Type 1: A Systematic Review

    International Nuclear Information System (INIS)

    Treglia, G.; Taralli, S.; Giordano, A.; Bertagna, F.; Salsano, M.; Maggi, F.; Muoio, B.; Novellis, P.; Vita, M.L.

    2012-01-01

    To systematically review the role of positron emission tomography (PET) with fluorine-18-fluorodeoxyglucose (FDG) in patients with neurofibromatosis type 1 (NF1). Methods. A comprehensive literature search of published studies regarding FDG-PET and PET/CT in patients with NF1 was performed. No beginning date limit and language restriction were used; the search was updated until December 2011. Only those studies or subsets in studies including whole-body FDG-PET or PET/CT scans performed in patients with NF1 were included. Results. We identified 12 studies including 352 NF1 patients. Qualitative evaluation was performed in about half of the studies and semiquantitative analysis, mainly based on different values of SUV cutoff, in the others. Most of the studies evaluated the role of FDG-PET for differentiating benign from malignant peripheral nerve sheath tumors (MPNSTs). Malignant lesions were detected with a sensitivity ranging between 100% and 89%, but with lower specificity, ranging between 100% and 72%. Moreover, FDG-PET seems to be an important imaging modality for predicting the progression to MPNST and the outcome in patients with MPNST. Two studies evaluated the role of FDG-PET in pediatric patients with NF1. Conclusions. FDG-PET and PET/CT are useful methods to identify malignant change in neurogenic tumors in NF1 and to discriminate malignant from benign neurogenic lesions

  2. Spatiotemporal dynamics and epistatic interaction sites in dengue virus type 1: a comprehensive sequence-based analysis.

    Directory of Open Access Journals (Sweden)

    Pei-Yu Chu

    Full Text Available The continuing threat of dengue fever necessitates a comprehensive characterisation of its epidemiological trends. Phylogenetic and recombination events were reconstructed based on 100 worldwide dengue virus (DENV type 1 genome sequences with an outgroup (prototypes of DENV2-4. The phylodynamic characteristics and site-specific variation were then analysed using data without the outgroup. Five genotypes (GI-GV and a ladder-like structure with short terminal branch topology were observed in this study. Apparently, the transmission of DENV1 was geographically random before gradual localising with human activity as GI-GIII in South Asia, GIV in the South Pacific, and GV in the Americas. Genotypes IV and V have recently shown higher population densities compared to older genotypes. All codon regions and all tree branches were skewed toward a negative selection, which indicated that their variation was restricted by protein function. Notably, multi-epistatic interaction sites were found in both PrM 221 and NS3 1730. Recombination events accumulated in regions E, NS3-NS4A, and particularly in region NS5. The estimated coevolution pattern also highlights the need for further study of the biological role of protein PrM 221 and NS3 1730. The recent transmission of emergent GV sublineages into Central America and Europe mandates closely monitoring of genotype interaction and succession.

  3. Impact of Angiotensin Type 1A Receptors in Principal Cells of the Collecting Duct on Blood Pressure and Hypertension.

    Science.gov (United States)

    Chen, Daian; Stegbauer, Johannes; Sparks, Matthew A; Kohan, Donald; Griffiths, Robert; Herrera, Marcela; Gurley, Susan B; Coffman, Thomas M

    2016-06-01

    The main actions of the renin-angiotensin system to control blood pressure (BP) are mediated by the angiotensin type 1 receptors (AT1Rs). The major murine AT1R isoform, AT1AR, is expressed throughout the nephron, including the collecting duct in both principal and intercalated cells. Principal cells play the major role in sodium and water reabsorption. Although aldosterone is considered to be the dominant regulator of sodium reabsorption by principal cells, recent studies suggest a role for direct actions of AT1R. To specifically examine the contributions of AT1AR in principal cells to BP regulation and the development of hypertension in vivo, we generated inbred 129/SvEv mice with deletion of AT1AR from principal cells (PCKO). At baseline, we found that BPs measured by radiotelemetry were similar between PCKOs and controls. During 1-week of low-salt diet (hypertension, there was a modest but significant attenuation of hypertension in PCKOs (163±6 mm Hg) compared with controls (178±2 mm Hg; Phypertension and epithelial sodium channel activation. © 2016 American Heart Association, Inc.

  4. Critical point of gas-liquid type phase transition and phase equilibrium functions in developed two-component plasma model.

    Science.gov (United States)

    Butlitsky, M A; Zelener, B B; Zelener, B V

    2014-07-14

    A two-component plasma model, which we called a "shelf Coulomb" model has been developed in this work. A Monte Carlo study has been undertaken to calculate equations of state, pair distribution functions, internal energies, and other thermodynamics properties. A canonical NVT ensemble with periodic boundary conditions was used. The motivation behind the model is also discussed in this work. The "shelf Coulomb" model can be compared to classical two-component (electron-proton) model where charges with zero size interact via a classical Coulomb law. With important difference for interaction of opposite charges: electrons and protons interact via the Coulomb law for large distances between particles, while interaction potential is cut off on small distances. The cut off distance is defined by an arbitrary ɛ parameter, which depends on system temperature. All the thermodynamics properties of the model depend on dimensionless parameters ɛ and γ = βe(2)n(1/3) (where β = 1/kBT, n is the particle's density, kB is the Boltzmann constant, and T is the temperature) only. In addition, it has been shown that the virial theorem works in this model. All the calculations were carried over a wide range of dimensionless ɛ and γ parameters in order to find the phase transition region, critical point, spinodal, and binodal lines of a model system. The system is observed to undergo a first order gas-liquid type phase transition with the critical point being in the vicinity of ɛ(crit) ≈ 13(T(*)(crit) ≈ 0.076), γ(crit) ≈ 1.8(v(*)(crit) ≈ 0.17), P(*)(crit) ≈ 0.39, where specific volume v* = 1/γ(3) and reduced temperature T(*) = ɛ(-1).

  5. Quo vadis GRI? A (critical) assessment of GRI 3.1 A+ non-financial reports and implications for credibility and standardization

    OpenAIRE

    Knebel Sebastian; Seele Peter

    2015-01-01

    Purpose – The purpose of this paper is to examine the status of non financial reporting according to the Global Reporting Initiative (GRI) 3.1 A+ standard. By examining the comprehensiveness of the GRI performance in corporate non financial reports classified as A+ the authors challenge the external assurance system imposed by GRI 3.1 A+ and discuss future directions for the application of GRI 4.0 particularly with regard to the standardized corporate reporting software language XBRL. Des...

  6. COL1A2 gene analysis in a Czech osteogenesis imperfecta patient: a candidate novel mutation in a patient affected by osteogenesis imperfecta type 3

    Directory of Open Access Journals (Sweden)

    Hrušková L

    2015-08-01

    Full Text Available Lucie Hrušková,1 Ivo Mařík,2,3 Stella Mazurová,1 Pavel Martásek,1 Ivan Mazura1 1Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic; 2Ambulant Centre for Defects of Locomotor Apparatus 1.1.c., Prague, Czech Republic; 3Faculty of Medical Studies, West Bohemia University, Pilsen, Czech RepublicAbstract: Osteogenesis imperfecta is a heritable bone fragility disease with a heterogenic genetic origin. Most cases result from mutations of either the COL1A1 gene or the COL1A2 gene. We identified a novel COL1A2 gene mutation in a Czech patient, born to unaffected parents, who was diagnosed according to clinical and anthropometric findings and radiographic features as having type 3 osteogenesis imperfecta, which is a severe form of this disease. The identified Gly814Trp mutation was predicted by a number of complementary bioinformatic programs to result in functional alteration of the protein. This case report provides both evidence of a novel COL1A2 mutation resulting in type 3 osteogenesis imperfecta and a genotype:phenotype correlation in this affected individual. Keywords: osteogenesis imperfecta type 3, collagen, alpha-2 (I chain, substitution, sequencing 

  7. Genetic relationships between clinical and non-clinical strains of Yersinia enterocolitica biovar 1A as revealed by multilocus enzyme electrophoresis and multilocus restriction typing

    Directory of Open Access Journals (Sweden)

    Virdi Jugsharan S

    2010-05-01

    Full Text Available Abstract Background Genetic relationships among 81 strains of Y. enterocolitica biovar 1A isolated from clinical and non-clinical sources were discerned by multilocus enzyme electrophoresis (MLEE and multilocus restriction typing (MLRT using six loci each. Such studies may reveal associations between the genotypes of the strains and their sources of isolation. Results All loci were polymorphic and generated 62 electrophoretic types (ETs and 12 restriction types (RTs. The mean genetic diversity (H of the strains by MLEE and MLRT was 0.566 and 0.441 respectively. MLEE (DI = 0.98 was more discriminatory and clustered Y. enterocolitica biovar 1A strains into four groups, while MLRT (DI = 0.77 identified two distinct groups. BURST (Based Upon Related Sequence Types analysis of the MLRT data suggested aquatic serotype O:6,30-6,31 isolates to be the ancestral strains from which, clinical O:6,30-6,31 strains might have originated by host adaptation and genetic change. Conclusion MLEE revealed greater genetic diversity among strains of Y. enterocolitica biovar 1A and clustered strains in four groups, while MLRT grouped the strains into two groups. BURST analysis of MLRT data nevertheless provided newer insights into the probable evolution of clinical strains from aquatic strains.

  8. Angiotensin II type 1a receptor-deficient mice develop angiotensin II-induced oxidative stress and DNA damage without blood pressure increase.

    Science.gov (United States)

    Zimnol, Anna; Amann, Kerstin; Mandel, Philipp; Hartmann, Christina; Schupp, Nicole

    2017-12-01

    Hypertensive patients have an increased risk of developing kidney cancer. We have shown in vivo that besides elevating blood pressure, angiotensin II causes DNA damage dose dependently. Here, the role of blood pressure in the formation of DNA damage is studied. Mice lacking one of the two murine angiotensin II type 1 receptor (AT1R) subtypes, AT1aR, were equipped with osmotic minipumps, delivering angiotensin II during 28 days. Parameters of oxidative stress and DNA damage of kidneys and hearts of AT1aR-knockout mice were compared with wild-type (C57BL/6) mice receiving angiotensin II, and additionally, with wild-type mice treated with candesartan, an antagonist of both AT1R subtypes. In wild-type mice, angiotensin II induced hypertension, reduced kidney function, and led to a significant formation of reactive oxygen species (ROS). Furthermore, genomic damage was markedly increased in this group. All these responses to angiotensin II could be attenuated by concurrent administration of candesartan. In AT1aR-deficient mice treated with angiotensin II, systolic pressure was not increased, and renal function was not affected. However, angiotensin II still led to an increase of ROS in kidneys and hearts of these animals. Additionally, genomic damage in the form of double-strand breaks was significantly induced in kidneys of AT1aR-deficient mice. Our results show that angiotensin II induced ROS production and DNA damage even without the presence of AT1aR and independently of blood pressure changes. Copyright © 2017 the American Physiological Society.

  9. Comparative studies of vertebrate scavenger receptor class B type 1: a high-density lipoprotein binding protein

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    Holmes RS

    2012-06-01

    Full Text Available Roger S Holmes,1,2 Laura A Cox11Department of Genetics and Southwest National Primate Research Center, Texas Biomedical Research Institute, San Antonio, TX, USA; 2School of Biomolecular and Physical Sciences, Griffith University, Nathan, Queensland, AustraliaAbstract: Scavenger receptor class B type 1 protein (SCARB1 plays an essential role in cholesterol homeostasis and functions in binding high density lipoprotein cholesterol (HDL in liver and other tissues of the body. SCARB1 also functions in lymphocyte homeostasis and in the uptake of hepatitis C virus (HCV by the liver. A genetic deficiency of this protein results in autoimmune disorders and significant changes in blood cholesterol phenotype. Comparative SCARB1 amino acid sequences and structures and SCARB1 gene locations were examined using data from several vertebrate genome projects. Vertebrate SCARB1 sequences shared 50%–99% identity as compared with 28%–31% sequence identities with other CD36-like superfamily members, ie, SCARB2 and SCARB3 (also called CD36. At least eight N-glycosylation sites were conserved among most of the vertebrate SCARB1 proteins examined. Sequence alignments, key amino acid residues, and conserved predicted secondary structures were also studied, including: cytoplasmic, transmembrane, and exoplasmic sequences; conserved N-terminal and C-terminal transmembrane glycines which participate in oligomer formation; conserved cystine disulfides and a free SH residue which participates in lipid transport; carboxyl terminal PDZ-binding domain sequences (Ala507-Arg/Lys508-Leu509; and 30 conserved proline and 18 conserved glycine residues, which may contribute to short loop formation within the exoplasmic HDL-binding sequence. Vertebrate SCARB1 genes usually contained 12 coding exons. The human SCARB1 gene contained CpG islands, micro RNA binding sites, and several transcription factor binding sites (including PPARG which may contribute to the high level (13.7 times

  10. Immunophenotypic and Ultrastructural Analysis of Mast Cells in Hermansky-Pudlak Syndrome Type-1: A Possible Connection to Pulmonary Fibrosis.

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    Arnold S Kirshenbaum

    Full Text Available Hermansky-Pudlak Syndrome type-1 (HPS-1 is an autosomal recessive disorder caused by mutations in HPS1 which result in reduced expression of the HPS-1 protein, defective lysosome-related organelle (LRO transport and absence of platelet delta granules. Patients with HPS-1 exhibit oculocutaneous albinism, colitis, bleeding and pulmonary fibrosis postulated to result from a dysregulated immune response. The effect of the HPS1 mutation on human mast cells (HuMCs is unknown. Since HuMC granules classify as LROs along with platelet granules and melanosomes, we set out to determine if HPS-1 cutaneous and CD34+ culture-derived HuMCs have distinct granular and cellular characteristics. Cutaneous and cultured CD34+-derived HuMCs from HPS-1 patients were compared with normal cutaneous and control HuMCs, respectively, for any morphological and functional differences. One cytokine-independent HPS-1 culture was expanded, cloned, designated the HP proMastocyte (HPM cell line and characterized. HPS-1 and idiopathic pulmonary fibrosis (IPF alveolar interstitium showed numerous HuMCs; HPS-1 dermal mast cells exhibited abnormal granules when compared to healthy controls. HPS-1 HuMCs showed increased CD63, CD203c and reduced mediator release following FcɛRI aggregation when compared with normal HuMCs. HPM cells also had the duplication defect, expressed FcɛRI and intracytoplasmic proteases and exhibited less mediator release following FcɛRI aggregation. HPM cells constitutively released IL-6, which was elevated in patients' serum, in addition to IL-8, fibronectin-1 (FN-1 and galectin-3 (LGALS3. Transduction with HPS1 rescued the abnormal HPM morphology, cytokine and matrix secretion. Microarray analysis of HPS-1 HuMCs and non-transduced HPM cells confirmed upregulation of differentially expressed genes involved in fibrogenesis and degranulation. Cultured HPS-1 HuMCs appear activated as evidenced by surface activation marker expression, a decrease in mediator

  11. Angiotensin type 1a receptors in the paraventricular nucleus of the hypothalamus control cardiovascular reactivity and anxiety-like behavior in male mice.

    Science.gov (United States)

    Wang, Lei; Hiller, Helmut; Smith, Justin A; de Kloet, Annette D; Krause, Eric G

    2016-09-01

    This study tested the hypothesis that deletion of angiotensin type 1a receptors (AT1a) from the paraventricular nucleus of hypothalamus (PVN) attenuates anxiety-like behavior, hypothalamic-pituitary-adrenal (HPA) axis activity, and cardiovascular reactivity. We used the Cre/LoxP system to generate male mice with AT1a specifically deleted from the PVN. Deletion of the AT1a from the PVN reduced anxiety-like behavior as indicated by increased time spent in the open arms of the elevated plus maze. In contrast, PVN AT1a deletion had no effect on HPA axis activation subsequent to an acute restraint challenge but did reduce hypothalamic mRNA expression for corticotropin-releasing hormone (CRH). To determine whether PVN AT1a deletion inhibits cardiovascular reactivity, we measured systolic blood pressure, heart rate, and heart rate variability (HRV) using telemetry and found that PVN AT1a deletion attenuated restraint-induced elevations in systolic blood pressure and elicited changes in HRV indicative of reduced sympathetic nervous activity. Consistent with the decreased HRV, PVN AT1a deletion also decreased adrenal weight, suggestive of decreased adrenal sympathetic outflow. Interestingly, the altered stress responsivity of mice with AT1a deleted from the PVN was associated with decreased hypothalamic microglia and proinflammatory cytokine expression. Collectively, these results suggest that deletion of AT1a from the PVN attenuates anxiety, CRH gene transcription, and cardiovascular reactivity and reduced brain inflammation may contribute to these effects. Copyright © 2016 the American Physiological Society.

  12. Complications impaired endothelial progenitor cell function in Type 2 diabetic patients with or without critical leg ischaemia: implication for impaired neovascularization in diabetes.

    Science.gov (United States)

    Chen, M-C; Sheu, J-J; Wang, P-W; Chen, C-Y; Kuo, M-C; Hsieh, C-J; Chen, J-F; Chang, H-W

    2009-02-01

    This study tested the hypothesis that migratory function of endothelial progenitor cells (EPCs) is impaired in Type 2 diabetic patients with or without critical leg ischaemia. Seventy-four patients were classified into four groups: Type 2 diabetic (n = 21) and non-diabetic patients (n = 10) with critical leg ischaemia and Type 2 diabetic patients without lower extremity vascular disease (n = 30) and healthy subjects (n = 13). The number and functional activity of circulating and cultured EPCs were determined. The migratory function of cultured EPCs was significantly impaired in diabetic patients without (median, 48, interquartile range, 46, 49 count/view/well) and with (median, 51, interquartile range, 46, 60 count/view/well) critical leg ischaemia and non-diabetic patients with critical leg ischaemia (median, 49, interquartile range, 47, 55 count/view/well) compared with healthy subjects (median, 63, interquartile range, 57, 65 count/view/well) (P interquartile range, 1600, 6600/10(6) cytometric events) than Type 2 diabetic patients with critical leg ischaemia (median, 5300, interquartile range, 2400, 11,100/10(6) cytometric events), non-diabetic patients with critical leg ischaemia (median, 5550, interquartile range, 2000, 32,100/10(6) cytometric events) and healthy subjects (median, 5400, interquartile range, 2700, 8700/10(6) cytometric events) (P = 0.413). The migratory function of EPCs is impaired in patients with Type 2 diabetes, even in those without critical leg ischaemia. These findings present an important new insight into the pathogenesis of impaired neovascularization and critical limb ischaemia in diabetic patients and provide avenues of future clinical study.

  13. Genome wide identification of wheat and Brachypodium type one protein phosphatases and functional characterization of durum wheat TdPP1a.

    Directory of Open Access Journals (Sweden)

    Mariem Bradai

    Full Text Available Reversible phosphorylation is an essential mechanism regulating signal transduction during development and environmental stress responses. An important number of dephosphorylation events in the cell are catalyzed by type one protein phosphatases (PP1, which catalytic activity is driven by the binding of regulatory proteins that control their substrate specificity or subcellular localization. Plants harbor several PP1 isoforms accounting for large functional redundancies. While animal PP1s were reported to play relevant roles in controlling multiple cellular processes, plant orthologs remain poorly studied. To decipher the role of plant PP1s, we compared PP1 genes from three monocot species, Brachypodium, common wheat and rice at the genomic and transcriptomic levels. To gain more insight into the wheat PP1 proteins, we identified and characterized TdPP1a, the first wheat type one protein phosphatase from a Tunisian durum wheat variety Oum Rabiaa3. TdPP1a is highly conserved in sequence and structure when compared to mammalian, yeast and other plant PP1s. We demonstrate that TdPP1a is an active, metallo-dependent phosphatase in vitro and is able to interact with AtI2, a typical regulator of PP1 functions. Also, TdPP1a is capable to complement the heat stress sensitivity of the yeast mutant indicating that TdPP1a is functional also in vivo. Moreover, transient expression of TdPP1a::GFP in tobacco leaves revealed that it is ubiquitously distributed within the cell, with a strong accumulation in the nucleus. Finally, transcriptional analyses showed similar expression levels in roots and leaves of durum wheat seedlings. Interestingly, the expression in leaves is significantly induced following salinity stress, suggesting a potential role of TdPP1a in wheat salt stress response.

  14. 4D-π-1A type β-substituted ZnII-porphyrins: ideal green sensitizers for building-integrated photovoltaics.

    Science.gov (United States)

    Covezzi, A; Orbelli Biroli, A; Tessore, F; Forni, A; Marinotto, D; Biagini, P; Di Carlo, G; Pizzotti, M

    2016-10-18

    Two novel green β-substituted Zn II -porphyrins, G1 and G2, based on a 4D-π-1A type substitution pattern have been synthesized. Their enhanced push-pull character, by reduction of H-L energy gaps, promotes broadening and red-shifting of absorption bands. The effective synthetic pathway and the remarkable spectroscopic properties make G2 ideal for BIPV application.

  15. Hsp72 (HSPA1A Prevents Human Islet Amyloid Polypeptide Aggregation and Toxicity: A New Approach for Type 2 Diabetes Treatment.

    Directory of Open Access Journals (Sweden)

    Paola C Rosas

    Full Text Available Type 2 diabetes is a growing public health concern and accounts for approximately 90% of all the cases of diabetes. Besides insulin resistance, type 2 diabetes is characterized by a deficit in β-cell mass as a result of misfolded human islet amyloid polypeptide (h-IAPP which forms toxic aggregates that destroy pancreatic β-cells. Heat shock proteins (HSP play an important role in combating the unwanted self-association of unfolded proteins. We hypothesized that Hsp72 (HSPA1A prevents h-IAPP aggregation and toxicity. In this study, we demonstrated that thermal stress significantly up-regulates the intracellular expression of Hsp72, and prevents h-IAPP toxicity against pancreatic β-cells. Moreover, Hsp72 (HSPA1A overexpression in pancreatic β-cells ameliorates h-IAPP toxicity. To test the hypothesis that Hsp72 (HSPA1A prevents aggregation and fibril formation, we established a novel C. elegans model that expresses the highly amyloidogenic human pro-IAPP (h-proIAPP that is implicated in amyloid formation and β-cell toxicity. We demonstrated that h-proIAPP expression in body-wall muscles, pharynx and neurons adversely affects C. elegans development. In addition, we demonstrated that h-proIAPP forms insoluble aggregates and that the co-expression of h-Hsp72 in our h-proIAPP C. elegans model, increases h-proIAPP solubility. Furthermore, treatment of transgenic h-proIAPP C. elegans with ADAPT-232, known to induce the expression and release of Hsp72 (HSPA1A, significantly improved the growth retardation phenotype of transgenic worms. Taken together, this study identifies Hsp72 (HSPA1A as a potential treatment to prevent β-cell mass decline in type 2 diabetic patients and establishes for the first time a novel in vivo model that can be used to select compounds that attenuate h-proIAPP aggregation and toxicity.

  16. Osteogenesis imperfecta Type IV: a newly identified variant at position c.560 (G > T; p.Gly187Val) in the COL1A2 gene.

    Science.gov (United States)

    Usta, Akin; Karademir, Dilay; Sen, Eylem; Yazici, Selcuk; Adali, Ertan; Erdem, Erkan; Karacan, Meric

    2017-01-01

    Osteogenesis imperfecta is a clinically heterogenous disease caused by defective collagen syntesis associated with a mutation in the COL1A1 or COL1A2 genes. In this report, we present a case of osteogenesis imperfecta (OI) type IV, seen in a female fetus with incurved femurs at 18 weeks of gestation. Molecular analysis of the newborn revealed a novel mutation at position c.560 (c.560 G > T) of the exon 12 in the COL1A2 gene; which lead to the glycine modification with valine (p.Gly187Val) at codon 187. The pregnancy follow-up was uneventful. After delivery, the newborn underwent biphosponat therapy and no fracture was detected until 1 year old.

  17. Qualitative Parameters of the Colonic Flora in Patients with HNF1A-MODY Are Different from Those Observed in Type 2 Diabetes Mellitus.

    Science.gov (United States)

    Mrozinska, Sandra; Radkowski, Piotr; Gosiewski, Tomasz; Szopa, Magdalena; Bulanda, Malgorzata; Ludwig-Galezowska, Agnieszka H; Morawska, Iwona; Sroka-Oleksiak, Agnieszka; Matejko, Bartlomiej; Kapusta, Przemyslaw; Salamon, Dominika; Malecki, Maciej T; Wolkow, Pawel; Klupa, Tomasz

    2016-01-01

    Background . Type 2 diabetes mellitus (T2DM) is determined by genetic and environmental factors. There have been many studies on the relationship between the composition of the gastrointestinal bacterial flora, T2DM, and obesity. There are no data, however, on the gut microbiome structure in monogenic forms of the disease including Maturity Onset Diabetes of the Young (MODY). Methods . The aim of the investigation was to compare the qualitative parameters of the colonic flora in patients with HNF1A - MODY and T2DM and healthy individuals. 16S sequencing of bacterial DNA isolated from the collected fecal samples using the MiSeq platform was performed. Results . There were significant between-group differences in the bacterial profile. At the phylum level, the amount of Proteobacteria was higher ( p = 0.0006) and the amount of Bacteroidetes was lower ( p = 0.0005) in T2DM group in comparison to the control group. In HNF1A-MODY group, the frequency of Bacteroidetes was lower than in the control group ( p = 0.0143). At the order level, Turicibacterales was more abundant in HNF1A-MODY group than in T2DM group. Conclusions . It appears that there are differences in the gut microbiome composition between patients with HNF1A-MODY and type 2 diabetes. Further investigation on this matter should be conducted.

  18. Qualitative Parameters of the Colonic Flora in Patients with HNF1A-MODY Are Different from Those Observed in Type 2 Diabetes Mellitus

    Directory of Open Access Journals (Sweden)

    Sandra Mrozinska

    2016-01-01

    Full Text Available Background. Type 2 diabetes mellitus (T2DM is determined by genetic and environmental factors. There have been many studies on the relationship between the composition of the gastrointestinal bacterial flora, T2DM, and obesity. There are no data, however, on the gut microbiome structure in monogenic forms of the disease including Maturity Onset Diabetes of the Young (MODY. Methods. The aim of the investigation was to compare the qualitative parameters of the colonic flora in patients with HNF1A-MODY and T2DM and healthy individuals. 16S sequencing of bacterial DNA isolated from the collected fecal samples using the MiSeq platform was performed. Results. There were significant between-group differences in the bacterial profile. At the phylum level, the amount of Proteobacteria was higher (p=0.0006 and the amount of Bacteroidetes was lower (p=0.0005 in T2DM group in comparison to the control group. In HNF1A-MODY group, the frequency of Bacteroidetes was lower than in the control group (p=0.0143. At the order level, Turicibacterales was more abundant in HNF1A-MODY group than in T2DM group. Conclusions. It appears that there are differences in the gut microbiome composition between patients with HNF1A-MODY and type 2 diabetes. Further investigation on this matter should be conducted.

  19. Immunology in the clinic review series; focus on type 1 diabetes and viruses: the enterovirus link to type 1 diabetes: critical review of human studies

    Science.gov (United States)

    Stene, L C; Rewers, M

    2012-01-01

    OTHER THEMES PUBLISHED IN THIS IMMUNOLOGY IN THE CLINIC REVIEW SERIES Metabolic diseases, host responses, cancer, autoinflammatory diseases, allergy. The hypothesis that under some circumstances enteroviral infections can lead to type 1 diabetes (T1D) was proposed several decades ago, based initially on evidence from animal studies and sero-epidemiology. Subsequently, enterovirus RNA has been detected more frequently in serum of patients than in control subjects, but such studies are susceptible to selection bias and reverse causality. Here, we review critically recent evidence from human studies, focusing on longitudinal studies with potential to demonstrate temporal association. Among seven longitudinal birth cohort studies, the evidence that enterovirus infections predict islet autoimmunity is quite inconsistent in our interpretation, due partially, perhaps, to heterogeneity in study design and a limited number of subjects studied. An association between enterovirus and rapid progression from autoimmunity to T1D was reported by one longitudinal study, but although consistent with evidence from animal models, this novel observation awaits replication. It is possible that a potential association with initiation and/or progression of islet autoimmunity can be ascribed to a subgroup of the many enterovirus serotypes, but this has still not been investigated properly. There is a need for larger studies with frequent sample intervals and collection of specimens of sufficient quality and quantity for detailed characterization of enterovirus. More research into the molecular epidemiology of enteroviruses and enterovirus immunity in human populations is also warranted. Ultimately, this knowledge may be used to devise strategies to reduce the risk of T1D in humans. PMID:22385232

  20. State of art report for critical flow model to analyze a break flow in pressurizer of integral type reactor

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Yeon Moon; Lee, D. J.; Yoon, J. H.; Kim, J. P.; Kim, H. Y

    1999-03-01

    At a critical flow condition, the flow rate can't exceed a maximum value for given upstream conditions and the limited flow rate is called as a critical flow rate. The phenomena of critical flow occur at the discharge of a single phase gas or subcooled water through nozzles and pipes. Among the previous researches on critical flow, many accurate correlations on pressure, temperature and flow rate are represented for the single phase gas. However, for the two phase critical flow, the results of previous work showed that there was a large discrepancy between the analytical and experimental data and the data were in agreement for the limited thermodynamic conditions. Thus, further studies are required to enhance the two phase critical flow model. In the integral reactor, the critical flows of nitrogen gas and subcooled water are expected for the break of gas cylinder pipeline connected to the pressurizer. It requires that the inlet shape of the pipe and the nitrogen gas effect should be considered for the critical flow of integral reactor. The nitrogen gas exist in the pressurizer may affect the flow rate of primary coolant, which has been considered only for a few previous researches. Thus, the evaluation of the effect of the nitrogen on the critical flow gas should be preceded for the proper analysis of the critical flow in the integral reactor. In this report, not only the essences of previous work on critical flow were investigated and summarized but also the effect of nitrogen gas and the inlet shape of the pipe on the critical flow were also investigated. (author)

  1. State of art report for critical flow model to analyze a break flow in pressurizer of integral type reactor

    International Nuclear Information System (INIS)

    Kang, Yeon Moon; Lee, D. J.; Yoon, J. H.; Kim, J. P.; Kim, H. Y.

    1999-03-01

    At a critical flow condition, the flow rate can't exceed a maximum value for given upstream conditions and the limited flow rate is called as a critical flow rate. The phenomena of critical flow occur at the discharge of a single phase gas or subcooled water through nozzles and pipes. Among the previous researches on critical flow, many accurate correlations on pressure, temperature and flow rate are represented for the single phase gas. However, for the two phase critical flow, the results of previous work showed that there was a large discrepancy between the analytical and experimental data and the data were in agreement for the limited thermodynamic conditions. Thus, further studies are required to enhance the two phase critical flow model. In the integral reactor, the critical flows of nitrogen gas and subcooled water are expected for the break of gas cylinder pipeline connected to the pressurizer. It requires that the inlet shape of the pipe and the nitrogen gas effect should be considered for the critical flow of integral reactor. The nitrogen gas exist in the pressurizer may affect the flow rate of primary coolant, which has been considered only for a few previous researches. Thus, the evaluation of the effect of the nitrogen on the critical flow gas should be preceded for the proper analysis of the critical flow in the integral reactor. In this report, not only the essences of previous work on critical flow were investigated and summarized but also the effect of nitrogen gas and the inlet shape of the pipe on the critical flow were also investigated. (author)

  2. Urinary calcium to creatinine ratio: a potential marker of secondary hyperparathyroidism in patients with vitamin D-dependent rickets type 1A.

    Science.gov (United States)

    Miyai, Kentaro; Onishi, Toshikazu; Kashimada, Kenichi; Hasegawa, Yukihiro

    2015-01-01

    Patients with vitamin D-dependent rickets type 1A (VDDR1A) are usually treated with alfacalcidol, an analog of vitamin D. Around puberty, an increased dose of alfacalcidol is recommended for these patients to avoid hypocalcemia and secondary hyperparathyroidism. However, no indicators of secondary hyperparathyroidism except for PTH are presently known. The aim of this study is to evaluate whether urinary calcium to creatinine ratio (U-Ca/Cr) is useful as a biomarker of secondary hyperparathyroidism in VDDR1A patients in order to determine the proper dose of alfacalcidol. Two brothers with VDDR1A were recruited who had null mutations of CYP27B1 which encodes 1-alpha-hydroxylase of vitamin D. We investigated the relationship between U-Ca/Cr and intact-PTH around puberty when the brothers showed hypocalcemia with secondary hyperparathyroidism. The results were compared to those of five patients with vitamin D deficiency (VDD). As a result, high intact-PTH levels were observed when U-Ca/Cr decreased to less than 0.1 (mg/mg) in both VDDR1A brothers. This relationship was also observed in the VDD patients. However, it is necessary to take into account body calcium status, either in depletion or in excess, to accurately evaluate the relationship between U-Ca/Cr and secondary hyperparathyroidism. First, low U-Ca/Cr was detected in situations with calcium depletion without hyperparathyroidism in the VDDR1A patients. Second, high U-Ca/Cr with hyperparathyroidism could be detected theoretically in a condition of excess calcium supply. In conclusion, a U-Ca/Cr ratio of less than 0.1 (mg/mg) in VDDR1A patients is useful to accurately evaluate calcium depletion and secondary hyperparathyroidism.

  3. Methods for the elicitation and use of expert opinion in risk assessment: Phase 1, A critical evaluation and directions for future research

    International Nuclear Information System (INIS)

    Mosleh, A.; Bier, V.M.; Apostolakis, G.

    1987-08-01

    The purpose of this work is to critically review and evaluate the elicitation and use of expert opinion in probabilistic risk assessment (PRA) in light of the available empirical and theoretical results on expert opinion use. PRA practice is represented by five case studies selected to represent a variety of aspects of the problem: assessments of component failure rates and maintenance data; recent assessments of seismic hazard rates; assessments of containment phenomenology; assessments of human error rates; and accident precursor studies. The review has yielded mixed results. On the negative side, there appears to be little reliance on normative expertise in structuring the process of expert opinion elicitation and use; most applications instead rely primarily on the common sense of the experts involved in the analysis, which is not always an adequate guide. On the positive side, however, there is evidence that expert opinions can in fact be used well in practical settings. Suggestions are given for Phase II work to enhance the applicability and use of appropriate expert opinion methods

  4. Glucose effectiveness is a critical pathogenic factor leading to glucose intolerance and type 2 diabetes: An ignored hypothesis.

    Science.gov (United States)

    Alford, F P; Henriksen, J E; Rantzau, C; Beck-Nielsen, H

    2018-02-16

    Although the ability of glucose to mediate its own in vivo metabolism is long documented, the quantitative measurement of whole body glucose-mediated glucose disposal at basal insulin levels (glucose effectiveness [GE]), followed the introduction of the Minimal Model intravenous glucose tolerance test technique. A literature review, combined with our own studies, of the role of GE in glucose metabolism in normal and "at risk" individuals, was undertaken to determine GE's contribution to glucose homeostasis. GE accounts for ~45% to 65% of glucose disposal in man. A negative association between GE and insulin meditated glucose disposal (Si), is present in normal subjects without a family history of type 2 diabetes mellitus but is absent in normoglycaemic "at risk" relatives with a positive family history of diabetes mellitus. Intracellular GE disposal is mediated by mass action of glucose through the skeletal muscle membrane via facilitated Glut 4 transporters. However, GE is frequently forgotten as a significant contributor to the development of glucose intolerance in "at risk" individuals. Only limited studies have examined the role of a lower GE in such normoglycemic subjects with preexisting mild insulin resistance and β-cell dysfunction. These studies demonstrate that in "at risk" individuals, an initial low GE is a key contributor and predictor of future glucose intolerance, whereas an initial raised GE is protective against future glucose intolerance. In "at risk" individuals, a low GE and genetically determined vulnerable β-cell function are more critical determinants of future glucose intolerance than their preexisting insulin-resistant state. Copyright © 2018 John Wiley & Sons, Ltd.

  5. An Amino Acid of Human Parainfluenza Virus Type 3 Nucleoprotein Is Critical for Template Function and Cytoplasmic Inclusion Body Formation

    Science.gov (United States)

    Zhang, Shengwei; Chen, Longyun; Zhang, Guangyuan; Yan, Qin; Yang, Xiaodan; Ding, Binbin; Tang, Qiaopeng; Sun, Shengjun; Hu, Zhulong

    2013-01-01

    The nucleoprotein (N) and phosphoprotein (P) interaction of nonsegmented negative-strand RNA viruses is essential for viral replication; this includes N0-P (N0, free of RNA) interaction and the interaction of N-RNA with P. The precise site(s) within N that mediates the N-P interaction and the detailed regulating mechanism, however, are less clear. Using a human parainfluenza virus type 3 (HPIV3) minigenome assay, we found that an N mutant (NL478A) did not support reporter gene expression. Using in vivo and in vitro coimmunoprecipitation, we found that NL478A maintains the ability to form NL478A0-P, to self-assemble, and to form NL478A-RNA but that NL478A-RNA does not interact with P. Using an immunofluorescence assay, we found that N-P interaction provides the minimal requirement for the formation of cytoplasmic inclusion bodies, which contain viral RNA, N, P, and polymerase in HPIV3-infected cells. NL478A was unable to form inclusion bodies when coexpressed with P, but the presence of N rescued the ability of NL478A to form inclusion bodies and the transcriptional function of NL478A, thereby suggesting that hetero-oligomers formed by N and NL478A are functional and competent to form inclusion bodies. Furthermore, we found that NL478A is also defective in virus growth. To our knowledge, we are the first to use a paramyxovirus to identify a precise amino acid within N that is critical for N-RNA and P interaction but not for N0-P interaction for the formation of inclusion bodies, which appear to be bona fide sites of RNA synthesis. PMID:24027324

  6. Differential expression of P-type ATPases in intestinal epithelial cells: Identification of putative new atp1a1 splice-variant

    International Nuclear Information System (INIS)

    Rocafull, Miguel A.; Thomas, Luz E.; Barrera, Girolamo J.; Castillo, Jesus R. del

    2010-01-01

    P-type ATPases are membrane proteins that couple ATP hydrolysis with cation transport across the membrane. Ten different subtypes have been described. In mammalia, 15 genes of P-type ATPases from subtypes II-A, II-B and II-C, that transport low-atomic-weight cations (Ca 2+ , Na + , K + and H + ), have been reported. They include reticulum and plasma-membrane Ca 2+ -ATPases, Na + /K + -ATPase and H + /K + -ATPases. Enterocytes and colonocytes show functional differences, which seem to be partially due to the differential expression of P-type ATPases. These enzymes have 9 structural motifs, being the phosphorylation (E) and the Mg 2+ ATP-binding (H) motifs the most preserved. These structural characteristics permitted developing a Multiplex-Nested-PCR (MN-PCR) for the simultaneous identification of different P-type ATPases. Thus, using MN-PCR, seven different cDNAs were cloned from enterocytes and colonocytes, including SERCA3, SERCA2, Na + /K + -ATPase α1-isoform, H + /K + -ATPase α2-isoform, PMCA1, PMCA4 and a cDNA-fragment that seems to be a new cassette-type splice-variant of the atp1a1 gen. PMCA4 in enterocytes and H + /K + -ATPase α2-isoform in colonocytes were differentially expressed. This cell-specific expression pattern is related with the distinctive enterocyte and colonocyte functions.

  7. Osteogenesis imperfecta type I: second-trimester diagnosis and incidental identification of a dominant COL1A1 deletion mutation in the paucisymptomatic father.

    Science.gov (United States)

    Chen, Chih-Ping; Su, Yi-Ning; Chang, Tung-Yao; Chern, Schu-Rern; Chen, Chen-Yu; Su, Jun-Wei; Wang, Wayseen

    2012-06-01

    To present second-trimester ultrasound and molecular diagnosis for osteogenesis imperfecta (OI) type I in a female fetus and incidental identification of a dominant COL1A1 deletion mutation in her paucisymptomatic father. A 30-year-old, primigravid woman was referred for genetic counseling in the second trimester because of bowing of the fetal lower limbs. She and her husband were non-consanguineous, and there was no family history of skeletal dysplasias. Prenatal ultrasound at 22 weeks of gestation revealed short and curved right femur and left tibia, and a short left fibula. The lengths of other long bones were normal. The husband was 158 cm tall, had blue sclerae, a history of habitual subluxation and dislocation of bilateral elbows and left knee, and an episode of left ulna fracture, and was not aware of his being affected with OI type I. The woman underwent amniocentesis. Cytogenetic analysis revealed a karyotype of 46,XX. Molecular analysis of the amniocytes revealed a heterozygous deletion mutation of c.1064_1068delCTGGT in exon 17 of the COL1A1 gene. By genetic testing the husband was found to carry the same mutation. Despite counseling of favorable outcome for OI type I with the parents, the woman elected to terminate the pregnancy. Postnatal skeletal X-ray findings were consistent with OI type I. Prenatal ultrasound diagnosis of mild forms of OI should include molecular analysis of type I collagen genes in both fetus and parents. Molecular genetic analysis of the family may incidentally identify a collagen gene mutation in the paucisymptomatic affected parent. Copyright © 2012. Published by Elsevier B.V.

  8. HDL cholesterol as a diagnostic tool for clinical differentiation of GCK-MODY from HNF1A-MODY and type 1 diabetes in children and young adults.

    Science.gov (United States)

    Fendler, Wojciech; Borowiec, Maciej; Antosik, Karolina; Szadkowska, Agnieszka; Deja, Grazyna; Jarosz-Chobot, Przemyslawa; Mysliwiec, Malgorzata; Wyka, Krystyna; Pietrzak, Iwona; Skupien, Jan; Malecki, Maciej T; Mlynarski, Wojciech

    2011-09-01

    Confirmation of monogenic diabetes caused by glucokinase mutations (GCK-MODY) allows pharmacogenetic intervention in the form of insulin discontinuation. This is especially important among paediatric and young adult populations where GCK-MODY is most prevalent. The study evaluated the utility of lipid parameters in screening for patients with GCK-MODY. Eighty-nine children with type 1 diabetes and 68 with GCK-MODY were screened for triglyceride (TG), total and HDL cholesterol levels. Standardization against a control group of 171 healthy children was applied to eliminate the effect of development. Clinical applicability and cut-off value were evaluated in all available patients with GCK-MODY (n = 148), hepatocyte nuclear factor 1-alpha-MODY (HNF1A MODY) (n = 37) or type 1 diabetes (n = 221). Lower lipid parameter values were observed in GCK-MODY than in patients with type 1 diabetes. Standard deviation scores were -0·22 ± 2·24 vs 1·31 ± 2·17 for HDL cholesterol (P MODY selection [sensitivity 87%, specificity 54%, negative predictive value (NPV) 86%, positive PV 56%]. A threshold HDL concentration of 1·56 mm offered significantly better diagnostic efficiency than total cholesterol (cut-off value 4·51 mm; NPV 80%; PPV 38%; P MODY and differentiation from T1DM and HNF1A-MODY, regardless of treatment or metabolic control. © 2011 Blackwell Publishing Ltd.

  9. Effects of Cooling Fluid Flow Rate on the Critical Heat Flux and Flow Stability in the Plate Fuel Type 2 MW TRIGA Reactor

    OpenAIRE

    H. P. Rahardjo; V. I. Sri Wardhani

    2017-01-01

    The conversion program of the 2 MW TRIGA reactor in Bandung consisted of the replacement of cylindrical fuel (produced by General Atomic) with plate fuel (produced by BATAN). The replacement led into the change of core cooling process from upward natural convection type to downward forced convection type, and resulted in different thermohydraulic safety criteria, such as critical heat flux (CHF) limit, boiling limit, and cooling fluid flow stability. In this paper, a thermohydraulic safety an...

  10. A single dose of dapagliflozin, an SGLT-2 inhibitor, induces higher glycosuria in GCK- and HNF1A-MODY than in type 2 diabetes mellitus.

    Science.gov (United States)

    Hohendorff, J; Szopa, M; Skupien, J; Kapusta, M; Zapala, B; Platek, T; Mrozinska, S; Parpan, T; Glodzik, W; Ludwig-Galezowska, A; Kiec-Wilk, B; Klupa, T; Malecki, M T

    2017-08-01

    SGLT2 inhibitors are a new class of oral hypoglycemic agents used in type 2 diabetes (T2DM). Their effectiveness in maturity onset diabetes of the young (MODY) is unknown. We aimed to assess the response to a single dose of 10 mg dapagliflozin in patients with Hepatocyte Nuclear Factor 1 Alpha (HNF1A)-MODY, Glucokinase (GCK)-MODY, and type 2 diabetes. We examined 14 HNF1A-MODY, 19 GCK-MODY, and 12 type 2 diabetes patients. All studied individuals received a single morning dose of 10 mg of dapagliflozin added to their current therapy of diabetes. To assess the response to dapagliflozin we analyzed change in urinary glucose to creatinine ratio and serum 1,5-Anhydroglucitol (1,5-AG) level. There were only four patients with positive urine glucose before dapagliflozin administration (one with HNF1A-MODY, two with GCK-MODY, and one with T2DM), whereas after SGLT-2 inhibitor use, glycosuria occurred in all studied participants. Considerable changes in mean glucose to creatinine ratio after dapagliflozin administration were observed in all three groups (20.51 ± 12.08, 23.19 ± 8.10, and 9.84 ± 6.68 mmol/mmol for HNF1A-MODY, GCK-MODY, and T2DM, respectively, p MODY, respectively), but not between the two MODY forms (p = 0.7231). Significant change in serum 1,5-AG was noticed only in T2DM and it was -6.57 ± 7.34 mg/ml (p = 0.04). A single dose of dapagliflozin, an SGLT-2 inhibitor, induces higher glycosuria in GCK- and HNF1A-MODY than in T2DM. Whether flozins are a valid therapeutic option in these forms of MODY requires long-term clinical studies.

  11. Perceived functioning and disability in adults with myotonic dystrophy type 1: a survey according to the International Classification Of Functioning, Disability and Health.

    Science.gov (United States)

    Kierkegaard, Marie; Harms-Ringdahl, Karin; Widén Holmqvist, Lotta; Tollbäck, Anna

    2009-06-01

    The purpose of this study was to describe and analyse self-rated perceived functioning, disability and environmental facilitators/barriers with regard to disease severity, using the International Classification of Functioning, Disability and Health (ICF) checklist, in adults with myotonic dystrophy type 1. Cross-sectional design. Forty-one women and 29 men with myotonic dystrophy type 1. A modified ICF checklist was used for self-rating of perceived problems in 29 body-function categories, difficulties in 52 activity and participation categories, and facilitators/barriers in 23 environmental-factor categories according to the verbal anchors of the ICF qualifiers. Disease severity classification was based on the muscular impairment rating scale. Of the persons with myotonic dystrophy type 1, 80% perceived problems of excessive daytime sleepiness, 76% of muscle power, and 66% of energy and drive functions, while over 59% perceived difficulties in physically demanding mobility activities. Disabilities in mobility, self-care and domestic life were more frequently reported by persons with severe disease. Support from the immediate family, medicines and social security services were perceived as facilitators for 50-60% of the participants. Disabilities and important environmental facilitators in adults with myotonic dystrophy type 1 were identified, and this clinically-relevant information can be used for developing health services for people with this condition.

  12. Comparison of Glomerular Filtration Rate Estimation from Serum Creatinine and Cystatin C in HNF1A-MODY and Other Types of Diabetes.

    Science.gov (United States)

    Szopa, Magdalena; Kapusta, Maria; Matejko, Bartlomiej; Klupa, Tomasz; Koblik, Teresa; Kiec-Wilk, Beata; Borowiec, Maciej; Malecki, Maciej T

    2015-01-01

    We previously showed that in HNF1A-MODY the cystatin C-based glomerular filtration rate (GFR) estimate is higher than the creatinine-based estimate. Currently, we aimed to replicate this finding and verify its clinical significance. The study included 72 patients with HNF1A-MODY, 72 with GCK-MODY, 53 with type 1 diabetes (T1DM), 70 with type 2 diabetes (T2DM), and 65 controls. Serum creatinine and cystatin C levels were measured. GFR was calculated from creatinine and cystatin C using the CKD-EPI creatinine equation (eGRF-cr) and CKD-EPI cystatin C equation (eGFR-cys), respectively. Cystatin C levels were lower (p MODY, GCK-MODY, and the controls (p = 0.004; p = 0.003; p MODY patients are higher compared to eGFR-cr. Some other differences were also described in diabetic groups. However, none of them appears to be clinically relevant.

  13. Differential expression of P-type ATPases in intestinal epithelial cells: Identification of putative new atp1a1 splice-variant

    Energy Technology Data Exchange (ETDEWEB)

    Rocafull, Miguel A., E-mail: mrocaful@ivic.ve [Lab. Fisiologia Molecular, Centro de Biofisica y Bioquimica, Instituto Venezolano de Investigaciones Cientificas (IVIC), Apartado 20632, Caracas 1020-A (Venezuela, Bolivarian Republic of); Thomas, Luz E.; Barrera, Girolamo J.; Castillo, Jesus R. del [Lab. Fisiologia Molecular, Centro de Biofisica y Bioquimica, Instituto Venezolano de Investigaciones Cientificas (IVIC), Apartado 20632, Caracas 1020-A (Venezuela, Bolivarian Republic of)

    2010-01-01

    P-type ATPases are membrane proteins that couple ATP hydrolysis with cation transport across the membrane. Ten different subtypes have been described. In mammalia, 15 genes of P-type ATPases from subtypes II-A, II-B and II-C, that transport low-atomic-weight cations (Ca{sup 2+}, Na{sup +}, K{sup +} and H{sup +}), have been reported. They include reticulum and plasma-membrane Ca{sup 2+}-ATPases, Na{sup +}/K{sup +}-ATPase and H{sup +}/K{sup +}-ATPases. Enterocytes and colonocytes show functional differences, which seem to be partially due to the differential expression of P-type ATPases. These enzymes have 9 structural motifs, being the phosphorylation (E) and the Mg{sup 2+}ATP-binding (H) motifs the most preserved. These structural characteristics permitted developing a Multiplex-Nested-PCR (MN-PCR) for the simultaneous identification of different P-type ATPases. Thus, using MN-PCR, seven different cDNAs were cloned from enterocytes and colonocytes, including SERCA3, SERCA2, Na{sup +}/K{sup +}-ATPase {alpha}1-isoform, H{sup +}/K{sup +}-ATPase {alpha}2-isoform, PMCA1, PMCA4 and a cDNA-fragment that seems to be a new cassette-type splice-variant of the atp1a1 gen. PMCA4 in enterocytes and H{sup +}/K{sup +}-ATPase {alpha}2-isoform in colonocytes were differentially expressed. This cell-specific expression pattern is related with the distinctive enterocyte and colonocyte functions.

  14. Comparison of Glomerular Filtration Rate Estimation from Serum Creatinine and Cystatin C in HNF1A-MODY and Other Types of Diabetes

    Directory of Open Access Journals (Sweden)

    Magdalena Szopa

    2015-01-01

    Full Text Available Introduction. We previously showed that in HNF1A-MODY the cystatin C-based glomerular filtration rate (GFR estimate is higher than the creatinine-based estimate. Currently, we aimed to replicate this finding and verify its clinical significance. Methods. The study included 72 patients with HNF1A-MODY, 72 with GCK-MODY, 53 with type 1 diabetes (T1DM, 70 with type 2 diabetes (T2DM, and 65 controls. Serum creatinine and cystatin C levels were measured. GFR was calculated from creatinine and cystatin C using the CKD-EPI creatinine equation (eGRF-cr and CKD-EPI cystatin C equation (eGFR-cys, respectively. Results. Cystatin C levels were lower (p<0.001 in the control (0.70±0.13 mg/L, HNF1A (0.75±0.21, and GCK (0.72±0.16 mg/L groups in comparison to those with either T1DM (0.87±0.15 mg/L or T2DM (0.9±0.23 mg/L. Moreover, eGFR-cys was higher than eGRF-cr in HNF1A-MODY, GCK-MODY, and the controls (p=0.004; p=0.003; p<0.0001. This corresponded to 8.9 mL/min/1.73 m2, 9.7 mL/min/1.73 m2, and 16.9 mL/min/1.73 m2 of difference. Additionally, T1DM patients had higher eGFR-cr than eGFR-cys (11.6 mL/min/1.73 m2; p=0.0004; no difference occurred in T2DM (p=0.91. Conclusions. We confirmed that eGFR-cys values in HNF1A-MODY patients are higher compared to eGFR-cr. Some other differences were also described in diabetic groups. However, none of them appears to be clinically relevant.

  15. Live Cell Imaging and 3D Analysis of Angiotensin Receptor Type 1a Trafficking in Transfected Human Embryonic Kidney Cells Using Confocal Microscopy.

    Science.gov (United States)

    Kadam, Parnika; McAllister, Ryan; Urbach, Jeffrey S; Sandberg, Kathryn; Mueller, Susette C

    2017-03-27

    Live-cell imaging is used to simultaneously capture time-lapse images of angiotensin type 1a receptors (AT1aR) and intracellular compartments in transfected human embryonic kidney-293 (HEK) cells following stimulation with angiotensin II (Ang II). HEK cells are transiently transfected with plasmid DNA containing AT1aR tagged with enhanced green fluorescent protein (EGFP). Lysosomes are identified with a red fluorescent dye. Live-cell images are captured on a laser scanning confocal microscope after Ang II stimulation and analyzed by software in three dimensions (3D, voxels) over time. Live-cell imaging enables investigations into receptor trafficking and avoids confounds associated with fixation, and in particular, the loss or artefactual displacement of EGFP-tagged membrane receptors. Thus, as individual cells are tracked through time, the subcellular localization of receptors can be imaged and measured. Images must be acquired sufficiently rapidly to capture rapid vesicle movement. Yet, at faster imaging speeds, the number of photons collected is reduced. Compromises must also be made in the selection of imaging parameters like voxel size in order to gain imaging speed. Significant applications of live-cell imaging are to study protein trafficking, migration, proliferation, cell cycle, apoptosis, autophagy and protein-protein interaction and dynamics, to name but a few.

  16. Bisphosphonates for treatment of Complex Regional Pain Syndrome type 1: A systematic literature review and meta-analysis of randomized controlled trials versus placebo.

    Science.gov (United States)

    Chevreau, Maxime; Romand, Xavier; Gaudin, Philippe; Juvin, Robert; Baillet, Athan

    2017-07-01

    Complex Regional Pain Syndrome Type 1 is a severely disabling pain syndrome with no definite established treatment. We have performed a systematic literature review and meta-analysis of all randomized controlled trials to assess the benefit of bisphosphonates on pain and function in patients with Complex Regional Pain Syndrome Type 1. A systematic literature search was performed in the Medline, Embase and Cochrane databases. Two authors selected independently blinded randomized trials comparing bisphosphonates to placebo on short-term (J30 to J40) and medium term pain (M2-M3), safety and function in patients with CRPS 1. The methodological quality of the studies was analyzed. Data were aggregated using the method of the inverse of the variance. 258 articles were identified. Four trials of moderate to good quality comprising 181 patients (90 in the bisphosphonate group and 91 in the placebo group) were included in this meta-analysis. Short-term pain Visual Analog Scale was significantly lower in the bisphosphonate group versus the placebo group (SMD=-2.6, 95%CI [-1.8, -3.4], Ppain (SMD=-2.5, 95%CI [-1.4, -3.6], Ppain in patients with Complex Regional Pain Syndrome type 1. Other studies are needed to determine their effectiveness. Copyright © 2017. Published by Elsevier SAS.

  17. Criticality Model

    International Nuclear Information System (INIS)

    Alsaed, A.

    2004-01-01

    The ''Disposal Criticality Analysis Methodology Topical Report'' (YMP 2003) presents the methodology for evaluating potential criticality situations in the monitored geologic repository. As stated in the referenced Topical Report, the detailed methodology for performing the disposal criticality analyses will be documented in model reports. Many of the models developed in support of the Topical Report differ from the definition of models as given in the Office of Civilian Radioactive Waste Management procedure AP-SIII.10Q, ''Models'', in that they are procedural, rather than mathematical. These model reports document the detailed methodology necessary to implement the approach presented in the Disposal Criticality Analysis Methodology Topical Report and provide calculations utilizing the methodology. Thus, the governing procedure for this type of report is AP-3.12Q, ''Design Calculations and Analyses''. The ''Criticality Model'' is of this latter type, providing a process evaluating the criticality potential of in-package and external configurations. The purpose of this analysis is to layout the process for calculating the criticality potential for various in-package and external configurations and to calculate lower-bound tolerance limit (LBTL) values and determine range of applicability (ROA) parameters. The LBTL calculations and the ROA determinations are performed using selected benchmark experiments that are applicable to various waste forms and various in-package and external configurations. The waste forms considered in this calculation are pressurized water reactor (PWR), boiling water reactor (BWR), Fast Flux Test Facility (FFTF), Training Research Isotope General Atomic (TRIGA), Enrico Fermi, Shippingport pressurized water reactor, Shippingport light water breeder reactor (LWBR), N-Reactor, Melt and Dilute, and Fort Saint Vrain Reactor spent nuclear fuel (SNF). The scope of this analysis is to document the criticality computational method. The criticality

  18. Low frequency variants in the exons only encoding isoform A of HNF1A do not contribute to susceptibility to type 2 diabetes.

    Directory of Open Access Journals (Sweden)

    Bahram Jafar-Mohammadi

    2009-08-01

    Full Text Available There is considerable interest in the hypothesis that low frequency, intermediate penetrance variants contribute to the proportion of Type 2 Diabetes (T2D susceptibility not attributable to the common variants uncovered through genome-wide association approaches. Genes previously implicated in monogenic and multifactorial forms of diabetes are obvious candidates in this respect. In this study, we focussed on exons 8-10 of the HNF1A gene since rare, penetrant mutations in these exons (which are only transcribed in selected HNF1A isoforms are associated with a later age of diagnosis of Maturity onset diabetes of the young (MODY than mutations in exons 1-7. The age of diagnosis in the subgroup of HNF1A-MODY individuals with exon 8-10 mutations overlaps with that of early multifactorial T2D, and we set out to test the hypothesis that these exons might also harbour low-frequency coding variants of intermediate penetrance that contribute to risk of multifactorial T2D.We performed targeted capillary resequencing of HNF1A exons 8-10 in 591 European T2D subjects enriched for genetic aetiology on the basis of an early age of diagnosis ( or =1 affected sibling. PCR products were sequenced and compared to the published HNF1A sequence. We identified several variants (rs735396 [IVS9-24T>C], rs1169304 [IVS8+29T>C], c.1768+44C>T [IVS9+44C>T] and rs61953349 [c.1545G>A, p.T515T] but no novel non-synonymous coding variants were detected.We conclude that low frequency, nonsynonymous coding variants in the terminal exons of HNF1A are unlikely to contribute to T2D-susceptibility in European samples. Nevertheless, the rationale for seeking low-frequency causal variants in genes known to contain rare, penetrant mutations remains strong and should motivate efforts to screen other genes in a similar fashion.

  19. Critical phosphorus loading of different types of shallow lakes and the consequences for management estimated with the ecosystem model PCLake

    NARCIS (Netherlands)

    Janse, J.H.; De Senerpont Domis, L.N.; Scheffer, M.; Lijklema, L.; Klinge, M.; Mooij, W.M.; Van Liere, L.

    2008-01-01

    Shallow lakes typically can be in one of two contrasting states: a clear state with submerged macrophytes or a turbid state dominated by phytoplankton. Eutrophication may cause a switch from the clear to the turbid state, if the phosphorus loading exceeds a critical value. Recovery of the clear

  20. The critical soil P levels for crop yield, soil fertility and environmental safety in different soil types

    NARCIS (Netherlands)

    Bai, Z.H.; Li, H.G.; Yang, X.Y.; Zhou, B.K.; Shi, X.J.; Wang, B.R.; Li, D.C.; Shen, J.B.; Chen, Q.; Qin, W.; Oenema, O.; Zhang, F.S.

    2013-01-01

    Sufficient soil phosphorus (P) is important for achieving optimal crop production, but excessive soil P levels may create a risk of P losses and associated eutrophication of surface waters. The aim of this study was to determine critical soil P levels for achieving optimal crop yields and minimal P

  1. A Novel CACNA1A Nonsense Variant [c.4054C>T (p.Arg1352⁎] Causing Episodic Ataxia Type 2

    Directory of Open Access Journals (Sweden)

    Sean Lance

    2018-01-01

    Full Text Available Episodic ataxia is a heterogenous group of uncommon neurological disorders characterised by recurrent episodes of vertigo, dysarthria, and ataxia for which a variety of different genetic variations have been implicated. Episodic ataxia type two (EA2 is the most common and also has the largest number of identified causative genetic variants. Treatment with acetazolamide is effective in improving symptoms, so accurate diagnosis is essential. However, a large proportion of patients with EA2 have negative genetic testing. We present a patient with a typical history of EA2 who had a novel variant in the CACNA1A gene not previously described. Report of such variations is important in learning more about the disease and improving diagnostic yield for the patient.

  2. A Novel CACNA1A Nonsense Variant [c.4054C>T (p.Arg1352⁎)] Causing Episodic Ataxia Type 2.

    Science.gov (United States)

    Lance, Sean; Mossman, Stuart; Poke, Gemma

    2018-01-01

    Episodic ataxia is a heterogenous group of uncommon neurological disorders characterised by recurrent episodes of vertigo, dysarthria, and ataxia for which a variety of different genetic variations have been implicated. Episodic ataxia type two (EA2) is the most common and also has the largest number of identified causative genetic variants. Treatment with acetazolamide is effective in improving symptoms, so accurate diagnosis is essential. However, a large proportion of patients with EA2 have negative genetic testing. We present a patient with a typical history of EA2 who had a novel variant in the CACNA1A gene not previously described. Report of such variations is important in learning more about the disease and improving diagnostic yield for the patient.

  3. SIRT1 deacetylates RFX5 and antagonizes repression of collagen type I (COL1A2) transcription in smooth muscle cells

    Energy Technology Data Exchange (ETDEWEB)

    Xia, Jun [Department of Respiratory Medicine, The First Affiliated Hospital of Nanjing Medical University (China); Department of Respiratory Medicine, Jiangsu Provincial Hospital of Chinese Traditional Medicine (China); Wu, Xiaoyan; Yang, Yuyu; Zhao, Yuhao [Atherosclerosis Research Center, Key Laboratory of Cardiovascular Disease and Molecular Intervention, Department of Pathophysiology, Nanjing Medical University (China); Fang, Mingming [Jiangsu Jiankang Vocational Institute (China); Xie, Weiping, E-mail: wpxienjmu@gmail.com [Department of Respiratory Medicine, The First Affiliated Hospital of Nanjing Medical University (China); Wang, Hong, E-mail: hwangnjmu@gmail.com [Department of Respiratory Medicine, The First Affiliated Hospital of Nanjing Medical University (China); Xu, Yong [Atherosclerosis Research Center, Key Laboratory of Cardiovascular Disease and Molecular Intervention, Department of Pathophysiology, Nanjing Medical University (China)

    2012-11-16

    Highlights: Black-Right-Pointing-Pointer SIRT1 interacts with and deacetylates RFX5. Black-Right-Pointing-Pointer SIRT1 activation attenuates whereas SIRT1 inhibition enhances collagen repression by RFX5 in vascular smooth muscle cells. Black-Right-Pointing-Pointer SIRT1 promotes cytoplasmic localization and proteasomal degradation of RFX5 and cripples promoter recruitment of RFX5. Black-Right-Pointing-Pointer IFN-{gamma} represses SIRT1 expression in vascular smooth muscle cells. Black-Right-Pointing-Pointer SIRT1 agonist alleviates collagen repression by IFN-{gamma} in vascular smooth muscle cells. -- Abstract: Decreased expression of collagen by vascular smooth muscle cells (SMCs) within the atherosclerotic plaque contributes to the thinning of the fibrous cap and poses a great threat to plaque rupture. Elucidation of the mechanism underlying repressed collagen type I (COL1A2) gene would potentially provide novel solutions that can prevent rupture-induced complications. We have previously shown that regulatory factor for X-box (RFX5) binds to the COL1A2 transcription start site and represses its transcription. Here we report that SIRT1, an NAD-dependent, class III deacetylase, forms a complex with RFX5. Over-expression of SIRT1 or NAMPT, which synthesizes NAD+ to activate SIRT1, or treatment with the SIRT1 agonist resveratrol decreases RFX5 acetylation and disrupts repression of the COL1A2 promoter activity by RFX5. On the contrary, knockdown of SIRT1 or treatment with SIRT1 inhibitors induces RFX5 acetylation and enhances the repression of collagen transcription. SIRT1 antagonizes RFX5 activity by promoting its nuclear expulsion and proteasomal degradation hence dampening its binding to the COL1A2 promoter. The pro-inflammatory cytokine IFN-{gamma} represses COL1A2 transcription by down-regulating SIRT1 expression in SMCs. Therefore, our data have identified as novel pathway whereby SIRT1 maintains collagen synthesis in SMCs by modulating RFX5 activity.

  4. Identification of a melatonin receptor type 1A gene ( AccMTNR1A) in Apis cerana cerana and its possible involvement in the response to low temperature stress

    Science.gov (United States)

    Li, Guilin; Zhang, Yanming; Ni, Yong; Wang, Ying; Xu, Baohua; Guo, Xingqi

    2018-04-01

    It is known that melatonin plays an indispensable role in the defense against some environment-induced stresses. The melatonin receptor (MTNR) is also closely linked to the environmental stress response in mammals. However, little is known about the function of the MTNR in insects, including honeybees. In this study, we identified a MTNR from Apis cerana cerana named AccMTNR1A, which contained a typical seven-transmembrane domain common to this family of receptors. A subcellular localization analysis showed that AccMTNR1A was localized in the cytomembrane. Additionally, we found that cold stress apparently boosted AccMTNR1A transcription, indicating that AccMTNR1A possibly connects to the cold stress response. The knockdown of AccMTNR1A attenuated the expression level of some genes associated with the cold stress response, suggesting that AccMTNR1A likely plays an analogous role with these genes during low temperature stress response. Moreover, silencing of AccMTNR1A also suppressed the transcription of some antioxidant genes, prompting the possibility that the response of AccMTNR1A to cold stress response may be related to antioxidant signaling pathways. Collectively, the findings presented here provide evidence that AccMTNR1A may play essential roles in protecting Apis cerana cerana from cold stress.

  5. Protein Kinase A (PKA) Type I Interacts with P-Rex1, a Rac Guanine Nucleotide Exchange Factor: EFFECT ON PKA LOCALIZATION AND P-Rex1 SIGNALING.

    Science.gov (United States)

    Chávez-Vargas, Lydia; Adame-García, Sendi Rafael; Cervantes-Villagrana, Rodolfo Daniel; Castillo-Kauil, Alejandro; Bruystens, Jessica G H; Fukuhara, Shigetomo; Taylor, Susan S; Mochizuki, Naoki; Reyes-Cruz, Guadalupe; Vázquez-Prado, José

    2016-03-18

    Morphology of migrating cells is regulated by Rho GTPases and fine-tuned by protein interactions and phosphorylation. PKA affects cell migration potentially through spatiotemporal interactions with regulators of Rho GTPases. Here we show that the endogenous regulatory (R) subunit of type I PKA interacts with P-Rex1, a Rac guanine nucleotide exchange factor that integrates chemotactic signals. Type I PKA holoenzyme interacts with P-Rex1 PDZ domains via the CNB B domain of RIα, which when expressed by itself facilitates endothelial cell migration. P-Rex1 activation localizes PKA to the cell periphery, whereas stimulation of PKA phosphorylates P-Rex1 and prevents its activation in cells responding to SDF-1 (stromal cell-derived factor 1). The P-Rex1 DEP1 domain is phosphorylated at Ser-436, which inhibits the DH-PH catalytic cassette by direct interaction. In addition, the P-Rex1 C terminus is indirectly targeted by PKA, promoting inhibitory interactions independently of the DEP1-PDZ2 region. A P-Rex1 S436A mutant construct shows increased RacGEF activity and prevents the inhibitory effect of forskolin on sphingosine 1-phosphate-dependent endothelial cell migration. Altogether, these results support the idea that P-Rex1 contributes to the spatiotemporal localization of type I PKA, which tightly regulates this guanine exchange factor by a multistep mechanism, initiated by interaction with the PDZ domains of P-Rex1 followed by direct phosphorylation at the first DEP domain and putatively indirect regulation of the C terminus, thus promoting inhibitory intramolecular interactions. This reciprocal regulation between PKA and P-Rex1 might represent a key node of integration by which chemotactic signaling is fine-tuned by PKA. © 2016 by The American Society for Biochemistry and Molecular Biology, Inc.

  6. Critical current, pinning and resistive state of superconducting single-crystal niobium with different types of defect structure

    International Nuclear Information System (INIS)

    Sokolenko, V.I.; Starodubov, Ya.D.

    2005-01-01

    Critical current pinning and resistive state of single crystal niobium of texture orientation are studied for different structural states obtained by rolling at 20 K by 42% and polishing the surface layers. It is found that the heterogeneous structures typical of the strained sample even after its thinning down to approx 10% display a lower current-carrying capability due to an increase of the thermomagnetic instability within the fragmented structure sections in the near-surface layers. For a homogeneous defect structure of the sample core with the density of equilibrium distributed dislocations of 1.3 centre dot 10 11 cm -2 , a correlation between the normal current density and the critical current density in the resistive state is found, in agreement with the concepts of flux creep due to the scatter of local values of J c

  7. Critical Conditions for Liquid Chromatography of Statistical Copolymers: Functionality Type and Composition Distribution Characterization by UP-LCCC/ESI-MS.

    Science.gov (United States)

    Epping, Ruben; Panne, Ulrich; Falkenhagen, Jana

    2017-02-07

    Statistical ethylene oxide (EO) and propylene oxide (PO) copolymers of different monomer compositions and different average molar masses additionally containing two kinds of end groups (FTD) were investigated by ultra high pressure liquid chromatography under critical conditions (UP-LCCC) combined with electrospray ionization time-of flight mass spectrometry (ESI-TOF-MS). Theoretical predictions of the existence of a critical adsorption point (CPA) for statistical copolymers with a given chemical and sequence distribution1 could be studied and confirmed. A fundamentally new approach to determine these critical conditions in a copolymer, alongside the inevitable chemical composition distribution (CCD), with mass spectrometric detection, is described. The shift of the critical eluent composition with the monomer composition of the polymers was determined. Due to the broad molar mass distribution (MMD) and the presumed existence of different end group functionalities as well as monomer sequence distribution (MSD), gradient separation only by CCD was not possible. Therefore, isocratic separation conditions at the CPA of definite CCD fractions were developed. Although the various present distributions partly superimposed the separation process, the goal of separation by end group functionality was still achieved on the basis of the additional dimension of ESI-TOF-MS. The existence of HO-H besides the desired allylO-H end group functionalities was confirmed and their amount estimated. Furthermore, indications for a MSD were found by UPLC/MS/MS measurements. This approach offers for the first time the possibility to obtain a fingerprint of a broad distributed statistical copolymer including MMD, FTD, CCD, and MSD.

  8. Eventual sport performance level: What about the role of type of sport, perception of critical life events, and practice quality?

    Science.gov (United States)

    Toering, Tynke

    2017-01-01

    The target article describes an interesting study, which provides some challenging findings regarding athletes' pathway to excellence. The suggested links between critical life events, need for success, personal characteristics, and eventual performance level make sense from a psychodynamic perspective. This commentary will discuss some critical points related to the application of the findings in talent identification and -development programs. These are (1) the possible effect of the selection of participants on the results (including the impact of dependence on others for team sport athletes and the opportunity for multiple medal attainment), (2) a lack of detail in the description of how the athletes approached the critical life events (the perception of an event may contribute more to development than the event itself), and (3) a lack of detail in the description of the practice process throughout development. Some interesting differences were found in the motivation for and approach to practice, but little detail was given about what the athletes exactly were doing at the time. The concept of self-regulated learning may be useful in explaining how super-elite athletes action their goals through quality practice. © 2017 Elsevier B.V. All rights reserved.

  9. Criticality safety

    International Nuclear Information System (INIS)

    Walker, G.

    1983-01-01

    When a sufficient quantity of fissile material is brought together a self-sustaining neutron chain reaction will be started in it and will continue until some change occurs in the fissile material to stop the chain reaction. The quantity of fissile material required is the 'Critical Mass'. This is not a fixed quantity even for a given type of fissile material but varies between quite wide limits depending on a number of factors. In a nuclear reactor the critical mass of fissile material is assembled under well-defined condition to produce a controllable chain reaction. The same materials have to be handled outside the reactor in all stages of fuel element manufacture, storage, transport and irradiated fuel reprocessing. At any stage it is possible (at least in principle) to assemble a critical mass and thus initiate an accidental and uncontrollable chain reaction. Avoiding this is what criticality safety is all about. A system is just critical when the rate of production of neutrons balances the rate of loss either by escape or by absorption. The factors affecting criticality are, therefore, those which effect neutron production and loss. The principal ones are:- type of nuclide and enrichment (or isotopic composition), moderation, reflection, concentration (density), shape and interaction. Each factor is considered in detail. (author)

  10. A study of critical heat flux in the fuel assembly dummies with various types of mixing grids

    International Nuclear Information System (INIS)

    Bezrukov, Yu. A.; Lisenkov, E. A.; Astakhov, V. I.; Vasilchenko, I. N.

    2013-01-01

    The report deals with the results of a study The report deals with the results of a study of critical heat fluxes (CHF) on the rod bundles equipped with mixing grids – intensifier of heat exchange. The study was carried out on FA dummies equipped with various kinds of mixing grids: “Vikhr” and “Sector screw die”. The tests were carried out at the following parameters: pressure 14 and 16 MPa; mass velocity 2000, 3000 and 4000 kg/(m2⋅s); relative enthalpy from minus 0,07 up to 0,18. Bundles with uniform and non-uniform axial heat generation were tested. The results of the experiments are intended for the elaboration of a correlation to be used in design development.of critical heat fluxes (CHF) on the rod bundles equipped with mixing grids – intensifier of heat exchange. The study was carried out on FA dummies equipped with various kinds of mixing grids: “Vikhr” and “Sector screw die”. The tests were carried out at the following parameters: pressure 14 and 16 MPa; mass velocity 2000, 3000 and 4000 kg/(m 2 ⋅s); relative enthalpy from minus 0,07 up to 0,18. Bundles with uniform and non-uniform axial heat generation were tested. The results of the experiments are intended for the elaboration of a correlation to be used in design development. (authors)

  11. The first critical experiment with a new type of fuel assemblies IRT-3M on the training reactor VR-I

    International Nuclear Information System (INIS)

    Matejka, Karel; Sklenka, Lubomir

    1997-01-01

    The paper 'The first critical experiment with a new type of fuel assemblies IRT-3M on training reactor VR-1 presents basic information about the replacement of fuel on the reactor VR-1 run on FJFI CVUT in Prague. In spring 1997 the IRT-2M fuel type used till then was replaced by the IRT-3M type. When the fuel was replaced, no change in its enrichment was made, i.e. its level remained as 36% 235 U. The replacement itself was carried out in tight co-operation with the Nuclear Research Institute Rez plc., as related to the operation of the research reactor LVR-15. The fuel replacement on the VR-I reactor is a part of the international program RERTR (Reduced Enrichment for Research and Test Reactors) in which the Czech Republic participates. (author)

  12. Promoter Variation and Expression Levels of Inflammatory Genes IL1A, IL1B, IL6 and TNF in Blood of Spinocerebellar Ataxia Type 3 (SCA3) Patients.

    Science.gov (United States)

    Raposo, Mafalda; Bettencourt, Conceição; Ramos, Amanda; Kazachkova, Nadiya; Vasconcelos, João; Kay, Teresa; Bruges-Armas, Jácome; Lima, Manuela

    2017-03-01

    Age at onset in spinocerebellar ataxia type 3 (SCA3/MJD) is incompletely explained by the size of the CAG tract at the ATXN3 gene, implying the existence of genetic modifiers. A role of inflammation in SCA3 has been postulated, involving altered cytokines levels; promoter variants leading to alterations in cytokines expression could influence onset. Using blood from 86 SCA3 patients and 106 controls, this work aimed to analyse promoter variation of four cytokines (IL1A, IL1B, IL6 and TNF) and to investigate the association between variants detected and their transcript levels, evaluated by quantitative PCR. Moreover, the effect of APOE isoforms, known to modulate cytokines, was investigated. Correlations between cytokine variants and onset were tested; the cumulative modifier effects of cytokines and APOE were analysed. Patients carrying the IL6*C allele had a significant earlier onset (4 years in average) than patients carrying the G allele, in agreement with lower mRNA levels produced by IL6*C carriers. The presence of APOE*ɛ2 allele seems to anticipate onset in average 10 years in patients carrying the IL6*C allele; a larger number of patients will be needed to confirm this result. These results highlight the pertinence of conducting further research on the role of cytokines as SCA3 modulators, pointing to the presence of shared mechanisms involving IL6 and APOE.

  13. In vivo hepatic lipid quantification using MRS at 7 Tesla in a mouse model of glycogen storage disease type 1a.

    Science.gov (United States)

    Ramamonjisoa, Nirilanto; Ratiney, Helene; Mutel, Elodie; Guillou, Herve; Mithieux, Gilles; Pilleul, Frank; Rajas, Fabienne; Beuf, Olivier; Cavassila, Sophie

    2013-07-01

    The assessment of liver lipid content and composition is needed in preclinical research to investigate steatosis and steatosis-related disorders. The purpose of this study was to quantify in vivo hepatic fatty acid content and composition using a method based on short echo time proton magnetic resonance spectroscopy (MRS) at 7 Tesla. A mouse model of glycogen storage disease type 1a with inducible liver-specific deletion of the glucose-6-phosphatase gene (L-G6pc(-/-)) mice and control mice were fed a standard diet or a high-fat/high-sucrose (HF/HS) diet for 9 months. In control mice, hepatic lipid content was found significantly higher with the HF/HS diet than with the standard diet. As expected, hepatic lipid content was already elevated in L-G6pc(-/-) mice fed a standard diet compared with control mice. L-G6pc(-/-) mice rapidly developed steatosis which was not modified by the HF/HS diet. On the standard diet, estimated amplitudes from olefinic protons were found significantly higher in L-G6pc(-/-) mice compared with that in control mice. L-G6pc(-/-) mice showed no noticeable polyunsaturation from diallylic protons. Total unsaturated fatty acid indexes measured by gas chromatography were in agreement with MRS measurements. These results showed the great potential of high magnetic field MRS to follow the diet impact and lipid alterations in mouse liver.

  14. SLAH1, a homologue of the slow type anion channel SLAC1, modulates shoot Cl − accumulation and salt tolerance in Arabidopsis thaliana

    KAUST Repository

    Qiu, Jiaen; Henderson, Sam W; Tester, Mark A.; Roy, Stuart J; Gilliham, Mathew

    2016-01-01

    Salinity tolerance is correlated with shoot chloride (Cl–) exclusion in multiple crops, but the molecular mechanisms of long-distance Cl– transport are poorly defined. Here, we characterize the in planta role of AtSLAH1 (a homologue of the slow type anion channel-associated 1 (SLAC1)). This protein, localized to the plasma membrane of root stelar cells, has its expression reduced by salt or ABA, which are key predictions for a protein involved with loading Cl– into the root xylem. Artificial microRNA knockdown mutants of AtSLAH1 had significantly reduced shoot Cl− accumulation when grown under low Cl–, whereas shoot Cl– increased and the shoot nitrate/chloride ratio decreased following AtSLAH1 constitutive or stelar-specific overexpression when grown in high Cl–. In both sets of overexpression lines a significant reduction in shoot biomass over the null segregants was observed under high Cl– supply, but not low Cl– supply. Further in planta data showed AtSLAH3 overexpression increased the shoot nitrate/chloride ratio, consistent with AtSLAH3 favouring nitrate transport. Heterologous expression of AtSLAH1 in Xenopus laevis oocytes led to no detectible transport, suggesting the need for post-translational modifications for AtSLAH1 to be active. Our in planta data are consistent with AtSLAH1 having a role in controlling root-to-shoot Cl– transport.

  15. Implications of genetic research on the role of the serotonin in depression: emphasis on the serotonin type 1A receptor and the serotonin transporter.

    Science.gov (United States)

    Neumeister, Alexander; Young, Theresa; Stastny, Juergen

    2004-08-01

    Serotonin systems appear to play a key role in the pathophysiology of major depressive disorder. Consequently, ongoing research determines whether serotonin related genes account for the very robust differential behavioral and neural mechanisms that discriminate patients with depression from healthy controls. Serotonin type 1(A) receptors and the serotonin transporters are reduced in depression, and recent genetic research in animals and humans has implicated both in depression. Preclinical studies have utilized a variety of animal models that have been used to explain pathophysiological mechanisms in humans, although it is not clear at all whether these models constitute relevant models for depression in humans. However, data from preclinical studies can generate hypotheses that are tested in humans by combining genetic data with behavioral and physiological challenge paradigms and neuroimaging. These studies will enhance our understanding about combined influences from multiple interacting genes, as well as from environmental factors on brain circuits and their function, and about how these mechanisms may contribute to the pathophysiology of neuropsychiatric disorders.

  16. SLAH1, a homologue of the slow type anion channel SLAC1, modulates shoot Cl − accumulation and salt tolerance in Arabidopsis thaliana

    KAUST Repository

    Qiu, Jiaen

    2016-06-23

    Salinity tolerance is correlated with shoot chloride (Cl–) exclusion in multiple crops, but the molecular mechanisms of long-distance Cl– transport are poorly defined. Here, we characterize the in planta role of AtSLAH1 (a homologue of the slow type anion channel-associated 1 (SLAC1)). This protein, localized to the plasma membrane of root stelar cells, has its expression reduced by salt or ABA, which are key predictions for a protein involved with loading Cl– into the root xylem. Artificial microRNA knockdown mutants of AtSLAH1 had significantly reduced shoot Cl− accumulation when grown under low Cl–, whereas shoot Cl– increased and the shoot nitrate/chloride ratio decreased following AtSLAH1 constitutive or stelar-specific overexpression when grown in high Cl–. In both sets of overexpression lines a significant reduction in shoot biomass over the null segregants was observed under high Cl– supply, but not low Cl– supply. Further in planta data showed AtSLAH3 overexpression increased the shoot nitrate/chloride ratio, consistent with AtSLAH3 favouring nitrate transport. Heterologous expression of AtSLAH1 in Xenopus laevis oocytes led to no detectible transport, suggesting the need for post-translational modifications for AtSLAH1 to be active. Our in planta data are consistent with AtSLAH1 having a role in controlling root-to-shoot Cl– transport.

  17. NpPDR1, a Pleiotropic Drug Resistance-Type ATP-Binding Cassette Transporter from Nicotiana plumbaginifolia, Plays a Major Role in Plant Pathogen Defense1

    Science.gov (United States)

    Stukkens, Yvan; Bultreys, Alain; Grec, Sébastien; Trombik, Tomasz; Vanham, Delphine; Boutry, Marc

    2005-01-01

    Nicotiana plumbaginifolia NpPDR1, a plasma membrane pleiotropic drug resistance-type ATP-binding cassette transporter formerly named NpABC1, has been suggested to transport the diterpene sclareol, an antifungal compound. However, direct evidence for a role of pleiotropic drug resistance transporters in the plant defense is still lacking. In situ immunolocalization and histochemical analysis using the gusA reporter gene showed that NpPDR1 was constitutively expressed in the whole root, in the leaf glandular trichomes, and in the flower petals. However, NpPDR1 expression was induced in the whole leaf following infection with the fungus Botrytis cinerea, and the bacteria Pseudomonas syringae pv tabaci, Pseudomonas fluorescens, and Pseudomonas marginalis pv marginalis, which do not induce a hypersensitive response in N. plumbaginifolia, whereas a weaker response was observed using P. syringae pv syringae, which does induce a hypersensitive response. Induced NpPDR1 expression was more associated with the jasmonic acid than the salicylic acid signaling pathway. These data suggest that NpPDR1 is involved in both constitutive and jasmonic acid-dependent induced defense. Transgenic plants in which NpPDR1 expression was prevented by RNA interference showed increased sensitivity to sclareol and reduced resistance to B. cinerea. These data show that NpPDR1 is involved in pathogen resistance and thus demonstrate a new role for the ATP-binding cassette transporter family. PMID:16126865

  18. NpPDR1, a pleiotropic drug resistance-type ATP-binding cassette transporter from Nicotiana plumbaginifolia, plays a major role in plant pathogen defense.

    Science.gov (United States)

    Stukkens, Yvan; Bultreys, Alain; Grec, Sébastien; Trombik, Tomasz; Vanham, Delphine; Boutry, Marc

    2005-09-01

    Nicotiana plumbaginifolia NpPDR1, a plasma membrane pleiotropic drug resistance-type ATP-binding cassette transporter formerly named NpABC1, has been suggested to transport the diterpene sclareol, an antifungal compound. However, direct evidence for a role of pleiotropic drug resistance transporters in the plant defense is still lacking. In situ immunolocalization and histochemical analysis using the gusA reporter gene showed that NpPDR1 was constitutively expressed in the whole root, in the leaf glandular trichomes, and in the flower petals. However, NpPDR1 expression was induced in the whole leaf following infection with the fungus Botrytis cinerea, and the bacteria Pseudomonas syringae pv tabaci, Pseudomonas fluorescens, and Pseudomonas marginalis pv marginalis, which do not induce a hypersensitive response in N. plumbaginifolia, whereas a weaker response was observed using P. syringae pv syringae, which does induce a hypersensitive response. Induced NpPDR1 expression was more associated with the jasmonic acid than the salicylic acid signaling pathway. These data suggest that NpPDR1 is involved in both constitutive and jasmonic acid-dependent induced defense. Transgenic plants in which NpPDR1 expression was prevented by RNA interference showed increased sensitivity to sclareol and reduced resistance to B. cinerea. These data show that NpPDR1 is involved in pathogen resistance and thus demonstrate a new role for the ATP-binding cassette transporter family.

  19. Efficacy of basal-bolus insulin regimens in the inpatient management of non-critically ill patients with type 2 diabetes

    DEFF Research Database (Denmark)

    Christensen, Merete B.; Gotfredsen, Anders; Nørgaard, Kirsten

    2017-01-01

    Hyperglycemia during hospitalization is associated with increased rates of complications and longer hospital stays. Various insulin regimens are used in the inpatient diabetes management of non-critically ill patients. In this systematic review and meta-analysis, we aimed to assess the efficacy...... with SSI therapy in hospitalized non-critically ill patients with type 2 diabetes. Primary outcome was mean daily blood glucose (BG) during admission. Secondary outcomes were incidence of hypoglycemia and length of hospital stay. Results of included randomized controlled trials (RCT) were pooled and meta......-analysed to provide estimates of the efficacy of BBI therapy. Five RCTs and seven observational studies were included in the review. Meta-analysis of RCTs showed significantly lower mean daily BG with BBI than SSI. Mean difference in daily BG between the two regimens ranged from 14 to 29 mg/dl. BBI therapy...

  20. The growth hormone-insulin-like growth factor axis in glycogen storage disease type 1: evidence of different growth patterns and insulin-like growth factor levels in patients with glycogen storage disease type 1a and 1b.

    Science.gov (United States)

    Melis, Daniela; Pivonello, Rosario; Parenti, Giancarlo; Della Casa, Roberto; Salerno, Mariacarolina; Balivo, Francesca; Piccolo, Pasquale; Di Somma, Carolina; Colao, Annamaria; Andria, Generoso

    2010-04-01

    To investigate the growth hormone (GH)-insulin-like growth factor (IGF) system in patients with glycogen storage disease type 1 (GSD1). This was a prospective, case-control study. Ten patients with GSD1a and 7 patients with GSD1b who were given dietary treatment and 34 sex-, age-, body mass index-, and pubertal stage-matched control subjects entered the study. Auxological parameters were correlated with circulating GH, either at basal or after growth hormone releasing hormone plus arginine test, insulin-like growth factors (IGF-I and IGF-II), and anti-pituitary antibodies (APA). Short stature was detected in 10.0% of patients with GSD1a, 42.9% of patients with GSD1b (P = .02), and none of the control subjects. Serum IGF-I levels were lower in patients with GSD1b (P = .0001). An impaired GH secretion was found in 40% of patients with GSD1a (P = .008), 57.1% of patients with GSD1b (P = .006), and none of the control subjects. Short stature was demonstrated in 3 of 4 patients with GSD1b and GH deficiency. The prevalence of APA was significantly higher in patients with GSD1b than in patients with GSD1a (P = .02) and control subjects (P = .03). The GH response to the provocative test inversely correlated with the presence of APA (P = .003). Compared with levels in control subjects, serum IGF-II and insulin levels were higher in both groups of patients, in whom IGF-II levels directly correlated with height SD scores (P = .003). Patients with GSD1a have an impaired GH secretion associated with reference range serum IGF-I levels and normal stature, whereas in patients with GSD1b, the impaired GH secretion, probably because of the presence of APA, was associated with reduced IGF-I levels and increased prevalence of short stature. The increased IGF-II levels, probably caused by increased insulin levels, in patients with GSD1 are presumably responsible for the improved growth pattern observed in patients receiving strict dietary treatment. Copyright 2010 Mosby, Inc. All

  1. Dryout-type critical heat flux in vertical upward annular flow: effects of entrainment rate, initial entrained fraction and diameter

    Science.gov (United States)

    Wu, Zan; Wadekar, Vishwas; Wang, Chenglong; Sunden, Bengt

    2018-01-01

    This study aims to reveal the effects of liquid entrainment, initial entrained fraction and tube diameter on liquid film dryout in vertical upward annular flow for flow boiling. Entrainment and deposition rates of droplets were included in mass conservation equations to estimate the local liquid film mass flux in annular flow, and the critical vapor quality at dryout conditions. Different entrainment rate correlations were evaluated using flow boiling data of water and organic liquids including n-pentane, iso-octane and R134a. Effect of the initial entrained fraction (IEF) at the churn-to-annular flow transition was also investigated. A transitional Boiling number was proposed to separate the IEF-sensitive region at high Boiling numbers and the IEF-insensitive region at low Boiling numbers. Besides, the diameter effect on dryout vapor quality was studied. The dryout vapor quality increases with decreasing tube diameter. It needs to be pointed out that the dryout characteristics of submillimeter channels might be different because of different mechanisms of dryout, i.e., drying of liquid film underneath long vapor slugs and flow boiling instabilities.

  2. Overexpression of PvPin1, a Bamboo Homolog of PIN1-Type Parvulin 1, Delays Flowering Time in Transgenic Arabidopsis and Rice

    Directory of Open Access Journals (Sweden)

    Zhigang Zheng

    2017-09-01

    Full Text Available Because of the long and unpredictable flowering period in bamboo, the molecular mechanism of bamboo flowering is unclear. Recent study showed that Arabidopsis PIN1-type parvulin 1 (Pin1At is an important floral activator and regulates floral transition by facilitating the cis/trans isomerization of the phosphorylated Ser/Thr residues preceding proline motifs in suppressor of overexpression of CO 1 (SOC1 and agamous-like 24 (AGL24. Whether bamboo has a Pin1 homolog and whether it works in bamboo flowering are still unknown. In this study, we cloned PvPin1, a homolog of Pin1At, from Phyllostachys violascens (Bambusoideae. Bioinformatics analysis showed that PvPin1 is closely related to Pin1-like proteins in monocots. PvPin1 was widely expressed in all tested bamboo tissues, with the highest expression in young leaf and lowest in floral bud. Moreover, PvPin1 expression was high in leaves before bamboo flowering then declined during flower development. Overexpression of PvPin1 significantly delayed flowering time by downregulating SOC1 and AGL24 expression in Arabidopsis under greenhouse conditions and conferred a significantly late flowering phenotype by upregulating OsMADS56 in rice under field conditions. PvPin1 showed subcellular localization in both the nucleus and cytolemma. The 1500-bp sequence of the PvPin1 promoter was cloned, and cis-acting element prediction showed that ABRE and TGACG-motif elements, which responded to abscisic acid (ABA and methyl jasmonate (MeJA, respectively, were characteristic of P. violascens in comparison with Arabidopsis. On promoter activity analysis, exogenous ABA and MeJA could significantly inhibit PvPin1 expression. These findings suggested that PvPin1 may be a repressor in flowering, and its delay of flowering time could be regulated by ABA and MeJA in bamboo.

  3. Overexpression of PvPin1, a Bamboo Homolog of PIN1-Type Parvulin 1, Delays Flowering Time in Transgenic Arabidopsis and Rice.

    Science.gov (United States)

    Zheng, Zhigang; Yang, Xiaoming; Fu, Yaping; Zhu, Longfei; Wei, Hantian; Lin, Xinchun

    2017-01-01

    Because of the long and unpredictable flowering period in bamboo, the molecular mechanism of bamboo flowering is unclear. Recent study showed that Arabidopsis PIN1-type parvulin 1 (Pin1At) is an important floral activator and regulates floral transition by facilitating the cis/trans isomerization of the phosphorylated Ser/Thr residues preceding proline motifs in suppressor of overexpression of CO 1 (SOC1) and agamous-like 24 (AGL24). Whether bamboo has a Pin1 homolog and whether it works in bamboo flowering are still unknown. In this study, we cloned PvPin1 , a homolog of Pin1At , from Phyllostachys violascens (Bambusoideae). Bioinformatics analysis showed that PvPin1 is closely related to Pin1-like proteins in monocots. PvPin1 was widely expressed in all tested bamboo tissues, with the highest expression in young leaf and lowest in floral bud. Moreover, PvPin1 expression was high in leaves before bamboo flowering then declined during flower development. Overexpression of PvPin1 significantly delayed flowering time by downregulating SOC1 and AGL24 expression in Arabidopsis under greenhouse conditions and conferred a significantly late flowering phenotype by upregulating OsMADS56 in rice under field conditions. PvPin1 showed subcellular localization in both the nucleus and cytolemma. The 1500-bp sequence of the PvPin1 promoter was cloned, and cis -acting element prediction showed that ABRE and TGACG-motif elements, which responded to abscisic acid (ABA) and methyl jasmonate (MeJA), respectively, were characteristic of P. violascens in comparison with Arabidopsis . On promoter activity analysis, exogenous ABA and MeJA could significantly inhibit PvPin1 expression. These findings suggested that PvPin1 may be a repressor in flowering, and its delay of flowering time could be regulated by ABA and MeJA in bamboo.

  4. Criticism and Counter-Criticism of Public Management: Strategy Models

    OpenAIRE

    Luis C. Ortigueira

    2007-01-01

    Critical control is very important in scientific management. This paper presents models of critical and counter-critical public-management strategies, focusing on the types of criticism and counter-criticism manifested in parliamentary political debates. The paper includes: (i) a normative model showing how rational criticism can be carried out; (ii) a normative model for oral critical intervention; and (iii) a general motivational strategy model for criticisms and counter-criticisms. The pap...

  5. Cellular ATP synthesis mediated by type III sodium-dependent phosphate transporter Pit-1 is critical to chondrogenesis.

    Science.gov (United States)

    Sugita, Atsushi; Kawai, Shinji; Hayashibara, Tetsuyuki; Amano, Atsuo; Ooshima, Takashi; Michigami, Toshimi; Yoshikawa, Hideki; Yoneda, Toshiyuki

    2011-01-28

    Disturbed endochondral ossification in X-linked hypophosphatemia indicates an involvement of P(i) in chondrogenesis. We studied the role of the sodium-dependent P(i) cotransporters (NPT), which are a widely recognized regulator of cellular P(i) homeostasis, and the downstream events in chondrogenesis using Hyp mice, the murine homolog of human X-linked hypophosphatemia. Hyp mice showed reduced apoptosis and mineralization in hypertrophic cartilage. Hyp chondrocytes in culture displayed decreased apoptosis and mineralization compared with WT chondrocytes, whereas glycosaminoglycan synthesis, an early event in chondrogenesis, was not altered. Expression of the type III NPT Pit-1 and P(i) uptake were diminished, and intracellular ATP levels were also reduced in parallel with decreased caspase-9 and caspase-3 activity in Hyp chondrocytes. The competitive NPT inhibitor phosphonoformic acid and ATP synthesis inhibitor 3-bromopyruvate disturbed endochondral ossification with reduced apoptosis in vivo and suppressed apoptosis and mineralization in conjunction with reduced P(i) uptake and ATP synthesis in WT chondrocytes. Overexpression of Pit-1 in Hyp chondrocytes reversed P(i) uptake and ATP synthesis and restored apoptosis and mineralization. Our results suggest that cellular ATP synthesis consequent to P(i) uptake via Pit-1 plays an important role in chondrocyte apoptosis and mineralization, and that chondrogenesis is ATP-dependent.

  6. Infinitely many solutions for Schrodinger-Kirchhoff type equations involving the fractional p-Laplacian and critical exponent

    Directory of Open Access Journals (Sweden)

    Li Wang

    2016-12-01

    Full Text Available In this article, we show the existence of infinitely many solutions for the fractional p-Laplacian equations of Schrodinger-Kirchhoff type equation $$ M([u]_{s, p}^p (-\\Delta _p^s u+V(x|u|^{p-2}u= \\alpha |u|^{ p_s^{*}-2 }u+\\beta k(x|u|^{q-2}u \\quad x\\in \\mathbb{R}^N, $$ where $(-\\Delta ^s_p$ is the fractional p-Laplacian operator, $[u]_{s,p}$ is the Gagliardo p-seminorm, $0 sp$, $1

  7. Wild-type MIC distributions of four fluoroquinolones active against Mycobacterium tuberculosis in relation to current critical concentrations and available pharmacokinetic and pharmacodynamic data.

    Science.gov (United States)

    Angeby, K A; Jureen, P; Giske, C G; Chryssanthou, E; Sturegård, E; Nordvall, M; Johansson, A G; Werngren, J; Kahlmeter, G; Hoffner, S E; Schön, T

    2010-05-01

    To describe wild-type distributions of the MIC of fluoroquinolones for Mycobacterium tuberculosis in relation to current critical concentrations used for drug susceptibility testing and pharmacokinetic/pharmacodynamic (PK/PD) data. A 96-stick replicator on Middlebrook 7H10 medium was used to define the MICs of ciprofloxacin, ofloxacin, moxifloxacin and levofloxacin for 90 consecutive clinical strains and 24 drug-resistant strains. The MICs were compared with routine BACTEC 460 susceptibility results and with MIC determinations in the BACTEC MGIT 960 system in a subset of strains using ofloxacin as a class representative. PK/PD data for each drug were reviewed in relation to the wild-type MIC distribution. The wild-type MICs of ciprofloxacin, ofloxacin, moxifloxacin and levofloxacin were distributed from 0.125 to 1, 0.25 to 1, 0.032 to 0.5 and 0.125 to 0.5 mg/L, respectively. The MIC data correlated well with the BACTEC 960 MGIT and BACTEC 460 results. PD indices were the most favourable for levofloxacin, followed by moxifloxacin, ofloxacin and ciprofloxacin. We propose S (susceptible) critical concentrations of S

  8. Absence of the common gamma chain (γ(c)), a critical component of the Type I IL-4 receptor, increases the severity of allergic lung inflammation.

    Science.gov (United States)

    Dasgupta, Preeta; Qi, Xiulan; Smith, Elizabeth P; Keegan, Achsah D

    2013-01-01

    The T(H)2 cytokines, IL-4 and IL-13, play critical roles in inducing allergic lung inflammation and drive the alternative activation of macrophages (AAM). Although both cytokines share receptor subunits, IL-4 and IL-13 have differential roles in asthma pathogenesis: IL-4 regulates T(H)2 cell differentiation, while IL-13 regulates airway hyperreactivity and mucus production. Aside from controlling T(H)2 differentiation, the unique contribution of IL-4 signaling via the Type I receptor in airway inflammation remains unclear. Therefore, we analyzed responses in mice deficient in gamma c (γ(c)) to elucidate the role of the Type I IL-4 receptor. OVA primed CD4⁺ OT-II T cells were adoptively transferred into RAG2⁻/⁻ and γ(c)⁻/⁻ mice and allergic lung disease was induced. Both γ(c)⁻/⁻ and γcxRAG2⁻/⁻ mice developed increased pulmonary inflammation and eosinophilia upon OVA challenge, compared to RAG2⁻/⁻ mice. Characteristic AAM proteins FIZZ1 and YM1 were expressed in lung epithelial cells in both mouse strains, but greater numbers of FIZZ1+ or YM1+ airways were present in γ(c)⁻/⁻ mice. Absence of γc in macrophages, however, resulted in reduced YM1 expression. We observed higher T(H)2 cytokine levels in the BAL and an altered DC phenotype in the γ(c)⁻/⁻ recipient mice suggesting the potential for dysregulated T cell and dendritic cell (DC) activation in the γ(c)-deficient environment. These results demonstrate that in absence of the Type I IL-4R, the Type II R can mediate allergic responses in the presence of T(H)2 effectors. However, the Type I R regulates AAM protein expression in macrophages.

  9. Factors that could explain the increasing prevalence of type 2 diabetes among adults in a Canadian province: a critical review and analysis

    Directory of Open Access Journals (Sweden)

    Véronique Thibault

    2016-11-01

    Full Text Available Abstract Background The prevalence of diabetes has increased since the last decade in New Brunswick. Identifying factors contributing to the increase in diabetes prevalence will help inform an action plan to manage the condition. The objective was to describe factors that could explain the increasing prevalence of type 2 diabetes in New Brunswick since 2001. Methods A critical literature review was conducted to identify factors potentially responsible for an increase in prevalence of diabetes. Data from various sources were obtained to draw a repeated cross-sectional (2001–2014 description of these factors concurrently with changes in the prevalence of type 2 diabetes in New Brunswick. Linear regressions, Poisson regressions and Cochran Armitage analysis were used to describe relationships between these factors and time. Results Factors identified in the review were summarized in five categories: individual-level risk factors, environmental risk factors, evolution of the disease, detection effect and global changes. The prevalence of type 2 diabetes has increased by 120% between 2001 and 2014. The prevalence of obesity, hypertension, prediabetes, alcohol consumption, immigration and urbanization increased during the study period and the consumption of fruits and vegetables decreased which could represent potential factors of the increasing prevalence of type 2 diabetes. Physical activity, smoking, socioeconomic status and education did not present trends that could explain the increasing prevalence of type 2 diabetes. During the study period, the mortality rate and the conversion rate from prediabetes to diabetes decreased and the incidence rate increased. Suggestion of a detection effect was also present as the number of people tested increased while the HbA1c and the age at detection decreased. Period and birth cohort effect were also noted through a rise in the prevalence of type 2 diabetes across all age groups, but greater increases were

  10. A-type central stars of planetary nebulae. 1. A radial-velocity study of the central stars of NGC2346 and NGC3132

    Energy Technology Data Exchange (ETDEWEB)

    Mendez, R H; Niemela, V S [Instituto de Astronomia y Fisica del Espacio, Succuoa, Buenos Aires (Argentina); Lee, P

    1978-08-01

    Radial-velocity measurements of the A-type central stars of NGC2346 and NGC3132 are presented. The first one is almost certainly a spectroscopic binary; no definite statement can be made about the second.

  11. CriticalEd

    DEFF Research Database (Denmark)

    Kjellberg, Caspar Mølholt; Meredith, David

    2014-01-01

    . Since the comments are not input sequentially, with regard to position, but in arbitrary order, this list must be sorted by copy/pasting the rows into place—an error-prone and time-consuming process. Scholars who produce critical editions typically use off-the-shelf music notation software......The best text method is commonly applied among music scholars engaged in producing critical editions. In this method, a comment list is compiled, consisting of variant readings and editorial emendations. This list is maintained by inserting the comments into a document as the changes are made......, consisting of a Sibelius plug-in, a cross-platform application, called CriticalEd, and a REST-based solution, which handles data storage/retrieval. A prototype has been tested at the Danish Centre for Music Publication, and the results suggest that the system could greatly improve the efficiency...

  12. Prevalence of autoantibodies in the course of Gaucher disease type 1: A multicenter study comparing Gaucher disease patients to healthy subjects.

    Science.gov (United States)

    Serratrice, Christine; Bensalah, Nesma; Penaranda, Guillaume; Bardin, Nathalie; Belmatoug, Nadia; Masseau, Agathe; Rose, Christian; Lidove, Olivier; Camou, Fabrice; Maillot, François; Leguy, Vanessa; Magy-Bertrand, Nadine; Marie, Isabelle; Cherin, Patrick; Bengherbia, Monia; Carballo, Sebastian; Boucraut, José; Serratrice, Jacques; Berger, Marc; Verrot, Denis

    2018-01-01

    Type 1 Gaucher disease may be related to the presence of autoantibodies. Their clinical significance is questioned. Primary endpoint was to compare the prevalence of autoantibodies in type 1 Gaucher disease patients with healthy subjects, seeking correlations with autoimmune characteristics. Secondary endpoints were to determine whether patients with autoantibodies reported autoimmunity-related symptoms and if genotype, splenectomy or treatment influenced autoantibodies presence. Type 1 Gaucher disease patients and healthy volunteers were included in this national multicenter exploratory study. Autoantibodies presence was compared in both groups and assessed regarding to genotype, splenectomy, Gaucher disease treatment and autoimmunity-related symptoms. Twenty healthy subjects and 40 type 1 Gaucher disease patients were included. Of the studied group: 15 patients undergone splenectomy, 37 were treated either with enzyme replacement therapy (34) or with substrate reduction therapy (3), 25 were homozygous/heterozygous for the N370S mutation. In type 1 Gaucher disease group (studied group), 52% had positive autoantibodies versus 26% in control group. Antiphospholipid antibodies were more frequent in the studied group (30% vs. 5%), but without correlation to thrombosis, osteonecrosis or bone infarcts. In the studied group, antinuclear antibodies were more frequent (25% vs. 16%). None of the patients with autoantibodies had clinical manifestations of autoimmune diseases. Autoantibodies were not correlated with treatment, genotype, or splenectomy, except for anticardiolipid, more frequent in splenectomized patients. In type 1 Gaucher disease, autoantibodies were more frequent compared to a healthy population. However, they were not associated with an increased prevalence of clinical active autoimmune diseases. Copyright © 2016 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

  13. Neurofibroma of the breast detected on position emission tomography-CT in a patient with neurofibromatosis type 1: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ho Seok; Kim, Hye Jung; Yang, Bang; Lee So Mi; Jeong, Ji Yun [Kyungpook National University Hospital, Daegu(Korea, Republic of)

    2016-02-15

    Neurofibromas of the breast are rare. They are commonly found as solitary lesions or parts of neurofibromatosis type 1 and are usually located on the nipple-areolar area. We describe a case of neurofibroma of the breast in the retromammary space, which was detected on positron emission tomography-computed tomography after hysterectomy due to endometrial cancer.

  14. Osteogenesis imperfecta type I: Second-trimester diagnosis and incidental identification of a dominant COL1A1 deletion mutation in the paucisymptomatic father

    Directory of Open Access Journals (Sweden)

    Chih-Ping Chen

    2012-06-01

    Conclusion: Prenatal ultrasound diagnosis of mild forms of OI should include molecular analysis of type I collagen genes in both fetus and parents. Molecular genetic analysis of the family may incidentally identify a collagen gene mutation in the paucisymptomatic affected parent.

  15. Genome wide identification of wheat and Brachypodium type one protein phosphatases and functional characterization of durum wheat TdPP1a

    OpenAIRE

    Bradai, Mariem; Mahjoubi, Habib; Chini, Andrea; Chabouté, Marie-Edith; Hanin, Moez; Ebel, Chantal

    2018-01-01

    Reversible phosphorylation is an essential mechanism regulating signal transduction during development and environmental stress responses. An important number of dephosphorylation events in the cell are catalyzed by type one protein phosphatases (PP1), which catalytic activity is driven by the binding of regulatory proteins that control their substrate specificity or subcellular localization. Plants harbor several PP1 isoforms accounting for large functional redundancies. While animal PP1s we...

  16. The central globular domain of the nucleocapsid protein of human immunodeficiency virus type 1 is critical for virion structure and infectivity.

    Science.gov (United States)

    Ottmann, M; Gabus, C; Darlix, J L

    1995-03-01

    The nucleocapsid protein NCp7 of human immunodeficiency virus type 1 (HIV-1) is a 72-amino-acid peptide containing two CCHC-type zinc fingers linked by a short basic sequence, 29RAPRKKG35, which is conserved in HIV-1 and simian immunodeficiency virus. The complete three-dimensional structure of NCp7 has been determined by 1H-nuclear magnetic resonance spectroscopy (N. Morellet, H. de Rocquigny, Y. Mely, N. Jullian, H. Demene, M. Ottmann, D. Gerard, J. L. Darlix, M. C. Fournié-Zaluski, and B. P. Roques, J. Mol. Biol. 235:287-301, 1994) and revealed a central globular domain where the two zinc fingers are brought in close proximity by the RAPRKKG linker. To examine the role of this globular structure and more precisely of the RAPRKKG linker in virion structure and infectivity, we generated HIV-1 DNA mutants in the RAPRKK sequence of NCp7 and analyzed the mutant virions produced by transfected cells. Mutations that probably alter the structure of NCp7 structure led to the formation of very poorly infectious virus (A30P) or noninfectious virus (P31L and R32G). In addition, the P31L mutant did not contain detectable amounts of reverse transcriptase and had an immature core morphology, as determined by electron microscopy. On the other hand, mutations changing the basic nature of NCp7 had poor effect. R29S had a wild-type phenotype, and the replacement of 32RKK34 by SSS (S3 mutant) resulted in a decrease by no more than 100-fold of the virus titer. These results clearly show that the RAPRKKG linker contains residues that are critical for virion structure and infectivity.

  17. Critical role of types 2 and 3 deiodinases in the negative regulation of gene expression by T₃in the mouse cerebral cortex.

    Science.gov (United States)

    Hernandez, Arturo; Morte, Beatriz; Belinchón, Mónica M; Ceballos, Ainhoa; Bernal, Juan

    2012-06-01

    Thyroid hormones regulate brain development and function through the control of gene expression, mediated by binding of T(3) to nuclear receptors. Brain T(3) concentration is tightly controlled by homeostatic mechanisms regulating transport and metabolism of T(4) and T(3). We have examined the role of the inactivating enzyme type 3 deiodinase (D3) in the regulation of 43 thyroid hormone-dependent genes in the cerebral cortex of 30-d-old mice. D3 inactivation increased slightly the expression of two of 22 positively regulated genes and significantly decreased the expression of seven of 21 negatively regulated genes. Administration of high doses of T(3) led to significant changes in the expression of 12 positive genes and three negative genes in wild-type mice. The response to T(3) treatment was enhanced in D3-deficient mice, both in the number of genes and in the amplitude of the response, demonstrating the role of D3 in modulating T(3) action. Comparison of the effects on gene expression observed in D3 deficiency with those in hypothyroidism, hyperthyroidism, and type 2 deiodinase (D2) deficiency revealed that the negative genes are more sensitive to D2 and D3 deficiencies than the positive genes. This observation indicates that, in normal physiological conditions, D2 and D3 play critical roles in maintaining local T(3) concentrations within a very narrow range. It also suggests that negatively and positively regulated genes do not have the same physiological significance or that their regulation by thyroid hormone obeys different paradigms at the molecular or cellular levels.

  18. Cognitive behavioural therapy with optional graded exercise therapy in patients with severe fatigue with myotonic dystrophy type 1: a multicentre, single-blind, randomised trial.

    Science.gov (United States)

    Okkersen, Kees; Jimenez-Moreno, Cecilia; Wenninger, Stephan; Daidj, Ferroudja; Glennon, Jeffrey; Cumming, Sarah; Littleford, Roberta; Monckton, Darren G; Lochmüller, Hanns; Catt, Michael; Faber, Catharina G; Hapca, Adrian; Donnan, Peter T; Gorman, Gráinne; Bassez, Guillaume; Schoser, Benedikt; Knoop, Hans; Treweek, Shaun; van Engelen, Baziel G M

    2018-06-18

    Myotonic dystrophy type 1 is the most common form of muscular dystrophy in adults and leads to severe fatigue, substantial physical functional impairment, and restricted social participation. In this study, we aimed to determine whether cognitive behavioural therapy optionally combined with graded exercise compared with standard care alone improved the health status of patients with myotonic dystrophy type 1. We did a multicentre, single-blind, randomised trial, at four neuromuscular referral centres with experience in treating patients with myotonic dystrophy type 1 located in Paris (France), Munich (Germany), Nijmegen (Netherlands), and Newcastle (UK). Eligible participants were patients aged 18 years and older with a confirmed genetic diagnosis of myotonic dystrophy type 1, who were severely fatigued (ie, a score of ≥35 on the checklist-individual strength, subscale fatigue). We randomly assigned participants (1:1) to either cognitive behavioural therapy plus standard care and optional graded exercise or standard care alone. Randomisation was done via a central web-based system, stratified by study site. Cognitive behavioural therapy focused on addressing reduced patient initiative, increasing physical activity, optimising social interaction, regulating sleep-wake patterns, coping with pain, and addressing beliefs about fatigue and myotonic dystrophy type 1. Cognitive behavioural therapy was delivered over a 10-month period in 10-14 sessions. A graded exercise module could be added to cognitive behavioural therapy in Nijmegen and Newcastle. The primary outcome was the 10-month change from baseline in scores on the DM1-Activ-c scale, a measure of capacity for activity and social participation (score range 0-100). Statistical analysis of the primary outcome included all participants for whom data were available, using mixed-effects linear regression models with baseline scores as a covariate. Safety data were presented as descriptives. This trial is registered

  19. Generation of an induced pluripotent stem cell (iPSC line from a patient with maturity-onset diabetes of the young type 3 (MODY3 carrying a hepatocyte nuclear factor 1-alpha (HNF1A mutation

    Directory of Open Access Journals (Sweden)

    Frank Griscelli

    2018-05-01

    Full Text Available Heterozygous non-synonymous (p.S142F mutation in HNF1A leads to maturity-onset diabetes of the young (MODY type 3, which is a subtype of dominant inherited young-onset non-autoimmune diabetes due to the defect of insulin secretion from pancreatic beta cells. We generated induced pluripotent stem cells (iPSCs from a patient with HNF1A p.S142F mutation. Cells from this patient, which were reprogrammed by non-integrative viral transduction had normal karyotype, harboured the HNF1A p.S142F mutation, expressed pluripotency hallmarks.

  20. Changes of gait pattern in children with Charcot-Marie-Tooth disease type 1A: a 18 months follow-up study.

    Science.gov (United States)

    Ferrarin, Maurizio; Lencioni, Tiziana; Rabuffetti, Marco; Moroni, Isabella; Pagliano, Emanuela; Pareyson, Davide

    2013-07-02

    In a previous study we identified 3 different gait patterns in a group of children with CMT1A disease: Normal-like (NL), Foot-drop (FD), Foot-drop and Push-off Deficit (FD&POD). Goal of the present study was to perform a follow-up evaluation of the same group of patients to analyze possible changes of gait features in relation to disease progression or specific therapy. Nineteen children with CMT1A were evaluated clinically (CMT-Examination Score and Overall Neuropathy Limitation Scale) and through gait analysis 18.2±1.5 months after a baseline evaluation. Meanwhile, 3 of them had foot surgery. Fifteen out of the 16 non-operated patients significantly changed at least one of the two parameters associated to primary signs (FD and/or POD). Eleven participants worsened at least one parameter and 9 improved one parameter. CMTES significantly worsened for the group of non-operated patients. However, there was no change in CMTES score in 4 patients and in ONLS score in 11. At subgroup level, participants originally belonging to NL group showed a trend towards a foot-drop deficit (-15%, ns); FD and FD&POD subgroups did not change their primary signs, although significant changes were identified individually. All 3 patients operated have improved push-off and proximal joint patterns during walking. Clinical scores did not change within any sub-group. Subtle changes occurring in 1.5 year in gait features of CMT1A children can be instrumentally identified. Such changes show a large inter-subject variability, with some patients even improving their walking pattern. There is anecdotal evidence that foot surgery may improve the push-off phase of gait.

  1. MaHog1, a Hog1-type mitogen-activated protein kinase gene, contributes to stress tolerance and virulence of the entomopathogenic fungus Metarhizium acridum.

    Science.gov (United States)

    Jin, Kai; Ming, Yue; Xia, Yu Xian

    2012-12-01

    Fungal biocontrol agents have great potential in integrated pest management. However, poor efficacy and sensitivity to various adverse factors have hampered their wide application. In eukaryotic cells, Hog1 kinase plays a critical role in stress responses. In this study, MaHog1 (GenBank accession no. EFY85878), encoding a member of the Hog1/Sty1/p38 mitogen-activated protein kinase family in Metarhizium (Me.) acridum, was identified. Targeted gene disruption was used to analyse the role of MaHog1 in virulence and tolerance of adverse factors. Mutants with MaHog1 depletion showed increased sensitivity to high osmotic stress, high temperature and oxidative stress, and exhibited remarkable resistance to cell wall-disturbing agents. These results suggest that Hog1 kinase has a conserved function in regulating multistress responses among fungi, and that MaHog1 might influence cell wall biogenesis in Me. acridum. Bioassays conducted with topical inoculation and intrahaemocoel injection revealed that MaHog1 is required for both penetration and postpenetration development of Me. acridum. MaHog1 disruption resulted in a significant reduction in virulence, likely due to the combination of a decrease in conidial germination, a reduction in appressorium formation and a decline in growth rate in insect haemolymph, which might be caused by impairing fungal tolerance of various stresses during infection.

  2. Nuclear-criticality safety analysis of the Oak Ridge Y-12 plant birdcage-type containers for intraplant storage and transportation

    International Nuclear Information System (INIS)

    Stachowiak, R.V.

    1983-01-01

    The Oak Ridge Y-12 Plant birdcage-type containers include a family of cubic (20-, 24-, and 30-inch) open-framed containers used for the in-house storage and transfer of unirradiated enriched uranium metal. This paper provides insight into the nuclear criticality safety analysis for birdcage usage. All credible contingencies (abnormal events) were analyzed and proven safe (subcritical) in accordance with the requirements and procedures of nuclear criticality safety standards. Examples of the contingencies considered in the analysis include, but are not limited to, full water reflection of any single uranium mass loading, double batching of a loading, water moderation, and misuse of the birdcage. These and other applicable contingencies determine the maximum uranium mass for the 20- and 24-inch birdcages, which is 20 and 28 kilograms, respectively. The maximum number of birdcages stored at one location and the storage array configuration are also determined by the credible contingencies. Stacking restrictions for birdcage storage are three high for the 20-inch birdcage and two high for the 24-inch birdcage. A maximum size for square-based arrays is ten feet by ten feet. Any number of these arrays may be used provided a twelve-foot separation is maintained between each array. Such a storage arrangement results in a floor utilization of 0.42 birdcages per square foot. Better floor utilization, i.e., more birdcages per square foot, is possible with other array configurations that are not square-based. Physical as well as administrative controls, procedures, training, and audits are used to ensure these basic criteria are observed. 1 table

  3. Fetal antigen 1, a member of the epidermal growth factor superfamily, in neurofibromas and serum from patients with neurofibromatosis type 1

    DEFF Research Database (Denmark)

    Jensen, Charlotte Harken; Schroder, H D; Teisner, B

    1999-01-01

    Fetal antigen 1 (FA1) is a 26-32 kDa glycoprotein containing six epidermal growth factor-like repeats closely related to the delta/notch/serrate proteins in Drosophila. FA1 has been shown to be involved in cell differentiation in a juxtacrine/paracrine manner. As neurofibromatosis type 1 (NF-1......), also called von Recklinghausen disease, involves aberrant growth of tissues derived from the neural crest, the expression of FA1 was examined in neurofibroma skin biopsies and serum from patients with NF-1. FA1 was found in the spindle cells of all (n = 10) skin tumour specimens from adult NF-1...

  4. GIS-based identification of areas that have resource potential for critical minerals in six selected groups of deposit types in Alaska

    Science.gov (United States)

    Karl, Susan M.; Jones, James V.; Hayes, Timothy S.

    2016-11-16

    Alaska has considerable potential for undiscovered mineral resources. This report evaluates potential for undiscovered critical minerals in Alaska. Critical minerals are those for which the United States imports more than half of its total supply and which are largely derived from nations that cannot be considered reliable trading partners. In this report, estimated resource potential and certainty for the state of Alaska are analyzed and mapped for the following six selected mineral deposit groups that may contain one or more critical minerals: (1) rare earth elements-thorium-yttrium-niobium(-uranium-zirconium) [REE-Th-Y-Nb(-U-Zr)] deposits associated with peralkaline to carbonatitic igneous intrusive rocks; (2) placer and paleoplacer gold (Au) deposits that in some places might also produce platinum group elements (PGE), chromium (Cr), tin (Sn), tungsten (W), silver (Ag), or titanium (Ti); (3) platinum group elements(-cobalt-chromium-nickel-titanium-vanadium) [PGE(-Co-Cr-Ni-Ti-V)] deposits associated with mafic to ultramafic intrusive rocks; (4) carbonate-hosted copper(-cobalt-silver-germanium-gallium) [Cu(-Co-Ag-Ge-Ga)] deposits; (5) sandstone-hosted uranium(-vanadium-copper) [U(-V-Cu)] deposits; and (6) tin-tungsten-molybdenum(-tantalum-indium-fluorspar) [Sn-W-Mo(-Ta-In-fluorspar)] deposits associated with specialized granites.This study used a data-driven, geographic information system (GIS)-implemented method to identify areas that have mineral resource potential in Alaska. This method systematically and simultaneously analyzes geoscience data from multiple geospatially referenced datasets and uses individual subwatersheds (12-digit hydrologic units) as the spatial unit of classification. The final map output uses a red, yellow, green, and gray color scheme to portray estimated relative potential (High, Medium, Low, Unknown) for each of the six groups of mineral deposit types, and it indicates the relative certainty (High, Medium, Low) of that estimate for

  5. Undifferentiated Pleomorphic Sarcoma and the Importance of Considering the Oncogenic and Immune-Suppressant Role of the Human T-Cell Lymphotropic Virus Type 1: A Case Report

    Directory of Open Access Journals (Sweden)

    Sergio Lupo

    2017-05-01

    Full Text Available IntroductionSoft-tissue sarcomas account for 0.7% of all malignant tumors, with an incidence rate of 3 per 100,000 persons/year. The undifferentiated pleomorphic sarcoma (UPS with giant cells, a high grade tumor of soft tissue, is very unusual, especially in young adults before the age of 40. Human T-cell lymphotropic virus type 1 (HTLV-1 is a human retrovirus, classified as group 1 human carcinogens by The International Agency for Research on Cancer, that causes an aggressive malignancy known as adult T-cell lymphoma/leukemia and a progressive chronic inflammatory neurological disease named HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP. HTLV-1 causes accumulation of genetic mutations in the host genome that could contribute to cellular transformation, one of the oncogenic features of HTLV-1.Case reportWe describe a case of a young woman with UPS who suffered from HAM/TSP with 3 years of evolution. In 2013, the patient started with neurological symptoms: weakness in the legs and bladder dysfunction. One year later, the patient developed a mild paraparesis in both extremities, anti-HTLV-1 antibodies were detected in plasma and in cerebrospinal fluid, and HAM/TSP was confirmed. In November 2015, a benign ganglion cyst was first suspected without intervention and by March 2016 a sarcoma was diagnosed. Three weeks after surgical resection, the tumor aroused in deep tissue and behaved aggressively, implicating a curative wide resection of the fibula, joint reconstruction, and soft-tissue graft. Histopathological examination confirmed UPS with giant cells.Concluding remarksThe unapparent subclinical immunodeficiency state due to HTLV-1 infection deserves to be considered in order to carefully monitor the possibility of developing any type of cancer. Besides, reaching an accurate and timely diagnosis of UPS can be challenging due to the difficulty in diagnosis/classification and delayed consultation. In this particular case

  6. A new treatment for human malignant melanoma targeting L-type amino acid transporter 1 (LAT1): A pilot study in a canine model

    International Nuclear Information System (INIS)

    Fukumoto, Shinya; Hanazono, Kiwamu; Fu, Dah-Renn; Endo, Yoshifumi; Kadosawa, Tsuyoshi; Iwano, Hidetomo; Uchide, Tsuyoshi

    2013-01-01

    Highlights: •LAT1 is highly expressed in tumors but at low levels in normal tissues. •We examine LAT1 expression and function in malignant melanoma (MM). •LAT1 expression in MM tissues and cell lines is higher than those in normal tissues. •LAT1 selective inhibitors inhibit amino acid uptake and cell growth in MM cells. •New chemotherapeutic protocols including LAT1 inhibitors are effective for treatment. -- Abstract: L-type amino acid transporter 1 (LAT1), an isoform of amino acid transport system L, transports branched or aromatic amino acids essential for fundamental cellular activities such as cellular growth, proliferation and maintenance. This amino acid transporter recently has received attention because of its preferential and up-regulated expression in a variety of human tumors in contrast to its limited distribution and low-level expression in normal tissues. In this study, we explored the feasibility of using LAT1 inhibitor as a new therapeutic agent for human malignant melanomas (MM) using canine spontaneous MM as a model for human MM. A comparative study of LAT expression was performed in 48 normal tissues, 25 MM tissues and five cell lines established from MM. The study observed LAT1 mRNA levels from MM tissues and cell lines that were significantly (P 3 H]L-Leucine uptake and cellular growth activities in CMeC-1 were inhibited in a dose-dependent manner by selective LAT1 inhibitors (2-amino-2-norbornane-carboxylic acid, BCH and melphalan, LPM). Inhibitory growth activities of various conventional anti-cancer drugs, including carboplatin, cyclophosphamide, dacarbazine, doxorubicin, mitoxantrone, nimustine, vinblastine and vincristine, were significantly (P < 0.05) enhanced by combination use with BCH or LPM. These findings suggest that LAT1 could be a new therapeutic target for MM

  7. A new treatment for human malignant melanoma targeting L-type amino acid transporter 1 (LAT1): A pilot study in a canine model

    Energy Technology Data Exchange (ETDEWEB)

    Fukumoto, Shinya; Hanazono, Kiwamu [Veterinary Internal Medicine, Department of Small Animal Clinical Sciences, School of Veterinary Medicine, Rakuno Gakuen University, Ebetsu, Hokkaido 069-8501 (Japan); Fu, Dah-Renn; Endo, Yoshifumi; Kadosawa, Tsuyoshi [Veterinary Oncology, Department of Small Animal Clinical Sciences, School of Veterinary Medicine, Rakuno Gakuen University, Ebetsu, Hokkaido 069-8501 (Japan); Iwano, Hidetomo [Veterinary Biochemistry, Department of Basic Veterinary Medicine, School of Veterinary Medicine, Rakuno Gakuen University, Ebetsu, Hokkaido 069-8501 (Japan); Uchide, Tsuyoshi, E-mail: uchide@rakuno.ac.jp [Veterinary Internal Medicine, Department of Small Animal Clinical Sciences, School of Veterinary Medicine, Rakuno Gakuen University, Ebetsu, Hokkaido 069-8501 (Japan)

    2013-09-13

    Highlights: •LAT1 is highly expressed in tumors but at low levels in normal tissues. •We examine LAT1 expression and function in malignant melanoma (MM). •LAT1 expression in MM tissues and cell lines is higher than those in normal tissues. •LAT1 selective inhibitors inhibit amino acid uptake and cell growth in MM cells. •New chemotherapeutic protocols including LAT1 inhibitors are effective for treatment. -- Abstract: L-type amino acid transporter 1 (LAT1), an isoform of amino acid transport system L, transports branched or aromatic amino acids essential for fundamental cellular activities such as cellular growth, proliferation and maintenance. This amino acid transporter recently has received attention because of its preferential and up-regulated expression in a variety of human tumors in contrast to its limited distribution and low-level expression in normal tissues. In this study, we explored the feasibility of using LAT1 inhibitor as a new therapeutic agent for human malignant melanomas (MM) using canine spontaneous MM as a model for human MM. A comparative study of LAT expression was performed in 48 normal tissues, 25 MM tissues and five cell lines established from MM. The study observed LAT1 mRNA levels from MM tissues and cell lines that were significantly (P < 0.01) higher than in normal tissues. Additionally, MM with distant metastasis showed a higher expression than those without distant metastasis. Functional analysis of LAT1 was performed on one of the five cell lines, CMeC-1. [{sup 3}H]L-Leucine uptake and cellular growth activities in CMeC-1 were inhibited in a dose-dependent manner by selective LAT1 inhibitors (2-amino-2-norbornane-carboxylic acid, BCH and melphalan, LPM). Inhibitory growth activities of various conventional anti-cancer drugs, including carboplatin, cyclophosphamide, dacarbazine, doxorubicin, mitoxantrone, nimustine, vinblastine and vincristine, were significantly (P < 0.05) enhanced by combination use with BCH or LPM

  8. Experimental determination of critical data of multi-component mixtures containing potential gasoline additives 2-butanol by a flow-type apparatus

    International Nuclear Information System (INIS)

    He, Maogang; Xin, Nan; Wang, Chengjie; Liu, Yang; Zhang, Ying; Liu, Xiangyang

    2016-01-01

    Graphical abstract: Experimental critical pressures of 2-butanol + hexane + heptane system. - Highlights: • Critical properties of six binary systems and two ternary systems were measured. • Six binary systems containing 2-butanol show non-ideal behavior in their T c –x 1 curves. • Non-ideal behavior of mixtures with 2-butanol relies on azeotropy. • Experimental data for binary systems were fitted well with Redlich–Kister equation. • Critical surfaces of ternary systems were plotted using the Cibulka’s expressions. - Abstract: In this work, we used a flow method for measurement of critical properties of six binary mixtures (2-butanol + cyclohexane, 2-butanol + hexane, 2-butanol + heptane, 2-butanol + octane, 2-butanol + nonane and 2-butanol + decane) and two ternary mixtures (2-butanol + hexane + heptane and 2-butanol + octane + decane). The critical properties were determined by observing the disappearance and reappearance of the gas–liquid phase meniscus in a quartz glass tube. The standard uncertainties of temperatures and pressures for both binary and ternary mixtures were estimated to be less than 0.2 K and 5.2 kPa, respectively. These critical data provide the boundaries of the two-phase regions of the related mixture systems. Six binary systems show non-ideal behaviors in the loci of critical temperatures. We used the Redlich–Kister equations to correlate the critical temperatures and pressures of these systems and listed the binary interaction parameters. The maximum average absolute deviation (AAD) of each binary system between experimental data and calculated results from Redlich–Kister equations is 0.038% for critical temperatures, and 0.244% for critical pressures. Moreover, the two ternary systems were newly reported and correlated by Cibulka’s and Singh’s expressions. The maximum AAD of critical temperatures and critical pressures are 0.103% and 0.433%, respectively.

  9. Vascular smooth muscle cells express the alpha(1A) subunit of a P-/Q-type voltage-dependent Ca(2+)Channel, and It is functionally important in renal afferent arterioles

    DEFF Research Database (Denmark)

    Hansen, Pernille B. Lærkegaard; Jensen, Boye L.; Andreasen, D

    2000-01-01

    In the present study, we tested whether the alpha(1A) subunit, which encodes a neuronal isoform of voltage-dependent Ca(2+) channels (VDCCs) (P-/Q-type), was present and functional in vascular smooth muscle and renal resistance vessels. By reverse transcription-polymerase chain reaction...... preglomerular resistance vessels and aorta, as well as mesangial cells, and that P-type VDCCs contribute to Ca(2+) influx in aortic and renal VSMCs and are involved in depolarization-mediated contraction in renal afferent arterioles....

  10. The nuclear factor-erythroid 2-related factor/heme oxygenase-1 axis is critical for the inflammatory features of type 2 diabetes-associated osteoarthritis.

    Science.gov (United States)

    Vaamonde-Garcia, Carlos; Courties, Alice; Pigenet, Audrey; Laiguillon, Marie-Charlotte; Sautet, Alain; Houard, Xavier; Kerdine-Römer, Saadia; Meijide, Rosa; Berenbaum, Francis; Sellam, Jérémie

    2017-09-01

    Epidemiological findings support the hypothesis that type 2 diabetes mellitus (T2DM) is a risk factor for osteoarthritis (OA). Moreover, OA cartilage from patients with T2DM exhibits a greater response to inflammatory stress, but the molecular mechanism is unclear. To investigate whether the antioxidant defense system participates in this response, we examined here the expression of nuclear factor-erythroid 2-related factor (Nrf-2), a master antioxidant transcription factor, and of heme oxygenase-1 (HO-1), one of its main target genes, in OA cartilage from T2DM and non-T2DM patients as well as in murine chondrocytes exposed to high glucose (HG). Ex vivo experiments indicated that Nrf-2 and HO-1 expression is reduced in T2DM versus non-T2DM OA cartilage (0.57-fold Nrf-2 and 0.34-fold HO-1), and prostaglandin E 2 (PGE 2 ) release was increased in samples with low HO-1 expression. HG-exposed, IL-1β-stimulated chondrocytes had lower Nrf-2 levels in vitro , particularly in the nuclear fraction, than chondrocytes exposed to normal glucose (NG). Accordingly, HO-1 levels were also decreased (0.49-fold) in these cells. The HO-1 inducer cobalt protoporphyrin IX more efficiently attenuated PGE 2 and IL-6 release in HG+IL-1β-treated cells than in NG+IL-1β-treated cells. Greater reductions in HO-1 expression and increase in PGE 2 /IL-6 production were observed in HG+IL-1β-stimulated chondrocytes from Nrf-2 -/- mice than in chondrocytes from wild-type mice. We conclude that the Nrf-2/HO-1 axis is a critical pathway in the hyperglucidic-mediated dysregulation of chondrocytes. Impairments in this antioxidant system may explain the greater inflammatory responsiveness of OA cartilage from T2DM patients and may inform treatments of such patients. © 2017 by The American Society for Biochemistry and Molecular Biology, Inc.

  11. Toward critical bioethics.

    Science.gov (United States)

    Árnason, Vilhjálmur

    2015-04-01

    This article deals with the question as to what makes bioethics a critical discipline. It considers different senses of criticism and evaluates their strengths and weaknesses. A primary method in bioethics as a philosophical discipline is critical thinking, which implies critical evaluation of concepts, positions, and arguments. It is argued that the type of analytical criticism that restricts its critical role to critical thinking of this type often suffers from other intellectual flaws. Three examples are taken to demonstrate this: premature criticism, uncritical self-understanding of theoretical assumptions, and narrow framing of bioethical issues. Such flaws can lead both to unfair treatment of authors and to uncritical discussion of topics. In this context, the article makes use of Häyry's analysis of different rationalities in bioethical approaches and argues for the need to recognize the importance of communicative rationality for critical bioethics. A radically different critical approach in bioethics, rooted in social theory, focuses on analyses of power relations neglected in mainstream critical thinking. It is argued that, although this kind of criticism provides an important alternative in bioethics, it suffers from other shortcomings that are rooted in a lack of normative dimensions. In order to complement these approaches and counter their shortcomings, there is a need for a bioethics enlightened by critical hermeneutics. Such hermeneutic bioethics is aware of its own assumptions, places the issues in a wide context, and reflects critically on the power relations that stand in the way of understanding them. Moreover, such an approach is dialogical, which provides both a critical exercise of speech and a normative dimension implied in the free exchange of reasons and arguments. This discussion is framed by Hedgecoe's argument that critical bioethics needs four elements: to be empirically rooted, theory challenging, reflexive, and politely skeptical.

  12. Adolescent Scoliosis 1A001: Radiographic Results of Selecting the Touched Vertebra as the Lowest Instrumented Vertebra in Lenke Type 1 (Main Thoracic) & Type 2 (Double Thoracic) Curves at a Minimum 5-year Follow-up

    OpenAIRE

    Lenke, Lawrence; Newton, Peter; Lehman, Ronald; Kelly, Michael; Clements, David; Errico, Thomas; Betz, Randall; Samdani, Amer; Blanke, Kathy; Oggiano, Leonardo; Sessa, Sergio; Rosa, Guido La; Guler, Umit Ozgur; Ozalay, Metin; Eyvazov, Kamil

    2017-01-01

    Introduction: A prior study showed the touched vertebra (TV), defined as the most cephalad thoracolumbar/lumbar vertebra “touched” by the center sacral vertical line (CSVL), as a potential landmark vertebra & recommended lowest instrumented vertebra (LIV) as well. We evaluated a large cohort of Lenke type 1 & 2 cases to determine if selecting the TV as the LIV will produce optimal positioning at a min. 5 yrs postoperative. Our hypothesis was that it would and that fusing short of the TV would...

  13. High-sensitivity C-reactive protein does not improve the differential diagnosis of HNF1A-MODY and familial young-onset type 2 diabetes: A grey zone analysis.

    Science.gov (United States)

    Bellanné-Chantelot, C; Coste, J; Ciangura, C; Fonfrède, M; Saint-Martin, C; Bouché, C; Sonnet, E; Valéro, R; Lévy, D-J; Dubois-Laforgue, D; Timsit, J

    2016-02-01

    Low plasma levels of high-sensitivity C-reactive protein (hs-CRP) have been suggested to differentiate hepatocyte nuclear factor 1 alpha-maturity-onset diabetes of the young (HNF1A-MODY) from type 2 diabetes (T2D). Yet, differential diagnosis of HNF1A-MODY and familial young-onset type 2 diabetes (F-YT2D) remains a difficult challenge. Thus, this study assessed the added value of hs-CRP to distinguish between the two conditions. This prospective multicentre study included 143 HNF1A-MODY patients, 310 patients with a clinical history suggestive of HNF1A-MODY, but not confirmed genetically (F-YT2D), and 215 patients with T2D. The ability of models, including clinical characteristics and hs-CRP to predict HNF1A-MODY was analyzed, using the area of the receiver operating characteristic (AUROC) curve, and a grey zone approach was used to evaluate these models in clinical practice. Median hs-CRP values were lower in HNF1A-MODY (0.25mg/L) than in F-YT2D (1.14mg/L) and T2D (1.70mg/L) patients. Clinical parameters were sufficient to differentiate HNF1A-MODY from classical T2D (AUROC: 0.99). AUROC analyses to distinguish HNF1A-MODY from F-YT2D were 0.82 for clinical features and 0.87 after including hs-CRP. For the grey zone analysis, the lower boundary was set to missMODY with F-YT2D, 65% of patients were classified in between these categories - in the zone of diagnostic uncertainty - even after adding hs-CRP to clinical parameters. hs-CRP does not improve the differential diagnosis of HNF1A-MODY and F-YT2D. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  14. Thinking Critically about Critical Thinking

    Science.gov (United States)

    Mulnix, Jennifer Wilson

    2012-01-01

    As a philosophy professor, one of my central goals is to teach students to think critically. However, one difficulty with determining whether critical thinking can be taught, or even measured, is that there is widespread disagreement over what critical thinking actually is. Here, I reflect on several conceptions of critical thinking, subjecting…

  15. Criticality Studies in a Pilot Plant for Processing MTR-Type Irradiated Fuels; Estudios de Criticidad de una Planta Piloto para el Tratamiento de Combustibles Irradiados Tipo ' MTR '

    Energy Technology Data Exchange (ETDEWEB)

    Pereira Sanchez, G.; Uriarte Hueda, A. [Junta de Energia Nuclear, Division de Materiales Madrid (Spain)

    1966-05-15

    A number of theoretical studies on nuclear safety have been carried out in a pilot plant being constructed at the Junta de Energia Nuclear in Madrid for processing irradiated fuels from the MTR-type experimental reactor JEN-1. The study was carried out working with aqueous and organic solutions at two levels of {sup 235}U enrichment - 20% and 93%. The paper is divided into two main parts: the first deals with the individual items of equipment, and the interactions between these are studied in the second part. The calculations in this second part have been made using three different methods to make it more certain that the system as a whole can never be critical. The first method employed is based on the solid angle concept and makes it possible to fix the maximum {sup 235}U concentrations within the system. The second method, based on the albedo, supplies the value of the multiplication factor K of the whole assembly as a function of the concentration of {sup 235}U. In the last part, the distribution of the equipment is compared with other similar systems and experimental tests from other sources. Finally, the paper specifies the conditions for working the installation which ensure that a nuclear accident can never occur. (author) [Spanish] Se ha efectuado una serie de estudios teoricos sobre la seguridad nuclear de una planta piloto, que se encuentra en construccion en la Junta de Energfa Nuclear situada en Madrid, para el tratamiento de combustibles irradiados procedentes del reactor experimental JEN-1 del tipo MTR. El estudio se ha realizado utilizando disoluciones, tanto acuosas como organicas, con dos grados de enriquecimiento, 20% y 93% en {sup 235}U. Este trabajo comprende dos partes principales: en la primera se han considerado las distintas unidades del equipo individualmente y en la segunda se han estudiado las interacciones entre ellas. El calculo de esta segunda parte se ha hecho por tres metodos diferentes para tener una mayor seguridad de que el

  16. Thermal-hydraulic behavior of physical quantities at critical velocities in a nuclear research reactor core channel using plate type fuel

    Directory of Open Access Journals (Sweden)

    Sidi Ali Kamel

    2012-01-01

    Full Text Available The thermal-hydraulic study presented here relates to a channel of a nuclear reactor core. This channel is defined as being the space between two fuel plates where a coolant fluid flows. The flow velocity of this coolant should not generate vibrations in fuel plates. The aim of this study is to know the distribution of the temperature in the fuel plates, in the cladding and in the coolant fluid at the critical velocities of Miller, of Wambsganss, and of Cekirge and Ural. The velocity expressions given by these authors are function of the geometry of the fuel plate, the mechanical characteristics of the fuel plate’s material and the thermal characteristics of the coolant fluid. The thermal-hydraulic study is made under steady-state; the equation set-up of the thermal problem is made according to El Wakil and to Delhaye. Once the equation set-up is validated, the three critical velocities are calculated and then used in the calculations of the different temperature profiles. The average heat flux and the critical heat flux are evaluated for each critical velocity and their ratio reported. The recommended critical velocity to be used in nuclear channel calculations is that of Wambsganss. The mathematical model used is more precise and all the physical quantities, when using this critical velocity, stay in safe margins.

  17. Genetic screening of a pedigree with osteogenesis imperfecta type Ⅰand identification of a novel mutation in COL1A2 pathogenic gene%成骨不全Ⅰ型家系的基因检测和COL1A2基因新突变的致病性鉴定

    Institute of Scientific and Technical Information of China (English)

    李荣; 郭源平; 潘敬新; 郭奕斌

    2015-01-01

    To uncover the molecular pathogenic mechanism of congenital osteogenesis imperfecta (OI) type I, all the 103 exons of the COL1A1 (Collagen, type Ⅰ, alpha 1) and COL1A2 (Collagen, type Ⅰ, alpha 2) genes in a child with OI type Ⅰ were screened using PCR-DNA direct sequencing. The results showed no pathological mutation in COL1A1 gene, but a novel mutation c.946G>T/p.G316C in the exon 19 of COL1A2 gene, which was inherited from her father. This mutation was not found in her mother and other six phenotypically normal relatives. By denaturing high perfor-mance liquid chromatography (DHPLC) screening, the abnormal double-peak was visualized in PCR products of exon 19 of COL1A2 gene in the proband and her father, while the normal single-peak was shown in those of her mother and all the healthy controls. Using allele specific amplification (ASA) screening, a specific band of 391 bp in COL1A2 exon 19 was amplified only in the proband and her father, but not in other samples. The amino acid encoded by the mutation site is evolutionarily highly conserved, and this mutation was a“damaging”or“probably damaging”factor to OI type Ⅰ, based on the predicting results using SIFT and Polyphen-2 softwares. In conclusion, the novel c.946G>T/p.G316C mutation in COL1A2 gene is a pathogenic mutation that could result in OI type Ⅰ. If the couple wants to get pregnant again, it is necessary to screen the mutation site in COL1A2 gene through the prenatal genetic diagnosis in the first trimester or through preimplantation genetic diagnosis (PGD) in the progestation.%为了揭示成骨不全(Osteogenesis imperfecta, OI)Ⅰ型家系的分子遗传学发生机制,文章采用PCR-DNA直接测序法,对患儿 COL1A1和 COL1A2基因共103个外显子(E)进行突变检测。结果显示:患儿 COL1A1基因未发现任何病理性突变,而在 COL1A2基因 E19内发现一新的杂合错义突变(p.G316C),该突变来自其父,而其母正常,其他表型正常的6位

  18. Critical Care

    Science.gov (United States)

    Critical care helps people with life-threatening injuries and illnesses. It might treat problems such as complications from surgery, ... attention by a team of specially-trained health care providers. Critical care usually takes place in an ...

  19. Enterovirus Exposure Uniquely Discriminates Type 1 Diabetes Patients with a Homozygous from a Heterozygous Melanoma Differentiation-Associated Protein 5/Interferon Induced with Helicase C Domain 1 A946T Genotype.

    Science.gov (United States)

    Schulte, Barbara M; Gielen, Paul R; Kers-Rebel, Esther D; Prosser, Amy C; Lind, Katharina; Flodström-Tullberg, Malin; Tack, Cees J; Elving, Lammy D; Adema, Gosse J

    2016-09-01

    In children at risk for type 1 diabetes, innate immune activity is detected before seroconversion. Enterovirus infections have been linked to diabetes development, and a polymorphism (A946T) in the innate immune sensor recognizing enterovirus RNA, interferon-induced with helicase C domain 1/melanoma differentiation-associated protein 5, predisposes to disease. We hypothesized that the strength of innate antienteroviral responses is affected in autoimmune type 1 diabetes patients and linked to the A946T polymorphism. We compared induction of interferon-stimulated genes (ISGs) in peripheral blood mononuclear cells (PBMCs) and dendritic cells (DCs) in healthy individuals and diabetes patients upon stimulation with enterovirus, enterovirus-antibody complexes, or ligands mimicking infection in relation to the A946T polymorphism. Overall, PBMCs of diabetes patients and healthy donors showed comparable ISG induction upon stimulation. No differences were observed in DCs. Interestingly, the data imply that the magnitude of responses to enterovirus and enterovirus-antibody complexes in PBMCs is critically influenced by the A946T polymorphism and elevated in heterozygotes compared to TT homozygous individuals in autoimmune diabetes patients, but not healthy controls. These data imply an intrinsic difference in the responses to enterovirus and enterovirus-antibody complexes in diabetes patients carrying a TT risk genotype compared to heterozygotes that may influence control of enterovirus clearance.

  20. How Critical Is Critical Thinking?

    Science.gov (United States)

    Shaw, Ryan D.

    2014-01-01

    Recent educational discourse is full of references to the value of critical thinking as a 21st-century skill. In music education, critical thinking has been discussed in relation to problem solving and music listening, and some researchers suggest that training in critical thinking can improve students' responses to music. But what exactly is…

  1. Tank farms criticality safety manual

    International Nuclear Information System (INIS)

    FORT, L.A.

    2003-01-01

    This document defines the Tank Farms Contractor (TFC) criticality safety program, as required by Title 10 Code of Federal Regulations (CFR-), Subpart 830.204(b)(6), ''Documented Safety Analysis'' (10 CFR- 830.204 (b)(6)), and US Department of Energy (DOE) 0 420.1A, Facility Safety, Section 4.3, ''Criticality Safety.'' In addition, this document contains certain best management practices, adopted by TFC management based on successful Hanford Site facility practices. Requirements in this manual are based on the contractor requirements document (CRD) found in Attachment 2 of DOE 0 420.1A, Section 4.3, ''Nuclear Criticality Safety,'' and the cited revisions of applicable standards published jointly by the American National Standards Institute (ANSI) and the American Nuclear Society (ANS) as listed in Appendix A. As an informational device, requirements directly imposed by the CRD or ANSI/ANS Standards are shown in boldface. Requirements developed as best management practices through experience and maintained consistent with Hanford Site practice are shown in italics. Recommendations and explanatory material are provided in plain type

  2. Criticality accident in Argentina

    International Nuclear Information System (INIS)

    Oliveira, A.R. de.

    1984-01-01

    A recent criticality type accident, ocurred in Argetina, is commented. Considerations about the nature of the facility where this accident took place, its genesis, type of operation carried out on the day of the event, and the medical aspects involved are done. (Author) [pt

  3. Anatomical and histological profile of bronchus-associated lymphoid tissue and localization of melatonin receptor types (Mel 1a and Mel 1b) in the lung-associated immune system of a tropical bird, Perdicula asiatica.

    Science.gov (United States)

    Kumar Kharwar, Rajesh; Haldar, Chandana

    2011-05-01

    The histological distribution of the lung-associated immune system (LAIS) and the expressional pattern of melatonin receptors are still unknown in birds. The aim of the present study was to determine the localization of the bronchus-associated lymphoid tissue (BALT nodule) in a tropical bird, the Indian jungle bush quail, Perdicula asiatica. We also demonstrate the expression of melatonin receptor types (Mel(1a) and Mel(1b)) in order to propose an immunomodulatory role of melatonin in LAIS. Localization of melatonin receptors in the lung of the Indian jungle bush quail, P. asiatica was supported immunohistochemically and by Western blot analysis using specific antibodies for those receptors. Immunolocalization for Mel(1b) receptor was noted in the bronchial region of the lungs, in finger-like projections of mucosal foldings, in lymphocytes in the BALT nodule as well as in free form. In contrast, immunolocalization for Mel(1a) receptor was noted in various areas of the lung instead of in the bronchial region. Western blot analysis showed a single band at 37 and 39kDa for Mel(1a) and Mel(1b) receptors, respectively, with the latter showing higher expression. The results demonstrate a well-developed LAIS and region-specific distribution of melatonin receptors in the lung and provide evidence for a possible functional role for melatonin in the LAIS of birds. Copyright © 2010 Elsevier GmbH. All rights reserved.

  4. Critical Jostling

    Directory of Open Access Journals (Sweden)

    Pippin Barr

    2016-11-01

    Full Text Available Games can serve a critical function in many different ways, from serious games about real world subjects to self-reflexive commentaries on the nature of games themselves. In this essay we discuss critical possibilities stemming from the area of critical design, and more specifically Carl DiSalvo’s adversarial design and its concept of reconfiguring the remainder. To illustrate such an approach, we present the design and outcomes of two games, Jostle Bastard and Jostle Parent. We show how the games specifically engage with two previous games, Hotline Miami and Octodad: Dadliest Catch, reconfiguring elements of those games to create interactive critical experiences and extensions of the source material. Through the presentation of specific design concerns and decisions, we provide a grounded illustration of a particular critical function of videogames and hope to highlight this form as another valuable approach in the larger area of videogame criticism.

  5. Upregulation of the Nr2f1-A830082K12Rik gene pair in murine neural crest cells results in a complex phenotype reminiscent of Waardenburg syndrome type 4.

    Science.gov (United States)

    Bergeron, Karl-F; Nguyen, Chloé M A; Cardinal, Tatiana; Charrier, Baptiste; Silversides, David W; Pilon, Nicolas

    2016-11-01

    Waardenburg syndrome is a neurocristopathy characterized by a combination of skin and hair depigmentation, and inner ear defects. In the type 4 form, these defects show comorbidity with Hirschsprung disease, a disorder marked by an absence of neural ganglia in the distal colon, triggering functional intestinal obstruction. Here, we report that the Spot mouse line - obtained through an insertional mutagenesis screen for genes involved in neural crest cell (NCC) development - is a model for Waardenburg syndrome type 4. We found that the Spot insertional mutation causes overexpression of an overlapping gene pair composed of the transcription-factor-encoding Nr2f1 and the antisense long non-coding RNA A830082K12Rik in NCCs through a mechanism involving relief of repression of these genes. Consistent with the previously described role of Nr2f1 in promoting gliogenesis in the central nervous system, we further found that NCC-derived progenitors of the enteric nervous system fail to fully colonize Spot embryonic guts owing to their premature differentiation in glial cells. Taken together, our data thus identify silencer elements of the Nr2f1-A830082K12Rik gene pair as new candidate loci for Waardenburg syndrome type 4. © 2016. Published by The Company of Biologists Ltd.

  6. Upregulation of the Nr2f1-A830082K12Rik gene pair in murine neural crest cells results in a complex phenotype reminiscent of Waardenburg syndrome type 4

    Directory of Open Access Journals (Sweden)

    Karl-F. Bergeron

    2016-11-01

    Full Text Available Waardenburg syndrome is a neurocristopathy characterized by a combination of skin and hair depigmentation, and inner ear defects. In the type 4 form, these defects show comorbidity with Hirschsprung disease, a disorder marked by an absence of neural ganglia in the distal colon, triggering functional intestinal obstruction. Here, we report that the Spot mouse line – obtained through an insertional mutagenesis screen for genes involved in neural crest cell (NCC development – is a model for Waardenburg syndrome type 4. We found that the Spot insertional mutation causes overexpression of an overlapping gene pair composed of the transcription-factor-encoding Nr2f1 and the antisense long non-coding RNA A830082K12Rik in NCCs through a mechanism involving relief of repression of these genes. Consistent with the previously described role of Nr2f1 in promoting gliogenesis in the central nervous system, we further found that NCC-derived progenitors of the enteric nervous system fail to fully colonize Spot embryonic guts owing to their premature differentiation in glial cells. Taken together, our data thus identify silencer elements of the Nr2f1-A830082K12Rik gene pair as new candidate loci for Waardenburg syndrome type 4.

  7. Critical Proximity

    OpenAIRE

    Simon, Jane

    2010-01-01

    This essay considers how written language frames visual objects. Drawing on Michel Foucault’s response to Raymond Roussel’s obsessive description, the essay proposes a model of criticism where description might press up against its objects. This critical closeness is then mapped across the conceptual art practice and art criticism of Ian Burn. Burn attends to the differences between seeing and reading, and considers the conditions which frame how we look at images, including how w...

  8. Critical frequencies of the ionospheric F1 and F2 layers during the last four solar cycles: Sunspot group type dependencies

    Science.gov (United States)

    Yiǧit, Erdal; Kilcik, Ali; Elias, Ana Georgina; Dönmez, Burçin; Ozguc, Atila; Yurchshyn, Vasyl; Rozelot, Jean-Pierre

    2018-06-01

    The long term solar activity dependencies of ionospheric F1 and F2 regions' critical frequencies (f0F1 and f0F2) are analyzed for the last four solar cycles (1976-2015). We show that the ionospheric F1 and F2 regions have different solar activity dependencies in terms of the sunspot group (SG) numbers: F1 region critical frequency (f0F1) peaks at the same time with the small SG numbers, while the f0F2 reaches its maximum at the same time with the large SG numbers, especially during the solar cycle 23. The observed differences in the sensitivity of ionospheric critical frequencies to sunspot group (SG) numbers provide a new insight into the solar activity effects on the ionosphere and space weather. While the F1 layer is influenced by the slow solar wind, which is largely associated with small SGs, the ionospheric F2 layer is more sensitive to Coronal Mass Ejections (CMEs) and fast solar winds, which are mainly produced by large SGs and coronal holes. The SG numbers maximize during of peak of the solar cycle and the number of coronal holes peaks during the sunspot declining phase. During solar minimum there are relatively less large SGs, hence reduced CME and flare activity. These results provide a new perspective for assessing how the different regions of the ionosphere respond to space weather effects.

  9. Web-Based Intervention for Women With Type 1 Diabetes in Pregnancy and Early Motherhood: Critical Analysis of Adherence to Technological Elements and Study Design.

    Science.gov (United States)

    Berg, Marie; Linden, Karolina; Adolfsson, Annsofie; Sparud Lundin, Carina; Ranerup, Agneta

    2018-05-02

    Numerous Web-based interventions have been implemented to promote health and health-related behaviors in persons with chronic conditions. Using randomized controlled trials to evaluate such interventions creates a range of challenges, which in turn can influence the study outcome. Applying a critical perspective when evaluating Web-based health interventions is important. The objective of this study was to critically analyze and discuss the challenges of conducting a Web-based health intervention as a randomized controlled trial. The MODIAB-Web study was critically examined using an exploratory case study methodology and the framework for analysis offered through the Persuasive Systems Design model. Focus was on technology, study design, and Web-based support usage, with special focus on the forum for peer support. Descriptive statistics and qualitative content analysis were used. The persuasive content and technological elements in the design of the randomized controlled trial included all four categories of the Persuasive Systems Design model, but not all design principles were implemented. The study duration was extended to a period of four and a half years. Of 81 active participants in the intervention group, a maximum of 36 women were simultaneously active. User adherence varied greatly with a median of 91 individual log-ins. The forum for peer support was used by 63 participants. Although only about one-third of the participants interacted in the forum, there was a fairly rich exchange of experiences and advice between them. Thus, adherence in terms of social interactions was negatively affected by limited active participation due to prolonged recruitment process and randomization effects. Lessons learned from this critical analysis are that technology and study design matter and might mutually influence each other. In Web-based interventions, the use of design theories enables utilization of the full potential of technology and promotes adherence. The

  10. Critical Review

    DEFF Research Database (Denmark)

    Rosenbaum, Ralph K.; Olsen, Stig Irving

    2018-01-01

    Manipulation and mistakes in LCA studies are as old as the tool itself, and so is its critical review. Besides preventing misuse and unsupported claims, critical review may also help identifying mistakes and more justifiable assumptions as well as generally improve the quality of a study. It thus...... supports the robustness of an LCA and increases trust in its results and conclusions. The focus of this chapter is on understanding what a critical review is, how the international standards define it, what its main elements are, and what reviewer qualifications are required. It is not the objective...... of this chapter to learn how to conduct a critical review, neither from a reviewer nor from a practitioner perspective. The foundation of this chapter and the basis for any critical review of LCA studies are the International Standards ISO 14040:2006, ISO 14044:2006 and ISO TS 14071:2014....

  11. Maximization of a reflected slab type reactor, and with double criticality as function of the position of the interface core-reflector

    International Nuclear Information System (INIS)

    Ierardi, Maria Clara Filippini

    1980-01-01

    Using optimal control techniques, it is obtained the fuel distribution which maximizes the power of an infinite, reflected slab nuclear reactor. The reactor is permissible power density and the maximum permissible fuel introduces the reflector into the reactor in a non-conventional way. Numerical results for both two and four energy groups are presented. It is observed a double criticality depending on the position of the core-reflector interface, however described by a diffusion theory model. Both the maximum concentration are the only restraints. (author)

  12. Unconventional Quantum Critical Points

    OpenAIRE

    Xu, Cenke

    2012-01-01

    In this paper we review the theory of unconventional quantum critical points that are beyond the Landau's paradigm. Three types of unconventional quantum critical points will be discussed: (1). The transition between topological order and semiclassical spin ordered phase; (2). The transition between topological order and valence bond solid phase; (3). The direct second order transition between different competing orders. We focus on the field theory and universality class of these unconventio...

  13. Third harmonic frequency generation by type-I critically phase-matched LiB3O5 crystal by means of optically active quartz crystal.

    Science.gov (United States)

    Gapontsev, Valentin P; Tyrtyshnyy, Valentin A; Vershinin, Oleg I; Davydov, Boris L; Oulianov, Dmitri A

    2013-02-11

    We present a method of third harmonic generation at 355 nm by frequency mixing of fundamental and second harmonic radiation of an ytterbium nanosecond pulsed all-fiber laser in a type-I phase-matched LiB(3)O(5) (LBO) crystal where originally orthogonal polarization planes of the fundamental and second harmonic beams are aligned by an optically active quartz crystal. 8 W of ultraviolet light at 355 nm were achieved with 40% conversion efficiency from 1064 nm radiation. The conversion efficiency obtained in a type-I phase-matched LBO THG crystal was 1.6 times higher than the one achieved in a type-II LBO crystal at similar experimental conditions. In comparison to half-wave plates traditionally used for polarization alignment the optically active quartz crystal has much lower temperature dependence and requires simpler optical alignment.

  14. Molecular analysis of critical sequences within the EBNA-2 type 1 gene from Epstein-Barr virus isolates from patients with infectious mononucleosis, tonsillar hyperplasia, and HIV infection

    NARCIS (Netherlands)

    Al-Homsi, A. S.; Berger, C.; van Baarle, D.; Kersten, M. J.; Klein, M. R.; McQuain, C.; van Oers, R.; Knecht, H.

    1998-01-01

    EBNA-2 is the first protein to be detected after infection of primary B lymphocytes by Epstein-Barr virus (EBV) and plays an essential role as transcriptional activator in EBV-induced lymphocyte transformation. We analysed by PCR and sequencing regions of the EBNA-2 type 1 gene from isolates from 13

  15. Critical Arts

    African Journals Online (AJOL)

    both formal and informal) in culture and social theory. CRITICAL ARTS aims to challenge and ... Book Review: Brian McNair, An Introduction to Political Communication (3rd edition), London: Routledge, 2003, ISBN 0415307082, 272pp. Phil Joffe ...

  16. Critical Proximity

    Directory of Open Access Journals (Sweden)

    Jane Simon

    2010-09-01

    Full Text Available This essay considers how written language frames visual objects. Drawing on Michel Foucault’s response to Raymond Roussel’s obsessive description, the essay proposes a model of criticism where description might press up against its objects. This critical closeness is then mapped across the conceptual art practice and art criticism of Ian Burn. Burn attends to the differences between seeing and reading, and considers the conditions which frame how we look at images, including how we look at, and through words. The essay goes on to consider Meaghan Morris’s writing on Lynn Silverman’s photographs. Both Morris and Burn offer an alternative to a parasitic model of criticism and enact a patient way of looking across and through visual landscapes.

  17. Critical proximity

    Directory of Open Access Journals (Sweden)

    Simon, Jane

    2010-01-01

    Full Text Available This essay considers how written language frames visual objects. Drawing on Michel Foucault’s response to Raymond Roussel’s obsessive description, the essay proposes a model of criticism where description might press up against its objects. This critical closeness is then mapped across the conceptual art practice and art criticism of Ian Burn. Burn attends to the differences between seeing and reading, and considers the conditions which frame how we look at images, including how we look at, and through words. The essay goes on to consider Meaghan Morris’s writing on Lynn Silverman’s photographs. Both Morris and Burn offer an alternative to a parasitic model of criticism and enact a patient way of looking across and through visual landscapes.

  18. Criticality alarm device

    International Nuclear Information System (INIS)

    Kasai, Kenji.

    1994-01-01

    The device of the present invention is utilized, for example, to a reprocessing facility for storing and processing nuclear fuels and measures and controls the nuclear fuel assembly system so as not to exceed criticality. That is, a conventional criticality alarm device applies a predetermined processing to neutron fluxes generated from a nuclear fuel assembly system containing nuclear fuels and outputs an alarm. The device of the present invention comprises (1) a neutron flux supply source for increasing and decreasing neutron fluxes periodically and supplying them to nuclear fuel assemblies, (2) a detector for detecting neutron fluxes in the nuclear fuel assemblies, (3) a critical state judging section for judging the critical state of the nuclear fuel assemblies based on the periodically changing signals obtained from the detector (2) and (4) an alarm section for outputting criticality alarms depending on the result of the judgement. The device of the present invention can accurately recognize the critical state of the nuclear fuel assembly system and can forecast reaching of the nuclear fuel assembly to criticality or prompt neutron critical state. (I.S.)

  19. The Macrophage Galactose-Type Lectin-1 (MGL1 Recognizes Taenia crassiceps Antigens, Triggers Intracellular Signaling, and Is Critical for Resistance to This Infection

    Directory of Open Access Journals (Sweden)

    Daniel Montero-Barrera

    2015-01-01

    Full Text Available C-type lectins are multifunctional sugar-binding molecules expressed on dendritic cells (DCs and macrophages that internalize antigens for processing and presentation. Macrophage galactose-type lectin 1 (MGL1 recognizes glycoconjugates expressing Lewis X structures which contain galactose residues, and it is selectively expressed on immature DCs and macrophages. Helminth parasites contain large amounts of glycosylated components, which play a role in the immune regulation induced by such infections. Macrophages from MGL1−/− mice showed less binding ability toward parasite antigens than their wild-type (WT counterparts. Exposure of WT macrophages to T. crassiceps antigens triggered tyrosine phosphorylation signaling activity, which was diminished in MGL1−/− macrophages. Following T. crassiceps infection, MGL1−/− mice failed to produce significant levels of inflammatory cytokines early in the infection compared to WT mice. In contrast, MGL1−/− mice developed a Th2-dominant immune response that was associated with significantly higher parasite loads, whereas WT mice were resistant. Flow cytometry and RT-PCR analyses showed overexpression of the mannose receptors, IL-4Rα, PDL2, arginase-1, Ym1, and RELM-α on MGL1−/− macrophages. These studies indicate that MGL1 is involved in T. crassiceps recognition and subsequent innate immune activation and resistance.

  20. Critical amino acids within the human immunodeficiency virus type 1 envelope glycoprotein V4 N- and C-terminals contribute to virus entry.

    Directory of Open Access Journals (Sweden)

    Yan Li

    Full Text Available The importance of the fourth variable (V4 region of the human immunodeficiency virus 1 (HIV-1 envelope glycoprotein (Env in virus infection has not been well clarified, though the polymorphism of this region has been found to be associated with disease progression to acquired immunodeficiency syndrome (AIDS. In the present work, we focused on the correlation between HIV-1 gp120 V4 region polymorphism and the function of the region on virus entry, and the possible mechanisms for how the V4 region contributes to virus infectivity. Therefore, we analyzed the differences in V4 sequences along with coreceptor usage preference from CCR5 to CXCR4 and examined the importance of the amino acids within the V4 region for CCR5- and CXCR4-tropic virus entry. In addition, we determined the influence of the V4 amino acids on Env expression and gp160 processing intracellularly, as well as the amount of Env on the pseudovirus surface. The results indicated that V4 tended to have a shorter length, fewer potential N-linked glycosylation sites (PNGS, greater evolutionary distance, and a lower negative net charge when HIV-1 isolates switched from a coreceptor usage preference for CCR5 to CXCR4. The N- and C-terminals of the HIV-1 V4 region are highly conserved and critical to maintain virus entry ability, but only the mutation at position 417 in the context of ADA (a R5-tropic HIV-1 strain resulted in the ability to utilize CXCR4. In addition, 390L, 391F, 414I, and 416L are critical to maintain gp160 processing and maturation. It is likely that the hydrophobic properties and the electrostatic surface potential of gp120, rather than the conformational structure, greatly contribute to this V4 functionality. The findings provide information to aid in the understanding of the functions of V4 in HIV-1 entry and offer a potential target to aid in the development of entry inhibitors.

  1. A critical review of the use and performance of different function types for modeling temperature-dependent development of arthropod larvae.

    Science.gov (United States)

    Quinn, Brady K

    2017-01-01

    Temperature-dependent development influences production rates of arthropods, including crustaceans important to fisheries and agricultural pests. Numerous candidate equation types (development functions) exist to describe the effect of temperature on development time, yet most studies use only a single type of equation and there is no consensus as to which, if any model predicts development rates better than the others, nor what the consequences of selecting a potentially incorrect model equation are on predicted development times. In this study, a literature search was performed of studies fitting development functions to development data of arthropod larvae (99 species). The published data of most (79) of these species were then fit with 33 commonly-used development functions. Overall performance of each function type and consequences of using a function other than the best one to model data were assessed. Performance was also related to taxonomy and the range of temperatures examined. The majority (91.1%) of studies were found to not use the best function out of those tested. Using the incorrect model lead to significantly less accurate (e.g., mean difference±SE 85.9±27.4%, range: -1.7 to 1725.5%) predictions of development times than the best function. Overall, more complex functions performed poorly relative to simpler ones. However, performance of some complex functions improved when wide temperature ranges were tested, which tended to be confined to studies of insects or arachnids compared with those of crustaceans. Results indicate the biological significance of choosing the best-fitting model to describe temperature-dependent development time data. Copyright © 2016 Elsevier Ltd. All rights reserved.

  2. Identification of the NC1 domain of {alpha}3 chain as critical for {alpha}3{alpha}4{alpha}5 type IV collagen network assembly.

    Science.gov (United States)

    LeBleu, Valerie; Sund, Malin; Sugimoto, Hikaru; Birrane, Gabriel; Kanasaki, Keizo; Finan, Elizabeth; Miller, Caroline A; Gattone, Vincent H; McLaughlin, Heather; Shield, Charles F; Kalluri, Raghu

    2010-12-31

    The network organization of type IV collagen consisting of α3, α4, and α5 chains in the glomerular basement membrane (GBM) is speculated to involve interactions of the triple helical and NC1 domain of individual α-chains, but in vivo evidence is lacking. To specifically address the contribution of the NC1 domain in the GBM collagen network organization, we generated a mouse with specific loss of α3NC1 domain while keeping the triple helical α3 chain intact by connecting it to the human α5NC1 domain. The absence of α3NC1 domain leads to the complete loss of the α4 chain. The α3 collagenous domain is incapable of incorporating the α5 chain, resulting in the impaired organization of the α3α4α5 chain-containing network. Although the α5 chain can assemble with the α1, α2, and α6 chains, such assembly is incapable of functionally replacing the α3α4α5 protomer. This novel approach to explore the assembly type IV collagen in vivo offers novel insights in the specific role of the NC1 domain in the assembly and function of GBM during health and disease.

  3. The critical role of conflict resolution in teams: a close look at the links between conflict type, conflict management strategies, and team outcomes.

    Science.gov (United States)

    Behfar, Kristin J; Peterson, Randall S; Mannix, Elizabeth A; Trochim, William M K

    2008-01-01

    This article explores the linkages between strategies for managing different types of conflict and group performance and satisfaction. Results from a qualitative study of 57 autonomous teams suggest that groups that improve or maintain top performance over time share 3 conflict resolution tendencies: (a) focusing on the content of interpersonal interactions rather than delivery style, (b) explicitly discussing reasons behind any decisions reached in accepting and distributing work assignments, and (c) assigning work to members who have the relevant task expertise rather than assigning by other common means such as volunteering, default, or convenience. The authors' results also suggest that teams that are successful over time are likely to be both proactive in anticipating the need for conflict resolution and pluralistic in developing conflict resolution strategies that apply to all group members. 2008 APA

  4. Comment on critical instability

    International Nuclear Information System (INIS)

    King, S.F.; Suzuki, Mahiko

    1992-01-01

    We discuss the problem of the mass splitting between top and bottom quarks, within the context of Nambu-Jona-Lasinio type models involving top and bottom quark condensates. We interpret the phenomenon of 'critical instability' recently proposed to account for such a mass splitting as the fine-tuning of two vacuum expectation values in a composite two-Higgs doublet model. (orig.)

  5. Critical Vidders

    DEFF Research Database (Denmark)

    Svegaard, Robin Sebastian Kaszmarczyk

    2015-01-01

    This article will introduce and take a look at a specific subset of the fan created remix videos known as vids, namely those that deal with feminist based critique of media. Through examples, it will show how fans construct and present their critique, and finally broach the topic of the critical ...

  6. Are Tesco customers exhibiting a more social type of loyalty towards Tesco and Tesco Clubcard? A critical analysis of the nature and type of Tesco customer loyalty to Tesco in Dundee.

    OpenAIRE

    Turner, Jason James

    2012-01-01

    The aims of the thesis are two-fold. The first aim is to evaluate the antecedents which influence loyalty to Tesco and Tesco Clubcard, contending that customer loyalty is influenced by factors of both a social and marketing nature. The second aim is to assess the nature and type of loyalty exhibited by Tesco customers towards Tesco and Tesco Clubcard. These two aims derive from the research question ‘What are the antecedents of loyalty exhibited by Tesco customers towards Tesco...

  7. Cav2.3 (R-Type Calcium Channels are Critical for Mediating Anticonvulsive and Neuroprotective Properties of Lamotrigine In Vivo

    Directory of Open Access Journals (Sweden)

    Maxine Dibué-Adjei

    2017-11-01

    Full Text Available Background/Aims: Lamotrigine (LTG is a popular modern antiepileptic drug (AED, however, its mechanism of action has yet to be fully understood, as it is known to modulate many members of several ion channel families. In heterologous systems, LTG inhibits Cav2.3 (R-type calcium currents, which contribute to kainic-acid- (KA induced epilepsy in vivo. To gain insight into the role of R-type currents in LTG drug action in vivo, we compared the effects of LTG to topiramate and lacosamide in Cav2.3-deficient mice and controls on KA-induced seizures. Methods: Behavioral seizure rating and quantitative electrocorticography were performed after injection of 20 mg/kg [and 30 mg/kg] KA. One hour before KA injection, mice were pretreated with either 30 mg/kg LTG, 50 mg/kg topiramate (TPM or 30 mg/kg lacosamide (LSM. Results: Ablation of Cav2.3 reduced total seizure scores by 28.6% (p=0.0012 and pretreatment with LTG reduced seizure activity of control mice by 23.2% (p=0.02. In Cav2.3-deficient mice LTG pretreatment increased seizure activity by 22.1% (p=0.018 and increased the percentage of degenerated CA1 pyramidal neurons (p=0.02. All three tested AEDs reduced seizure activity in control mice, however only the non-calcium channel modulating AED, LSM had an anticonvulsive effect in Cav2.3-deficient mice. Furthermore LTG altered electrocorticographic parameters differently in the two genotypes, decreasing relative power of ictal spikes in control mice compared to Cav2.3-defcient mice. Conclusion: These findings give first in vivo evidence for an essential role for Cav2.3 in LTG pharmacology and shed light on a paradoxical effect of LTG in their absence. Furthermore, LTG appears to promote ictal activity in Cav2.3-deficient mice resulting in increased neurotoxicity in the CA1 region. This paradoxical mechanism, possibly reflecting rebound hyperexcitation of pyramidal CA1 neurons after increased inhibition, may be key in understanding LTG-induced seizure

  8. Identification of two novel critical mutations in PCNT gene resulting in microcephalic osteodysplastic primordial dwarfism type II associated with multiple intracranial aneurysms.

    Science.gov (United States)

    Li, Fei-Feng; Wang, Xu-Dong; Zhu, Min-Wei; Lou, Zhi-Hong; Zhang, Qiong; Zhu, Chun-Yu; Feng, Hong-Lin; Lin, Zhi-Guo; Liu, Shu-Lin

    2015-12-01

    Microcephalic osteodysplastic primordial dwarfism type II (MOPD II) is a highly detrimental human autosomal inherited recessive disorder. The hallmark characteristics of this disease are intrauterine and postnatal growth restrictions, with some patients also having cerebrovascular problems such as cerebral aneurysms. The genomic basis behind most clinical features of MOPD II remains largely unclear. The aim of this work was to identify the genetic defects in a Chinese family with MOPD II associated with multiple intracranial aneurysms. The patient had typical MOPD II syndrome, with subarachnoid hemorrhage and multiple intracranial aneurysms. We identified three novel mutations in the PCNT gene, including one single base alteration (9842A>C in exon 45) and two deletions (Del-C in exon 30 and Del-16 in exon 41). The deletions were co-segregated with the affected individual in the family and were not present in the control population. Computer modeling demonstrated that the deletions may cause drastic changes on the secondary and tertiary structures, affecting the hydrophilicity and hydrophobicity of the mutant proteins. In conclusion, we identified two novel mutations in the PCNT gene associated with MOPD II and intracranial aneurysms, and the mutations were expected to alter the stability and functioning of the protein by computer modeling.

  9. Hypoxia Is a Critical Parameter for Chondrogenic Differentiation of Human Umbilical Cord Blood Mesenchymal Stem Cells in Type I/III Collagen Sponges

    Directory of Open Access Journals (Sweden)

    Tangni Gómez-Leduc

    2017-09-01

    Full Text Available Umbilical cord blood (UCB is an attractive alternative to bone marrow for isolation of mesenchymal stem cells (MSCs to treat articular cartilage defects. Here, we set out to determine the growth factors (bone morphogenetic protein 2 (BMP-2 and transforming growth factor-β (TGF-β1 and oxygen tension effects during chondrogenesis of human UCB-MSCs for cartilage engineering. Chondrogenic differentiation was induced using 3D cultures in type I/III collagen sponges with chondrogenic factors in normoxia (21% O2 or hypoxia (<5% O2 for 7, 14 and 21 days. Our results show that UCB-MSCs can be committed to chondrogenesis in the presence of BMP-2+TGF-β1. Normoxia induced the highest levels of chondrocyte-specific markers. However, hypoxia exerted more benefit by decreasing collagen X and matrix metalloproteinase-13 (MMP13 expression, two chondrocyte hypertrophy markers. However, a better chondrogenesis was obtained by switching oxygen conditions, with seven days in normoxia followed by 14 days in hypoxia, since these conditions avoid hypertrophy of hUCB-MSC-derived chondrocytes while maintaining the expression of chondrocyte-specific markers observed in normoxia. Our study demonstrates that oxygen tension is a key factor for chondrogenesis and suggests that UBC-MSCs 3D-culture should begin in normoxia to obtain a more efficient chondrocyte differentiation before placing them in hypoxia for chondrocyte phenotype stabilization. UCB-MSCs are therefore a reliable source for cartilage engineering.

  10. Critical reading and critical thinking Critical reading and critical thinking

    Directory of Open Access Journals (Sweden)

    Loni Kreis Taglieber

    2008-04-01

    Full Text Available The purpose of this paper is to provide, for L1 and L2 reading and writing teachers, a brief overview of the literature about critical reading and higher level thinking skills. The teaching of these skills is still neglected in some language classes in Brazil, be it in L1 or in L2 classes. Thus, this paper may also serve as a resource guide for L1 and/or L2 reading and writing teachers who want to incorporate critical reading and thinking into their classes. In modern society, even in everyday life people frequently need to deal with complicated public and political issues, make decisions, and solve problems. In order to do this efficiently and effectively, citizens must be able to evaluate critically what they see, hear, and read. Also, with the huge amount of printed material available in all areas in this age of “information explosion” it is easy to feel overwhelmed. But often the information piled up on people’s desks and in their minds is of no use due to the enormous amount of it. The purpose of this paper is to provide, for L1 and L2 reading and writing teachers, a brief overview of the literature about critical reading and higher level thinking skills. The teaching of these skills is still neglected in some language classes in Brazil, be it in L1 or in L2 classes. Thus, this paper may also serve as a resource guide for L1 and/or L2 reading and writing teachers who want to incorporate critical reading and thinking into their classes. In modern society, even in everyday life people frequently need to deal with complicated public and political issues, make decisions, and solve problems. In order to do this efficiently and effectively, citizens must be able to evaluate critically what they see, hear, and read. Also, with the huge amount of printed material available in all areas in this age of “information explosion” it is easy to feel overwhelmed. But often the information piled up on people’s desks and in their minds is of

  11. The upstream enhancer elements of the G6PC promoter are critical for optimal G6PC expression in murine glycogen storage disease type Ia.

    Science.gov (United States)

    Lee, Young Mok; Pan, Chi-Jiunn; Koeberl, Dwight D; Mansfield, Brian C; Chou, Janice Y

    2013-11-01

    Glycogen storage disease type-Ia (GSD-Ia) patients deficient in glucose-6-phosphatase-α (G6Pase-α or G6PC) manifest impaired glucose homeostasis characterized by fasting hypoglycemia, growth retardation, hepatomegaly, nephromegaly, hyperlipidemia, hyperuricemia, and lactic acidemia. Two efficacious recombinant adeno-associated virus pseudotype 2/8 (rAAV8) vectors expressing human G6Pase-α have been independently developed. One is a single-stranded vector containing a 2864-bp of the G6PC promoter/enhancer (rAAV8-GPE) and the other is a double-stranded vector containing a shorter 382-bp minimal G6PC promoter/enhancer (rAAV8-miGPE). To identify the best construct, a direct comparison of the rAAV8-GPE and the rAAV8-miGPE vectors was initiated to determine the best vector to take forward into clinical trials. We show that the rAAV8-GPE vector directed significantly higher levels of hepatic G6Pase-α expression, achieved greater reduction in hepatic glycogen accumulation, and led to a better toleration of fasting in GSD-Ia mice than the rAAV8-miGPE vector. Our results indicated that additional control elements in the rAAV8-GPE vector outweigh the gains from the double-stranded rAAV8-miGPE transduction efficiency, and that the rAAV8-GPE vector is the current choice for clinical translation in human GSD-Ia. © 2013.

  12. L-type calcium channels play a critical role in maintaining lens transparency by regulating phosphorylation of aquaporin-0 and myosin light chain and expression of connexins.

    Science.gov (United States)

    Maddala, Rupalatha; Nagendran, Tharkika; de Ridder, Gustaaf G; Schey, Kevin L; Rao, Ponugoti Vasantha

    2013-01-01

    Homeostasis of intracellular calcium is crucial for lens cytoarchitecture and transparency, however, the identity of specific channel proteins regulating calcium influx within the lens is not completely understood. Here we examined the expression and distribution profiles of L-type calcium channels (LTCCs) and explored their role in morphological integrity and transparency of the mouse lens, using cDNA microarray, RT-PCR, immunoblot, pharmacological inhibitors and immunofluorescence analyses. The results revealed that Ca (V) 1.2 and 1.3 channels are expressed and distributed in both the epithelium and cortical fiber cells in mouse lens. Inhibition of LTCCs with felodipine or nifedipine induces progressive cortical cataract formation with time, in association with decreased lens weight in ex-vivo mouse lenses. Histological analyses of felodipine treated lenses revealed extensive disorganization and swelling of cortical fiber cells resembling the phenotype reported for altered aquaporin-0 activity without detectable cytotoxic effects. Analysis of both soluble and membrane rich fractions from felodipine treated lenses by SDS-PAGE in conjunction with mass spectrometry and immunoblot analyses revealed decreases in β-B1-crystallin, Hsp-90, spectrin and filensin. Significantly, loss of transparency in the felodipine treated lenses was preceded by an increase in aquaporin-0 serine-235 phosphorylation and levels of connexin-50, together with decreases in myosin light chain phosphorylation and the levels of 14-3-3ε, a phosphoprotein-binding regulatory protein. Felodipine treatment led to a significant increase in gene expression of connexin-50 and 46 in the mouse lens. Additionally, felodipine inhibition of LTCCs in primary cultures of mouse lens epithelial cells resulted in decreased intracellular calcium, and decreased actin stress fibers and myosin light chain phosphorylation, without detectable cytotoxic response. Taken together, these observations reveal a crucial

  13. Criticality accident:

    International Nuclear Information System (INIS)

    Canavese, Susana I.

    2000-01-01

    A criticality accident occurred at 10:35 on September 30, 1999. It occurred in a precipitation tank in a Conversion Test Building at the JCO Tokai Works site in Tokaimura (Tokai Village) in the Ibaraki Prefecture of Japan. STA provisionally rated this accident a 4 on the seven-level, logarithmic International Nuclear Event Scale (INES). The September 30, 1999 criticality accident at the JCO Tokai Works Site in Tokaimura, Japan in described in preliminary, technical detail. Information is based on preliminary presentations to technical groups by Japanese scientists and spokespersons, translations by technical and non-technical persons of technical web postings by various nuclear authorities, and English-language non-technical reports from various news media and nuclear-interest groups. (author)

  14. Critical dynamics

    International Nuclear Information System (INIS)

    Dekker, H.

    1980-01-01

    It is shown how to solve the master equation for a Markov process including a critical point by means of successive approximations in terms of a small parameter. A critical point occurs if, by adjusting an externally controlled quantity, the system shows a transition from normal monostable to bistable behaviour. The fundamental idea of the theory is to separate the master equation into its proper irreducible part and a corrective remainder. The irreducible or zeroth order stochastic approximation will be a relatively simple Fokker-Planck equation that contains the essential features of the process. Once the solution of this irreducible equation is known, the higher order corrections in the original master equation can be incorporated in a systematic manner. (Auth.)

  15. Critical scattering

    International Nuclear Information System (INIS)

    Stirling, W.G.; Perry, S.C.

    1996-01-01

    We outline the theoretical and experimental background to neutron scattering studies of critical phenomena at magnetic and structural phase transitions. The displacive phase transition of SrTiO 3 is discussed, along with examples from recent work on magnetic materials from the rare-earth (Ho, Dy) and actinide (NpAs, NpSb, USb) classes. The impact of synchrotron X-ray scattering is discussed in conclusion. (author) 13 figs., 18 refs

  16. Nuclear multifragmentation critical exponents

    International Nuclear Information System (INIS)

    Bauer, W.; Friedman, W.A.; Univ. of Wisconsin, Madison, WI

    1995-01-01

    In a recent Letter, cited in a reference, the EoS collaboration presented data of fragmentation of 1 A GeV gold nuclei incident on carbon. By analyzing moments of the fragment charge distribution, the authors claim to determine the values of the critical exponents γ, β, and τ for finite nuclei. These data represent a crucial step forward in the understanding of the physics of nuclear fragmentation. However, as shown in this paper, the analysis presented in the cited reference is not sufficient to support the claim that the critical exponents for nuclear fragmentation have been unambiguously determined

  17. Effect of engine parameters and type of gaseous fuel on the performance of dual-fuel gas diesel engines. A critical review

    Energy Technology Data Exchange (ETDEWEB)

    Sahoo, B.B. [Centre for Energy, Indian Institute of Technology, Guwahati 781039 (India); Sahoo, N.; Saha, U.K. [Department of Mechanical Engineering, Indian Institute of Technology, Guwahati 781039 (India)

    2009-08-15

    Petroleum resources are finite and, therefore, search for their alternative non-petroleum fuels for internal combustion engines is continuing all over the world. Moreover gases emitted by petroleum fuel driven vehicles have an adverse effect on the environment and human health. There is universal acceptance of the need to reduce such emissions. Towards this, scientists have proposed various solutions for diesel engines, one of which is the use of gaseous fuels as a supplement for liquid diesel fuel. These engines, which use conventional diesel fuel and gaseous fuel, are referred to as 'dual-fuel engines'. Natural gas and bio-derived gas appear more attractive alternative fuels for dual-fuel engines in view of their friendly environmental nature. In the gas-fumigated dual-fuel engine, the primary fuel is mixed outside the cylinder before it is inducted into the cylinder. A pilot quantity of liquid fuel is injected towards the end of the compression stroke to initiate combustion. When considering a gaseous fuel for use in existing diesel engines, a number of issues which include, the effects of engine operating and design parameters, and type of gaseous fuel, on the performance of the dual-fuel engines, are important. This paper reviews the research on above issues carried out by various scientists in different diesel engines. This paper touches upon performance, combustion and emission characteristics of dual-fuel engines which use natural gas, biogas, producer gas, methane, liquefied petroleum gas, propane, etc. as gaseous fuel. It reveals that 'dual-fuel concept' is a promising technique for controlling both NO{sub x} and soot emissions even on existing diesel engine. But, HC, CO emissions and 'bsfc' are higher for part load gas diesel engine operations. Thermal efficiency of dual-fuel engines improve either with increased engine speed, or with advanced injection timings, or with increased amount of pilot fuel. The ignition

  18. Adenovirus type 5 E1A and E6 proteins of low-risk cutaneous beta-human papillomaviruses suppress cell transformation through interaction with FOXK1/K2 transcription factors.

    Science.gov (United States)

    Komorek, Jessica; Kuppuswamy, Mohan; Subramanian, T; Vijayalingam, S; Lomonosova, Elena; Zhao, Ling-Jun; Mymryk, Joe S; Schmitt, Kimberly; Chinnadurai, G

    2010-03-01

    The adenovirus (Adv) oncoprotein E1A stimulates cell proliferation and inhibits differentiation. These activities are primarily linked to the N-terminal region (exon 1) of E1A, which interacts with multiple cellular protein complexes. The C terminus (exon 2) of E1A antagonizes these processes, mediated in part through interaction with C-terminal binding proteins 1 and 2 (CtBP1/2). To identify additional cellular E1A targets that are involved in the modulation of E1A C-terminus-mediated activities, we undertook tandem affinity purification of E1A-associated proteins. Through mass spectrometric analysis, we identified several known E1A-interacting proteins as well as novel E1A targets, such as the forkhead transcription factors, FOXK1/K2. We identified a Ser/Thr-containing sequence motif in E1A that mediated interaction with FOXK1/K2. We demonstrated that the E6 proteins of two beta-human papillomaviruses (HPV14 and HPV21) associated with epidermodysplasia verruciformis also interacted with FOXK1/K2 through a motif similar to that of E1A. The E1A mutants deficient in interaction with FOXK1/K2 induced enhanced cell proliferation and oncogenic transformation. The hypertransforming activity of the mutant E1A was suppressed by HPV21 E6. An E1A-E6 chimeric protein containing the Ser/Thr domain of the E6 protein in E1A interacted efficiently with FOXK1/K2 and inhibited cell transformation. Our results suggest that targeting FOXK1/K2 may be a common mechanism for certain beta-HPVs and Adv5. E1A exon 2 mutants deficient in interaction with the dual-specificity kinases DYRK1A/1B and their cofactor HAN11 also induced increased cell proliferation and transformation. Our results suggest that the E1A C-terminal region may suppress cell proliferation and oncogenic transformation through interaction with three different cellular protein complexes: FOXK1/K2, DYRK(1A/1B)/HAN11, and CtBP1/2.

  19. Non-Critical Covariant Superstrings

    CERN Document Server

    Grassi, P A

    2005-01-01

    We construct a covariant description of non-critical superstrings in even dimensions. We construct explicitly supersymmetric hybrid type variables in a linear dilaton background, and study an underlying N=2 twisted superconformal algebra structure. We find similarities between non-critical superstrings in 2n+2 dimensions and critical superstrings compactified on CY_(4-n) manifolds. We study the spectrum of the non-critical strings, and in particular the Ramond-Ramond massless fields. We use the supersymmetric variables to construct the non-critical superstrings sigma-model action in curved target space backgrounds with coupling to the Ramond-Ramond fields. We consider as an example non-critical type IIA strings on AdS_2 background with Ramond-Ramond 2-form flux.

  20. Education Studies: Issues & Critical Perspectives

    Science.gov (United States)

    Kassem, Derek; Mufti, Emmanuel; Robinson, John

    2006-01-01

    This major text for Education Studies students provides a critical account of key issues in education today. The text features: (1) A critical analysis of key issues in Education Studies to encourage students' thinking about education in the broadest terms; (2) Themed sections with introductions to link the issues discussed in each chapter; (3)…

  1. Critical Mass

    CERN Multimedia

    AUTHOR|(CDS)2070299

    2017-01-01

    Critical Mass is a cycling event typically held on the last Friday of every month; its purpose is not usually formalized beyond the direct action of meeting at a set location and time and traveling as a group through city or town streets on bikes. The event originated in 1992 in San Francisco; by the end of 2003, the event was being held in over 300 cities around the world. At CERN it is held once a year in conjunction with the national Swiss campaing "Bike to work".

  2. Measuring Psychological Critical Thinking: An Update

    Science.gov (United States)

    Lawson, Timothy J.; Jordan-Fleming, Mary Kay; Bodle, James H.

    2015-01-01

    Critical thinking is widely considered an important skill for psychology majors. However, few measures exist of the types of critical thinking that are specific to psychology majors. Lawson (1999) designed the Psychological Critical Thinking Exam (PCTE) to measure students' ability to "think critically, or evaluate claims, in a way that…

  3. A tryptophan-rich motif in the human parainfluenza virus type 2 V protein is critical for the blockade of toll-like receptor 7 (TLR7)- and TLR9-dependent signaling.

    Science.gov (United States)

    Kitagawa, Yoshinori; Yamaguchi, Mayu; Zhou, Min; Komatsu, Takayuki; Nishio, Machiko; Sugiyama, Tsuyoshi; Takeuchi, Kenji; Itoh, Masae; Gotoh, Bin

    2011-05-01

    Plasmacytoid dendritic cells (pDCs) do not produce alpha interferon (IFN-α) unless viruses cause a systemic infection or overcome the first-line defense provided by conventional DCs and macrophages. We show here that even paramyxoviruses, whose infections are restricted to the respiratory tract, have a V protein able to prevent Toll-like receptor 7 (TLR7)- and TLR9-dependent IFN-α induction specific to pDCs. Mutational analysis of human parainfluenza virus type 2 demonstrates that the second Trp residue of the Trp-rich motif (Trp-X(3)-Trp-X(9)-Trp) in the C-terminal domain unique to V, a determinant for IRF7 binding, is critical for the blockade of TLR7/9-dependent signaling.

  4. Dictionary criticism

    DEFF Research Database (Denmark)

    Nielsen, Sandro

    2018-01-01

    Dictionary criticism is part of the lexicographical universe and reviewing of electronic and printed dictionaries is not an exercise in linguistics or in subject fields but an exercise in lexicography. It does not follow from this that dictionary reviews should not be based on a linguistic approach......, but that the linguistic approach is only one of several approaches to dictionary reviewing. Similarly, the linguistic and factual competences of reviewers should not be relegated to an insignificant position in the review process. Moreover, reviewers should define the object of their reviews, the dictionary, as a complex...... information tool with several components and in terms of significant lexicographical features: lexicographical functions, data and structures. This emphasises the fact that dictionaries are much more than mere vessels of linguistic categories, namely lexicographical tools that have been developed to fulfil...

  5. The role of genetic variants in CYP2C8, LPIN1, PPARGC1A and PPARγ on the trough steady-state plasma concentrations of rosiglitazone and on glycosylated haemoglobin A1c in type 2 diabetes

    DEFF Research Database (Denmark)

    Stage, Tore B; Christensen, Mette M H; Feddersen, Søren

    2013-01-01

    OBJECTIVE: The aim of this study was to examine the effect of single nucleotide polymorphisms in CYP2C8, LPIN1, PPARGC1A and PPARγ on rosiglitazone's (i) trough steady-state plasma concentration (C(ss,min)), (ii) on glycosylated haemoglobin A1c (HbA1c) and (iii) the risk of developing adverse eve...

  6. Steelhead Critical Habitat, Coast - NOAA [ds122

    Data.gov (United States)

    California Department of Resources — This layer depicts areas designated for Steelhead Critical Habitat as well as habitat type and quality in the Coastal California Steelhead ESUs (evolutionarily...

  7. Enterovirus Exposure Uniquely Discriminates Type 1 Diabetes Patients with a Homozygous from a Heterozygous Melanoma Differentiation-Associated Protein 5/Interferon Induced with Helicase C Domain 1 A946T Genotype

    NARCIS (Netherlands)

    Schulte, B.M.; Gielen, P.R.; Kers-Rebel, E.D.; Prosser, A.C.; Lind, K.; Flodstrom-Tullberg, M.; Tack, C.J.J.; Elving, L.D.; Adema, G.J.

    2016-01-01

    In children at risk for type 1 diabetes, innate immune activity is detected before seroconversion. Enterovirus infections have been linked to diabetes development, and a polymorphism (A946T) in the innate immune sensor recognizing enterovirus RNA, interferon-induced with helicase C domain 1/melanoma

  8. Facts about Type 2

    Medline Plus

    Full Text Available ... Us in the Fight for a Cure Your tax-deductible gift today can fund critical diabetes research ... Care of Type 2 Diabetes This two-page introduction to type 2 diabetes is in PDF format ...

  9. Effects of IFN-β1a and IFN-β1b treatment on the expression of cytokines, inducible NOS (NOS type II), and myelin proteins in animal model of multiple sclerosis.

    Science.gov (United States)

    Lubina-Dąbrowska, Natalia; Stepień, Adam; Sulkowski, Grzegorz; Dąbrowska-Bouta, Beata; Langfort, Józef; Chalimoniuk, Małgorzata

    2017-08-01

    The aim of this study was to investigate the effects of interferon (IFN)-β1a and IFN-β1b treatment on inflammatory factors and myelin protein levels in the brain cortex of the Lewis rat experimental autoimmune encephalomyelitis (EAE), animal model of multiple sclerosis. To induce EAE, rat were immunized with inoculums containing spinal cord guinea pig homogenized in phosphate-buffered saline and emulsified in Freund's complete adjuvant containing 110 µg of the appropriate antigen in 100 µl of an emulsion and additionally 4-mg/ml Mycobacterium tuberculosis (H37Ra). The rats were treated three times per week with subcutaneous applications of 300,000 units IFN-β1a or IFN-β1b. The treatments were started 8 days prior to immunization and continued until day 14 after immunization. The rats were killed on the 14th day of the experiment. EAE induced dramatic increase in interleukin (IL)-1β, IL-6, and tumor necrosis factor (TNF)-concentrations and inducible nitric oxide synthase (iNOS) expression in the brain, which closely corresponded to the course of neurological symptoms and the loss of weight. Both IFN-β1b and IFN-β1a treatments inhibited the pro-inflammatory cytokines (IL-6, IL-1β, TNF-α and IFN-γ), decreased the activation of astrocytes, increased the myelin protein level in the brain cortex, and improved the neurological status of EAE rats by different mechanisms; IFN-β1a reduced iNOS expression, at least in part, by the enhancement of IL-10, while IFN-β1b diminished IL-10 concentration and did not decrease EAE-induced iNOS expression.

  10. Residue Phe112 of the Human-Type Corrinoid Adenosyltransferase (PduO) Enzyme of Lactobacillus reuteri Is Critical to the Formation of the Four-Coordinate Co(II) Corrinoid Substrate and to the Activity of the Enzyme

    Energy Technology Data Exchange (ETDEWEB)

    Mera, Paola E.; St. Maurice, Martin; Rayment, Ivan; Escalante-Semerena, Jorge C.; UW

    2009-06-08

    ATP:Corrinoid adenosyltransferases (ACAs) catalyze the transfer of the adenosyl moiety from ATP to cob(I)alamin via a four-coordinate cob(II)alamin intermediate. At present, it is unknown how ACAs promote the formation of the four-coordinate corrinoid species needed for activity. The published high-resolution crystal structure of the ACA from Lactobacillus reuteri (LrPduO) in complex with ATP and cob(II)alamin shows that the environment around the alpha face of the corrin ring consists of bulky hydrophobic residues. To understand how these residues promote the generation of the four-coordinate cob(II)alamin, variants of the human-type ACA enzyme from L. reuteri (LrPduO) were kinetically and structurally characterized. These studies revealed that residue Phe112 is critical in the displacement of 5,6-dimethylbenzimidazole (DMB) from its coordination bond with the Co ion of the ring, resulting in the formation of the four-coordinate species. An F112A substitution resulted in a 80% drop in the catalytic efficiency of the enzyme. The explanation for this loss of activity was obtained from the crystal structure of the mutant protein, which showed cob(II)alamin bound in the active site with DMB coordinated to the cobalt ion. The crystal structure of an LrPduO(F112H) variant showed a DMB-off/His-on interaction between the corrinoid and the enzyme, whose catalytic efficiency was 4 orders of magnitude lower than that of the wild-type protein. The analysis of the kinetic parameters of LrPduO(F112H) suggests that the F112H substitution negatively impacts product release. Substitutions of other hydrophobic residues in the Cbl binding pocket did not result in significant defects in catalytic efficiency in vitro; however, none of the variant enzymes analyzed in this work supported AdoCbl biosynthesis in vivo.

  11. A Habermasian Approach to Critical Reading

    Science.gov (United States)

    Lee, Cheu-jey

    2016-01-01

    This article explores the connection between critical reading and Jurgen Habermas's theory of communicative action. It proposes that Habermas's criteria used for evaluating validity claims in communicative action can be applied in reading texts critically. Analyses of different types of texts are presented to show how critical reading is done in a…

  12. Critical behavior of collapsing surfaces

    DEFF Research Database (Denmark)

    Olsen, Kasper; Sourdis, C.

    2009-01-01

    We consider the mean curvature evolution of rotationally symmetric surfaces. Using numerical methods, we detect critical behavior at the threshold of singularity formation resembling that of gravitational collapse. In particular, the mean curvature simulation of a one-parameter family of initial...... data reveals the existence of a critical initial surface that develops a degenerate neckpinch. The limiting flow of the type II singularity is accurately modeled by the rotationally symmetric translating soliton....

  13. Critical Zen art history

    Directory of Open Access Journals (Sweden)

    Gregory P. A. Levin

    2016-12-01

    Full Text Available This essay sketches a history of the study of Zen art from the late nineteenth century to post-war reconsiderations, leading towards what I term “critical Zen art studies.” The latter, I suggest, has been undertaken by historians of art and others to challenge normative definitions of Zen art based on modern constructs, revise understanding of the types and functions of visual art important to Chan/Sŏn/Zen Buddhist monasteries, and study iconographies and forms not as a transparent aesthetic indices to Zen Mind or No Mind but as rhetorically, ritually, and socially complex, even unruly, events of representation.

  14. Síndrome de microdeleción en la neurofibromatosis tipo- 1: presentación de un caso Microdeletion syndrome in neurofibromatosis type-1: a case report

    Directory of Open Access Journals (Sweden)

    Miladys Orraca Castillo

    2011-12-01

    Full Text Available La neurofibromatosis tipo 1 es una enfermedad genética neuroectodérmica, en la que han sido descritas diferentes tipos de mutaciones en el gen NF1, cuyo locus está en el cromosoma 17 y en este mapean miles de genes; algunos de ellos se encuentran en regiones muy próximas al gen de la enfermedad. En este trabajo se revisó la literatura médica sobre el tema y presenta un caso con características clínicas propias del síndrome de microdeleción del gen NF1, constituyendo un caso novedoso para la ciencia médica cubana.Neurofibromatosis type-1 is a neuroectodermic genetic condition, where different types of mutations in NF1 gene have been described. Its locus is located at chromosome-17, thousand ALIGN="JUSTIFY">of genes are mapping, and some of them are found in very near regions of the gene of this disease. Medical literature about the topic was reviewed; a case presenting the exact clinical characteristics of a microdeletion syndrome of NF1 gene is reported, which constitutes a new case to the Cuban medical science.

  15. OsLOL1, a C2C2-type zinc finger protein, interacts with OsbZIP58 to promote seed germination through the modulation of gibberellin biosynthesis in Oryza sativa.

    Science.gov (United States)

    Wu, Jiahe; Zhu, Chuanfeng; Pang, Jinhuan; Zhang, Xiangrong; Yang, Chunlin; Xia, Guixian; Tian, Yingchuan; He, Chaozu

    2014-12-01

    Seed germination is a key developmental process in the plant life cycle that is influenced by various environmental cues and phytohormones through gene expression and a series of metabolism pathways. In the present study, we investigated a C2C2-type finger protein, OsLOL1, which promotes gibberellin (GA) biosynthesis and affects seed germination in Oryza sativa (rice). We used OsLOL1 antisense and sense transgenic lines to explore OsLOL1 functions. Seed germination timing in antisense plants was restored to wild type when exogenous GA3 was applied. The reduced expression of the GA biosynthesis gene OsKO2 and the accumulation of ent-kaurene were observed during germination in antisense plants. Based on yeast two-hybrid and firefly luciferase complementation analyses, OsLOL1 interacted with the basic leucine zipper protein OsbZIP58. The results from electrophoretic mobility shift and dual-luciferase reporter assays showed that OsbZIP58 binds the G-box cis-element of the OsKO2 promoter and activates LUC reporter gene expression, and that interaction between OsLOL1 and OsbZIP58 activates OsKO2 gene expression. In addition, OsLOL1 decreased SOD1 gene expression and accelerated programmed cell death (PCD) in the aleurone layer of rice grains. These findings demonstrate that the interaction between OsLOL1 and OsbZIP58 influences GA biosynthesis through the activation of OsKO2 via OsbZIP58, thereby stimulating aleurone PCD and seed germination. © 2014 The Authors The Plant Journal © 2014 John Wiley & Sons Ltd.

  16. GhZFP1, a novel CCCH-type zinc finger protein from cotton, enhances salt stress tolerance and fungal disease resistance in transgenic tobacco by interacting with GZIRD21A and GZIPR5.

    Science.gov (United States)

    Guo, Ying-Hui; Yu, Yue-Ping; Wang, Dong; Wu, Chang-Ai; Yang, Guo-Dong; Huang, Jin-Guang; Zheng, Cheng-Chao

    2009-01-01

    * Zinc finger proteins are a superfamily involved in many aspects of plant growth and development. However, CCCH-type zinc finger proteins involved in plant stress tolerance are poorly understood. * A cDNA clone designated Gossypium hirsutum zinc finger protein 1 (GhZFP1), which encodes a novel CCCH-type zinc finger protein, was isolated from a salt-induced cotton (G. hirsutum) cDNA library using differential hybridization screening and further studied in transgenic tobacco Nicotiana tabacum cv. NC89. Using yeast two-hybrid screening (Y2H), proteins GZIRD21A (GhZFP1 interacting and responsive to dehydration protein 21A) and GZIPR5 (GhZFP1 interacting and pathogenesis-related protein 5), which interacted with GhZFP1, were isolated. * GhZFP1 contains two typical zinc finger motifs (Cx8Cx5Cx3H and Cx5Cx4Cx3H), a putative nuclear export sequence (NES) and a potential nuclear localization signal (NLS). Transient expression analysis using a GhZFP1::GFP fusion gene in onion epidermal cells indicated a nuclear localization for GhZFP1. RNA blot analysis showed that the GhZFP1 transcript was induced by salt (NaCl), drought and salicylic acid (SA). The regions in GhZFP1 that interact with GZIRD21A and GZIPR5 were identified using truncation mutations. * Overexpression of GhZFP1 in transgenic tobacco enhanced tolerance to salt stress and resistance to Rhizoctonia solani. Therefore, it appears that GhZFP1 might be involved as an important regulator in plant responses to abiotic and biotic stresses.

  17. Dictionary criticism and lexicographical function theory

    DEFF Research Database (Denmark)

    Tarp, Sven

    2017-01-01

    This contribution discusses dictionary criticism in the light of the function theory. It starts analyzing the objective of dictionary criticism and lists eight of the most important purposes with which criticism has been made by supporters of the function theory. It then discusses the two main...... types of dictionary criticism, namely criticism of other authors’ dictionaries and self-criticism of one’s own dictionaries. Based on this discussion, it proceeds to a definition of the concept of dictionary criticism which is above all considered a theory-based activity, the outcome of which may...... by the supporters of the function theory, and the way it could be presented in order to create debate. Finally, the contribution indicates the important role dictionary criticism has had in the development of the function theory and endorses an open and critical discussion culture within lexicography....

  18. {sup 18}F-FBPA as a tumor-specific probe of L-type amino acid transporter 1 (LAT1): a comparison study with {sup 18}F-FDG and {sup 11}C-Methionine PET

    Energy Technology Data Exchange (ETDEWEB)

    Watabe, Tadashi [Osaka University Graduate School of Medicine, Department of Nuclear Medicine and Tracer Kinetics, Osaka (Japan); Osaka University Graduate School of Medicine, PET Molecular Imaging Center, Osaka (Japan); Ikeda, Hayato; Aoki, Masanao [Osaka University Graduate School of Medicine, Department of Nuclear Medicine and Tracer Kinetics, Osaka (Japan); Nagamori, Shushi; Wiriyasermkul, Pattama; Tanaka, Yoko; Hagiwara, Kohei; Kanai, Yoshikatsu [Osaka University Graduate School of Medicine, Department of Bio-system Pharmacology, Osaka (Japan); Naka, Sadahiro [Osaka University, Osaka University Graduate School of Medicine, Osaka University Hospital, Osaka (Japan); Kanai, Yasukazu [Osaka University Graduate School of Medicine, PET Molecular Imaging Center, Osaka (Japan); Osaka University Graduate School of Medicine, Department of Molecular Imaging in Medicine, Osaka (Japan); Shimosegawa, Eku [Osaka University Graduate School of Medicine, Department of Nuclear Medicine and Tracer Kinetics, Osaka (Japan); Osaka University Graduate School of Medicine, PET Molecular Imaging Center, Osaka (Japan); Osaka University, Osaka University Graduate School of Medicine, Osaka University Hospital, Osaka (Japan); Hatazawa, Jun [Osaka University Graduate School of Medicine, Department of Nuclear Medicine and Tracer Kinetics, Osaka (Japan); Osaka University Graduate School of Medicine, PET Molecular Imaging Center, Osaka (Japan); Osaka University, Immunology Frontier Research Center, Osaka (Japan)

    2017-02-15

    The purpose of this study was to evaluate the usefulness of L-4-borono-2-{sup 18}F-fluoro-phenylalanine ({sup 18}F-FBPA) as a tumor-specific probe, in comparison to {sup 18}F-FDG and {sup 11}C-methionine (Met), focusing on its transport selectivity by L-type amino acid transporter 1 (LAT1), which is highly upregulated in cancers. Cellular analyses of FBPA were performed to evaluate the transportability and K{sub m} value. PET studies were performed in rat xenograft models of C6 glioma (n = 12) and in rat models of turpentine oil-induced subcutaneous inflammation (n = 9). The kinetic parameters and uptake values on static PET images were compared using the one-tissue compartment model (K{sub 1}, k{sub 2}) and maximum standardized uptake value (SUVmax). The cellular analyses showed that FBPA had a lower affinity to a normal cell-type transporter LAT2 and induced less efflux through LAT2 among FBPA, Met, and BPA, while the efflux through LAT1 induced by FBPA was similar among the three compounds. The K{sub m} value of {sup 18}F-FBPA for LAT1 (196.8 ± 11.4 μM) was dramatically lower than that for LAT2 (2813.8 ± 574.5 μM), suggesting the higher selectivity of {sup 18}F-FBPA for LAT1. K{sub 1} and k{sub 2} values were significantly smaller in {sup 18}F-FBPA PET (K{sub 1} = 0.04 ± 0.01 ml/ccm/min and k{sub 2} = 0.07 ± 0.01 /min) as compared to {sup 11}C-Met PET (0.22 ± 0.09 and 0.52 ± 0.10, respectively) in inflammatory lesions. Static PET analysis based on the SUVmax showed significantly higher accumulation of {sup 18}F-FDG in the tumor and inflammatory lesions (7.2 ± 2.1 and 4.6 ± 0.63, respectively) as compared to both {sup 18}F-FBPA (3.2 ± 0.40 and 1.9 ± 0.19) and {sup 11}C-Met (3.4 ± 0.43 and 1.6 ± 0.11). No significant difference was observed between {sup 18}F-FBPA and {sup 11}C-Met in the static PET images. This study shows the utility of {sup 18}F-FBPA as a tumor-specific probe of LAT1 with low accumulation in the inflammatory lesions. (orig.)

  19. "1"8F-FBPA as a tumor-specific probe of L-type amino acid transporter 1 (LAT1): a comparison study with "1"8F-FDG and "1"1C-Methionine PET

    International Nuclear Information System (INIS)

    Watabe, Tadashi; Ikeda, Hayato; Aoki, Masanao; Nagamori, Shushi; Wiriyasermkul, Pattama; Tanaka, Yoko; Hagiwara, Kohei; Kanai, Yoshikatsu; Naka, Sadahiro; Kanai, Yasukazu; Shimosegawa, Eku; Hatazawa, Jun

    2017-01-01

    The purpose of this study was to evaluate the usefulness of L-4-borono-2-"1"8F-fluoro-phenylalanine ("1"8F-FBPA) as a tumor-specific probe, in comparison to "1"8F-FDG and "1"1C-methionine (Met), focusing on its transport selectivity by L-type amino acid transporter 1 (LAT1), which is highly upregulated in cancers. Cellular analyses of FBPA were performed to evaluate the transportability and K_m value. PET studies were performed in rat xenograft models of C6 glioma (n = 12) and in rat models of turpentine oil-induced subcutaneous inflammation (n = 9). The kinetic parameters and uptake values on static PET images were compared using the one-tissue compartment model (K_1, k_2) and maximum standardized uptake value (SUVmax). The cellular analyses showed that FBPA had a lower affinity to a normal cell-type transporter LAT2 and induced less efflux through LAT2 among FBPA, Met, and BPA, while the efflux through LAT1 induced by FBPA was similar among the three compounds. The K_m value of "1"8F-FBPA for LAT1 (196.8 ± 11.4 μM) was dramatically lower than that for LAT2 (2813.8 ± 574.5 μM), suggesting the higher selectivity of "1"8F-FBPA for LAT1. K_1 and k_2 values were significantly smaller in "1"8F-FBPA PET (K_1 = 0.04 ± 0.01 ml/ccm/min and k_2 = 0.07 ± 0.01 /min) as compared to "1"1C-Met PET (0.22 ± 0.09 and 0.52 ± 0.10, respectively) in inflammatory lesions. Static PET analysis based on the SUVmax showed significantly higher accumulation of "1"8F-FDG in the tumor and inflammatory lesions (7.2 ± 2.1 and 4.6 ± 0.63, respectively) as compared to both "1"8F-FBPA (3.2 ± 0.40 and 1.9 ± 0.19) and "1"1C-Met (3.4 ± 0.43 and 1.6 ± 0.11). No significant difference was observed between "1"8F-FBPA and "1"1C-Met in the static PET images. This study shows the utility of "1"8F-FBPA as a tumor-specific probe of LAT1 with low accumulation in the inflammatory lesions. (orig.)

  20. Short-term impact of a stress management and health promotion program on perceived stress, parental stress, health locus of control, and cortisol levels in parents of children and adolescents with diabetes type 1: a pilot randomized controlled trial.

    Science.gov (United States)

    Tsiouli, Eleni; Pavlopoulos, Vassilis; Alexopoulos, Evangelos C; Chrousos, George; Darviri, Christina

    2014-01-01

    Parents of children and adolescents with diabetes type 1 (DT1) usually experience high stress levels, as they have to cope with multiple demands in their everyday life. Different complex interventions have been implemented, which sometimes have led to opposite results. The purpose of this study was to assess stress levels in parents of children and adolescents with DT1 and to evaluate the effectiveness of a stress management program (progressive muscle relaxation combined with diaphragmatic breathing) in reducing perceived and parenting stress, increasing internal locus of control, promoting healthy lifestyle, and normalizing cortisol levels. Randomized controlled trial. A total of 44 parents were randomly assigned to the intervention group (performing relaxation for eight weeks, n = 19) and control group (n = 25). Pre-post measurements included cortisol levels, lifestyle characteristics, perceived stress, perception of health, and parenting stress. A statistically significant decrease in perceived stress (from 27.21 to 19.00, P = .001), as well as in parenting stress (from 85.79 to 73.68, P = .003), was observed in the intervention group. A statistically significant difference was found in perceived stress between the two groups after the intervention (Dmean = 6.64, P = .010). No significant difference was revealed between or within the groups in cortisol levels. Significant improvement was reported by the subjects of the intervention group in various lifestyle parameters. Relaxation techniques seem to have a positive impact on stress and on various lifestyle factors in parents of children and adolescents with DT1. Future research on long-term benefits of an intervention program comprising of various relaxation schemes is warranted. Copyright © 2014 Elsevier Inc. All rights reserved.

  1. Biochemical characterization of the triticale TsPAP1, a new type of plant prolyl aminopeptidase, and its impact on proline content and flowering time in transgenic Arabidopsis plants.

    Science.gov (United States)

    Zdunek-Zastocka, Edyta; Grabowska, Agnieszka; Branicki, Tomasz; Michniewska, Beata

    2017-07-01

    Proline aminopeptidase (PAP, EC 3.4.11.5) is the only enzyme that effectively releases proline from the N-termini of peptides. The amino acid sequence of the PAP from Triticosecale, TsPAP1, comprises conserved regions, characteristic of the monomeric forms of PAP found in bacteria but not yet identified in plants. Therefore, we aimed to obtain and biochemically characterize the TsPAP1 protein. The recombinant TsPAP1 protein was received through heterologous expression of the TsPAP1 coding sequence in a bacterial expression system and purified with affinity chromatography. Gel filtration chromatography and SDS electrophoresis revealed that TsPAP1 is a monomer with a molecular mass of 37.5 kDa. TsPAP1 prefers substrates with proline at the N-terminus but is also capable of hydrolyzing β-naphthylamides of hydroxyproline and alanine. Among the peptides tested, the most preferred were di- and tripeptides, especially those with glycine in the Y position. The use of diagnostic inhibitors indicated that TsPAP1 is a serine peptidase; however, further characterization revealed that the SH residues are also important for maintaining its activity. To examine the role of TsPAP1 under physiological conditions, we developed transgenic Arabidopsis plants overexpressing TsPAP1. Compared with wild-type plants, the transgenic lines accumulated more proline, flowered an average of 3.5 days earlier, and developed more siliques than did untransformed controls. Our paper is the first to describe the biochemical properties of a novel monomeric plant PAP and contributes to the functional characterization of PAP proteins in plants. Copyright © 2017 Elsevier Masson SAS. All rights reserved.

  2. Causes of death and prognostic factors in multiple endocrine neoplasia type 1: a prospective study: comparison of 106 MEN1/Zollinger-Ellison syndrome patients with 1613 literature MEN1 patients with or without pancreatic endocrine tumors.

    Science.gov (United States)

    Ito, Tetsuhide; Igarashi, Hisato; Uehara, Hirotsugu; Berna, Marc J; Jensen, Robert T

    2013-05-01

    Multiple endocrine neoplasia type 1 (MEN1) is classically characterized by the development of functional or nonfunctional hyperplasia or tumors in endocrine tissues (parathyroid, pancreas, pituitary, adrenal). Because effective treatments have been developed for the hormone excess state, which was a major cause of death in these patients in the past, coupled with the recognition that nonendocrine tumors increasingly develop late in the disease course, the natural history of the disease has changed. An understanding of the current causes of death is important to tailor treatment for these patients and to help identify prognostic factors; however, it is generally lacking.To add to our understanding, we conducted a detailed analysis of the causes of death and prognostic factors from a prospective long-term National Institutes of Health (NIH) study of 106 MEN1 patients with pancreatic endocrine tumors with Zollinger-Ellison syndrome (MEN1/ZES patients) and compared our results to those from the pooled literature data of 227 patients with MEN1 with pancreatic endocrine tumors (MEN1/PET patients) reported in case reports or small series, and to 1386 patients reported in large MEN1 literature series. In the NIH series over a mean follow-up of 24.5 years, 24 (23%) patients died (14 MEN1-related and 10 non-MEN1-related deaths). Comparing the causes of death with the results from the 227 patients in the pooled literature series, we found that no patients died of acute complications due to acid hypersecretion, and 8%-14% died of other hormone excess causes, which is similar to the results in 10 large MEN1 literature series published since 1995. In the 2 series (the NIH and pooled literature series), two-thirds of patients died from an MEN1-related cause and one-third from a non-MEN1-related cause, which agrees with the mean values reported in 10 large MEN1 series in the literature, although in the literature the causes of death varied widely. In the NIH and pooled literature

  3. Nuclear criticality safety guide

    International Nuclear Information System (INIS)

    Pruvost, N.L.; Paxton, H.C.

    1996-09-01

    This technical reference document cites information related to nuclear criticality safety principles, experience, and practice. The document also provides general guidance for criticality safety personnel and regulators

  4. Nuclear criticality safety guide

    Energy Technology Data Exchange (ETDEWEB)

    Pruvost, N.L.; Paxton, H.C. [eds.

    1996-09-01

    This technical reference document cites information related to nuclear criticality safety principles, experience, and practice. The document also provides general guidance for criticality safety personnel and regulators.

  5. Super critical water reactors

    International Nuclear Information System (INIS)

    Dumaz, P.; Antoni, O; Arnoux, P.; Bergeron, A; Renault, C.; Rimpault, G.

    2005-01-01

    Water is used as a calori-porter and moderator in the most major nuclear centers which are actually in function. In the pressurized water reactor (PWR) and boiling water reactor (BWR), water is maintained under critical point of water (21 bar, 374 Centigrade) which limits the efficiency of thermodynamic cycle of energy conversion (yield gain of about 33%) Crossing the critical point, one can then use s upercritical water , the obtained pressure and temperature allow a significant yield gains. In addition, the supercritical water offers important properties. Particularly there is no more possible coexistence between vapor and liquid. Therefore, we don't have more boiling problem, one of the phenomena which limits the specific power of PWR and BWR. Since 1950s, the reactor of supercritical water was the subject of studies more or less detailed but neglected. From the early 1990s, this type of conception benefits of some additional interests. Therefore, in the international term G eneration IV , the supercritical water reactors had been considered as one of the big options for study as Generation IV reactors. In the CEA, an active city has engaged from 1930 with the participation to a European program: The HPWR (High Performance Light Water Reactor). In this contest, the R and D studies are focused on the fields of neutrons, thermodynamic and materials. The CEA intends to pursue a limited effort of R and D in this field, in the framework of international cooperation, preferring the study of versions of rapid spectrum. (author)

  6. Identification of HNF4A Mutation p.T130I and HNF1A Mutations p.I27L and p.S487N in a Han Chinese Family with Early-Onset Maternally Inherited Type 2 Diabetes

    Directory of Open Access Journals (Sweden)

    Ying Yang

    2016-01-01

    Full Text Available Maturity-onset diabetes of the young (MODY is characterized by the onset of diabetes before the age of 25 years, positive family history, high genetic predisposition, monogenic mutations, and an autosomal dominant mode of inheritance. Here, we aimed to investigate the mutations and to characterize the phenotypes of a Han Chinese family with early-onset maternally inherited type 2 diabetes. Detailed clinical assessments and genetic screening for mutations in the HNF4α, GCK, HNF-1α, IPF-1, HNF1β, and NEUROD1 genes were carried out in this family. One HNF4A mutation (p.T130I and two HNF1A polymorphisms (p.I27L and p.S487N were identified. Mutation p.T130I was associated with both early-onset and late-onset diabetes and caused downregulated HNF4A expression, whereas HNF1A polymorphisms p.I27L and p.S487N were associated with the age of diagnosis of diabetes. We demonstrated that mutation p.T130I in HNF4A was pathogenic as were the predicted polymorphisms p.I27L and p.S487N in HNF1A by genetic and functional analysis. Our results show that mutations in HNF4A and HNF1A genes might account for this early-onset inherited type 2 diabetes.

  7. The vaccines for Bovine Herpesvirus Type 1: A review

    African Journals Online (AJOL)

    Administrator

    2011-09-05

    Sep 5, 2011 ... disease, causing great economic losses in the livestock industry. Vaccine is a ... virus vaccines. Key words: BoHV-1, vaccine, DIVA, recombinant virus. ... may cause latent infection and immune suppression. (Yates, 1982).

  8. Prevention of criticality accidents

    International Nuclear Information System (INIS)

    Canavese, S.I.

    1982-01-01

    These notes used in the postgraduate course on Radiological Protection and Nuclear Safety discuss macro-and microscopic nuclear constants for fissile materials systems. Critical systems: their definition; criteria to analyze the critical state; determination of the critical size; analysis of practical problems about prevention of criticality. Safety of isolated units and of sets of units. Application of standards. Conception of facilities from the criticality control view point. (author) [es

  9. Facts about Type 2

    Medline Plus

    Full Text Available ... Us in the Fight for a Cure Your tax-deductible gift today can fund critical diabetes research ... Type 2 Diabetes Program Food & Fitness Food Recipes Planning Meals What Can I Eat Weight Loss Fitness ...

  10. WHSC1, a 90 kb SET domain-containing gene, expressed in early development and homologous to a Drosophila dysmorphy gene maps in the Wolf-Hirschhorn syndrome critical region and is fused to IgH in t(4;14) multiple myeloma

    NARCIS (Netherlands)

    Stec, I.; Wright, T. J.; van Ommen, G. J.; de Boer, P. A.; van Haeringen, A.; Moorman, A. F.; Altherr, M. R.; den Dunnen, J. T.

    1998-01-01

    Wolf-Hirschhorn syndrome (WHS) is a malformation syndrome associated with a hemizygous deletion of the distal short arm of chromosome 4 (4p16.3). The smallest region of overlap between WHS patients, the WHS critical region, has been confined to 165 kb, of which the complete sequence is known. We

  11. Enhancing Critical Thinking Skills among Authoritarian Students

    Science.gov (United States)

    Henderson Hurley, Martha; Hurley, David

    2013-01-01

    This article focuses on assignments designed to enhance critical thinking skills for authoritarian personality types. This paper seeks to add to the literature by exploring instructional methods to overcome authoritarian traits that could inhibit the development of critical thinking skills. The article presents a strategy which can be employed…

  12. Chinook Critical Habitat, Coast - NOAA [ds124

    Data.gov (United States)

    California Department of Resources — This layer depicts areas designated for Chinook Critical Habitat as well as habitat type and quality in the California Coastal Evolutionary Significant Unit (ESU -...

  13. Post-Industrial Cultural Criticism

    DEFF Research Database (Denmark)

    Kammer, Aske

    2015-01-01

    hierarchies within journalism. The article maps which Danish websites conduct arts and culture reviews, asks what features these websites have that facilitate public discourse, and measures the actual discussion on the websites. While academic diagnoses of the state of the online public sphere have generally......Integrating perspectives from research into cultural and post-industrial journalism, this article presents a pilot study of websites with reviews of arts and culture conducted by amateurs. Such websites constitute a popular space for cultural criticism, and one that challenges traditional...... reviewers have highly specialized knowledge of culture and, on that basis, argues that the emergence of this type of critic might represent a qualitative strengthening of cultural criticism....

  14. Critical thinking: Not all that critical

    Directory of Open Access Journals (Sweden)

    Bruce Dietrick Price

    2016-09-01

    Full Text Available Critical Thinking basically says to be suspicious of everything, except the fad known as Critical Thinking. It is perhaps best understood as a new and watered-down version of an earlier fad called Deconstruction. That was just a fancy word for debunking. After you strip away all the high-minded rhetoric, Critical Thinking is typically used to tell students that they should not trust conventional wisdom, tradition, religion, parents, and all that irrelevant, old-fashioned stuff. Critical Thinking, somewhat surprisingly, also turns out to be highly contemptuous of facts and knowledge. The formulation in public schools goes like this: children must learn how to think, not what to think. WHAT is, of course, all the academic content and scholarly knowledge that schools used to teach.

  15. Critical/non-critical system methodology report

    International Nuclear Information System (INIS)

    1989-01-01

    The method used to determine how the waste Isolation Pilot Plant (WIPP) facilities/systems were classified as critical or non-critical to the receipt of CH waste is described within this report. All WIPP critical facilities/systems are listed in the Operational Readiness Review Dictionary. Using the Final Safety Analysis Report (FSAR) as a guide to define the boundaries of the facilities/systems, a direct correlation of the ORR Dictionary to the FSAR can be obtained. The critical facilities/systems are those which are directly related to or have a critical support role in the receipt of CH waste. The facility/systems must meet one of the following requirements to be considered critical: (a) confinement or measure of the release of radioactive materials; (b) continued receipt and/or storage of transuranic waste (TRU) without an interruption greater than one month according to the shipping plan schedule; (c) the environmental and occupational safety of personnel meets the established site programs; and (d) the physical security of the WIPP facilities

  16. California Condor Critical Habitat

    Data.gov (United States)

    California Natural Resource Agency — These Data identify (in general) the areas where critical habitat for the California Condor occur. Critical habitat for the species consists of the following 10...

  17. Surgical Critical Care Initiative

    Data.gov (United States)

    Federal Laboratory Consortium — The Surgical Critical Care Initiative (SC2i) is a USU research program established in October 2013 to develop, translate, and validate biology-driven critical care....

  18. Critical Care Team

    Science.gov (United States)

    ... often uphold the patient's wishes. The critical care nurse becomes an important part of decision-making with the patient, the family and the care team. A registered nurse (RN) who is certified in critical care is ...

  19. The Ways of Criticism

    NARCIS (Netherlands)

    Krabbe, Erik C. W.; van Laar, Jan Albert

    This paper attempts to systematically characterize critical reactions in argumentative discourse, such as objections, critical questions, rebuttals, refutations, counterarguments, and fallacy charges, in order to contribute to the dialogical approach to argumentation. We shall make use of four

  20. Education by Criticism.

    Science.gov (United States)

    Tanner, Stephen L.

    1986-01-01

    Argues that students should exercise criticism in the classroom, but this criticism should not take the form of mere training in technical skills, indoctrination into a particular conceptual system, or theoretical speculation ungrounded in reality. (SRT)

  1. Reflector-moderated critical assemblies

    International Nuclear Information System (INIS)

    Paxton, H.C.; Jarvis, G.A.; Byers, C.C.

    1975-07-01

    Experiments with reflector-moderated critical assemblies were part of the Rover Program at the Los Alamos Scientific Laboratory (LASL). These assemblies were characterized by thick D 2 O or beryllium reflectors surrounding large cavities that contained highly enriched uranium at low average densities. Because interest in this type of system has been revived by LASL Plasma Cavity Assembly studies, more detailed descriptions of the early assemblies than had been available in the unclassified literature are provided. (U.S.)

  2. Reimagining Critical Theory

    Science.gov (United States)

    Rexhepi, Jevdet; Torres, Carlos Alberto

    2011-01-01

    This paper discusses Critical Theory, a model of theorizing in the field of the political sociology of education. We argue for a "reimagined" Critical Theory to herald an empowering, liberatory education that fosters curiosity and critical thinking, and a means for successful bottom-up, top-down political engagement. We present arguments…

  3. Foundations for Critical Thinking

    Science.gov (United States)

    Bers, Trudy; Chun, Marc; Daly, William T.; Harrington, Christine; Tobolowsky, Barbara F.

    2015-01-01

    "Foundations for Critical Thinking" explores the landscape of critical-thinking skill development and pedagogy through foundational chapters and institutional case studies involving a range of students in diverse settings. By establishing a link between active learning and improved critical thinking, this resource encourages all higher…

  4. Creating a Critical Thinker

    Science.gov (United States)

    Piergiovanni, Polly R.

    2014-01-01

    A college education is expected to improve students' critical thinking skills. Keeping students active in class--through writing activities and class discussion--has been shown to help students think critically. In this article, creative hands-on activities, which are common in engineering courses, are shown to improve students' critical thinking…

  5. International critical perspectives

    NARCIS (Netherlands)

    Sambrook, S.A.; Poell, R.F.

    2014-01-01

    The Problem Critical perspectives on human resource development (HRD) have emerged, across Europe and America, hailed as the future of the field. However, we note the paucity of critical perspectives globally, the problematic dominance of critical HRD activities in Western sites of theory and

  6. Dynamic critical behaviour and scaling

    International Nuclear Information System (INIS)

    Oezoguz, B.E.

    2001-01-01

    Traditionally the scaling is the property of dynamical systems at thermal equilibrium. In second order phase transitions scaling behaviour is due to the infinite correlation length around the critical point. In first order phase transitions however, the correlation length remains finite and a different type of scaling can be observed. For first order phase transitions all singularities are governed by the volume of the system. Recently, a different type of scaling, namely dynamic scaling has attracted attention in second order phase transitions. In dynamic scaling, when a system prepared at high temperature is quenched to the critical temperature, it exhibits scaling behaviour. Dynamic scaling has been applied to various spin systems and the validity of the arguments are shown. Firstly, in this thesis project the dynamic scaling is applied to 4-dimensional using spin system which exhibits second order phase transition with mean-field critical indices. Secondly, it is shown that although the dynamic is quite different, first order phase transitions also has a different type of dynamic scaling

  7. Transportable criticality alarm system

    International Nuclear Information System (INIS)

    Clem, W.E.

    1988-09-01

    The Transportable Criticality Alarm System was developed at the Hanford Site in 1982 to comply with the requirements of US Department of Energy Order DOE 5480.1, 12/18/80, and ANSI/ANS-8.3- 1979. The portable unit that it replaced failed to comply with the new requirements in that it did not provide the necessary warning of malfunctions, nor did it provide the Hanford Site standard criticality alarm signal. Modern technology allowed the Transportable Criticality Alarm System to comply with the criticality requirements cited and to incorporate other features that make it more usable, maintainable, and reliable. The Transportable Criticality Alarm System (TCAS) provides temporary criticality coverage in manned areas where the facility criticality alarm system is not operable. This gamma radiation-sensitive system has been in use for the past 6 yr at the Hanford Site. 2 refs., 4 figs., 1 tab

  8. Criticality handbook. Pt. 1

    International Nuclear Information System (INIS)

    Heinicke, W.; Krug, H.; Thomas, W.; Weber, W.; Gmal, B.

    1985-12-01

    The GRS Criticality Handbook is intended as a source of information on criticality problems for the persons concerned in industry, authorities, or research laboratories. It is to serve as a guide allowing quick and appropriate evaluation of criticality problems during design or erection of nuclear installations. This present issue replaces the one published in 1979, presenting revised and new data in a modified construction, but within the framework of the proven basic structure of the Handbook. Some fundamental knowledge is required of criticality problems and the relevant terms and definitions of nuclear safety, in order to fully deploy the information given. Part 1 of the Handbook therefore first introduces terminology and definitions, followed by experimental methods and calculation models for criticality calculations. The next chapters deal with the function and efficiency of neutron reflectors and neutron absorbers, measuring methods for criticality monitoring, organisational safety measures, and criticality accidents and their subsequent analysis. (orig./HP) [de

  9. From Critical Theory to Critical Hermeneutics

    Directory of Open Access Journals (Sweden)

    Øjvind Larsen

    2014-06-01

    Full Text Available From their beginning in the 1930s, critical theory and the Frankfurt school had their focus on a critique of disturbed social relations in western society dominated by totalitarian political regimes like Stalinism, Fascism, Nazism, and by capitalism as an oppressive and destructive economic system and culture. Now, 80 years later, this has all become history and thus it is time to leave the concept of critical theory behind us, and instead bring the concept of critique to a broader theoretical framework like hermeneutics. This allows the possibility of retaining the theoretical intentions of the old Frankfurt school and at the same time there will be no boundaries by specific dominant theoretical perspectives. In this paper, such a framework for a critical hermeneutics is discussed on the basis of Weber’s, Gadamer’s, and Habermas’ theories on hermeneutics within the social sciences.

  10. The incretin effect in critically ill patients

    DEFF Research Database (Denmark)

    Nielsen, Signe Tellerup; Janum, Susanne; Krogh-Madsen, Rikke

    2015-01-01

    INTRODUCTION: Patients admitted to the intensive care unit often develop hyperglycaemia, but the underlying mechanisms have not been fully described. The incretin effect is reduced in patients with type 2 diabetes. Type 2 diabetes and critical illness have phenotypical similarities, such as hyper...

  11. Critical Psychologies for Critical Health Literacies

    Science.gov (United States)

    Corcoran, Tim

    2014-01-01

    Health education is largely informed by psychological theories and practices that pursue reductionist views of people learning. However, critical attention is moving to understand health in ways that reconsider relationships to context and the forms of life within which everyday living takes place. This shift is apparent in theoretical…

  12. Surveillance and Critical Theory

    Directory of Open Access Journals (Sweden)

    Christian Fuchs

    2015-09-01

    Full Text Available In this comment, the author reflects on surveillance from a critical theory approach, his involvement in surveillance research and projects, and the status of the study of surveillance. The comment ascertains a lack of critical thinking about surveillance, questions the existence of something called “surveillance studies” as opposed to a critical theory of society, and reflects on issues such as Edward Snowden’s revelations, and Foucault and Marx in the context of surveillance.

  13. Extending Critical Performativity

    DEFF Research Database (Denmark)

    Spicer, André; Alvesson, Mats; Kärreman, Dan

    2016-01-01

    In this article we extend the debate about critical performativity. We begin by outlining the basic tenets of critical performativity and how this has been applied in the study of management and organization. We then address recent critiques of critical performance. We note these arguments suffer...... of public importance; engaging with non-academic groups using dialectical reasoning; scaling up insights through movement building; and propagating deliberation...

  14. Nuclear criticality predictability

    International Nuclear Information System (INIS)

    Briggs, J.B.

    1999-01-01

    As a result of lots of efforts, a large portion of the tedious and redundant research and processing of critical experiment data has been eliminated. The necessary step in criticality safety analyses of validating computer codes with benchmark critical data is greatly streamlined, and valuable criticality safety experimental data is preserved. Criticality safety personnel in 31 different countries are now using the 'International Handbook of Evaluated Criticality Safety Benchmark Experiments'. Much has been accomplished by the work of the ICSBEP. However, evaluation and documentation represents only one element of a successful Nuclear Criticality Safety Predictability Program and this element only exists as a separate entity, because this work was not completed in conjunction with the experimentation process. I believe; however, that the work of the ICSBEP has also served to unify the other elements of nuclear criticality predictability. All elements are interrelated, but for a time it seemed that communications between these elements was not adequate. The ICSBEP has highlighted gaps in data, has retrieved lost data, has helped to identify errors in cross section processing codes, and has helped bring the international criticality safety community together in a common cause as true friends and colleagues. It has been a privilege to associate with those who work so diligently to make the project a success. (J.P.N.)

  15. Critical point predication device

    International Nuclear Information System (INIS)

    Matsumura, Kazuhiko; Kariyama, Koji.

    1996-01-01

    An operation for predicting a critical point by using a existent reverse multiplication method has been complicated, and an effective multiplication factor could not be plotted directly to degrade the accuracy for the prediction. The present invention comprises a detector counting memory section for memorizing the counting sent from a power detector which monitors the reactor power, a reverse multiplication factor calculation section for calculating the reverse multiplication factor based on initial countings and current countings of the power detector, and a critical point prediction section for predicting the criticality by the reverse multiplication method relative to effective multiplication factors corresponding to the state of the reactor core previously determined depending on the cases. In addition, a reactor core characteristic calculation section is added for analyzing an effective multiplication factor depending on the state of the reactor core. Then, if the margin up to the criticality is reduced to lower than a predetermined value during critical operation, an alarm is generated to stop the critical operation when generation of a period of more than a predetermined value predicted by succeeding critical operation. With such procedures, forecasting for the critical point can be easily predicted upon critical operation to greatly mitigate an operator's burden and improve handling for the operation. (N.H.)

  16. Introduction: Critical Visual Theory

    Directory of Open Access Journals (Sweden)

    Peter Ludes

    2014-03-01

    Full Text Available The studies selected for publication in this special issue on Critical Visual Theory can be divided into three thematic groups: (1 image making as power making, (2 commodification and recanonization, and (3 approaches to critical visual theory. The approaches to critical visual theory adopted by the authors of this issue may be subsumed under the following headings (3.1 critical visual discourse and visual memes in general and Anonymous visual discourse in particular, (3.2 collective memory and gendered gaze, and (3.3 visual capitalism, global north and south.

  17. Benefits and risks of testosterone treatment for hypoactive sexual desire disorder in women: a critical review of studies published in the decades preceding and succeeding the advent of phosphodiesterase type 5 inhibitors

    Directory of Open Access Journals (Sweden)

    Sandra Léa Bonfim Reis

    2014-04-01

    Full Text Available With advancing age, there is an increase in the complaints of a lack of a libido in women and erectile dysfunction in men. The efficacy of phosphodiesterase type 5 inhibitors, together with their minimal side effects and ease of administration, revolutionized the treatment of erectile dysfunction. For women, testosterone administration is the principal treatment for hypoactive sexual desire disorder. We sought to evaluate the use of androgens in the treatment of a lack of libido in women, comparing two periods, i.e., before and after the advent of the phosphodiesterase type 5 inhibitors. We also analyzed the risks and benefits of androgen administration. We searched the Latin-American and Caribbean Health Sciences Literature, Cochrane Library, Excerpta Medica, Scientific Electronic Library Online, and Medline (PubMed databases using the search terms disfunção sexual feminina/female sexual dysfunction, desejo sexual hipoativo/female hypoactive sexual desire disorder, testosterona/testosterone, terapia androgênica em mulheres/androgen therapy in women, and sexualidade/sexuality as well as combinations thereof. We selected articles written in English, Portuguese, or Spanish. After the advent of phosphodiesterase type 5 inhibitors, there was a significant increase in the number of studies aimed at evaluating the use of testosterone in women with hypoactive sexual desire disorder. However, the risks and benefits of testosterone administration have yet to be clarified.

  18. Recommendations about criticality accident dosimetry

    International Nuclear Information System (INIS)

    1975-07-01

    The aims of criticality accident dosimetry and the characteristics peculiar to a critical burst being defined, the requirements to be fulfilled by a dosimetric system applied to this type of measurements are presented. The devices chosen by the C.E.A. Radiation Survey Divisions, simple and cheap, are described along with the main processes to be carried out in order to evaluate doses after an accident. The apparatus necessary for detector counting and the directions for use are presented in detail, allowing standardization of measurements. A set of linear formula enables to obtain, from these measurements, all required informations about neutron fluences and spectra, along with the suitable components of the dose at the irradiated people locations [fr

  19. Casuistry and its communitarian critics.

    Science.gov (United States)

    Kuczewski, Mark G

    1994-06-01

    Communitarian critics have derided case-based reasoning for ignoring the need to arrive at a shared hierarchy of goods prior to case resolution. They claim that such a failure means that casuistry depends on either a naive metaphysical realism or an ethical conventionalism. Casuistry does embrace a certain unobjectionable moral realism and can require appeals to narrative histories, but despite this dependence on the surrounding culture, casuists possess a way to remain critical of society through the concept of practical wisdom and the use of a moral taxonomy. Therefore, casuistry's viability depends upon the existence and employment of this Aristotelian virtue. Furthermore, the casuistry that emerges is a sophisticated type of communitarianism rather than a free-standing method.

  20. Empathy and the Critic

    Science.gov (United States)

    Jurecic, Ann

    2011-01-01

    "Empathy" is a much-discussed term in the humanities these days. While some critics value it and argue that literature desirably promotes it, other critics worry that appeals to this emotion will neglect important matters of social context. In the literature classroom, the best approach is to take time to consider how texts complicate the impulse…

  1. Critical Literacy: Foundational Notes

    Science.gov (United States)

    Luke, Allan

    2012-01-01

    The term "critical literacy" refers to use of the technologies of print and other media of communication to analyze, critique, and transform the norms, rule systems, and practices governing the social fields of everyday life (A. Luke, 2004). Since Freire's (1970) educational projects in Brazil, approaches to critical literacy have been…

  2. Critical Response Protocol

    Science.gov (United States)

    Ellingson, Charlene; Roehrig, Gillian; Bakkum, Kris; Dubinsky, Janet M.

    2016-01-01

    This article introduces the Critical Response Protocol (CRP), an arts-based technique that engages students in equitable critical discourse and aligns with the "Next Generation Science Standards" vision for providing students opportunities for language learning while advancing science learning (NGSS Lead States 2013). CRP helps teachers…

  3. Critical Chain Exercises

    Science.gov (United States)

    Doyle, John Kevin

    2010-01-01

    Critical Chains project management focuses on holding buffers at the project level vs. task level, and managing buffers as a project resource. A number of studies have shown that Critical Chain project management can significantly improve organizational schedule fidelity (i.e., improve the proportion of projects delivered on time) and reduce…

  4. Nuclear criticality safety guide

    International Nuclear Information System (INIS)

    Ro, Seong Ki; Shin, Hee Seong; Park, Seong Won; Shin, Young Joon.

    1997-06-01

    Nuclear criticality safety guide was described for handling, transportation and storage of nuclear fissile materials in this report. The major part of the report was excerpted frp, TID-7016(revision 2) and nuclear criticality safety written by Knief. (author). 16 tabs., 44 figs., 5 refs

  5. Critical Utopian Action Research

    DEFF Research Database (Denmark)

    Nielsen, Birger Steen; Nielsen, Kurt Aagaard

    2016-01-01

    The specific concept of critical utopian action research is presented and discussed, as to its origin, use and potentials. The inspiration from Robert Jungk and his future creating workshops is elaborated.......The specific concept of critical utopian action research is presented and discussed, as to its origin, use and potentials. The inspiration from Robert Jungk and his future creating workshops is elaborated....

  6. Critical Habitat :: NOAA Fisheries

    Science.gov (United States)

    occupied by the species at the time of listing, if they contain physical or biological features essential essential for conservation. Critical Habitat Maps NOTE: The critical habitat maps provided here are for Data Leatherback Turtle (U.S. West Coast) » Biological Report » Economic Report 2012 77 FR 4170 Go to

  7. Self organized criticality

    International Nuclear Information System (INIS)

    Creutz, M.

    1993-03-01

    Self organized criticality refers to the tendency of highly dissipative systems to drive themselves to a critical state. This has been proposed to explain why observed physics often displays a wide disparity of length and time scales. The phenomenon can be studied in simple cellular automaton models

  8. Critical Pedagogy and Faith

    Science.gov (United States)

    Neumann, Jacob W.

    2011-01-01

    Critical pedagogy has often been linked in the literature to faith traditions such as liberation theology, usually with the intent of improving or redirecting it. While recognizing and drawing from those previous linkages, Jacob Neumann goes further in this essay and develops the thesis that critical pedagogy can not just benefit from a connection…

  9. Critical parameters for ammonia

    International Nuclear Information System (INIS)

    Sato, M.; Masui, G.; Uematsu, M.

    2005-01-01

    (p, ρ, T) measurements and visual observations of the meniscus for ammonia were carried out carefully in the critical region over the range of temperatures: -1 K (T - T c ) 0.04 K, and of densities: -19 kg . m -3 (ρ - ρ c ) 19 kg . m -3 by a metal-bellows volumometer with an optical cell. Vapor pressures were also measured at T = (310, 350, and 400) K. The critical parameters of T c and ρ c were determined based on the results of observation of the critical opalescence. The critical pressure p c was determined from the present measurements at T c on the vapor pressure curve. Comparisons of the critical parameters with values given in the literature are presented

  10. Critical parameters for ammonia

    Energy Technology Data Exchange (ETDEWEB)

    Sato, M. [Center for Mechanical Engineering and Applied Mechanics, Keio University, Hiyoshi 3-14-1, Kohoku-ku, Yokohama 223-8522 (Japan); Masui, G. [Center for Mechanical Engineering and Applied Mechanics, Keio University, Hiyoshi 3-14-1, Kohoku-ku, Yokohama 223-8522 (Japan); Uematsu, M. [Center for Mechanical Engineering and Applied Mechanics, Keio University, Hiyoshi 3-14-1, Kohoku-ku, Yokohama 223-8522 (Japan)]. E-mail: uematsu@mech.keio.ac.jp

    2005-09-15

    (p, {rho}, T) measurements and visual observations of the meniscus for ammonia were carried out carefully in the critical region over the range of temperatures: -1 K (T - T {sub c}) 0.04 K, and of densities: -19 kg . m{sup -3} ({rho} - {rho} {sub c}) 19 kg . m{sup -3} by a metal-bellows volumometer with an optical cell. Vapor pressures were also measured at T = (310, 350, and 400) K. The critical parameters of T {sub c} and {rho} {sub c} were determined based on the results of observation of the critical opalescence. The critical pressure p {sub c} was determined from the present measurements at T {sub c} on the vapor pressure curve. Comparisons of the critical parameters with values given in the literature are presented.

  11. Taking Critical Ontology Seriously

    DEFF Research Database (Denmark)

    Wigger, Angela; Horn, Laura

    2017-01-01

    To be ‘critical’ has become fashionable among social scientists in various disciplines. Only a few decades ago, the prefix ‘critical’ was almost automatically associated with Western Marxism and in particular the Frankfurt School. Today, the term critical is no longer limited to a single...... theoretical approach, but pertains to a vast range of approaches, including feminist, reflexive, postcolonial, postmodern or poststructuralist studies, and studies committed to a post-positivist epistemology more generally. But what does critical social science actually mean? Which implications does critical...... a primer on a few core dimensions of critical social science and its central premises. It discusses first what critical social science is not, and clarifies key differences between what is commonly referred to as ‘mainstream’ and ‘critical’ social science perspectives. It addresses the role of normative...

  12. A Tryptophan-Rich Motif in the Human Parainfluenza Virus Type 2 V Protein Is Critical for the Blockade of Toll-Like Receptor 7 (TLR7)- and TLR9-Dependent Signaling▿

    OpenAIRE

    Kitagawa, Yoshinori; Yamaguchi, Mayu; Zhou, Min; Komatsu, Takayuki; Nishio, Machiko; Sugiyama, Tsuyoshi; Takeuchi, Kenji; Itoh, Masae; Gotoh, Bin

    2011-01-01

    Plasmacytoid dendritic cells (pDCs) do not produce alpha interferon (IFN-α) unless viruses cause a systemic infection or overcome the first-line defense provided by conventional DCs and macrophages. We show here that even paramyxoviruses, whose infections are restricted to the respiratory tract, have a V protein able to prevent Toll-like receptor 7 (TLR7)- and TLR9-dependent IFN-α induction specific to pDCs. Mutational analysis of human parainfluenza virus type 2 demonstrates that the second ...

  13. Control of criticality; Kawalan kegentingan

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1988-12-31

    The chapter briefly discussed the following subjects: basic and the principle of criticality, natural uranium, neutron utilization, criticality data for systems, criticality accidents, criticality control i.e. mass, volume and geometry control .

  14. The Possibility of Film Criticism.

    Science.gov (United States)

    Poague, Leland; Cadbury, William

    1989-01-01

    Examines the role of critical language in film criticism. Compares and contrasts Monroe Beardsley's philosophy on film aesthetics with the New Criticism. Outlines some of the contributions Beardsley has made to the study of film criticism. (KM)

  15. Preclosure Criticality Analysis Process Report

    International Nuclear Information System (INIS)

    Thomas, D.A.

    1999-01-01

    The design approach for criticality of the disposal container and waste package will be dictated by existing regulatory requirements. This conclusion is based on the fact that preclosure operations and facilities have significant similarities to existing facilities and operations currently regulated by the NRC. The major difference would be the use of a risk-informed approach with burnup credit. This approach could reduce licensing delays and costs of the repository. The probability of success for this proposed seamless licensing strategy is increased, since there is precedence of regulation (10 CFR Part 63 and NUREG 1520) and commercial precedence for allowing burnup credit at sites similar to Yucca Mountain during preclosure. While NUREG 1520 is not directly applicable to a facility for handling spent nuclear fuel, the risk-informed approach to criticality analysis in NUREG 1520 is considered indicative of how the NRC will approach risk-informed criticality analysis at spent fuel facilities in the future. The types of design basis events which must be considered during the criticality safety analysis portion of the Integrated Safety Analysis (ISA) are those events which result in unanticipated moderation, loss of neutron absorber, geometric changes in the critical system, or administrative errors in waste form placement (loading) of the disposal container. The specific events to be considered must be based on the review of the system's design, as discussed in Section 3.2. A transition of licensing approach (e.g., deterministic versus risk-informed, performance-based) is not obvious and will require analysis. For commercial spent nuclear fuel, the probability of interspersed moderation may be low enough to allow nearly the same Critical Limit for both preclosure and postclosure, though an administrative margin will be applied to preclosure and possibly not to postclosure. Similarly the Design Basis Events for the waste package may be incredible and therefore not

  16. Preclosure Criticality Analysis Process Report

    International Nuclear Information System (INIS)

    Thomas, D.A.

    1999-01-01

    The design approach for criticality of the disposal container and waste package will be dictated by existing regulatory requirements. This conclusion is based on the fact that preclosure operations and facilities have significant similarities to existing facilities and operations currently regulated by the NRC. The major difference would be the use of a risk-informed approach with burnup credit. This approach could reduce licensing delays and costs of the repository. The probability of success for this proposed seamless licensing strategy is increased, since there is precedence of regulation (10 CFR Part 63 and NUREG 1520) and commercial precedence for allowing burnup credit at sites similar to Yucca Mountain during preclosure. While NUREG 1520 is not directly applicable to a facility for handling spent nuclear fuel, the risk-informed approach to criticality analysis in NUREG 1520 is considered indicative of how the NRC will approach risk-informed criticality analysis at spent fuel facilities in the future. The types of design basis events which must be considered during the criticality safety analysis portion of the Integrated Safety Analysis (ISA) are those events which result in unanticipated moderation, loss of neutron absorber, geometric changes in the critical system, or administrative errors in waste form placement (loading) of the disposal container. The specific events to be considered must be based on the review of the system's design, as discussed in Section 3.2. A transition of licensing approach (e.g., deterministic versus risk-informed, performance-based) is not obvious and will require analysis. For commercial spent nuclear fuel, the probability of interspersed moderation may be low enough to allow nearly the same Critical Limit for both preclosure and postclosure, though an administrative margin will be applied to preclosure and possibly not to postclosure. Similarly the Design Basis Events for the waste package may be incredible and therefore not

  17. Human chimera-type galectin-3: defining the critical tail length for high-affinity glycoprotein/cell surface binding and functional competition with galectin-1 in neuroblastoma cell growth regulation.

    Science.gov (United States)

    Kopitz, Jürgen; Vértesy, Sabine; André, Sabine; Fiedler, Sabine; Schnölzer, Martina; Gabius, Hans-Joachim

    2014-09-01

    Many human proteins have a modular design with receptor and structural domains. Using adhesion/growth-regulatory galectin-3 as model, we describe an interdisciplinary strategy to define the functional significance of its tail established by nine non-triple helical collagen-like repeats (I-IX) and the N-terminal peptide. Genetic engineering with sophisticated mass spectrometric product analysis provided the tools for biotesting, i.e. eight protein variants with different degrees of tail truncation. Evidently,various aspects of galectin-3 activity (cis binding and cell bridging) are affected by tail shortening in a different manner. Thus, this combined approach reveals an unsuspected complexity of structure-function relationship, encouraging further application beyond this chimera-type galectin. Copyright © 2014 Elsevier Masson SAS. All rights reserved.

  18. The french criticality handbook

    International Nuclear Information System (INIS)

    Maubert, L.; Puit, J.C.

    1987-01-01

    The french criticality handbook, published in 1978 by the ''Commissariat a l'Energie Atomique'', is presented with the main targets aimed by the writer and the main choices taken relating to fissile mediums, reflection conditions, dilution curves. The validation of the critical values is presented as one of the most important aspects of this handbook which is mainly intended, in the mind of the author, to specialists well advertised in the field of criticality. The complements which have been introduced since 1978 and those which are foreseen in a near future are also detailed. (author)

  19. Frustration and quantum criticality

    Science.gov (United States)

    Vojta, Matthias

    2018-06-01

    This review article is devoted to the interplay between frustrated magnetism and quantum critical phenomena, covering both theoretical concepts and ideas as well as recent experimental developments in correlated-electron materials. The first part deals with local-moment magnetism in Mott insulators and the second part with frustration in metallic systems. In both cases, frustration can either induce exotic phases accompanied by exotic quantum critical points or lead to conventional ordering with unconventional crossover phenomena. In addition, the competition of multiple phases inherent to frustrated systems can lead to multi-criticality.

  20. Frustration and quantum criticality.

    Science.gov (United States)

    Vojta, Matthias

    2018-03-15

    This review article is devoted to the interplay between frustrated magnetism and quantum critical phenomena, covering both theoretical concepts and ideas as well as recent experimental developments in correlated-electron materials. The first part deals with local-moment magnetism in Mott insulators and the second part with frustration in metallic systems. In both cases, frustration can either induce exotic phases accompanied by exotic quantum critical points or lead to conventional ordering with unconventional crossover phenomena. In addition, the competition of multiple phases inherent to frustrated systems can lead to multi-criticality. © 2018 IOP Publishing Ltd.

  1. Minimum critical mass systems

    International Nuclear Information System (INIS)

    Dam, H. van; Leege, P.F.A. de

    1987-01-01

    An analysis is presented of thermal systems with minimum critical mass, based on the use of materials with optimum neutron moderating and reflecting properties. The optimum fissile material distributions in the systems are obtained by calculations with standard computer codes, extended with a routine for flat fuel importance search. It is shown that in the minimum critical mass configuration a considerable part of the fuel is positioned in the reflector region. For 239 Pu a minimum critical mass of 87 g is found, which is the lowest value reported hitherto. (author)

  2. PREPP criticality control

    International Nuclear Information System (INIS)

    Cargo, C.H.; Becker, G.K.; Berglund, G.R.; O'Connor, N.J.

    1989-03-01

    There is a general lack of data available with regards to criticality control in the use of incineration for full scale processing of TRU waste. In addition to the new analytical tools, such as PRA, are only now starting to be applied to criticality control. These factors have made the development of the PREPP Criticality Control Plan somewhat unique. It is hoped that the PREPP experiences gained in these efforts will provide new insight which will be useful to future facilities processing waste material containing fissile material. 1 fig

  3. The evaluation of set of criticality parameters using scale system

    International Nuclear Information System (INIS)

    Abe, Alfredo; Sanchez, Andrea; Yamaguchi, Mistuo

    2009-01-01

    In evaluating the criticality safety of the nuclear fuel facility, it is important to apply a consistent methodology, which consider every aspects concerning various types of criticality parameters. Usually, the critical parameters are compiled and arranged into handbooks, and these handbooks are based on experience with nuclear facilities, experimental data from criticality safety research facilities, and theoretical studies performed using numerical simulations. Most of criticality safety evaluation can be addressed using the criticality parameters data directly from handbook, but some critical parameters for a specific chemical mixtures and/or enrichment are not be available. Consequently, not available parameters has to be evaluated. This work present the methodology to evaluate a set of critical parameters using SCALE system for various types of mixtures present at nuclear fuel cycle facilities for two different level of enrichment, the results are verified in the independent calculation using MCNP Monte Carlo Code. (author)

  4. Designated Critical Habitat

    Data.gov (United States)

    Kansas Data Access and Support Center — Critical habitats include those areas documented as currently supporting self-sustaining populations of any threatened or endangered species of wildlife as well as...

  5. Coastal Critical Habitat Designations

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — The Endangered Species Act (ESA) requires the Federal government to designate critical habitat, areas of habitat essential to the species' conservation, for ESA...

  6. Silencing criticism in Mexico

    Directory of Open Access Journals (Sweden)

    Ximena Suárez

    2017-10-01

    Full Text Available Journalists and human rights defenders in Mexico are being attacked in an attempt to silence their criticism. Many are forced to flee or risk being assassinated. The consequences are both personal and of wider social significance.

  7. Smalltooth Sawfish Critical Habitat

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — These data represent the critical habitat for smalltooth sawfish (Pristis pectinatat) as designated by 74 FR 45353, September 2, 2009, Rules and Regulations.

  8. Critical Robotic Lunar Missions

    Science.gov (United States)

    Plescia, J. B.

    2018-04-01

    Perhaps the most critical missions to understanding lunar history are in situ dating and network missions. These would constrain the volcanic and thermal history and interior structure. These data would better constrain lunar evolution models.

  9. Critical-point nuclei

    International Nuclear Information System (INIS)

    Clark, R.M.

    2004-01-01

    It has been suggested that a change of nuclear shape may be described in terms of a phase transition and that specific nuclei may lie close to the critical point of the transition. Analytical descriptions of such critical-point nuclei have been introduced recently and they are described briefly. The results of extensive searches for possible examples of critical-point behavior are presented. Alternative pictures, such as describing bands in the candidate nuclei using simple ΔK = 0 and ΔK = 2 rotational-coupling models, are discussed, and the limitations of the different approaches highlighted. A possible critical-point description of the transition from a vibrational to rotational pairing phase is suggested

  10. Probabilistic Criticality Consequence Evaluation

    International Nuclear Information System (INIS)

    P. Gottlieb; J.W. Davis; J.R. Massari

    1996-01-01

    This analysis is prepared by the Mined Geologic Disposal System (MGDS) Waste Package Development (WPD) department with the objective of providing a comprehensive, conservative estimate of the consequences of the criticality which could possibly occur as the result of commercial spent nuclear fuel emplaced in the underground repository at Yucca Mountain. The consequences of criticality are measured principally in terms of the resulting changes in radionuclide inventory as a function of the power level and duration of the criticality. The purpose of this analysis is to extend the prior estimates of increased radionuclide inventory (Refs. 5.52 and 5.54), for both internal and external criticality. This analysis, and similar estimates and refinements to be completed before the end of fiscal year 1997, will be provided as input to Total System Performance Assessment-Viability Assessment (TSPA-VA) to demonstrate compliance with the repository performance objectives

  11. Right Whale Critical Habitat

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — These data represent the critical habitat for Right Whale as designated by Federal Register Vol. 59, No. 28805, May 19, 1993, Rules and Regulations.

  12. Johnsons Seagrass Critical Habitat

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — These data represent the critical habitat for Johnson's Seagrass as designated by Federal Register Vol. 65, No. 66, Wednesday, April 5, 2000, Rules and Regulations.

  13. Green Turtle Critical Habitat

    Data.gov (United States)

    National Oceanic and Atmospheric Administration, Department of Commerce — These data represent the critical habitat for green turtle as designated by Federal Register Vol. 63, No. 46701, September 2, 1998, Rules and Regulations.

  14. Neutrophils in critical illness.

    Science.gov (United States)

    McDonald, Braedon

    2018-03-01

    During critical illness, dramatic alterations in neutrophil biology are observed including abnormalities of granulopoeisis and lifespan, cell trafficking and antimicrobial effector functions. As a result, neutrophils transition from powerful antimicrobial protectors into dangerous mediators of tissue injury and organ dysfunction. In this article, the role of neutrophils in the pathogenesis of critical illness (sepsis, trauma, burns and others) will be explored, including pathological changes to neutrophil function during critical illness and the utility of monitoring aspects of the neutrophil phenotype as biomarkers for diagnosis and prognostication. Lastly, we review findings from clinical trials of therapies that target the harmful effects of neutrophils, providing a bench-to-bedside perspective on neutrophils in critical illness.

  15. Scale of Critical Thinking

    OpenAIRE

    Semerci, Nuriye

    2000-01-01

    The main purpose of this study is to develop the scale for critical thinking. The Scale of Critical Thinking was applied to 200 student. In this scale, there are total 55 items, four of which are negative and 51 of which are positive. The KMO (Kaiser-Meyer-Olkin) value is 0.75, the Bartlett test value is 7145.41, and the Cronbach Alpha value is 0.90.

  16. Furthering critical institutionalism

    Directory of Open Access Journals (Sweden)

    Frances Dalton Cleaver

    2015-03-01

    Full Text Available This special issue furthers the study of natural resource management from a critical institutional perspective. Critical institutionalism (CI is a contemporary body of thought that explores how institutions dynamically mediate relationships between people, natural resources and society. It focuses on the complexity of institutions entwined in everyday social life, their historical formation, the interplay between formal and informal, traditional and modern arrangements, and the power relations that animate them. In such perspectives a social justice lens is often used to scrutinise the outcomes of institutional processes. We argue here that critical institutional approaches have potentially much to offer commons scholarship, particularly through the explanatory power of the concept of bricolage for better understanding institutional change.  Critical institutional approaches, gathering momentum over the past 15 years or so, have excited considerable interest but the insights generated from different disciplinary perspectives remain insufficiently synthesised. Analyses emphasising complexity can be relatively illegible to policy-makers, a fact which lessens their reach. This special issue therefore aims to synthesise critical institutional ideas and so to lay the foundation for moving beyond the emergent stage to make meaningful academic and policy impact. In bringing together papers here we define and synthesise key themes of critical institutionalism, outline the concept of institutional bricolage and identity some key challenges facing this school of thought.

  17. The Critical Bricolage

    Directory of Open Access Journals (Sweden)

    Kim McMillan

    2015-10-01

    Full Text Available Change in health care has become rapid and continuous. Much decision-making processes guiding change management are derived from organizational literature which is heavily reflective of managerial perspectives. These perspectives represent and aim to serve only a small subgroup of organizational members. However, change is complex, fragile, and has higher rates of success and sustainability when change management strategies reflect a multitude of organizational voices. There is a dearth of literature exploring the intersect between organizational and nursing discourses on the subject of rapid and continuous change in health care. Multitheoretical, multimethodological, and multidisciplinary informed approaches to methodological decision making are needed to link organizational and nursing discourses in ways that will offer alternative perspectives on the subject of change. Furthermore, critically guided multitheoretical, multimethodological, and multidisciplinary methodologies are timely and important in organizational research. Critically guided research seeks to analyze taken-for-granted assumptions and institutionalized practices seeking alternative perspectives and alternative sources of organizational knowledge. Exploring alternative perspectives from a critical lens recognizes the impact predominant discursive influences have on change management and the subsequent impact on organizational members’ working lives. This article will explore how Kincheloe’s discussions of the critical bricolage serve to support combining critical organizational methodologies (guided by Alvesson and Deetz with a voice-centered relational method of data analysis (guided by Brown and Gilligan to create a critical interpretive methodology that explores the voices of nurses as they experience rapid and continuous change in health care.

  18. Down-regulation of Wild-type p53-induced Phosphatase 1 (Wip1) Plays a Critical Role in Regulating Several p53-dependent Functions in Premature Senescent Tumor Cells*

    Science.gov (United States)

    Crescenzi, Elvira; Raia, Zelinda; Pacifico, Francesco; Mellone, Stefano; Moscato, Fortunato; Palumbo, Giuseppe; Leonardi, Antonio

    2013-01-01

    Premature or drug-induced senescence is a major cellular response to chemotherapy in solid tumors. The senescent phenotype develops slowly and is associated with chronic DNA damage response. We found that expression of wild-type p53-induced phosphatase 1 (Wip1) is markedly down-regulated during persistent DNA damage and after drug release during the acquisition of the senescent phenotype in carcinoma cells. We demonstrate that down-regulation of Wip1 is required for maintenance of permanent G2 arrest. In fact, we show that forced expression of Wip1 in premature senescent tumor cells induces inappropriate re-initiation of mitosis, uncontrolled polyploid progression, and cell death by mitotic failure. Most of the effects of Wip1 may be attributed to its ability to dephosphorylate p53 at Ser15 and to inhibit DNA damage response. However, we also uncover a regulatory pathway whereby suppression of p53 Ser15 phosphorylation is associated with enhanced phosphorylation at Ser46, increased p53 protein levels, and induction of Noxa expression. On the whole, our data indicate that down-regulation of Wip1 expression during premature senescence plays a pivotal role in regulating several p53-dependent aspects of the senescent phenotype. PMID:23612976

  19. Identifying critical thinking indicators and critical thinker attributes in nursing practice.

    Science.gov (United States)

    Chao, Shu-Yuan; Liu, Hsing-Yuan; Wu, Ming-Chang; Clark, Mary Jo; Tan, Jung-Ying

    2013-09-01

    Critical thinking is an essential skill in the nursing process. Although several studies have evaluated the critical thinking skills of nurses, there is limited information related to the indicators of critical thinking or evaluation of critical thinking in the context of the nursing process. This study investigated the potential indicators of critical thinking and the attributes of critical thinkers in clinical nursing practice. Knowledge of these indicators can aid the development of tools to assess nursing students' critical thinking skills. The study was conducted between September 2009 and August 2010. In phase 1, a literature review and four focus groups were conducted to identify the indicators of critical thinking in the context of nursing and the attributes of critical thinkers. In phase 2, 30 nursing professionals participated in a modified Delphi research survey to establish consensus and the appropriateness of each indicator and attribute identified in phase 1. We identified 37 indicators of critical thinking and 10 attributes of critical thinkers. The indicators were categorized into five subscales within the context of the nursing process toreflect nursing clinical practice: assessment, 16 indicators of ability to apply professional knowledge and skills to analyze and interpret patient problems; diagnosis, five indicators of ability to propose preliminary suppositions; planning, five indicators of ability to develop problem-solving strategies; implementation, five indicators of ability to implement planning; and evaluation, six indicators of ability to self-assess and reflect. The study operationalized critical thinking into a practical indicator suitable for nursing contexts in which critical thinking is required for clinical problem solving. Identified indicators and attributes can assist clinical instructors to evaluate student critical thought skills and development-related teaching strategies.

  20. Critical sizes and critical characteristics of nanoclusters, nanostructures and nanomaterials

    International Nuclear Information System (INIS)

    Suzdalev, I.P.

    2005-01-01

    Full text: Critical sizes and characteristics of nanoclusters and nanostructures are introduced as the parameters of nanosystems and nanomaterials. The next critical characteristics are considered: atomic and electronic 'magic number', critical size of cluster nucleation, critical size of melting-freezing of cluster, critical size of quantum (laser) radiation, critical sizes for the single electron conductivity, critical energy and magnetic field for the magnetic tunneling, critical cluster sizes for the giant magnetic resistance, critical size of the first order magnetic phase transition. The critical characteristics are estimated by thermodynamic approaches, by Moessbauer spectroscopy, AFM, heat capacity, SQUID magnetometry and other technique, The influence of cluster-cluster interactions, cluster-matrix interactions and cluster defects on cluster atomic dynamics, cluster melting, cluster critical sizes, Curie or Neel points and the character of magnetic phase transitions were investigated. The applications of critical size and critical characteristic parameters for the nanomaterial characterization are considered

  1. Homozygous EEF1A2 mutation causes dilated cardiomyopathy, failure to thrive, global developmental delay, epilepsy and early death.

    Science.gov (United States)

    Cao, Siqi; Smith, Laura L; Padilla-Lopez, Sergio R; Guida, Brandon S; Blume, Elizabeth; Shi, Jiahai; Morton, Sarah U; Brownstein, Catherine A; Beggs, Alan H; Kruer, Michael C; Agrawal, Pankaj B

    2017-09-15

    Eukaryotic elongation factor 1A (EEF1A), is encoded by two distinct isoforms, EEF1A1 and EEF1A2; whereas EEF1A1 is expressed almost ubiquitously, EEF1A2 expression is limited such that it is only detectable in skeletal muscle, heart, brain and spinal cord. Currently, the role of EEF1A2 in normal cardiac development and function is unclear. There have been several reports linking de novo dominant EEF1A2 mutations to neurological issues in humans. We report a pair of siblings carrying a homozygous missense mutation p.P333L in EEF1A2 who exhibited global developmental delay, failure to thrive, dilated cardiomyopathy and epilepsy, ultimately leading to death in early childhood. A third sibling also died of a similar presentation, but DNA was unavailable to confirm the mutation. Functional genomic analysis was performed in S. cerevisiae and zebrafish. In S. cerevisiae, there was no evidence for a dominant-negative effect. Previously identified putative de novo mutations failed to complement yeast strains lacking the EEF1A ortholog showing a major growth defect. In contrast, the introduction of the mutation seen in our family led to a milder growth defect. To evaluate its function in zebrafish, we knocked down eef1a2 expression using translation blocking and splice-site interfering morpholinos. EEF1A2-deficient zebrafish had skeletal muscle weakness, cardiac failure and small heads. Human EEF1A2 wild-type mRNA successfully rescued the morphant phenotype, but mutant RNA did not. Overall, EEF1A2 appears to be critical for normal heart function in humans, and its deficiency results in clinical abnormalities in neurologic function as well as in skeletal and cardiac muscle defects. © The Author 2017. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  2. Music critic Gustav Michel

    Directory of Open Access Journals (Sweden)

    Vasić Aleksandar N.

    2004-01-01

    Full Text Available The writers whose real vocation was not music left significant traces in the history of Serbian music critics and essayism of the late 19th century and the first half of the 20th century. Numerous authors, literary historians theoreticians and critics, jurists and theatre historians, wrote successfully on music in Serbian daily newspapers, literary and other magazines, until the Second World War. This study is devoted to Gustav Michel (1868 - 1926, one of the music amateurs who ought to be remembered in the history of Serbian music critics. Gustav Michel was a pharmacist by vocation. He ran a private pharmacy in Belgrade all his life. But he was a musician as well. He played the viola in the second (in chronological order of foundation Serbian String Quartet. The ensemble mostly consisted of amateurs, and it performed standard pieces of chamber music (W. A. Mozart L. v. Beethoven, F. Schubert, F. Mendelsohn-Bartholdy, A. Dvořžak. These musicians had performed public concerts in Belgrade since 1900 up until Michel’s death. Belgrade music critics prised the performances of this string ensemble highly. Gustav Michel was also a music critic. Until now only seven articles, published by this author between 1894 and 1903, in Order (Red, Folk Newspaper (Narodne novine and Serbian Literary Magazine (Srpski književni glasnik have been found. Michel’s preserved articles unambiguously prove that their author had a solid knowledge of music theory and history, the knowledge that exceeded amateurism. Nevertheless, Michel did not burden his first critics with expert language of musicology. Later on, in Serbian Literary Magazine, the magazine which left enough room for music, Michel penetrated more into musical terminology, thus educating slowly forming Serbian concert-going public. The analysis of Michel’s texts showed that he was not, in contrast to the majority of professional music critics, an opponent of virtuosity. Gentle and liberal, he did not

  3. Evaluación de los factores de riesgo y los tipos de superficie para el desarrollo de las úlceras por presión en el enfermo crítico Assessment of risk factors and the types of surface for the development of pressure ulcers on critical ill patients

    Directory of Open Access Journals (Sweden)

    Concepció Fuentes i Pumarola

    2007-06-01

    Full Text Available Objetivos: Evaluar los factores de riesgo y los tipos de superficie para el desarrollo de las úlceras por presión (UPP en el enfermo crítico ingresado en una unidad de cuidados intensivos (UCI. Material y métodos: Estudio prospectivo de incidencia de UPP. Población: Enfermos ingresados en una UCI con EMINA© de alto riesgo, estancia mínima de 48 horas y que no presentaran UPP al ingreso. Se registraron los datos demográficos, de gravedad al ingreso (SAPS II y APACHE III, diabetes, estado séptico, ventilación mecánica, incidencia y grado de UPP, y tipo de superficie de apoyo (alternante [SA] o estática [SE]. Conclusiones: Los factores de riesgo asociados con el desarrollo de UPP en el enfermo crítico fueron la edad, la estancia media y el tipo de superficie de apoyo, siendo muy significativa la disminución de la incidencia de UPP con las superficies alternantes.Objectives: To assess the risk factors and the types of surface for the development of pressure ulcers (PU on critical ill patients in an Intensive Care Unit (ICU. Material and methods: Prospective study about the incidence of PU. Population: All patients on ICU with EMINA® of high risk, who stay 48 h. at least and don't have PU in the moment of admission. Information about demographic characteristics, gravity in the moment of admitting was recorded: SAPS II and APACHE III, diabetes, sepsis, mechanical ventilation, incidence and graduation of PU, and the type of support surface: alternate (AS or static (SS. Conclusions: The risk factors associated with the development of PU on critical ill patients were the age, the average time in ICU and the type of support surface, being the reduction on incidence of PU with the alternate surfaces very important.

  4. Nuclear criticality information system

    International Nuclear Information System (INIS)

    Koponen, B.L.; Hampel, V.E.

    1981-01-01

    The nuclear criticality safety program at LLNL began in the 1950's with a critical measurements program which produced benchmark data until the late 1960's. This same time period saw the rapid development of computer technology useful for both computer modeling of fissile systems and for computer-aided management and display of the computational benchmark data. Database management grew in importance as the amount of information increased and as experimental programs were terminated. Within the criticality safety program at LLNL we began at that time to develop a computer library of benchmark data for validation of computer codes and cross sections. As part of this effort, we prepared a computer-based bibliography of criticality measurements on relatively simple systems. However, it is only now that some of these computer-based resources can be made available to the nuclear criticality safety community at large. This technology transfer is being accomplished by the DOE Technology Information System (TIS), a dedicated, advanced information system. The NCIS database is described

  5. Fermion-induced quantum critical points.

    Science.gov (United States)

    Li, Zi-Xiang; Jiang, Yi-Fan; Jian, Shao-Kai; Yao, Hong

    2017-08-22

    A unified theory of quantum critical points beyond the conventional Landau-Ginzburg-Wilson paradigm remains unknown. According to Landau cubic criterion, phase transitions should be first-order when cubic terms of order parameters are allowed by symmetry in the Landau-Ginzburg free energy. Here, from renormalization group analysis, we show that second-order quantum phase transitions can occur at such putatively first-order transitions in interacting two-dimensional Dirac semimetals. As such type of Landau-forbidden quantum critical points are induced by gapless fermions, we call them fermion-induced quantum critical points. We further introduce a microscopic model of SU(N) fermions on the honeycomb lattice featuring a transition between Dirac semimetals and Kekule valence bond solids. Remarkably, our large-scale sign-problem-free Majorana quantum Monte Carlo simulations show convincing evidences of a fermion-induced quantum critical points for N = 2, 3, 4, 5 and 6, consistent with the renormalization group analysis. We finally discuss possible experimental realizations of the fermion-induced quantum critical points in graphene and graphene-like materials.Quantum phase transitions are governed by Landau-Ginzburg theory and the exceptions are rare. Here, Li et al. propose a type of Landau-forbidden quantum critical points induced by gapless fermions in two-dimensional Dirac semimetals.

  6. The critical ionization velocity

    International Nuclear Information System (INIS)

    Raadu, M.A.

    1980-06-01

    The critical ionization velocity effect was first proposed in the context of space plasmas. This effect occurs for a neutral gas moving through a magnetized plasma and leads to rapid ionization and braking of the relative motion when a marginal velocity, 'the critical velocity', is exceeded. Laboratory experiments have clearly established the significance of the critical velocity and have provided evidence for an underlying mechanism which relies on the combined action of electron impact ionization and a collective plasma interaction heating electrons. There is experimental support for such a mechanism based on the heating of electrons by the modified two-stream instability as part of a feedback process. Several applications to space plasmas have been proposed and the possibility of space experiments has been discussed. (author)

  7. Critical Kinship Studies

    DEFF Research Database (Denmark)

    In recent decades the concept of kinship has been challenged and reinvigorated by the so-called “repatriation of anthropology” and by the influence of feminist studies, queer studies, adoption studies, and science and technology studies. These interdisciplinary approaches have been further...... developed by increases in infertility, reproductive travel, and the emergence of critical movements among transnational adoptees, all of which have served to question how kinship is now practiced. Critical Kinship Studies brings together theoretical and disciplinary perspectives and analytically sensitive...... in the Humanities and Social Sciences today. Critical Kinship Studies should be of particular interest to students and scholars in Anthropology, Sociology, Cultural Studies, Medical Humanities, Politics, Gender and Queer Studies and Globalization....

  8. Constructing criticality by classification

    DEFF Research Database (Denmark)

    Machacek, Erika

    2017-01-01

    " in the bureaucratic practice of classification: Experts construct material criticality in assessments as they allot information on the materials to the parameters of the assessment framework. In so doing, they ascribe a new set of connotations to the materials, namely supply risk, and their importance to clean energy......, legitimizing a criticality discourse.Specifically, the paper introduces a typology delineating the inferences made by the experts from their produced recommendations in the classification of rare earth element criticality. The paper argues that the classification is a specific process of constructing risk....... It proposes that the expert bureaucratic practice of classification legitimizes (i) the valorisation that was made in the drafting of the assessment framework for the classification, and (ii) political operationalization when enacted that might have (non-)distributive implications for the allocation of public...

  9. Critical Axial Load

    Directory of Open Access Journals (Sweden)

    Walt Wells

    2008-01-01

    Full Text Available Our objective in this paper is to solve a second order differential equation for a long, simply supported column member subjected to a lateral axial load using Heun's numerical method. We will use the solution to find the critical load at which the column member will fail due to buckling. We will calculate this load using Euler's derived analytical approach for an exact solution, as well as Euler's Numerical Method. We will then compare the three calculated values to see how much they deviate from one another. During the critical load calculation, it will be necessary to calculate the moment of inertia for the column member.

  10. Russian nuclear criticality experiments. Status and prospects

    International Nuclear Information System (INIS)

    Gagarinski, A.Yu.

    2003-01-01

    After the nuclear criticality had been reached on a uranium-graphite assembly for the first time in the Soviet Union on December 25, 1946, by I.V. Kurchatov and his team (1), the critical conditions in a great variety of multiplying media have been realized only in the Kurchatov Institute for at least several thousand times. Even the first Russian critical experiments carried out by Igor Kurchatov confirmed the unique merits of zero-power reactors: the most practically convenient range of parameters of kinetic response for variation of critical conditions, as well as invariability, over a wide range of the most important functions of neutron flux to reactor power. Neutron physics experiments have become a necessary stage in creation and improvement of nuclear reactors. Most critical experiments were performed mainly as a necessary stage of reactor design in the 60ies and 70ies, which has been the reactor 'golden age', when most of the total of over thousand nuclear reactors of various type and destination have been created worldwide. Though the ways of conducting critical measurements were very diversified, there are two main types of experiments. The first is so-called mock-up or prototype experiments when an exact (to the extent possible) simulation of the core is constructed to minimize the error in forecasting the operating reactor characteristics. Such experiments, which often represent the quality control of the core manufacturing and adjustment of core parameters to the design requirements, were carried out in Russia on critical assemblies of several plants, in design institutions (OKBM, Nizhni Novgorod; Electrostal and others), as well as in research centers (RRC 'Kurchatov Institute', etc.). Their results, which prevail today in the criticality database, even taking into account the capabilities provided by present-day calculation codes, are not well suited for new applications. It is hard to expect that the error resulting from inevitable idealization of

  11. MCNPTM criticality primer and training experiences

    International Nuclear Information System (INIS)

    Briesmeister, J.; Forster, R.A.; Busch, R.

    1995-01-01

    With the closure of many experimental facilities, the nuclear criticality safety analyst is increasingly required to rely on computer calculations to identify safe limits for the handling and storage of fissile materials. However, the analyst may have little experience with the specific codes available at his or her facility. Usually, the codes are quite complex, black boxes capable of analyzing numerous problems with a myriad of input options. Documentation for these codes is designed to cover all the possible configurations and types of analyses but does not give much detail on any particular type of analysis. For criticality calculations, the user of a code is primarily interested in the value of the effective multiplication factor for a system (k eff ). Most codes will provide this, and truckloads of other information that may be less pertinent to criticality calculations. Based on discussions with code users in the nuclear criticality safety community, it was decided that a simple document discussing the ins and outs of criticality calculations with specific codes would be quite useful. The Transport Methods Group, XTM, at Los Alamos National Laboratory (LANL) decided to develop a primer for criticality calculations with their Monte Carlo code, MCNP. This was a joint task between LANL with a knowledge and understanding of the nuances and capabilities of MCNP and the University of New Mexico with a knowledge and understanding of nuclear criticality safety calculations and educating first time users of neutronics calculations. The initial problem was that the MCNP manual just contained too much information. Almost everything one needs to know about MCNP can be found in the manual; the problem is that there is more information than a user requires to do a simple k eff calculation. The basic concept of the primer was to distill the manual to create a document whose only focus was criticality calculations using MCNP

  12. Trust and Critical Thinking

    Science.gov (United States)

    Kleinig, John

    2018-01-01

    This article discusses the tension between trust, as an expression of interpersonal commitment, and critical thinking, which includes a demand for reasons. It explores the importance of each for individual flourishing, and then seeks to establish some ways in which they intersect, drawing on ideas of authority and trustworthiness. It argues that…

  13. Posthuman Critical Theory

    NARCIS (Netherlands)

    Braidotti, R.

    2016-01-01

    This essay proposes a genealogical cartography of the emergence of a posthuman turn in critical theory, including feminist theory, based on the convergence of posthumanism with post-anthropocentrism. The former critiques the universalist posture of the idea of ‘Man’ as the alleged ‘measure of all

  14. Critical scattering by bubbles

    International Nuclear Information System (INIS)

    Fiedler-Ferrari, N.; Nussenzveig, H.M.

    1986-11-01

    We apply the complex angular momentum theory to the problem of the critical scattering of light by spherical cavities in the high frequency limit (permittivity greater than the external media) (e.g, air bubble in water) (M.W.O.) [pt

  15. The critical interface

    DEFF Research Database (Denmark)

    Pold, Søren

    2005-01-01

    In this paper, I argue that aesthetics might serve the role of critical reflection on representation within the field of computing, and I demonstrate through three analytic examples from computer art how this might look like and what the contribution might be....

  16. Reply to critics

    NARCIS (Netherlands)

    Lindahl, H.K.

    2014-01-01

    In my reply to critics I address a raft of issues raised by the commentators to Fault Lines of Globalization. These issues include: the radicalization of the Husserlian concept of intentionality as differance; the irreducibility of a-legality to (il)legality; the relation between legal orders and

  17. Critical Practice: Teaching "Shakespeare."

    Science.gov (United States)

    Mellor, Bronwyn; Patterson, Annette

    2000-01-01

    Describes how the authors taught their students to read "Hamlet" from a critical literacy perspective, analyzing how particular readings of texts and characters are constructed or produced; how they are determined by historical and cultural conventions; analyzing values that various readings support or challenge--rather than trying to…

  18. Alternative methods in criticality

    International Nuclear Information System (INIS)

    Pedicini, J.M.

    1982-01-01

    In this thesis two new methods of calculating the criticality of a nuclear system are introduced and verified. Most methods of determining the criticality of a nuclear system depend implicitly upon knowledge of the angular flux, net currents, or moments of the angular flux, on the system surface in order to know the leakage. For small systems, leakage is the predominant element in criticality calculations. Unfortunately, in these methods the least accurate fluxes, currents, or moments are those occurring near system surfaces or interfaces. This is due to a mathematical inability to satisfy rigorously with a finite order angular polynomial expansion or angular difference technique the physical boundary conditions which occur on these surfaces. Consequently, one must accept large computational effort or less precise criticality calculations. The methods introduced in this thesis, including a direct leakage operator and an indirect multiple scattering leakage operator, obviate the need to know angular fluxes accurately at system boundaries. Instead, the system wide scalar flux, an integral quantity which is substantially easier to obtain with good precision is sufficient to obtain production, absorption, scattering, and leakage rates

  19. Critical groups - basic concepts

    International Nuclear Information System (INIS)

    Carter, M.W.

    1992-01-01

    The potential exposure pathways from the land application site to man are presented. It is emphasised that the critical group is not necessary the population group closest to the source. It could be the group impact by the most significant pathways(s). Only by assessing the importance of each of these pathways and then combining them can a proper choice of critical group be made. It would be wrong to select a critical group on the basis that it seems the most probable one, before the pathways have been properly assessed. A calculation in Carter (1983) suggested that for the operating mine site, the annual doses to an Aboriginal person, a service worker and a local housewife, were all about the same and were in the range 0.1 to 0.2 mSv per year. Thus it may be that for the land application area, the critical group turns out to be non-Aboriginal rather than the expected Aboriginal group. 6 refs., 3 figs

  20. APS Quantum Critical Higgs

    CERN Document Server

    Bellazzini, Brando; Hubisz, Jay; Lee, Seung J.; Serra, Javi; Terning, John

    2016-01-01

    The appearance of the light Higgs boson at the LHC is difficult to explain, particularly in light of naturalness arguments in quantum field theory. However light scalars can appear in condensed matter systems when parameters (like the amount of doping) are tuned to a critical point. At zero temperature these quantum critical points are directly analogous to the finely tuned standard model. In this paper we explore a class of models with a Higgs near a quantum critical point that exhibits non-mean-field behavior. We discuss the parametrization of the effects of a Higgs emerging from such a critical point in terms of form factors, and present two simple realistic scenarios based on either generalized free fields or a 5D dual in AdS space. For both of these models we consider the processes $gg\\to ZZ$ and $gg\\to hh$, which can be used to gain information about the Higgs scaling dimension and IR transition scale from the experimental data.

  1. Alternative methods in criticality

    International Nuclear Information System (INIS)

    Pedicini, J.M.

    1982-01-01

    Two new methods of calculating the criticality of a nuclear system are introduced and verified. Most methods of determining the criticality of a nuclear system depend implicitly upon knowledge of the angular flux, net currents, or moments of the angular flux, on the system surface in order to know the leakage. For small systems, leakage is the predominant element in criticality calculations. Unfortunately, in these methods the least accurate fluxes, currents, or moments are those occuring near system surfaces or interfaces. This is due to a mathematical inability to satisfy rigorously with a finite order angular polynomial expansion or angular difference technique the physical boundary conditions which occur on these surfaces. Consequently, one must accept large computational effort or less precise criticality calculations. The methods introduced in this thesis, including a direct leakage operator and an indirect multiple scattering leakage operator, obviate the need to know angular fluxes accurately at system boundaries. Instead, the system wide scalar flux, an integral quantity which is substantially easier to obtain with good precision, is sufficient to obtain production, absorption, scattering, and leakage rates

  2. Quantum Critical Higgs

    Science.gov (United States)

    Bellazzini, Brando; Csáki, Csaba; Hubisz, Jay; Lee, Seung J.; Serra, Javi; Terning, John

    2016-10-01

    The appearance of the light Higgs boson at the LHC is difficult to explain, particularly in light of naturalness arguments in quantum field theory. However, light scalars can appear in condensed matter systems when parameters (like the amount of doping) are tuned to a critical point. At zero temperature these quantum critical points are directly analogous to the finely tuned standard model. In this paper, we explore a class of models with a Higgs near a quantum critical point that exhibits non-mean-field behavior. We discuss the parametrization of the effects of a Higgs emerging from such a critical point in terms of form factors, and present two simple realistic scenarios based on either generalized free fields or a 5D dual in anti-de Sitter space. For both of these models, we consider the processes g g →Z Z and g g →h h , which can be used to gain information about the Higgs scaling dimension and IR transition scale from the experimental data.

  3. Assessment of criticality safety

    International Nuclear Information System (INIS)

    Lloyd, R.C.; Heaberlin, S.W.; Clayton, E.D.; Carter, R.D.

    1979-01-01

    A study was made of 100 violations of criticality safety specifications reported over a 10-y period in the operations of fuel reprocessing plants. The seriousness of each rule violation was evaluated by assigning it a severity index value. The underlying causes or reasons, for the violations were identified. A criticality event tree was constructed using the parameters, causes, and reasons found in the analysis of the infractions. The event tree provides a means for visualizing the paths to an accidental criticality. Some 65% of the violations were caused by misinterpretation on the part of the operator, being attributed to a lack of clarity in the specification and insufficient training; 33% were attributed to lack of care, whereas only 2% were caused by mechanical failure. A fault tree was constructed by assembling the events that could contribute to an accident. With suitable data on the probabilities of contributing events, the probability of the accident's occurrence can be forecast. Estimated probabilities for criticality were made, based on the limited data available, that in this case indicate a minimum time span of 244 y of plant operation per accident ranging up to approx. 3000 y subject to the various underlying assumptions made. Some general suggestions for improvement are formulated based on the cases studied. Although conclusions for other plants may differ in detail, the general method of analysis and the fault tree logic should prove applicable. 4 figures, 8 tables

  4. Critical theory and holocaust

    Directory of Open Access Journals (Sweden)

    Krstić Predrag

    2006-01-01

    Full Text Available In this paper the author is attempting to establish the relationship - or the lack of it - of the Critical Theory to the "Jewish question" and justification of perceiving signs of Jewish religious heritage in the thought of the representatives of this movement. The holocaust marked out by the name of "Auschwitz", is here tested as a point where the nature of this relationship has been decided. In this encounter with the cardinal challenge for the contemporary social theory, the particularity of the Frankfurt School reaction is here revealed through Adorno installing Auschwitz as unexpected but lawful emblem of the ending of the course that modern history has assumed. The critique of this "fascination" with Auschwitz, as well as certain theoretical pacification and measured positioning of the holocaust into discontinued plane of "unfinished" and continuation and closure of the valued project, are given through communicative-theoretical pre-orientation of Jürgen Habermas’s Critical Theory and of his followers. Finally, through the work of Detlev Claussen, it is suggested that in the youngest generation of Adorno’s students there are signs of revision to once already revised Critical Theory and a kind of defractured and differentiated return to the initial understanding of the decisiveness of the holocaust experience. This shift in the attitude of the Critical Theory thinkers to the provocation of holocaust is not, however, particularly reflected towards the status of Jews and their tradition, but more to the age old questioning and explanatory patterns for which they served as a "model". The question of validity of the enlightenment project, the nature of occidental rationalism, (nonexistence of historical theology and understanding of the identity and emancipation - describe the circle of problems around which the disagreement is concentrated in the social critical theory.

  5. Achievement report for fiscal 1998. Development of an industrial rinsing equipment of environmentally harmonizing type using an ultra critical fluid (the second year); 1998 nendo seika hokokusho. Chorinkai ryutai wo mochiita kankyo chowagata kogyo senjo sochi no kaihatsu (dai 2 nendo)

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1999-03-01

    This project is intended for environment friendly and pollution-free rinsing by using an ultra critical fluid of CO2 being an atmospheric constituent. It also has a purpose of establishing a new rinsing method implemented by an ultra precision component machine and device using such features possessed by the ultra critical fluid as high permeability and disinfecting activities. It is further intended of developing, fabricating, and practically using a rinsing equipment that makes possible precision rinsing of mechanical, electronic, optical and aeronautical components. Using the results of experiments performed in the previous year as the base, discussions were given on the relationship between the two aspects of the hardware aspect such as equipment configuration and piping of the rinsing equipment and the software aspect such as operating conditions and procedures and the rising effect. As a result, a new rinsing equipment of solvent circulation type was proposed. The basic design software for the specific equipment was completed, and an experimental equipment was designed and fabricated under the clean specifications. The performance evaluation and verification experiments were finished in the end of fiscal 1998, and collection of fundamental data has begun. With regard to the rinsing evaluation method, qualitative and quantitative analyses were performed on the rinsing by using a surface shape measuring microscope and the infrared spectroscopy. (NEDO)

  6. Critical Education, Critical Pedagogies, Marxist Education in the United States

    Science.gov (United States)

    Foley, Jean Ann; Morris, Doug; Gounari, Panayota; Agostinone-Wilson, Faith

    2015-01-01

    As critical pedagogy becomes more mainstream on the educational landscape in the United States, it is important to revisit the original tenets of critical pedagogy and explore their current manifestations. Since the beginning of "criticalism" from the theoretical/foundational work of the Frankfurt School of Critical Social Theory,…

  7. Engineering Surface Critical Behavior of (2 +1 )-Dimensional O(3) Quantum Critical Points

    Science.gov (United States)

    Ding, Chengxiang; Zhang, Long; Guo, Wenan

    2018-06-01

    Surface critical behavior (SCB) refers to the singularities of physical quantities on the surface at the bulk phase transition. It is closely related to and even richer than the bulk critical behavior. In this work, we show that three types of SCB universality are realized in the dimerized Heisenberg models at the (2 +1 )-dimensional O(3) quantum critical points by engineering the surface configurations. The ordinary transition happens if the surface is gapped in the bulk disordered phase, while the gapless surface state generally leads to the multicritical special transition, even though the latter is precluded in classical phase transitions because the surface is in the lower critical dimension. An extraordinary transition is induced by the ferrimagnetic order on the surface of the staggered Heisenberg model, in which the surface critical exponents violate the results of the scaling theory and thus seriously challenge our current understanding of extraordinary transitions.

  8. Blood typing

    Science.gov (United States)

    ... detect these minor antigens. It is done before transfusions, except in emergency situations. Alternative Names Cross matching; Rh typing; ABO blood typing; Blood group; Anemia - immune hemolytic blood type; ...

  9. Spent fuel storage criticality safety

    Energy Technology Data Exchange (ETDEWEB)

    Amin, E M; Elmessiry, A M [National center of nuclear safety and radiation control atomic energy authority, (Egypt)

    1995-10-01

    The safety aspects of the spent fuel storage pool of the Egyptian test and research reactor one (ET-R R-1) has to be assessed as part of a general overall safety evaluation to be included in a safety analysis report (SAR) for this reactor. The present work treats the criticality safety of the spent fuel storage pool. Conservative calculations based on using fresh fuel has been performed, as well as less conservative using burned fuel. The calculations include cross library generation for burned and fresh fuel for the ET-R R-1 fuel type. The WIMS-D 4 code has been used in library generation and burn up calculation the critically calculations are performed using the one dimensional transport code (ANISN) and the two dimensional diffusion code (DIXY2). The possibility of increasing the storage efficiency either by insertion of absorber sheets of soluble boron salts or by reduction of fuel rod separation has been studied. 8 figs., 2 tabs.

  10. Spent fuel storage criticality safety

    International Nuclear Information System (INIS)

    Amin, E.M.; Elmessiry, A.M.

    1995-01-01

    The safety aspects of the spent fuel storage pool of the Egyptian test and research reactor one (ET-R R-1) has to be assessed as part of a general overall safety evaluation to be included in a safety analysis report (SAR) for this reactor. The present work treats the criticality safety of the spent fuel storage pool. Conservative calculations based on using fresh fuel has been performed, as well as less conservative using burned fuel. The calculations include cross library generation for burned and fresh fuel for the ET-R R-1 fuel type. The WIMS-D 4 code has been used in library generation and burn up calculation the critically calculations are performed using the one dimensional transport code (ANISN) and the two dimensional diffusion code (DIXY2). The possibility of increasing the storage efficiency either by insertion of absorber sheets of soluble boron salts or by reduction of fuel rod separation has been studied. 8 figs., 2 tabs

  11. Effect of critical field on screened dielectric breakdown growth

    International Nuclear Information System (INIS)

    Wang Wei.

    1993-10-01

    In this paper we have shown that due to a critical field, the patterns growing in the screened Laplacian field will be affected. When the critical field is zero, there is a transition from a dense growing to single branch of aggregate. At a higher critical field, the pattern shows a spiky-type aggregate, and for a small critical field the transition is cut. For an intermediate critical field, the result shows that below the transition, there no dense structure. (author). 9 refs, 2 figs

  12. A critical assessment of two types of personal UV dosimeters.

    Science.gov (United States)

    Seckmeyer, Gunther; Klingebiel, Marcus; Riechelmann, Stefan; Lohse, Insa; McKenzie, Richard L; Liley, J Ben; Allen, Martin W; Siani, Anna-Maria; Casale, Giuseppe R

    2012-01-01

    Doses of erythemally weighted irradiances derived from polysulphone (PS) and electronic ultraviolet (EUV) dosimeters have been compared with measurements obtained using a reference spectroradiometer. PS dosimeters showed mean absolute deviations of 26% with a maximum deviation of 44%, the calibrated EUV dosimeters showed mean absolute deviations of 15% (maximum 33%) around noon during several test days in the northern hemisphere autumn. In the case of EUV dosimeters, measurements with various cut-off filters showed that part of the deviation from the CIE erythema action spectrum was due to a small, but significant sensitivity to visible radiation that varies between devices and which may be avoided by careful preselection. Usually the method of calibrating UV sensors by direct comparison to a reference instrument leads to reliable results. However, in some circumstances the quality of measurements made with simple sensors may be over-estimated. In the extreme case, a simple pyranometer can be used as a UV instrument, providing acceptable results for cloudless skies, but very poor results under cloudy conditions. It is concluded that while UV dosimeters are useful for their design purpose, namely to estimate personal UV exposures, they should not be regarded as an inexpensive replacement for meteorological grade instruments. © 2011 Wiley Periodicals, Inc. Photochemistry and Photobiology © 2011 The American Society of Photobiology.

  13. "Typing Back": Social Media as Space for Critical Discourse

    Science.gov (United States)

    Careless, Erin Jennifer

    2015-01-01

    Social media applications such as Facebook and Twitter have become integrated into sociocultural practices for millions of people around the world, and are having an enduring impact on the field of adult education. As essentially free, virtually non-hierarchical tools that facilitate user-generated knowledge, these online spaces may be powerful…

  14. Unfolding of neutron spectra from Godiva type critical assemblies

    International Nuclear Information System (INIS)

    Harvey, J.T.; Meason, J.L.; Wright, H.L.

    1976-01-01

    The results from three experiments conducted at the White Sands Missile Range Fast Burst Reactor Facility are discussed. The experiments were designed to measure the ''free-field'' neutron leakage spectrum and the neutron spectra from mildly perturbed environments. SAND-II was used to calculate the neutron spectrum utilizing several different trial input spectra for each experiment. Comparisons are made between the unfolded neutron spectrum for each trial input on the basis of the following parameters: average neutron energy (above 10 KeV), integral fluence (above 10 KeV), spectral index and the hardness parameter, phi/sub eq//phi

  15. Sensitivity Analysis of Criticality for Different Nuclear Fuel Shapes

    International Nuclear Information System (INIS)

    Kang, Hyun Sik; Jang, Misuk; Kim, Seoung Rae

    2016-01-01

    Rod-type nuclear fuel was mainly developed in the past, but recent study has been extended to plate-type nuclear fuel. Therefore, this paper reviews the sensitivity of criticality according to different shapes of nuclear fuel types. Criticality analysis was performed using MCNP5. MCNP5 is well-known Monte Carlo codes for criticality analysis and a general-purpose Monte Carlo N-Particle code that can be used for neutron, photon, electron or coupled neutron / photon / electron transport, including the capability to calculate eigenvalues for critical systems. We performed the sensitivity analysis of criticality for different fuel shapes. In sensitivity analysis for simple fuel shapes, the criticality is proportional to the surface area. But for fuel Assembly types, it is not proportional to the surface area. In sensitivity analysis for intervals between plates, the criticality is greater as the interval increases, but if the interval is greater than 8mm, it showed an opposite trend that the criticality decrease by a larger interval. As a result, it has failed to obtain the logical content to be described in common for all cases. The sensitivity analysis of Criticality would be always required whenever subject to be analyzed is changed

  16. Sensitivity Analysis of Criticality for Different Nuclear Fuel Shapes

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Hyun Sik; Jang, Misuk; Kim, Seoung Rae [NESS, Daejeon (Korea, Republic of)

    2016-10-15

    Rod-type nuclear fuel was mainly developed in the past, but recent study has been extended to plate-type nuclear fuel. Therefore, this paper reviews the sensitivity of criticality according to different shapes of nuclear fuel types. Criticality analysis was performed using MCNP5. MCNP5 is well-known Monte Carlo codes for criticality analysis and a general-purpose Monte Carlo N-Particle code that can be used for neutron, photon, electron or coupled neutron / photon / electron transport, including the capability to calculate eigenvalues for critical systems. We performed the sensitivity analysis of criticality for different fuel shapes. In sensitivity analysis for simple fuel shapes, the criticality is proportional to the surface area. But for fuel Assembly types, it is not proportional to the surface area. In sensitivity analysis for intervals between plates, the criticality is greater as the interval increases, but if the interval is greater than 8mm, it showed an opposite trend that the criticality decrease by a larger interval. As a result, it has failed to obtain the logical content to be described in common for all cases. The sensitivity analysis of Criticality would be always required whenever subject to be analyzed is changed.

  17. Critical Digital Tourism Studies

    DEFF Research Database (Denmark)

    Gyimóthy, Szilvia; Munar, Ana María

    2013-01-01

    This paper advocates the need for a critical and cross-disciplinary research agenda on the field of digital technologies and tourism. The changing virtual landscape of tourism has received increased attention by tourism scholars. However, contemporary studies on information technologies (IT......) are approached mostly from a business administration perspective and informed by conceptual frameworks developed in management and marketing. IT studies in tourism are still at a stage similar to the first advocacy phase of tourism research in general (Jafari, 1990) and are seldom inspired by relevant...... to studying digital socio-technical systems and virtual mediation in tourism. Critical Digital Tourism Studies opens a new cross-disciplinary field where the sociality of virtual tourism interactions is examined (entailing the study of structures, social rules, ideologies, power relations, sustainability...

  18. Critical Virtue Ethics

    Directory of Open Access Journals (Sweden)

    Jochen Schmidt

    2014-06-01

    Full Text Available Since the publication of Anscombe’s famous paper “Modern Moral Philosophy” (1958, virtue ethics has become a matter of discussion among scholars. At least four charges have been raised against virtue ethics, one of which is the charge of promoting undue enthusiasm regarding the moral fitness of human beings. This article explores the limits of virtue ethics with regard to the frailty of human virtuousness. After giving a report of the charges raised against virtue ethics from the perspective of empirical ethics, the author presents the idea of what he would like to call critical virtue ethics as seen by three Lutheran thinkers: Immanuel Kant, Friedrich Nietzsche and Martin Luther himself. He will demonstrate that the empirical contestation of virtue ethics shows a remarkable resemblance to insights found in Luther, Kant and Nietzsche. And finally, the writer draws tentative conclusions about the future of critical virtue ethics.

  19. Criticality in Neuronal Networks

    Science.gov (United States)

    Friedman, Nir; Ito, Shinya; Brinkman, Braden A. W.; Shimono, Masanori; Deville, R. E. Lee; Beggs, John M.; Dahmen, Karin A.; Butler, Tom C.

    2012-02-01

    In recent years, experiments detecting the electrical firing patterns in slices of in vitro brain tissue have been analyzed to suggest the presence of scale invariance and possibly criticality in the brain. Much of the work done however has been limited in two ways: 1) the data collected is from local field potentials that do not represent the firing of individual neurons; 2) the analysis has been primarily limited to histograms. In our work we examine data based on the firing of individual neurons (spike data), and greatly extend the analysis by considering shape collapse and exponents. Our results strongly suggest that the brain operates near a tuned critical point of a highly distinctive universality class.

  20. Critical indices for reversible gamma-alpha phase transformation in metallic cerium

    Science.gov (United States)

    Soldatova, E. D.; Tkachenko, T. B.

    1980-08-01

    Critical indices for cerium have been determined within the framework of the pseudobinary solution theory along the phase equilibrium curve, the critical isotherm, and the critical isobar. The results obtained verify the validity of relationships proposed by Rushbrook (1963), Griffiths (1965), and Coopersmith (1968). It is concluded that reversible gamma-alpha transformation in metallic cerium is a critical-type transformation, and cerium has a critical point on the phase diagram similar to the critical point of the liquid-vapor system.

  1. Tolerization with BLP down-regulates HMGB1 a critical mediator of sepsis-related lethality.

    LENUS (Irish Health Repository)

    Coffey, J Calvin

    2012-02-03

    Tolerization with bacterial lipoprotein (BLP) affords a significant survival benefit in sepsis. Given that high mobility group box protein-1 (HMGB1) is a recognized mediator of sepsis-related lethality, we determined if tolerization with BLP leads to alterations in HMGB1. In vitro, BLP tolerization led to a reduction in HMGB1 gene transcription. This was mirrored at the protein level, as HMGB1 protein expression and release were reduced significantly in BLP-tolerized human THP-1 monocytic cells. BLP tolerance in vivo led to a highly significant, long-term survival benefit following challenge with lethal dose BLP in C57BL\\/6 mice. This was associated with an attenuation of HMGB1 release into the circulation, as evidenced by negligible serum HMGB1 levels in BLP-tolerized mice. Moreover, HMGB1 levels in peritoneal macrophages from BLP-tolerized mice were reduced significantly. Hence, tolerization with BLP leads to a down-regulation of HMGB1 protein synthesis and release. The improved survival associated with BLP tolerance could thus be explained by a reduction in HMGB1, were the latter associated with lethality in BLP-related sepsis. In testing this hypothesis, it was noted that neutralization of HMGB1, using anti-HMGB1 antibodies, abrogated BLP-associated lethality almost completely. To conclude, tolerization with BLP leads to a down-regulation of HMGB1, thus offering a novel means of targeting the latter. HMGB1 is also a mediator of lethality in BLP-related sepsis.

  2. Klebsiella Typing

    DEFF Research Database (Denmark)

    Hansen, D S; Skov, R; Benedí, J.V.

    2002-01-01

    OBJECTIVE: To compare pulsed-field gel electrophoresis (PFGE) typing and O:K-serotyping of Klebsiella in two different epidemiological settings. METHODS: One hundred and four bacteremia isolates without known epidemiological relation and 47 isolates from an outbreak in a neonatal intensive care...... unit (NICU) were K-typed by countercurrent immunoelectrophoresis (CCIE), O-typed by an inhibition enzyme-linked immunosorbent assay method, and typed by pulsed-field gel electrophoresis (PFGE) using the restriction enzyme XbaI. RESULTS: Typing data for the 104 bacteremia isolates were compared...... with regard to typability, number of types, maximum number of isolates per type, and the Discriminative Index (DI). O-typing combined with K-typing (DI 0.98) as O:K-serotyping (DI 0.99) gave a very discriminative typing system, whereas O-typing alone was not very discriminative (DI 0.76). PFGE (DI 1...

  3. The critical catastrophe revisited

    International Nuclear Information System (INIS)

    De Mulatier, Clélia; Rosso, Alberto; Dumonteil, Eric; Zoia, Andrea

    2015-01-01

    The neutron population in a prototype model of nuclear reactor can be described in terms of a collection of particles confined in a box and undergoing three key random mechanisms: diffusion, reproduction due to fissions, and death due to absorption events. When the reactor is operated at the critical point, and fissions are exactly compensated by absorptions, the whole neutron population might in principle go to extinction because of the wild fluctuations induced by births and deaths. This phenomenon, which has been named critical catastrophe, is nonetheless never observed in practice: feedback mechanisms acting on the total population, such as human intervention, have a stabilizing effect. In this work, we revisit the critical catastrophe by investigating the spatial behaviour of the fluctuations in a confined geometry. When the system is free to evolve, the neutrons may display a wild patchiness (clustering). On the contrary, imposing a population control on the total population acts also against the local fluctuations, and may thus inhibit the spatial clustering. The effectiveness of population control in quenching spatial fluctuations will be shown to depend on the competition between the mixing time of the neutrons (i.e. the average time taken for a particle to explore the finite viable space) and the extinction time

  4. Relative Critical Points

    Directory of Open Access Journals (Sweden)

    Debra Lewis

    2013-05-01

    Full Text Available Relative equilibria of Lagrangian and Hamiltonian systems with symmetry are critical points of appropriate scalar functions parametrized by the Lie algebra (or its dual of the symmetry group. Setting aside the structures – symplectic, Poisson, or variational – generating dynamical systems from such functions highlights the common features of their construction and analysis, and supports the construction of analogous functions in non-Hamiltonian settings. If the symmetry group is nonabelian, the functions are invariant only with respect to the isotropy subgroup of the given parameter value. Replacing the parametrized family of functions with a single function on the product manifold and extending the action using the (coadjoint action on the algebra or its dual yields a fully invariant function. An invariant map can be used to reverse the usual perspective: rather than selecting a parametrized family of functions and finding their critical points, conditions under which functions will be critical on specific orbits, typically distinguished by isotropy class, can be derived. This strategy is illustrated using several well-known mechanical systems – the Lagrange top, the double spherical pendulum, the free rigid body, and the Riemann ellipsoids – and generalizations of these systems.

  5. Plant critical concept

    International Nuclear Information System (INIS)

    O'Regan, P.J.

    1996-01-01

    The objective of the effort summarized in this paper is to support O and M cost reduction efforts by focusing resources on components and processes critical to plant performance. This effort will identify where resources on nonplant critical components and processes can be reduced or eliminated. This method will use a functional assessment as the basis for component-specific evaluations and ranking. This effort consists of two stages conducted in series. The first stage is to deterministically identify that set of plant components that are relevant from a plant performance perspective (i.e., safety, economics, reliability). The second stage probabilistically ranks that set of plant components from an importance perspective, where importance pertains to the particular application and is probabilistically weighted. The results of a pilot study identified that only a relatively small set of components are truly critical from an integrated plant performance perspective. These results are consistent with work being conducted at other nuclear power plants, as well as other commercial facilities. Initial implementation of this effort is estimated to reduce O and M costs on the order of $1 million per year. Subsequent applications are anticipated to increase that savings to $4--$5 million per year

  6. Criticality alarm device

    International Nuclear Information System (INIS)

    Sasaki, Takashi.

    1995-01-01

    In a critical alarm device comprising an n-number of radiation detectors and a 2/3 logic module, the 2/3 logic module comprises a rectifier inputting an output of an AC power and outputting it as a full wave-rectified, DC voltage, an analog/digital converter inputting the output from the rectifier, putting it to analog/digital conversion and continuously storing it into a memory, a latch circuit inputting critical signals from the 2/3 logic and holding them and outputting latch signals, and a means for outputting timing signals for data storage to a memory based on clocks at a predetermined period and outputting timing signals for stopping the data storage to the memory after passing a predetermined period from the inputting time point if the latch signals are inputted from the latch circuit. Then, judgement whether the generation of criticality is caused by an actual radiation detection or by noises from the power source is enabled, to provide extremely high reliability. (N.H.)

  7. Online discussion: Enhancing students' critical thinking skills

    Science.gov (United States)

    Rathakrishnan, Mohan; Ahmad, Rahayu; Suan, Choo Ling

    2017-10-01

    Online discussion has become one of the important strategies for the teacher to teach the students to think critically when conveying their ideas and become more proactive and creative. In this paper, padlet online discussion communication was conducted to examine its effectiveness in enhancing critical thinking. In this study, there are two types of critical thinking: macro and micro critical thinking. A total of 70 Universiti Utara Malaysia Management Foundation Programme students involved in this experimental research design. The students in treatment class are divided to few groups. Every group uses padlet online discussion to discuss the topic given. All the group members discuss and write their ideas in padlet. Ideas that are posted in padlet will be displayed in front of the class so that the entire group in the treatment class could see the given ideas. Paul's (1993) model was used to analyze student's macro and micro critical thinking in padlet online discussion and communication. The finding shows that students who used padlet online discussion backchannel communication have greater macro and micro critical thinking level than students who do not use online discussion.

  8. Critical Points in Distance Learning System

    Directory of Open Access Journals (Sweden)

    Airina Savickaitė

    2013-08-01

    Full Text Available Purpose – This article presents the results of distance learning system analysis, i.e. the critical elements of the distance learning system. The critical points of distance learning are a part of distance education online environment interactivity/community process model. The most important is the fact that the critical point is associated with distance learning participants. Design/methodology/approach – Comparative review of articles and analysis of distance learning module. Findings – A modern man is a lifelong learner and distance learning is a way to be a modern person. The focus on a learner and feedback is the most important thing of learning distance system. Also, attention should be paid to the lecture-appropriate knowledge and ability to convey information. Distance system adaptation is the way to improve the learner’s learning outcomes. Research limitations/implications – Different learning disciplines and learning methods may have different critical points. Practical implications – The information of analysis could be important for both lecturers and students, who studies distance education systems. There are familiar critical points which may deteriorate the quality of learning. Originality/value – The study sought to develop remote systems for applications in order to improve the quality of knowledge. Keywords: distance learning, process model, critical points. Research type: review of literature and general overview.

  9. ACRR fuel storage racks criticality safety analysis

    International Nuclear Information System (INIS)

    Bodette, D.E.; Naegeli, R.E.

    1997-10-01

    This document presents the criticality safety analysis for a new fuel storage rack to support modification of the Annular Core Research Reactor for production of molybdenum-99 at Sandia National Laboratories, Technical Area V facilities. Criticality calculations with the MCNP code investigated various contingencies for the criticality control parameters. Important contingencies included mix of fuel element types stored, water density due to air bubbles or water level for the over-moderated racks, interaction with existing fuel storage racks and fuel storage holsters in the fuel storage pool, neutron absorption of planned rack design and materials, and criticality changes due to manufacturing tolerances or damage. Some limitations or restrictions on use of the new fuel storage rack for storage operations were developed through the criticality analysis and are required to meet the double contingency requirements of criticality safety. As shown in the analysis, this system will remain subcritical under all credible upset conditions. Administrative controls are necessary for loading, moving, and handling the storage rack as well as for control of operations around it. 21 refs., 16 figs., 4 tabs

  10. Development of Critical Thinking in Pharmacy Education

    Directory of Open Access Journals (Sweden)

    Michael J Peeters

    2016-02-01

    Full Text Available The concept of development is ubiquitous throughout higher education. Development of critical thinking, problem-solving, and clinical reasoning are noted as important outcomes in higher education, including health professions education. In this era of widening scrutiny, demonstration of this outcome within programmatic assessment is becoming increasingly important. Programmatic assessment of critical thinking is complicated because of its multiple definitions, array of theoretical frameworks, and variety of measurement instruments. Additionally, recent guidelines and standards for pharmacy education have affirmed “habits of mind,” which are not new to education and encompass analytical critical thinking. In this paper, we sought to provide: 1 an overview of various critical thinking measurement instruments with their different associated critical thinking definitions, 2 a background and framework for thinking using the Dimensions of Learning model, 3 implications and applications for assessing cognitive development (critical and complex thinking within the context of pharmacy education, and 4 specific suggestions for assessment in pharmacy education.   Type: Idea Paper

  11. Controlling superconductivity by tunable quantum critical points.

    Science.gov (United States)

    Seo, S; Park, E; Bauer, E D; Ronning, F; Kim, J N; Shim, J-H; Thompson, J D; Park, Tuson

    2015-03-04

    The heavy fermion compound CeRhIn5 is a rare example where a quantum critical point, hidden by a dome of superconductivity, has been explicitly revealed and found to have a local nature. The lack of additional examples of local types of quantum critical points associated with superconductivity, however, has made it difficult to unravel the role of quantum fluctuations in forming Cooper pairs. Here, we show the precise control of superconductivity by tunable quantum critical points in CeRhIn5. Slight tin-substitution for indium in CeRhIn5 shifts its antiferromagnetic quantum critical point from 2.3 GPa to 1.3 GPa and induces a residual impurity scattering 300 times larger than that of pure CeRhIn5, which should be sufficient to preclude superconductivity. Nevertheless, superconductivity occurs at the quantum critical point of the tin-doped metal. These results underline that fluctuations from the antiferromagnetic quantum criticality promote unconventional superconductivity in CeRhIn5.

  12. Papers, Please as Critical Making: A Review

    Directory of Open Access Journals (Sweden)

    Eddie Lohmeyer

    2017-06-01

    Full Text Available This article examines Lucas Pope’s independent game Papers, Please as an instantiation of critical making within the discipline of the digital humanities. By confronting the player with moral decisions in their capacity as an immigration officer allowing or denying entry to immigrants within a totalitarian state, the game introduces an expressive form of game design in which conceptual practices are used to examine political and social realities. This type of critical media practice introduces a political ethic to the digital humanities that is arguably scarce within the discipline.

  13. Nuclear fission, chain reaction and criticality

    International Nuclear Information System (INIS)

    Reuss, Paul

    2016-01-01

    Criticality is, notably for nuclear reactors, the status which separates the case of a fission chain reaction which inexorably decays, from that of a reaction which grows faster and faster until a counter-reaction occurs. If this status is an objective in nuclear reactors, it must not be reached or exceeded in any case in other types of installations in which fissile materials are handled (fabrication, transports, nuclear fuel processing). The author proposes an insight into this notion of criticality, discusses elements of neutron science which allow the multiplication factor to be assessed, analyses accidental scenarios which may happen, and presents associated experiments and computation codes

  14. Glucose metabolism in critically ill patients

    DEFF Research Database (Denmark)

    Nielsen, Signe Tellerup; Krogh-Madsen, Rikke; Møller, Kirsten

    2015-01-01

    glucose (BG). This is taken advantage of in the treatment of patients with T2DM, for whom GLP-1 analogs have been introduced during the recent years. Infusion of GLP-1 also lowers the BG level in critically ill patients without causing severe hypoglycemia. The T2DM and critical illness share similar......, stimulates insulin secretion and inhibits glucagon release both in healthy individuals and in patients with type 2 diabetes (T2DM). Compared to insulin, GLP-1 appears to be associated with a lower risk of severe hypoglycemia, probably because the magnitude of its insulinotropic action is dependent on blood...

  15. Critical Phenomena Associated with Boson Stars

    OpenAIRE

    Hawley, Scott H.; Choptuik, Matthew W.

    2001-01-01

    We present a brief synopsis of related work (gr-qc/0007039), describing a study of black hole threshold phenomena for a self-gravitating, massive complex scalar field in spherical symmetry. We construct Type I critical solutions dynamically by tuning a one-parameter family of initial data consisting of a boson star and a massless real scalar field, and numerically evolving this data. The resulting critical solutions appear to correspond to boson stars on the unstable branch, as we show via co...

  16. Criticality accident alarm system

    International Nuclear Information System (INIS)

    Malenfant, R.E.

    1991-01-01

    The American National Standard ANSI/ANS-8.3-1986, Criticality Accident Alarm System provides guidance for the establishment and maintenance of an alarm system to initiate personnel evacuation in the event of inadvertent criticality. In addition to identifying the physical features of the components of the system, the characteristics of accidents of concern are carefully delineated. Unfortunately, this ANSI Standard has led to considerable confusion in interpretation, and there is evidence that the ''minimum accident of concern'' may not be appropriate. Furthermore, although intended as a guide, the provisions of the standard are being rigorously applied, sometimes with interpretations that are not consistent. Although the standard is clear in the use of absorbed dose in free air of 20 rad, at least one installation has interpreted the requirement to apply to dose in soft tissue. The standard is also clear in specifying the response to both neutrons and gamma rays. An assembly of uranyl fluoride enriched to 5% 235 U was operated to simulate a potential accident. The dose, delivered in a free run excursion 2 m from the surface of the vessel, was greater than 500 rad, without ever exceeding a rate of 20 rad/min, which is the set point for activating an alarm that meets the standard. The presence of an alarm system would not have prevented any of the five major accidents in chemical operations nor is it absolutely certain that the alarms were solely responsible for reducing personnel exposures following the accident. Nevertheless, criticality alarm systems are now the subject of great effort and expense. 13 refs

  17. Critical infrastructure protection

    Energy Technology Data Exchange (ETDEWEB)

    Bradley, F. [Canadian Electricity Association, Toronto, ON (Canada)

    2003-04-01

    The need to protect critical electrical infrastructure from terrorist attacks, or other physical damage, including weather related events, or the potential impact of computer viruses and other attacks on IT resources are discussed. Activities of the North American Electric Reliability Council (NERC) are highlighted which seek to safeguard the North American bulk electric power system principally through the Information Sharing and Analysis Sector (ES-ISAC). ES-ISAC serves the electricity sector by facilitating communication between electric sector participants, federal government and other critical infrastructure industries by disseminating threat indications, analyses and warnings, together with interpretations, to assist the industry in taking infrastructure protection actions. Attention is drawn to the numerous cyber incidents in recent years, which although resulted in no loss of service to electricity customers so far, in at least one instance (the January 25th SOL-Slammer worm incident) resulted in degradation of service in a number of sectors, including financial, transportation and telecommunication services. The increasing frequency of cyber-based attacks, coupled with the industry's growing dependence on e-commerce and electronic controls, are good reasons to believe that critical infrastructure protection (CIP) poses a serious challenge to the industry's risk management practices. The Canadian Electricity Association (CEA) is an active participant in ES-ISAC and works cooperatively with a range of partners, such as the Edison Electric Institute and the American Public Power Association to ensure coordination and effective protection program delivery for the electric power sector. The Early Warning System (EWS) developed by the CIP Working Group is one of the results of this cooperation. EWS uses the Internet, e-mail, web-enabled cell phones and Blackberry hand-held devices to deliver real-time threat information to members on a 24/7 basis. EWS

  18. Critical Thinking versus Neurosexism

    Directory of Open Access Journals (Sweden)

    Sonia Reverter-Bañón

    2017-02-01

    Full Text Available The aim of this paper is to offer a view of the assumptions that guide the practice of claiming sex differences in the brain. After the realization that much of the research and publication of neuroscientific findings assume such a difference, we found a great deal of what has been called neurosexism. As a way to overcome it and from a feminist theory with a political commitment we propose a critical approach to the neurosciences. This is understood as a reflective collaboration between disciplines that could provide a framework for overcoming prejudices in thinking and designing science.

  19. Critical cyberculture studies

    CERN Document Server

    Silver, David; Jones, Steve

    2006-01-01

    Starting in the early 1990s, journalists and scholars began responding to and trying to take account of new technologies and their impact on our lives. By the end of the decade, the full-fledged study of cyberculture had arrived. Today, there exists a large body of critical work on the subject, with cutting-edge studies probing beyond the mere existence of virtual communities and online identities to examine the social, cultural, and economic relationships that take place online. Taking stock of the exciting work that is being done and positing what cyberculture's future might look like, Criti

  20. Pathos in Criticism: Edwin Black's Communism-as-Cancer Metaphor

    Science.gov (United States)

    Condit, Celeste M.

    2013-01-01

    Edwin Black's essay on "The Second Persona," introduced to rhetorical critics a rationale and model for a type of ideological criticism. Because it ignored the role of pathos in both the rhetoric Black purported to critique and in the construction of his own audience, Black's essay mis-described key features of Robert Welch's "Blue Book", which…

  1. Chinook Critical Habitat, Central Valley - NOAA [ds125

    Data.gov (United States)

    California Department of Resources — This layer depicts areas designated for Chinook Critical Habitat as well as habitat type and quality in the Central Valley Spring-run Evolutionary Significant Unit...

  2. Chinook Critical Habitat, Central Valley - NOAA [ds125

    Data.gov (United States)

    California Natural Resource Agency — This layer depicts areas designated for Chinook Critical Habitat as well as habitat type and quality in the Central Valley Spring-run Evolutionary Significant Unit...

  3. Steelhead Critical Habitat, Central Valley - NOAA [ds123

    Data.gov (United States)

    California Natural Resource Agency — This layer depicts areas designated for Steelhead Critical Habitat as well as habitat type and quality in the California Central Valley Evolutionary Significant Unit...

  4. Genetic linkage analysis of type 1 diabetes mellitus to markers on chromosomes 2 and 11 in families from Antioquia, Colombia Análisis de ligamiento genético de la diabetes mellitus tipo 1, a marcadores de los cromosomas 2 y 11 en familias antioqueñas

    Directory of Open Access Journals (Sweden)

    Gabriel Bedoya Berrío

    2004-02-01

    Full Text Available DIABETES MELLITUS (DM comprises e heterogeneous group of hypoglycemic disorders, that are grouped according to their physiopathology and etiology; the most notorious ones are type 1 DM (DM1 and type 2 DM (DM2; DM1 is characterized by early onset and absolute lack of insulin; therefore, patients suffering from it depend on insulin since the beginning of their symptoms; in contrast, DM2 manifests during adult life and not all patients depend on insulin. DM1 is classified as DM1A when it results from an autoimmune response of pancreatic b cells, and DM1B if it is of unknown origin (idiopathic. Studies on the etiology of DM1 have revealed that both types have a strong genetic component but their inheritance pattern is complex since its pathogenesis may result from the interaction with environmental factors of variants in multiple genes. By means of genetic studies on DM1, susceptibility loci known as IDDM have been identified, namely: for DM1A the first locus (IDDM1 was found in the HLA-DR/QD region, located in 6p21, that modulates the effect of other genes involved in the disease; the second one (IDDM2 is located in 11p15, the site of the insulin gene. That means, DM1 exhibits wide genetic heterogeneity so that more than 18 loci involved in susceptibility to this disease have been identified, among them, 3 on chromosome 2 (IDDM 7, 12, and 13, and 1on chromosome 14 (IDDM11, the latter being associated to DM1B. The aim of this study was the search for loci on chromosomes 2 and 11 involved in the susceptibility to DM, in three families from Antioquia, Colombia; for that purpose, parametric linkage analysis was performed to 23 microsatellite markers on chromosome 2, and to 18 on chromosome 11. In order to determine the power for making linkage analysis, simulation was carried out on the families, coded as DM1 (11 affected members, family 1 (2 affected members, and family 10 (3 affected members; results demonstrated power enough for that purpose since

  5. Plant critical concept

    International Nuclear Information System (INIS)

    O'Regan, P.J.

    1995-01-01

    The achievement of operation and maintenance (O ampersand M) cost reductions is a prime concern for plant operators. Initiatives by the nuclear industry to address this concern are under way and/or in development. These efforts include plant reliability studies, reliability-centered maintenance, risk ranking and testing philosophies, performance-based testing philosophies, graded quality assurance, and so forth. This paper presents the results of an effort to develop a methodology that integrates and applies the common data and analysis requirements for a number of risk-based and performance-based initiatives. This initial phase of the effort applied the methodology and its results to two initiatives. These were the procurement function and the preventive maintenance function. This effort integrated multiple programs and functions to identify those components that are truly critical from an integrated plant performance perspective. The paper describes the scope of the effort, the development of a methodology to identify plant critical components, and the application of these results to the maintenance rule compliance, preventive maintenance, and procurement functions at the candidate plant

  6. Critical heat flux evaluation

    International Nuclear Information System (INIS)

    Banner, D.

    1995-01-01

    Critical heat flux (CHF) is of importance for nuclear safety and represents the major limiting factors for reactor cores. Critical heat flux is caused by a sharp reduction in the heat transfer coefficient located at the outer surface of fuel rods. Safety requires that this phenomenon also called the boiling crisis should be precluded under nominal or incidental conditions (Class I and II events). CHF evaluation in reactor cores is basically a two-step approach. Fuel assemblies are first tested in experimental loops in order to determine CHF limits under various flow conditions. Then, core thermal-hydraulic calculations are performed for safety evaluation. The paper will go into more details about the boiling crisis in order to pinpoint complexity and lack of fundamental understanding in many areas. Experimental test sections needed to collect data over wide thermal-hydraulic and geometric ranges are described CHF safety margin evaluation in reactors cores is discussed by presenting how uncertainties are mentioned. From basic considerations to current concerns, the following topics are discussed; knowledge of the boiling crisis, CHF predictors, and advances thermal-hydraulic codes. (authors). 15 refs., 4 figs

  7. Quantum critical Hall exponents

    CERN Document Server

    Lütken, C A

    2014-01-01

    We investigate a finite size "double scaling" hypothesis using data from an experiment on a quantum Hall system with short range disorder [1-3]. For Hall bars of width w at temperature T the scaling form is w(-mu)T(-kappa), where the critical exponent mu approximate to 0.23 we extract from the data is comparable to the multi-fractal exponent alpha(0) - 2 obtained from the Chalker-Coddington (CC) model [4]. We also use the data to find the approximate location (in the resistivity plane) of seven quantum critical points, all of which closely agree with the predictions derived long ago from the modular symmetry of a toroidal sigma-model with m matter fields [5]. The value nu(8) = 2.60513 ... of the localisation exponent obtained from the m = 8 model is in excellent agreement with the best available numerical value nu(num) = 2.607 +/- 0.004 derived from the CC-model [6]. Existing experimental data appear to favour the m = 9 model, suggesting that the quantum Hall system is not in the same universality class as th...

  8. Consideration of criticality in a nuclear waste repository

    International Nuclear Information System (INIS)

    Rechard, R.P.; Sanchez, L.C.; Stockman, C.T.; Ramsey, J.L. Jr.; Martell, M.

    1995-01-01

    The preliminary criticality analysis that was done suggests that the possibility of achieving critical conditions cannot be easily ruled out without looking at the geochemical process of assembly or the dynamics of the operation of a critical assembly. The evaluation of a critical assembly requires an integrated, consistent approach that includes evaluating the following: (1) the alteration rates of the layers of the container and spent fuel, (2) the transport of fissile material or neutron absorbers, and (3) the assembly mechanisms that can achieve critical conditions. The above is a non-trivial analysis and preliminary work suggests that with the loading assumed, enough fissile mass will leach from the HEU multi-purpose canisters to support a criticality. In addition, the consequences of an unpressurized Oklo type criticality would be insignificant to the performance of an unsaturated, tuff repository

  9. Non-critical pure spinor superstrings

    International Nuclear Information System (INIS)

    Adam, Ido; Grassi, Pietro Antonio; Mazzucato, Luca; Oz, Yaron; Yankielowicz, Shimon

    2007-01-01

    We construct non-critical pure spinor superstrings in two, four and six dimensions. We find explicitly the map between the RNS variables and the pure spinor ones in the linear dilaton background. The RNS variables map onto a patch of the pure spinor space and the holomorphic top form on the pure spinor space is an essential ingredient of the mapping. A basic feature of the map is the requirement of doubling the superspace, which we analyze in detail. We study the structure of the non-critical pure spinor space, which is different from the ten-dimensional one, and its quantum anomalies. We compute the pure spinor lowest lying BRST cohomology and find an agreement with the RNS spectra. The analysis is generalized to curved backgrounds and we construct as an example the non-critical pure spinor type IIA superstring on AdS 4 with RR 4-form flux

  10. Critical current enhancement in high Tc superconductors

    International Nuclear Information System (INIS)

    Jin, S.; Graebner, J.E.; Tiefel, T.H.

    1990-01-01

    Progress toward major technological applications of the bulk, high T c superconductors has been hindered by two major barriers, i.e., the Josephson weak-links at grain boundaries and the lack of sufficient intragrain flux pinning. It has been demonstrated that the weak link problem can be overcome by extreme alignment of grains such as in melt-textured-growth (MTG) materials. Modified or improved processing by various laboratories has produced further increased critical currents. However, the insufficient flux pinning seems to limit the critical current density in high fields to about 10 4 --10 5 A/cm 2 at 77K, which is not satisfactory for many applications. In this paper, processing, microstructure, and critical current behavior of the MTG type superconductors are described, and various processing possibilities for flux pinning enhancement are discussed

  11. Criticality management of Tokai reprocessing facility

    Energy Technology Data Exchange (ETDEWEB)

    Nojiri, Ichiro [Japan Nuclear Cycle Development Inst., Tokai, Ibaraki (Japan)

    2001-01-01

    In fuel cycle centers a number of equipment and vessels of various types and of complex design are used in several processes, i.e. dissolution of spent fuels, separation and storage of uranium and plutonium from fission products and transuranium elements. For each processes, Monte Carlo codes are frequently applied to manage the fuel criticality. Safety design depends largely on specific features of each facilities. The present report describes status of criticality management for main processes in Tokai Reprocessing Facility, JNC, and the criticality conditions specifically existing there. The guiding principle throughout consists of mass control, volume control, design (form) control, concentration control, and control due to employment of neutron poisons. (S. Ohno)

  12. 77 FR 69568 - Special Conditions: Bombardier Aerospace, Model BD-500-1A10 and BD-500-1A11 Airplanes; Sidestick...

    Science.gov (United States)

    2012-11-20

    ... Aerospace, Model BD-500-1A10 and BD-500-1A11 Airplanes; Sidestick Controllers AGENCY: Federal Aviation... conditions for the Bombardier Aerospace Model BD-500-1A10 and BD-500-1A11 airplanes. These airplanes will... on the comments we receive. Background On December 10, 2009, Bombardier Aerospace applied for a type...

  13. What Makes Critical Thinking Critical for Adult ESL Students

    Science.gov (United States)

    Miekley, Joshua P.

    2014-01-01

    Critical-thinking skills help to prepare adult education students for a successful transition to college degree programs and for job advancement. Yet fostering critical thinking poses a challenge to ESL instructors. Brookfield (2012) provides a way forward for adult educators when he explains that the crux of critical thinking is to discover one's…

  14. Critical Thinking in Criminology: Critical Reflections on Learning and Teaching

    Science.gov (United States)

    Howes, Loene M.

    2017-01-01

    Fostering critical thinking abilities amongst students is one component of preparing them to navigate uncertain and complex social lives and employment circumstances. One conceptualisation of critical thinking, valuable in higher education, draws from critical theory to promote social justice and redress power inequities. This study explored how…

  15. Blood Types

    Science.gov (United States)

    ... blood, safe blood transfusions depend on careful blood typing and cross-matching. There are four major blood ... cause exceptions to the above patterns. ABO blood typing is not sufficient to prove or disprove paternity ...

  16. Critical experiments AT Pacific Northwest Laboratory

    International Nuclear Information System (INIS)

    Clayton, E.D.; Bierman, S.R.

    1984-01-01

    After a short description of the facility, a brief listing of the principal types of fuel forms and assembly geometries is provided. A number of experiments have recently been performed on plain fissionable units, or isolated assemblies of single units, that include measurements on solutions composed of Pu-U mixtures and critical experiment data on lattices of low enriched uranium in water. Experiments have been performed on planar arrays of containers with Pu solutions because of the lack of data in this field concerning the safe storage of nuclear fuel; others have been conducted on arrays of low enriched U lattice assemblies. Neutronic measurements to date have shown they can be used to provide additional benchmark data for improvement and validation of criticality codes. Studies have previously been made to ascertain the need for critical experiments in support of fuel recycle operations. The result of an effort to update the list of needed critical experiments is summarized in this section. Experiments are listed in support of uranium based fuels and fast breeder reactor fuels. An effort is made to identify those areas within the fuel cycle wherein the critical experiment data would be applied and to identify the experiments (and data) required to fulfill the needs in each of these areas. The type and form of fuel on which the data would be obtained also are identified. In presenting this information, no attempt is made to describe the experiments in detail, or to define the actual number of critical experiments that might be needed to provide the required data

  17. Comparison of the transient behavior of lead-based advanced critical and sub-critical reactors

    International Nuclear Information System (INIS)

    Wang Gang; Gu Zhixing; Wang Zhen; Jin Ming; Bai Yunqing

    2014-01-01

    A lead-based reactor developed by FDS Team is proposed in 2011 and designed to be 10 MW. It is a pool type reactor and the primary coolant is driven by natural circulation. The reactor has two operation modes, which are a lead-based critical fast reactor mode and a lead-based sub-critical reactor mode. The conceptual designs of the two modes are both completed by 2013. In this paper, four transient accidents were simulated for both the critical and sub-critical reactors above by NTC-2D code, which is developed by FDS Team for advanced reactor safety analysis. The four accidents were protected and unprotected loss of heat sink accidents (PLOHS and ULOHS), protected and unprotected transient overpower accidents (PTOP and UTOP). The simulation results of the two reactors were compared and analyzed. The results showed that during PLOHS and PTOP accidents for both the two modes, all the key parameters (core power, fuel, cladding and coolant temperatures in the hottest channel) decreased to very small values after the reactor scrammed, which meant the reactors under the two modes were both safe. For ULOHS, the fuel, cladding and coolant temperatures of the sub-critical reactor increased bigger than those of the critical one. For UTOP, the parameters above of the critical fast reactor were much bigger than those of the sub-critical one. The analysis results showed different safety advantages of the lead-based critical fast and sub-critical reactors during different transient accidents. (author)

  18. Dependence of critical current on sample length analyzed by the variation of local critical current bent of BSCCO superconducting composite tape

    International Nuclear Information System (INIS)

    Matsubayashi, H.; Mukai, Y.; Shin, J.K.; Ochiai, S.; Okuda, H.; Osamura, K.; Otto, A.; Malozemoff, A.

    2008-01-01

    Using the high critical current type BSCCO composite tape fabricated at American Superconductor Corporation, the relation of overall critical current to the distribution of local critical current and the dependence of overall critical current on sample length of the bent samples were studied experimentally and analytically. The measured overall critical current was described well from the distribution of local critical current and n-value of the constituting short elements, by regarding the overall sample to be composed of local series circuits and applying the voltage summation model. Also the dependence of overall critical current on sample length could be reproduced in computer satisfactorily by the proposed simulation method

  19. A Response to Critics.

    Science.gov (United States)

    Hawley, Willis D.

    1987-01-01

    Provides a rejoinder to comments published in the February issue rebutting author's article on the risks and promises of extended teacher-preparation programs. Covers issues surrounding costs and benefits, the need for evaluative research before investment in programs, the differences between types of programs, and their objectives and possible…

  20. Activity in the Chronically Critically III

    Science.gov (United States)

    Winkelman, Chris; Higgins, Patricia A.; Kathy Chen, Yea-Jyh

    2006-01-01

    Although therapeutic activity prevents functional decline and reduces mortality, little is known about typical levels of activity among intensive care unit (ICU) patients. This report of a preliminary study describes typical therapeutic activity and compares the use of two measures of activity in a small sample of chronically critically ill adults. Type, frequency, and duration of therapeutic activity were measured simultaneously with direct observation and actigraphy. The only consistent activity documented was turning (frequency: 3 turns/8 hours; duration: mean average of 11 minutes). Analysis demonstrated acceptable agreement between the two measures of activity for both frequency and duration of therapeutic but not for type of activity. Congruence between measures for duration of activity was also supported. This study provides information for investigators and practitioners who are interested in measuring or implementing therapeutic activity in selected critically ill adults. PMID:16327517