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Sample records for kantowski sachs metric

  1. Kantowski--Sachs cosmological models as big-bang models

    International Nuclear Information System (INIS)

    Weber, E.

    1985-01-01

    In the presence of a nonzero cosmological constant Λ, we classify the anisotropic cosmological models of the Kantowski--Sachs type by means of the quantities epsilon 2 0 , q 0 , summation 0 corresponding, respectively, to the relative root-mean-square deviation from isotropy, the deceleration parameter, and the density parameter of the perfect fluid at a given time t = t 0 . We obtain for Λ>0 a set of big-bang models of zero measure as well as a set of cosmological models of nonzero measure evolving toward the de Sitter solution

  2. Gravitational, shear and matter waves in Kantowski-Sachs cosmologies

    Energy Technology Data Exchange (ETDEWEB)

    Keresztes, Zoltán; Gergely, László Á. [Department of Theoretical Physics, University of Szeged, Tisza Lajos krt 84-86, Szeged 6720 (Hungary); Forsberg, Mats; Bradley, Michael [Department of Physics, UmeåUniversity (Sweden); Dunsby, Peter K.S., E-mail: zkeresztes@titan.physx.u-szeged.hu, E-mail: forsberg.mats.a.b@gmail.com, E-mail: michael.bradley@physics.umu.se, E-mail: peter.dunsby@uct.ac.za, E-mail: gergely@physx.u-szeged.hu [Astrophysics, Cosmology and Gravity Centre (ACGC), University of Cape Town, Rondebosch 7701, Cape Town (South Africa)

    2015-11-01

    A general treatment of vorticity-free, perfect fluid perturbations of Kantowski-Sachs models with a positive cosmological constant are considered within the framework of the 1+1+2 covariant decomposition of spacetime. The dynamics is encompassed in six evolution equations for six harmonic coefficients, describing gravito-magnetic, kinematic and matter perturbations, while a set of algebraic expressions determine the rest of the variables. The six equations further decouple into a set of four equations sourced by the perfect fluid, representing forced oscillations and two uncoupled damped oscillator equations. The two gravitational degrees of freedom are represented by pairs of gravito-magnetic perturbations. In contrast with the Friedmann case one of them is coupled to the matter density perturbations, becoming decoupled only in the geometrical optics limit. In this approximation, the even and odd tensorial perturbations of the Weyl tensor evolve as gravitational waves on the anisotropic Kantowski-Sachs background, while the modes describing the shear and the matter density gradient are out of phase dephased by π /2 and share the same speed of sound.

  3. Kantowski-Sachs multidimensional cosmological models and dynamical dimensional reduction

    International Nuclear Information System (INIS)

    Demianski, M.; Rome Univ.; Golda, Z.A.; Heller, M.; Szydlowski, M.

    1988-01-01

    Einstein's field equations are solved for a multidimensional spacetime (KS) x Tsup(m), where (KS) is a four-dimensional Kantowski-Sachs spacetime and Tsup(m) is an m-dimensional torus. Among all possible vacuum solutions there is a large class of spacetimes in which the macroscopic space expands and the microscopic space contracts to a finite volume. We also consider a non-vacuum case and we explicitly solve the field equations for the matter satisfying the Zel'dovich equation of state. In non-vacuum models, with matter satisfying an equation of state p = γρ, O ≤ γ < 1, at a sufficiently late stage of evolution the microspace always expands and the dynamical dimensional reduction does not occur. (author)

  4. Kantowski-Sachs Einstein-æther perfect fluid models

    Energy Technology Data Exchange (ETDEWEB)

    Latta, Joey [Department of Mathematics and Statistics, Dalhousie University, Halifax, Nova Scotia, B3H 3J5 (Canada); Leon, Genly [Instituto de Física, Pontificia Universidad de Católica de Valparaíso, Casilla 4950, Valparaíso (Chile); Paliathanasis, Andronikos, E-mail: lattaj@mathstat.dal.ca, E-mail: genly.leon@pucv.cl, E-mail: anpaliat@phys.uoa.gr [Instituto de Ciencias Físicas y Matemáticas, Universidad Austral de Chile, Valdivia (Chile)

    2016-11-01

    We investigate Kantowski-Sachs models in Einstein-æ ther theory with a perfect fluid source using the singularity analysis to prove the integrability of the field equations and dynamical system tools to study the evolution. We find an inflationary source at early times, and an inflationary sink at late times, for a wide region in the parameter space. The results by A.A. Coley, G. Leon, P. Sandin and J. Latta ( JCAP 12 (2015) 010), are then re-obtained as particular cases. Additionally, we select other values for the non-GR parameters which are consistent with current constraints, getting a very rich phenomenology. In particular, we find solutions with infinite shear, zero curvature, and infinite matter energy density in comparison with the Hubble scalar. We also have stiff-like future attractors, anisotropic late-time attractors, or both, in some special cases. Such results are developed analytically, and then verified by numerics. Finally, the physical interpretation of the new critical points is discussed.

  5. Quantum field theory of the universe in the Kantowski-Sachs space-time

    International Nuclear Information System (INIS)

    Shen, Y.; Tan, Z.

    1996-01-01

    In this paper, the quantum field theory of the universe in the Kantowski-Sachs space-time is studied. An analogue of proceedings in quantum field theory is applied in curved space-time to the Kantowski-Sachs space-time, obtaining the wave function of the universe satisfied the Wheeler-DeWitt equation. Regarding the wave function as a universe field in the minisuperspace, the authors can not only overcome the difficulty of the probabilistic interpretation in quantum cosmology, but also come to the conclusion that there is multiple production of universes. The average number of the produced universes from nothing is calculated. The distribution of created universe is given. It is the Planckian distribution

  6. Exact Kantowski-Sachs and Bianchi types I and III cosmological models with a conformally invariant scalar field

    International Nuclear Information System (INIS)

    Accioly, A.J.

    1985-01-01

    Exact solutions of the Einstein-Conformally Invariant Scalar Field Equations are obtained for Kantowski-Sachs and Bianchi types I and III cosmologies. The presence of the conformally invariant scalar field is responsible for some interesting features of the solutions. In particular it is found that the Bianchi I model is consistent with the big-bang theory of cosmology. (Author) [pt

  7. Bianchi - I, II, VIII, IX and Kantowski-Sachs-like cosmological models with perfect fluid and electromagnetic fields with conductivity current

    International Nuclear Information System (INIS)

    Portugal, R.

    1984-01-01

    Three processes of solutions of the Einstein-Maxwell equations for Bianchi - I, II, VIII, IX and Kantowski-Sachs-like cosmological models with perfect fluid in magnetohydrolodynamical regimem are presented. Diagonal Bianchi-like models are considered with two anisotropy direction in the maximum. Solutions are found for Bianchi-II and IX-like models with energy conditions to be analyzed. Solutions are found for Bianchi-IX and Kantowski-Sachs-Like models with positive electric conductivity and satisfering to the predominant energy conditions. Solutions are formed for isotropic Kantowski-Sachs-Like models satisfering to the equation of state p=λρ, 0 0, admiting, in addition to the perfect fluid, electric field only. It is shown that a class of Bertotti-Robinson-like solutions is unstable by perturbations and it is carried in Kantowski-Sachs-like models with non-null electric conductivity. (L.C.) [pt

  8. Phenomenological dynamics of loop quantum cosmology in Kantowski-Sachs spacetime

    International Nuclear Information System (INIS)

    Chiou, D.-W.

    2008-01-01

    The fundamental theory and the semiclassical description of loop quantum cosmology (LQC) have been studied in the Friedmann-Robertson-Walker and Bianchi I models. As an extension to include both anisotropy and intrinsic curvature, this paper investigates the cosmological model of Kantowski-Sachs spacetime with a free massless scalar field at the level of phenomenological dynamics with the LQC discreteness corrections. The LQC corrections are implemented in two different improved quantization schemes. In both schemes, the big bang and big crunch singularities of the classical solution are resolved and replaced by the big bounces when the area or volume scale factor approaches the critical values in the Planck regime measured by the reference of the scalar field momentum. Symmetries of scaling are also noted and suggest that the fundamental spatial scale (area gap) may give rise to a temporal scale. The bouncing scenarios are in an analogous fashion of the Bianchi I model, naturally extending the observations obtained previously.

  9. Classification of Kantowski-Sachs and Bianchi Type III Space-Times According to Their Killing Vector Fields in Teleparallel Theory of Gravitation

    International Nuclear Information System (INIS)

    Shabbir, Ghulam; Khan, Suhail

    2010-01-01

    In this paper we classify Kantowski-Sachs and Bianchi type III space-times according to their teleparallel Killing vector fields using direct integration technique. It turns out that the dimension of the teleparallel Killing vector fields are 4 or 6, which are the same in numbers as in general relativity. In case of 4 the teleparallel Killing vector fields are multiple of the corresponding Killing vector fields in general relativity by some function of t. In the case of 6 Killing vector fields the metric functions become constants and the Killing vector fields in this case are exactly the same as in general relativity. Here we also discuss the Lie algebra in each case. (general)

  10. Time-dependent automorphism-inducing diffeomorphisms, open algebras and the generality of the Kantowski-Sachs vacuum geometry

    Science.gov (United States)

    Christodoulakis, T.; Papadopoulos, G. O.

    2002-10-01

    Following the spirit of a previous work of ours, we investigate the group of those general coordinate transformations (GCTs) which preserve manifest spatial homogeneity. In contrast to the case of Bianchi type models, here we permit an isometry group of motions G4 = SO(3) ⊗ Tr, where Tr is the translations group, along the radial direction, while SO(3) acts multiply transitively on each hypersurface of simultaneity Σt. The basis 1-forms cannot be invariant under the action of the entire isometry group and hence produce an open Lie algebra. In order for these GCTs to exist and have a nontrivial, well-defined action, certain integrability conditions have to be satisfied; their solutions, exhibiting the maximum expected 'gauge' freedom, can be used to simplify the generic, spatially homogeneous, line element. In this way an alternative proof of the generality of the Kantowski-Sachs (KS) vacuum is given, while its most general, manifestly homogeneous, form is explicitly presented.

  11. Tay-Sachs - resources

    Science.gov (United States)

    The following organizations provide information on Tay-Sachs disease : US National Library of Medicine -- ghr.nlm.nih.gov/condition/tay-sachs-disease March of Dimes -- www.marchofdimes.org/complications/tay-sachs- ...

  12. Tay-Sachs Disease

    Science.gov (United States)

    Tay-Sachs disease is a rare, inherited disease. It is a type of lipid metabolism disorder. It causes too ... cells, causing mental and physical problems. . Infants with Tay-Sachs disease appear to develop normally for the first few ...

  13. Energy in the Kantowski–Sachs space-time using teleparallel ...

    Indian Academy of Sciences (India)

    Energy in the Kantowski–Sachs space-time using teleparallel geometry ... Kantowski–Sachs metric; teleparallelism; gravitational energy. Abstract. The purpose of this paper is to examine the energy content of the inflationary Universe described by Kantowski–Sachs space-time in quasilocal approach of teleparallel gravity ...

  14. SAChES: Scalable Adaptive Chain-Ensemble Sampling.

    Energy Technology Data Exchange (ETDEWEB)

    Swiler, Laura Painton [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Ray, Jaideep [Sandia National Lab. (SNL-CA), Livermore, CA (United States); Ebeida, Mohamed Salah [Sandia National Lab. (SNL-NM), Albuquerque, NM (United States); Huang, Maoyi [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Hou, Zhangshuan [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Bao, Jie [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Ren, Huiying [Pacific Northwest National Lab. (PNNL), Richland, WA (United States)

    2017-08-01

    We present the development of a parallel Markov Chain Monte Carlo (MCMC) method called SAChES, Scalable Adaptive Chain-Ensemble Sampling. This capability is targed to Bayesian calibration of com- putationally expensive simulation models. SAChES involves a hybrid of two methods: Differential Evo- lution Monte Carlo followed by Adaptive Metropolis. Both methods involve parallel chains. Differential evolution allows one to explore high-dimensional parameter spaces using loosely coupled (i.e., largely asynchronous) chains. Loose coupling allows the use of large chain ensembles, with far more chains than the number of parameters to explore. This reduces per-chain sampling burden, enables high-dimensional inversions and the use of computationally expensive forward models. The large number of chains can also ameliorate the impact of silent-errors, which may affect only a few chains. The chain ensemble can also be sampled to provide an initial condition when an aberrant chain is re-spawned. Adaptive Metropolis takes the best points from the differential evolution and efficiently hones in on the poste- rior density. The multitude of chains in SAChES is leveraged to (1) enable efficient exploration of the parameter space; and (2) ensure robustness to silent errors which may be unavoidable in extreme-scale computational platforms of the future. This report outlines SAChES, describes four papers that are the result of the project, and discusses some additional results.

  15. Prenatal Diagnosis Of Tay-Sachs Disease

    Directory of Open Access Journals (Sweden)

    Özgür Özyüncü

    2010-04-01

    CONCLUSION: Tay-Sachs disease can be diagnosed prenatally by measuring hexosaminidase enzyme activity in fetal tissue samples with an acceptable complication rate. Prenatal diagnosis should be offered to families who have affected siblings with Tay-Sachs disease.

  16. Enfermedad de Tay-Sachs

    Directory of Open Access Journals (Sweden)

    Albia Josefina Pozo Alonso

    Full Text Available La enfermedad de Tay-Sachs es un trastorno neurodegenerativo progresivo de herencia autosómica recesiva. Se debe a la deficiencia de la enzima β-hexosaminidasa A, que provoca una acumulación de gangliósidos GM2 en los lisosomas. Se incluye dentro de las esfingolipidosis. De las esfingolipidosis que presentan mancha rojo cereza en la mácula, la enfermedad de Tay-Sachs es la única en la que no se evidencia hepatoesplenomegalia. La variante más frecuente se inicia en la lactancia. Se presenta un lactante del sexo masculino al que se le realizó el diagnóstico de esta entidad a los 8 meses de edad. A partir de los 4 meses comenzó a presentar una reacción de sobresalto. A los 6 meses comenzó a perder habilidades previamente adquiridas y crisis epilépticas mioclónicas. Se constató una disminución de la actividad específica de la enzima hexosaminidasa A en leucocitos.

  17. Relativistic cosmologies with closed, locally homogeneous space sections

    International Nuclear Information System (INIS)

    Fagundes, H.V.

    1985-01-01

    The homogeneous Bianchi and Kantowski-Sachs metrics of relativistic cosmology are investigated through their correspondence with recent geometrical results of Thurston. These allow a partial classification of the topologies for closed, locally homogeneous spaces according to Thurston's eight geometric types. Besides, which of the Bianchi-Kantowski-Sachs metrics can be imposed on closed space sections of cosmological models are learned. This is seen as a progress toward implementation of a postulate of the closure of space for both classical and quantum gravity. (Author) [pt

  18. Ethical Issues with Genetic Testing for Tay-Sachs.

    Science.gov (United States)

    Clayton, Tricia

    Several genetic disorders are specific to Jewish heritage; one of the most devastating is Tay-Sachs disease.Tay-Sachs is a fatal hereditary disease, causing progressive neurological problems for which there is no cure. Ethical issues surrounding genetic testing for Tay-Sachs within the Jewish community continue to be complex and multifaceted. A perspective of Tay-Sachs, using rights-based ethics and virtue ethics as a theoretical framework, is explored.

  19. Tay-Sachs and Sandhoff Diseases

    Science.gov (United States)

    ... more common in these groups: Creole of northern Argentina Metis Indians in Saskatchewan, Canada Lebanese If you or your partner is part of these ethnic groups, or you have a family history of Tay-Sachs disease or Sandhoff disease, tell ...

  20. Tay Sachs disease: an autopsy case report.

    Science.gov (United States)

    Jadhav, Meenal Vitthal; Landge, Meenal P; Sawaimoon, Satyakam K; Harke, Arun B; Deshmukh, Sanjay D

    2005-10-01

    This report describes a case report of a postmortem performed on a 5-year old patient of Tay-Sachs disease, presenting with failure to thrive, muscular flaccidity, and cherry-red spots on macula on fundoscopy. There was no history of similarly affected sibling or any other family member. The diagnosis was confirmed by enzyme studies. At postmortem, there was no organomegaly. The brain, on microscopy, showed vacuolated swollen neurons.

  1. The National Tay Sachs and Allied Diseases Association.

    Science.gov (United States)

    Zeitlin, Paula

    1986-01-01

    The National Tay-Sachs and Allied Diseases Association is involved in education, research, and prevention of Tay-Sachs, an inherited metabolic disorder which destroys the central nervous system, and over 30 related disorders. The group features a parent peer group network and a support group for carrier couples. (CL)

  2. National Tay-Sachs and Allied Diseases Association, Inc.

    Science.gov (United States)

    Exceptional Parent, 1977

    1977-01-01

    Reviewed are the history and organization, purpose and programs, and public services of the National Tay-Sachs and Allied Diseases Association, an organization geared toward eradicating Tay-Sachs disease (a hereditary disorder affecting primarily Jewish infants which generally leads to deterioration and death by the child's fifth year). (SBH)

  3. The metabolism of Tay-Sachs ganglioside: catabolic studies with lysosomal enzymes from normal and Tay-Sachs brain tissue

    Science.gov (United States)

    Tallman, John F.; Johnson, William G.; Brady, Roscoe O.

    1972-01-01

    The catabolism of Tay-Sachs ganglioside, N-acetylgalactosaminyl- (N-acetylneuraminosyl) -galactosylglucosylceramide, has been studied in lysosomal preparations from normal human brain and brain obtained at biopsy from Tay-Sachs patients. Utilizing Tay-Sachs ganglioside labeled with 14C in the N-acetylgalactosaminyl portion or 3H in the N-acetylneuraminosyl portion, the catabolism of Tay-Sachs ganglioside may be initiated by either the removal of the molecule of N-acetylgalactosamine or N-acetylneuraminic acid. The activity of the N-acetylgalactosamine-cleaving enzyme (hexosaminidase) is drastically diminished in such preparations from Tay-Sachs brain whereas the activity of the N-acetylneuraminic acid-cleaving enzyme (neuraminidase) is at a normal level. Total hexosaminidase activity as measured with an artificial fluorogenic substrate is increased in tissues obtained from patients with the B variant form of Tay-Sachs disease and it is virtually absent in the O-variant patients. The addition of purified neuraminidase and various purified hexosaminidases exerted only a minimal synergistic effect on the hydrolysis of Tay-Sachs ganglioside in the lysosomal preparations from the control or patient with the O variant of Tay-Sachs disease. Images PMID:4639018

  4. Survey on serum acetylecholinesterase (SAChE activity in selected farming communities at risk of organophosphate exposure, Southern Ghana

    Directory of Open Access Journals (Sweden)

    Ato Kwamena Tetteh

    2017-12-01

    Material and Methods ― The study was cross-sectional, involving 963 participants who consented to being screened for serum acetylcholinesterase (SAChE activity, using the dry chemistry test strips. The SAChE activity was then categorized into normal, low or high risk of developing symptoms associated with OP poisoning. Results ― The total group included 54.5% females and 45.5% males. Majority were crop farmers (46.3% and students/pupils (30.8%. The remaining 22.9% consisted mainly of traders, teachers, fishermen/fish processors, drivers and dressmakers. Very low SAChE activity unit of 30 U/ml. By this, a significant majority had either low or high risk of developing OP poisoning (χ2=4.43, 95%CI: 4.12–4.65, p=0.014 compared with those with normal activity. Most of the participants were in the 10–19 years age group of which 15.6% were in the high risk category, while 13.6% had low risk of OP poisoning. Reduced SAChE activity was predominant among all age groups compared with those with normal activity of the enzyme. As well, 27.3% who were females had reduced SAChE activity rate <30 U/ml (high risk of OP poisoning. Similarly, 23.4% of males were also at high risk of OP poisoning. There was however no statistical significance between the sex with regards to the numbers at risk of OP poisoning (χ2=4.80, 95% CI: 4.25–4.75, p=0.160. Conclusion ― This study provides some evidence of exposure, deduced from reduced SAChE activity, which potentially puts participants at a high risk of developing symptoms associated with OP poisoning. A follow-up study might be able to suggest to physicians in Ghana considering diagnostics of OP poisoning, especially for clients from farming communities where pesticides are used extensively.

  5. Tay Sachs and Related Storage Diseases: Family Planning

    Science.gov (United States)

    Schneiderman, Gerald; And Others

    1978-01-01

    Based on interviews with 24 families, the article discusses family planning and the choices available to those families in which a child has previously died from Tay-Sachs or related lipid storage diseases. (IM)

  6. Late-onset Tay-Sachs disease.

    Science.gov (United States)

    Barritt, Andrew W; Anderson, Stuart J; Leigh, P Nigel; Ridha, Basil H

    2017-10-01

    We discuss the assessment and differential diagnoses of a young adult Hungarian man with a 1-year history of a progressive and symmetric amyotrophic lateral sclerosis-like syndrome, along with irregular action tremor and stimulus-sensitive myoclonus of the arms. MR scan of the brain showed isolated cerebellar atrophy and formal neuropsychometric testing identified significant subclinical deficits in attention, processing speed and memory. We suspected a form of GM 2 gangliosidosis, and white cell enzyme analysis showed markedly reduced enzymatic activity of β-hexosaminidase A. Genetic testing subsequently revealed two heterozygous pathogenic mutations in the HEXA gene (c.1499delT p.(Leu500fs) and c.805G>A p.(Gly269Ser)), confirming the very rare diagnosis of adult-onset Tay-Sachs disease. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  7. A critique of Jeffrey D. Sachs's The end of poverty.

    Science.gov (United States)

    Henwood, Doug

    2006-01-01

    Jeffrey Sachs's The End of Poverty is a manifesto and how-to guide on ending extreme poverty around the world; it promotes the U.N. Millennium Development Goals. Sachs achieved fame with his policy package for the "stabilization" of Bolivia (which did nothing to relieve Bolivia's poverty), and became advisor to the Yeltsin government in Russia and to Poland, Slovenia, and Estonia as they began their transitions to capitalism (the last three mixed successes; Russia a thorough disaster). Sachs later became more prominent as a critic of development orthodoxy, and was economic advisor to the Jubilee 2000 movement. The End of Poverty is full of sharp critiques of Western imperialism, but his views on the rest of the development business are more conventional.

  8. Tay-Sachs disease in Jacob sheep.

    Science.gov (United States)

    Torres, Paola A; Zeng, Bai Jin; Porter, Brian F; Alroy, Joseph; Horak, Fred; Horak, Joan; Kolodny, Edwin H

    2010-12-01

    Autopsy studies of four Jacob sheep dying within their first 6-8 months of a progressive neurodegenerative disorder suggested the presence of a neuronal storage disease. Lysosomal enzyme studies of brain and liver from an affected animal revealed diminished activity of hexosaminidase A (Hex A) measured with an artificial substrate specific for this component of β-hexosaminidase. Absence of Hex A activity was confirmed by cellulose acetate electrophoresis. Brain lipid analyses demonstrated the presence of increased concentrations of G(M2)-ganglioside and asialo-G(M2)-ganglioside. The hexa cDNA of Jacob sheep was cloned and sequenced revealing an identical number of nucleotides and exons as in human HexA and 86% homology in nucleotide sequence. A missense mutation was found in the hexa cDNA of the affected sheep caused by a single nucleotide change at the end of exon 11 resulting in skipping of exon 11. Transfection of normal sheep hexa cDNA into COS1 cells and human Hex A-deficient cells led to expression of Hex S but no increase in Hex A indicating absence of cross-species dimerization of sheep Hex α-subunit with human Hex β-subunits. Using restriction site analysis, the heterozygote frequency of this mutation in Jacob sheep was determined in three geographically separate flocks to average 14%. This large naturally occurring animal model of Tay-Sachs disease is the first to offer promise as a means for trials of gene therapy applicable to human infants. Copyright © 2010 Elsevier Inc. All rights reserved.

  9. Planck 2015 results: XXI. The integrated Sachs-Wolfe effect

    DEFF Research Database (Denmark)

    Ade, P. A R; Aghanim, N.; Arnaud, M.

    2016-01-01

    This paper presents a study of the integrated Sachs-Wolfe (ISW) effect from the Planck 2015 temperature and polarization data release. This secondary cosmic microwave background (CMB) anisotropy caused by the large-scale time-evolving gravitational potential is probed from different perspectives....

  10. Rapid detection of fetal Mendelian disorders: Tay-Sachs disease.

    Science.gov (United States)

    Guetta, Esther; Peleg, Leah

    2008-01-01

    Tay-Sachs disease is an autosomal recessive storage disease caused by the impaired activity of the lysosomal enzyme hexosaminidase A. In this fatal disease, the sphingolipid GM2 ganglioside accumulates in the neurons. Due to high carrier rates and the severity of the disease, population screening and prenatal diagnosis of Tay-Sachs disease are routinely carried out in Israel. Laboratory diagnosis of Tay-Sachs is carried out with biochemical and DNA-based methods in peripheral and umbilical cord blood, amniotic fluid, and chorionic villi samples. The assay of hexosaminidase A (Hex A) activity is carried out with synthetic substrates, 4-methylumbelliferyl-6-sulfo-N-acetyl-beta-glucosaminide (4-MUGS) and 4-methylumbelliferil-N-acetyl-beta-glucosamine (4-MUG), and the DNA-based analysis involves testing for the presence of specific known mutations in the alpha-subunit gene of Hex A. Prenatal diagnosis of Tay-Sachs disease is accomplished within 24-48 h from sampling. The preferred strategy is to simultaneously carry out enzymatic analysis in the amniotic fluid supernatant or in chorionic villi and molecular DNA-based testing in an amniotic fluid cell-pellet or in chorionic villi.

  11. Midisuperspace-induced corrections to the Wheeler De Witt equation

    International Nuclear Information System (INIS)

    Mazzitelli, F.D.

    1992-04-01

    We consider the midisuperspace of four dimensional spherically symmetric metrics and the Kantowski-Sachs minisuperspace contained in it. We discuss the quantization of the midisuperspace using the fact that the dimensionally reduced Einstein Hilbert action becomes a scalar-tensor theory of gravity in two dimensions. We show that the covariant regularization procedure in the midisuperspace induces modifications into the minisuperspace Wheeler De Witt equation. (author). 8 refs

  12. Choroidal Coloboma in a Case of Tay-Sachs Disease

    OpenAIRE

    Ahmed, Nasreen Raees; Tripathy, Koushik; Kumar, Vivek; Gogia, Varun

    2014-01-01

    Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  13. Choroidal coloboma in a case of tay-sachs disease.

    Science.gov (United States)

    Ahmed, Nasreen Raees; Tripathy, Koushik; Kumar, Vivek; Gogia, Varun

    2014-01-01

    Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  14. Distribution of a pseudodeficiency allele among Tay-Sachs carriers

    Energy Technology Data Exchange (ETDEWEB)

    Tomczak, J.; Grebner, E.E. (Thomas Jefferson Univ., Philadelphia, PA (United States)); Boogen, C. (Univ. of Essen Medical School (Germany))

    1993-08-01

    Recently Triggs-Raine et al. (1992) identified a new mutation in the gene coding for the [alpha]-subunit of [beta]-hexosaminidase A (hex A), the enzyme whose deficiency causes Tay-Sachs disease. This mutation, a C[sub 739]-to-T transition in exon 7, results in an altered enzyme that is active (albeit at reduced levels) in cells but that has essentially no activity in serum. This so-called pseudodeficient allele was first detected in compound heterozygotes who also carried a Tay-Sachs disease allele and therefore had no detectable hex A in their serum but who were in good health. Carriers of this apparently benign mutation are generally indistinguishable from carriers of a lethal mutation by means of routine enzyme-based screening tests, because the product of the pseudodeficient allele is not detectable in serum and has decreased activity in cells. This suggests that some individuals who have been classified as Tay-Sachs carriers are actually carriers of the pseudodeficient allele and are not at risk to have a child affected with Tay-Sachs disease. The pseudodeficient allele may also be responsible for some inconclusive diagnoses, where leukocyte values fall below the normal range but are still above the carrier range. The fact that there are now two mutant alleles (the psuedodeficient and the adult) that are indistinguishable from the lethal infantile mutations by means of enzyme assay yet that are phenotypically very different and that together may account for as much as 12% of enzyme-defined carriers on the basis of the data here suggests that DNA analysis should be part of a comprehensive screening program. It will be particularly useful to identify the mutations in couples at risk, before they undergo prenatal diagnosis. DNA analysis will also resolve some inconclusive diagnoses.

  15. Choroidal Coloboma in a Case of Tay-Sachs Disease

    Directory of Open Access Journals (Sweden)

    Nasreen Raees Ahmed

    2014-01-01

    Full Text Available Coloboma as an ocular finding has been documented in various syndromes. Here we have a case of infantile Tay-Sachs disease associated with unilateral choroidal coloboma. To the best of our knowledge, such an association has not been documented in the literature. Whether such an association is a matter of chance or signifies the involvement of ganglioside metabolism in ocular embryogenesis remains to be elucidated.

  16. The white matter of the human cerebrum: part I The occipital lobe by Heinrich Sachs.

    Science.gov (United States)

    Forkel, Stephanie J; Mahmood, Sajedha; Vergani, Francesco; Catani, Marco

    2015-01-01

    This is the first complete translation of Heinrich Sachs' outstanding white matter atlas dedicated to the occipital lobe. This work is accompanied by a prologue by Prof Carl Wernicke who for many years was Sachs' mentor in Breslau and enthusiastically supported his work. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

  17. The white matter of the human cerebrum: Part I The occipital lobe by Heinrich Sachs

    Science.gov (United States)

    Forkel, Stephanie J.; Mahmood, Sajedha; Vergani, Francesco; Catani, Marco

    2015-01-01

    This is the first complete translation of Heinrich Sachs' outstanding white matter atlas dedicated to the occipital lobe. This work is accompanied by a prologue by Prof Carl Wernicke who for many years was Sachs' mentor in Breslau and enthusiastically supported his work. PMID:25527430

  18. Semantic metrics

    OpenAIRE

    Hu, Bo; Kalfoglou, Yannis; Dupplaw, David; Alani, Harith; Lewis, Paul; Shadbolt, Nigel

    2006-01-01

    In the context of the Semantic Web, many ontology-related operations, e.g. ontology ranking, segmentation, alignment, articulation, reuse, evaluation, can be boiled down to one fundamental operation: computing the similarity and/or dissimilarity among ontological entities, and in some cases among ontologies themselves. In this paper, we review standard metrics for computing distance measures and we propose a series of semantic metrics. We give a formal account of semantic metrics drawn from a...

  19. An inducible mouse model of late onset Tay-Sachs disease.

    Science.gov (United States)

    Jeyakumar, Mylvaganam; Smith, David; Eliott-Smith, Elena; Cortina-Borja, Mario; Reinkensmeier, Gabriele; Butters, Terry D; Lemm, Thorsten; Sandhoff, Konrad; Perry, V Hugh; Dwek, Raymond A; Platt, Frances M

    2002-08-01

    Mouse models of the G(M2) gangliosidoses, Tay-Sachs and Sandhoff disease, are null for the hexosaminidase alpha and beta subunits respectively. The Sandhoff (Hexb-/-) mouse has severe neurological disease and mimics the human infantile onset variant. However, the Tay-Sachs (Hexa-/-) mouse model lacks an overt phenotype as mice can partially bypass the blocked catabolic pathway and escape disease. We have investigated whether a subset of Tay-Sachs mice develop late onset disease. We have found that approximately 65% of the mice develop one or more clinical signs of the disease within their natural life span (n = 52, P disease at an earlier age (n = 21, P Tay-Sachs mice confirmed that pregnancy induces late onset Tay-Sachs disease. Onset of symptoms correlated with reduced up-regulation of hexosaminidase B, a component of the bypass pathway.

  20. Rapid identification of HEXA mutations in Tay-Sachs patients.

    Science.gov (United States)

    Giraud, Carole; Dussau, Jeanne; Azouguene, Emilie; Feillet, François; Puech, Jean-Philippe; Caillaud, Catherine

    2010-02-19

    Tay-Sachs disease (TSD) is a recessively inherited neurodegenerative disorder due to mutations in the HEXA gene resulting in a beta-hexosaminidase A (Hex A) deficiency. The purpose of this study was to characterize the molecular abnormalities in patients with infantile or later-onset forms of the disease. The complete sequencing of the 14 exons and flanking regions of the HEXA gene was performed with a unique technical condition in 10 unrelated TSD patients. Eleven mutations were identified, including five splice mutations, one insertion, two deletions and three single-base substitutions. Four mutations were novel: two splice mutations (IVS8+5G>A, IVS2+4delAGTA), one missense mutation in exon 6 (c.621T>G (p.D207E)) and one small deletion (c.1211-1212delTG) in exon 11 resulting in a premature stop codon at residue 429. The c.621T>G missense mutation was found in a patient presenting an infantile form. Its putative role in the pathogenesis of TSD is suspected as residue 207 is highly conserved in human, mouse and rat. Moreover, structural modelling predicted changes likely to affect substrate binding and catalytic activity of the enzyme. The time-saving procedure reported here could be useful for the characterization of Tay-Sachs-causing mutations, in particular in non-Ashkenazi patients mainly exhibiting rare mutations. Copyright (c) 2010 Elsevier Inc. All rights reserved.

  1. Tay-Sachs disease with conspicuous cranial computerized tomographic appearances

    International Nuclear Information System (INIS)

    Watanabe, Kishichiro; Mukawa, Akio; Muto, Kazuhiko; Nishikawa, Jiro; Takahashi, Shigeko.

    1985-01-01

    An autopsy case of a 3-year-old female infant with Tay-Sachs disease was presented. A cherry red spot in the fundus and a deficiency of N-acetyl-β-hexosaminidase A in the white blood cells were revealed soon after admission at the age of one year. Her parents and sister were found to be healthy carriers. The patient showed a typical clinical course with marked cranial swelling. In addition to the marked ballooning of neurons on light microscope, membranous cytoplasmic body (MCB) on electron microscope and abnormal accumulation of GM 2 ganglioside in the cerebral cortex by thin layer chromatography were confirmed in the autopsy specimens. In the late stage of her clinical course, the cranial computerized tomography (CT) demonstrated symmetric and deep-wavy hyperdense cerebral cortical zones, diffuse hypodensity and diminished volume of cerebral white matter, mild to moderate ventricular dilatation, and a small cerebellum and brainstem. These conspicuous appearances of the cranial CT seem to be characteristic of Tay-Sachs disease in the late stage, and they are derived from abnormal accumulation of GM 2 ganglioside in the cerebral cortex, and diffuse intense demyelination (dysmyelinating demyelination) of the cerebral white matter. (author)

  2. Structural consequences of amino acid substitutions causing Tay-Sachs disease.

    Science.gov (United States)

    Ohno, Kazuki; Saito, Seiji; Sugawara, Kanako; Sakuraba, Hitoshi

    2008-08-01

    To determine the structural changes in the alpha-subunit of beta-hexosaminidase due to amino acid substitutions causing Tay-Sachs disease, we built structural models of mutant alpha-subunits resulting from 33 missense mutations (24 infantile and 9 late-onset), and analyzed the influence of each amino acid replacement on the structure by calculating the number of atoms affected and determining the solvent-accessible surface area of the corresponding amino acid residue in the wild-type alpha-subunit. In the infantile Tay-Sachs group, the number of atoms influenced by a mutation was generally larger than that in the late-onset Tay-Sachs group in both the main chain and the side chain, and residues associated with the mutations found in the infantile Tay-Sachs group tended to be less solvent-accessible than those in the late-onset Tay-Sachs group. Furthermore, color imaging determined the distribution and degree of the structural changes caused by representative amino acid substitutions, and that there were also differences between the infantile and late-onset Tay-Sachs disease groups. Structural study is useful for elucidating the basis of Tay-Sachs disease.

  3. 'Cherry red spot' in a patient with Tay-Sachs disease: case report.

    Science.gov (United States)

    Aragão, Ricardo Evangelista Marrocos de; Ramos, Régia Maria Gondim; Pereira, Felipe Bezerra Alves; Bezerra, Andreya Ferreira Rodrigues; Fernandes, Daniel Nogueira

    2009-01-01

    Tay-Sachs disease is an autosomal recessive disorder of sphingolipid metabolism, caused by enzyme hexosaminidase A deficiency that leads to an accumulation of GM2 in neurocytes which results in progressive loss of neurological function. The accumulation of lipid in retinal ganglion cells that leads to a chalk-white appearance of the fundus called 'cherry red spot' is the hallmark of Tay-Sachs disease. It is also seen in others neurometabolic diseases as well as in central retinal artery occlusion. This case reports a child with Tay-Sachs disease in a family with four previous similar deaths without diagnostic.

  4. Atypical presentation of late-onset Tay-Sachs disease.

    Science.gov (United States)

    Deik, Andres; Saunders-Pullman, Rachel

    2014-05-01

    Late-onset Tay-Sachs disease (LOTS) is a lysosomal storage disease caused by deficient Beta-hexosaminidase A activity. We describe a 53-year-old woman who presented with adult-onset leg weakness, and whose initial diagnosis was progressive muscular atrophy without identifiable etiology. Development of cerebellar ataxia in mid-life prompted reassessment. Beta-hexosaminidase A quantification assay demonstrated absence of the isozyme. Genetic testing identified compound heterozygous mutations in the HEXA gene, confirming the diagnosis of LOTS. The phenotypic spectrum of LOTS includes motor neuronopathy, ataxia, choreoathetosis, neuropathy, and psychiatric symptoms in various combinations. This patient highlights the emergence of different clinical features over many years and emphasizes the need to consider LOTS in the differential diagnosis of progressive muscular atrophy. Copyright © 2013 Wiley Periodicals, Inc.

  5. Tay-Sachs disease: current perspectives from Australia.

    Science.gov (United States)

    Lew, Raelia M; Burnett, Leslie; Proos, Anné L; Delatycki, Martin B

    2015-01-01

    Tay-Sachs disease (TSD) is a fatal, recessively inherited neurodegenerative condition of infancy and early childhood. Although rare in most other populations, the carrier frequency is one in 25 in Ashkenazi Jews. Australian high-school-based TSD preconception genetic screening programs aim to screen, educate, and optimize reproductive choice for participants. These programs have demonstrated high uptake, low psychological morbidity, and have been shown to result in fewer than expected Jewish TSD-affected births over 18 years of operation. The majority of Jewish individuals of reproductive age outside of the high school screening program setting in Australia have not accessed screening. Recent recommendations advocate supplementing the community high school screening programs with general practitioner- and obstetrician-led genetic screening of Ashkenazi Jewish individuals for TSD and other severe recessive diseases for which this group is at risk. Massively parallel DNA sequencing is expected to become the testing modality of choice over the coming years.

  6. Tay-Sachs disease: current perspectives from Australia

    Directory of Open Access Journals (Sweden)

    Lew RM

    2015-01-01

    Full Text Available Raelia M Lew,1,7 Leslie Burnett,2,3,4 Anné L Proos,2 Martin B Delatycki5,6 1Department of Obstetrics and Gynecology, QEII Research Institute for Mothers and Infants, The University of Sydney, Australia; 2NSW Health Pathology North, Royal North Shore Hospital, St Leonards, Australia; 3SEALS, Prince of Wales Hospital, Randwick, Australia; 4Sydney Medical School-Northern, Royal North Shore Hospital E25, University of Sydney, Sydney, Australia; 5Department of Clinical Genetics, Austin Health, Heidelberg, Australia; 6Bruce Lefroy Centre for Genetic Health Research, Murdoch Childrens Research Institute, Parkville, Australia; 7Department of Obstetrics and Gynaecology, Dentistry and Health Sciences, Faculty of Medicine, The University of Melbourne, Melbourne, Australia Abstract: Tay-Sachs disease (TSD is a fatal, recessively inherited neurodegenerative condition of infancy and early childhood. Although rare in most other populations, the carrier frequency is one in 25 in Ashkenazi Jews. Australian high-school-based TSD preconception genetic screening programs aim to screen, educate, and optimize reproductive choice for participants. These programs have demonstrated high uptake, low psychological morbidity, and have been shown to result in fewer than expected Jewish TSD-affected births over 18 years of operation. The majority of Jewish individuals of reproductive age outside of the high school screening program setting in Australia have not accessed screening. Recent recommendations advocate supplementing the community high school screening programs with general practitioner- and obstetrician-led genetic screening of Ashkenazi Jewish individuals for TSD and other severe recessive diseases for which this group is at risk. Massively parallel DNA sequencing is expected to become the testing modality of choice over the coming years. Keywords: Tay-Sachs disease, genetic screening, Australia

  7. CMB anisotropies at all orders: the non-linear Sachs-Wolfe formula

    OpenAIRE

    Roldan, Omar

    2017-01-01

    We obtain the non-linear generalization of the Sachs-Wolfe + integrated Sachs-Wolfe (ISW) formula describing the CMB temperature anisotropies. Our formula is valid at all orders in perturbation theory, is also valid in all gauges and includes scalar, vector and tensor modes. A direct consequence of our results is that the maps of the logarithmic temperature anisotropies are much cleaner than the usual CMB maps, because they automatically remove many secondary anisotropies. This can for instan...

  8. Segregation of Tay-Sachs and Sandhoff alleles in a non-Jewish family.

    OpenAIRE

    Lane, A B; Young, E; Jenkins, T

    1980-01-01

    A non-Jewish family is presented in which the genes for Tay-Sachs disease and Sandhoff disease are segregating. Individuals heterozygous for both alleles have low serum and white cell total hexosaminidase levels together with a proportion of heat-labile hexosaminidase A (HEX A) which falls in the normal range. The individuals would not be detected as carriers of Tay-Sachs disease or Sandhoff disease in a population screening program.

  9. Segregation of Tay-Sachs and Sandhoff alleles in a non-Jewish family.

    Science.gov (United States)

    Lane, A B; Young, E; Jenkins, T

    1980-01-01

    A non-Jewish family is presented in which the genes for Tay-Sachs disease and Sandhoff disease are segregating. Individuals heterozygous for both alleles have low serum and white cell total hexosaminidase levels together with a proportion of heat-labile hexosaminidase A (HEX A) which falls in the normal range. The individuals would not be detected as carriers of Tay-Sachs disease or Sandhoff disease in a population screening program. PMID:7446530

  10. Tay-Sach disease with "cherry-red spot"--first reported case in Malaysia.

    Science.gov (United States)

    Chan, L Y; Balasubramaniam, S; Sunder, R; Jamalia, R; Karunakar, T V N; Alagaratnam, J

    2011-12-01

    We present a rare case of Tay-Sachs disease with retinal 'cherry-red spots' in a 19-month-old Malay child. Molecular genetic studies confirmed the diagnosis. The case highlights that 'cherry-red spot' is a useful clinical clue in Tay-Sachs disease and several other lysosomal storage disorders. It serves as an ideal illustration of the eye as a window to inborn error of metabolism.

  11. Metric learning

    CERN Document Server

    Bellet, Aurelien; Sebban, Marc

    2015-01-01

    Similarity between objects plays an important role in both human cognitive processes and artificial systems for recognition and categorization. How to appropriately measure such similarities for a given task is crucial to the performance of many machine learning, pattern recognition and data mining methods. This book is devoted to metric learning, a set of techniques to automatically learn similarity and distance functions from data that has attracted a lot of interest in machine learning and related fields in the past ten years. In this book, we provide a thorough review of the metric learnin

  12. Initial-boundary-value problem of the self-gravitating scalar field in the Bondi-Sachs gauge

    International Nuclear Information System (INIS)

    Frittelli, Simonetta; Gomez, Roberto

    2007-01-01

    It is shown that, in the Bondi-Sachs gauge that fixes the speed of incoming light rays to the value 1, the Einstein equations coupled to a scalar field in spherical symmetry are cast into a symmetric-hyperbolic system of equations for the scalar field, lapse and shift as fundamental variables. In this system of equations, the lapse and shift are incoming characteristic fields, and the scalar field has three components: incoming, outgoing and static. A constraint-preserving boundary condition is prescribed by imposing the projection of the Einstein equation normal to the boundary at the outer value of the radial coordinate. The boundary condition specifies one of the two incoming metric fields. The remaining incoming metric field and the incoming scalar field component need to be specified arbitrarily. Numerical simulations of the scattering of the scalar field by a black hole in the nonlinear regime are presented that illustrate interesting facts about black-hole physics and the behavior of the characteristic variables of the problem

  13. Metrication manual

    International Nuclear Information System (INIS)

    Harper, A.F.A.; Digby, R.B.; Thong, S.P.; Lacey, F.

    1978-04-01

    In April 1978 a meeting of senior metrication officers convened by the Commonwealth Science Council of the Commonwealth Secretariat, was held in London. The participants were drawn from Australia, Bangladesh, Britain, Canada, Ghana, Guyana, India, Jamaica, Papua New Guinea, Solomon Islands and Trinidad and Tobago. Among other things, the meeting resolved to develop a set of guidelines to assist countries to change to SI and to compile such guidelines in the form of a working manual

  14. Effective gene therapy in an authentic model of Tay-Sachs-related diseases.

    Science.gov (United States)

    Cachón-González, M Begoña; Wang, Susan Z; Lynch, Andrew; Ziegler, Robin; Cheng, Seng H; Cox, Timothy M

    2006-07-05

    Tay-Sachs disease is a prototypic neurodegenerative disease. Lysosomal storage of GM2 ganglioside in Tay-Sachs and the related disorder, Sandhoff disease, is caused by deficiency of beta-hexosaminidase A, a heterodimeric protein. Tay-Sachs-related diseases (GM2 gangliosidoses) are incurable, but gene therapy has the potential for widespread correction of the underlying lysosomal defect by means of the secretion-recapture cellular pathway for enzymatic complementation. Sandhoff mice, lacking the beta-subunit of hexosaminidase, manifest many signs of classical human Tay-Sachs disease and, with an acute course, die before 20 weeks of age. We treated Sandhoff mice by stereotaxic intracranial inoculation of recombinant adeno-associated viral vectors encoding the complementing human beta-hexosaminidase alpha and beta subunit genes and elements, including an HIV tat sequence, to enhance protein expression and distribution. Animals survived for >1 year with sustained, widespread, and abundant enzyme delivery in the nervous system. Onset of the disease was delayed with preservation of motor function; inflammation and GM2 ganglioside storage in the brain and spinal cord was reduced. Gene delivery of beta-hexosaminidase A by using adeno-associated viral vectors has realistic potential for treating the human Tay-Sachs-related diseases.

  15. [Tay-Sachs disease in non-Jewish infant in Israel].

    Science.gov (United States)

    Nadim, Nasser

    2012-01-01

    Tay-Sachs disease, also known as GM2 gangliosidosis or Hexosaminidase A deficiency is an autosomal recessive genetic fatal disorder. The disease is known to appear in East European Ashkenazi Jews, North African Jews, and Quebec French Canadians exclusively, but, with different frequency and type of mutation. Its most common variant is the infantile type Tay-Sachs disease. Juvenile and late-onset forms of the disease are infrequent and slowly progressive. At nearly 3 to 6 months old, a baby with Tay-Sachs progressively loses his motor skills and attentiveness. Startle responses and hyperreflexia become prominent, especially on eliciting deep patellar and Achilles reflexes, as a consequence of neurodegeneration of the upper motor neuron. Other systemic damage ensues gradually; seizures, blindness, spasticity of limbs, inability to swallow and breathe, and eventually the baby dies at 1-4 years of age. All Tay-Sachs patients have a "cherry red spot", easily seen in the macula area of the retina, using an ophthalmoscope. The "cherry red spot" is the only normal part of the retina in these sick babies. The case presented here emphasizes that Tay-Sachs disease is sometimes misdiagnosed at first visits even by an experienced clinician, because of his lack of awareness that this disease is not exclusively a Jewish disease.

  16. Basic versus applied research: Julius Sachs (1832-1897) and the experimental physiology of plants.

    Science.gov (United States)

    Kutschera, Ulrich

    2015-01-01

    The German biologist Julius Sachs was the first to introduce controlled, accurate, quantitative experimentation into the botanical sciences, and is regarded as the founder of modern plant physiology. His seminal monograph Experimental-Physiologie der Pflanzen (Experimental Physiology of Plants) was published 150 y ago (1865), when Sachs was employed as a lecturer at the Agricultural Academy in Poppelsdorf/Bonn (now part of the University). This book marks the beginning of a new era of basic and applied plant science. In this contribution, I summarize the achievements of Sachs and outline his lasting legacy. In addition, I show that Sachs was one of the first biologists who integrated bacteria, which he considered to be descendants of fungi, into the botanical sciences and discussed their interaction with land plants (degradation of wood etc.). This "plant-microbe-view" of green organisms was extended and elaborated by the laboratory botanist Wilhelm Pfeffer (1845-1920), so that the term "Sachs-Pfeffer-Principle of Experimental Plant Research" appears to be appropriate to characterize this novel way of performing scientific studies on green, photoautotrophic organisms (embryophytes, algae, cyanobacteria).

  17. Proton MR spectroscopy in three children with Tay-Sachs disease.

    Science.gov (United States)

    Aydin, Kubilay; Bakir, Baris; Tatli, Burak; Terzibasioglu, Ege; Ozmen, Meral

    2005-11-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM(2) gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease.

  18. Proton MR spectroscopy in three children with Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Aydin, Kubilay; Bakir, Baris; Terzibasioglu, Ege [Istanbul University, Neuroradiology Division, Department of Radiology, Istanbul (Turkey); Tatli, Burak; Ozmen, Meral [Istanbul University, Department of Paediatric Neurology, Istanbul (Turkey)

    2005-11-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM{sub 2} gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease. (orig.)

  19. Proton MR spectroscopy in three children with Tay-Sachs disease

    International Nuclear Information System (INIS)

    Aydin, Kubilay; Bakir, Baris; Terzibasioglu, Ege; Tatli, Burak; Ozmen, Meral

    2005-01-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM 2 gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease. (orig.)

  20. Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease.

    Science.gov (United States)

    Kodama, Takashi; Togawa, Tadayasu; Tsukimura, Takahiro; Kawashima, Ikuo; Matsuoka, Kazuhiko; Kitakaze, Keisuke; Tsuji, Daisuke; Itoh, Kohji; Ishida, Yo-Ichi; Suzuki, Minoru; Suzuki, Toshihiro; Sakuraba, Hitoshi

    2011-01-01

    To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2) levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex) B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-GM2 levels in the brain and plasma in Sandhoff mice were apparently increased compared with those in wild-type mice, and they decreased on intracerebroventricular administration of the modified Hex B. The lyso-GM2 levels in plasma of patients with Tay-Sachs disease and Sandhoff disease were increased, and the increase in lyso-GM2 was associated with a decrease in Hex A activity. Lyso-GM2 is expected to be a potential biomarker of Tay-Sachs disease and Sandhoff disease.

  1. [Sandhoff's and Tay-Sachs disease--based on our own cases].

    Science.gov (United States)

    Kokot, Witold; Raczyńska, Krystyna; Krajka-Lauer, Jarosława; Iwaszkiewicz-Bilikiewicz, Barbara; Wierzba, Jolanta

    2004-01-01

    The authors described two infant with Sandhoff's and Tay-Sachs disease. Tay-Sachs disease is well-known inherited disease leading to an accumulation of gangliosides in the brain and retina. Sandhoffs disease (GM2 gangliosidosis type 0) was diagnosed in an infant, in whom a progressive neurological disorder and cherry-red foveal spots were developed. In addition, to the general clinical examination, indirect ophthalnoscopy, blood white cells enzymatic examination are used to make definitive diagnosis. In this cases, the early eye fundus examination allowed us to make the proper diagnosis. The fundus change is characterized by the "cherry-red spot" in the central area.

  2. Planck 2015 results: XXI. The integrated Sachs-Wolfe effect

    International Nuclear Information System (INIS)

    Ade, P. A. R.; Aghanim, N.; Arnaud, M.; Ashdown, M.; Aumont, J.

    2016-01-01

    Here, this paper presents a study of the integrated Sachs-Wolfe (ISW) effect from the Planck 2015 temperature and polarization data release. This secondary cosmic microwave background (CMB) anisotropy caused by the large-scale time-evolving gravitational potential is probed from different perspectives. The CMB is cross-correlated with different large-scale structure (LSS) tracers: radio sources from the NVSS catalogue; galaxies from the optical SDSS and the infrared WISE surveys; and the Planck 2015 convergence lensing map. The joint cross-correlation of the CMB with the tracers yields a detection at 4σ where most of the signal-to-noise is due to the Planck lensing and the NVSS radio catalogue. In fact, the ISW effect is detected from the Planck data only at ≈3σ (through the ISW-lensing bispectrum), which is similar to the detection level achieved by combining the cross-correlation signal coming from all the galaxy catalogues mentioned above. We study the ability of the ISW effect to place constraints on the dark-energy parameters; in particular, we show that Ω Λ is detected at more than 3σ. This cross-correlation analysis is performed only with the Planck temperature data, since the polarization scales available in the 2015 release do not permit significant improvement of the CMB-LSS cross-correlation detectability. Nevertheless, the Planck polarization data are used to study the anomalously large ISW signal previously reported through the aperture photometry on stacked CMB features at the locations of known superclusters and supervoids, which is in conflict with ΛCDM expectations. We find that the current Planck polarization data do not exclude that this signal could be caused by the ISW effect. In addition, the stacking of the Planck lensing map on the locations of superstructures exhibits a positive cross-correlation with these large-scale structures. Finally, we have improved our previous reconstruction of the ISW temperature fluctuations by combining

  3. Black Hole Entropy from Bondi-Metzner-Sachs Symmetry at the Horizon.

    Science.gov (United States)

    Carlip, S

    2018-03-09

    Near the horizon, the obvious symmetries of a black hole spacetime-the horizon-preserving diffeomorphisms-are enhanced to a larger symmetry group with a three-dimensional Bondi-Metzner-Sachs algebra. Using dimensional reduction and covariant phase space techniques, I investigate this augmented symmetry and show that it is strong enough to determine the black hole entropy in any dimension.

  4. Shock absorption of below-knee prostheses : A comparison between the SACH and the Multiflex foot

    NARCIS (Netherlands)

    van Leeuwen, J. L.; Speth, L. A W M; Daanen, H. A M

    1990-01-01

    Shock waves were measured during walking on a treadmill on the metal tube of a below-knee KBM prosthesis, provided either with a SACH foot or with a Multiflex foot. Accelerations were measured in the axial direction and the dorso-ventral direction, about 160 mm proximal to the sole of the shoe. The

  5. 78 FR 24447 - Goldman Sachs Trust, et al.; Notice of Application

    Science.gov (United States)

    2013-04-25

    ... SECURITIES AND EXCHANGE COMMISSION [Investment Company Act Release No. 30471; 812-14075] Goldman Sachs Trust, et al.; Notice of Application April 19, 2013. AGENCY: Securities and Exchange Commission..., or cause more than 10% of the acquired company's voting stock to be owned by investment companies and...

  6. Tay-Sachs disease: high gene frequency in a non-Jewish population.

    Science.gov (United States)

    Kelly, T E; Chase, G A; Kaback, M M; Kumor, K; McKusick, V A

    1975-01-01

    A non-Amish "Pennsylvania Dutch" semi-isolate was found to have a high frequency of Tay-Sachs gene. This high frequency could be ascribed to founder effect and may represent, in microcosm, how this mechanism could have produced the high gene frequency among Ashkenazi Jews. PMID:803011

  7. Late-onset Tay-Sachs disease: adverse effects of medications and implications for treatment.

    Science.gov (United States)

    Shapiro, B E; Hatters-Friedman, S; Fernandes-Filho, J A; Anthony, K; Natowicz, M R

    2006-09-12

    The authors conducted a retrospective and brief prospective study of adverse effects of approximately 350 medications in 44 adults with late-onset Tay-Sachs disease (LOTS). Some medications were relatively safe, whereas others, particularly haloperidol, risperidone, and chlorpromazine, were associated with neurologic worsening.

  8. A pseudodeficiency allele common in non-Jewish Tay-Sachs carriers: Implications for carrier screening

    Energy Technology Data Exchange (ETDEWEB)

    Triggs-Raine, B.L.; Akerman, B.R.; Gravel, R.A. (McGill Univ.-Montreal Children' s Hospital Research Institute, Montreal, Quebec (Canada)); Mules, E.H.; Thomas, G.H.; Dowling, C.E. (Johns Hopkins School of Medicine, Baltimore, MD (United States)); Kaback, M.M.; Lim-Steele, J.S.T. (Univ. of California, San Diego, CA (United States)); Natowicz, M.R. (Eunice Kennedy Shriver Center for Mental Retardation, Waltham, MA (United States)); Grebner, E.E. (Thomas Jefferson Univ., Philadelphia, PA (United States)); Navon, R.R. (Tel-Aviv Univ., Kfar-Sava (Israel)); Welch, J.P. (Dalhousie Univ., Halifax, Nova, Scotia (Canada)); Greenberg, C.R. (Univ. of Manitoba, Winnipeg (Canada))

    1992-10-01

    Deficiency of [beta]-hexosaminidase A (Hex A) activity typically results in Tay-Sachs disease. However, healthy subjects found to be deficient in Hex A activity (i.e., pseudodeficient) by means of in vitro biochemical tests have been described. The authors analyzed the HEXA gene of one pseudodeficient subject and identified both a C[sub 739]-to-T substitution that changes Arg[sub 247][yields]Trp on one allele and a previously identified Tay-Sachs disease mutation of the second allele. Six additional pseudodeficient subjects were found to have the C[sub 739]-to-T but for none of 36 Jewish enzyme-defined carries who did not have one of three known mutations common to this group. The C[sub 739]-to-T allele, together with a [open quotes]true[close quotes] Tay-Sachs disease allele, causes Hex A pseudodeficiency. Given both the large proportion of non-Jewish carriers with this allele and that standard biochemical screening cannot differentiate between heterozygotes for the C[sub 739]-to-T mutations and Tay-Sachs disease carriers, DNA testing for this mutation in at-risk couples is essential. This could prevent unnecessary or incorrect prenatal diagnoses. 40 refs., 3 figs., 4 tabs.

  9. Energy in the Kantowski–Sachs space-time using teleparallel ...

    Indian Academy of Sciences (India)

    Inflation is again considered to be one of the early phases of the Universe [10]. For testing gravitational energy–momentum tensor of the teleparallel theory in quasilocal approach and also in the. Hamiltonian structure of TEGR, it is intended to calculate the total energy of inflationary. Kantowski–Sachs-type Universe. Again ...

  10. Outcomes of arthroscopic "Remplissage": capsulotenodesis of the engaging large Hill-Sachs lesion

    Directory of Open Access Journals (Sweden)

    Mayo Lee

    2011-06-01

    Full Text Available Abstract Background A Hill-Sachs lesion of the humeral head after a shoulder dislocation is clinically insignificant in most cases. However, a sizable defect will engage with the anterior rim of the glenoid and cause instability even after anterior glenoid reconstruction. The purpose of this study was to evaluate the outcome of arthroscopic capsulotenodesis of the posterior capsule and infraspinatus tendon ("remplissage" to seal a large engaging Hill-Sachs lesion in an unstable shoulder. Methods This was a prospective follow-up study of patients who underwent arthroscopic surgery for recurrent shoulder instability with a large engaging Hill-Sachs lesion from 2007 to 2009. The clinical results were measured preoperatively and postoperatively with the Simple Shoulder test (SST and the Rowe score for instability. Results Eleven patients met the inclusion criteria of this study. The mean follow-up time was 30 months (range 24 to 35 months. At the last follow-up, significant improvement was observed in both scores with no recurrent dislocations. The mean SST improved from 6.6 to 11 (p Conclusions Arthroscopic remplissage for shoulder instability is an effective soft tissue technique to seal a large engaging Hill-Sachs lesion with respect to recurrence rate, range of motion and shoulder function.

  11. Three-dimensional computed tomography measurement accuracy of varying Hill-Sachs lesion size.

    Science.gov (United States)

    Ho, Anthony; Kurdziel, Michael D; Koueiter, Denise M; Wiater, J Michael

    2018-02-01

    The glenoid track concept has been proposed to correlate shoulder stability with bone loss. Accurate assessment of Hill-Sachs lesion size preoperatively may affect surgical planning and postoperative outcomes; however, no measurement method has been universally accepted. This study aimed to assess the accuracy and reliability of measuring Hill-Sachs lesion sizes using 3-dimensional (3D) computed tomography (CT). Nine polyurethane humerus bone substitutes were used to create Hill-Sachs lesions of varying sizes with a combination of lesion depth (shallow, intermediate, and deep) and width (small, medium, and large). Specimens were scanned with a clinical CT scanner for size measurements and a micro-CT scanner for measurement of true lesion size. Six evaluators repeated measurements twice in a 2-week interval. Scans were measured by use of 3D CT reconstructions for length, width, and Hill-Sachs interval and with use of 2D CT for depth. The interclass correlation coefficient evaluated interobserver and intraobserver variability and percentage error, and Student t-tests assessed measurement accuracy. Interclass correlation coefficient reliability demonstrated strong agreement for all variables measured (0.856-0.975). Percentage error between measured length and measured depth and the true measurement significantly varied with respect to both lesion depth (P = .003 and P = .005, respectively) and lesion size (P = .049 and P = .004, respectively). The 3D CT imaging is effective and reproducible in determining lesion size. Determination of Hill-Sachs interval width is also reliable when it is applied to the glenoid track concept. Measured values on 3D and 2-dimensional imaging using a conventional CT scanner may slightly underestimate true measurements. Copyright © 2017 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.

  12. Sachês antimicrobianos em pós-colheita de manga

    Directory of Open Access Journals (Sweden)

    Eber Antonio Alves Medeiros

    2011-10-01

    Full Text Available Objetivou-se com este trabalho desenvolver sachês incorporados com óleos essenciais avaliando as propriedades físico-químicas e microbiológicas de mangas armazenadas em saco de papel contendo estes sachês em seu interior. Os óleos essenciais de orégano (Origanum vulgaris e capim-limão (Cymbopogon citratus foram incorporados em saches, e sua atividade antimicrobiana foi testada nos fungos Colletotrichum gloeosporides, Lasiodiplodia theobromae, Xanthomonas campestris pv. mangiferae indica, Alternaria alternata. Frutos de manga 'Tommy Atkins' foram acondicionados individualmente em sacos de papel contendo em seu interior um sachê antimicrobiano e mantidos a 25 °C ± 2 °C e UR 80% ± 5% por nove dias. Os parâmetros cor da epiderme, firmeza de polpa, sólidos solúveis, acidez titulável e pH sofreram pouca influência dos óleos essenciais de orégano e capim limão, indicando que a presença do óleo essencial não altera as características físico-químicas da polpa de manga. Os sachês ativos incorporados com óleos essenciais de orégano e capim limão apresentaram controle no crescimento dos microrganismos testados, sendo o capim-limão mais eficiente, reduzindo em aproximadamente 2 ciclos Log a contagem de mesófilos aeróbios e fungos filamentosos e leveduras em relação ao tratamento controle empregado.

  13. The frequency of Tay-Sachs disease causing mutations in the Brazilian Jewish population justifies a carrier screening program

    Directory of Open Access Journals (Sweden)

    Roberto Rozenberg

    Full Text Available CONTEXT: Tay-Sachs disease is an autosomal recessive disease characterized by progressive neurologic degeneration, fatal in early childhood. In the Ashkenazi Jewish population the disease incidence is about 1 in every 3,500 newborns and the carrier frequency is 1 in every 29 individuals. Carrier screening programs for Tay-Sachs disease have reduced disease incidence by 90% in high-risk populations in several countries. The Brazilian Jewish population is estimated at 90,000 individuals. Currently, there is no screening program for Tay-Sachs disease in this population. OBJECTIVE: To evaluate the importance of a Tay-Sachs disease carrier screening program in the Brazilian Jewish population by determining the frequency of heterozygotes and the acceptance of the program by the community. SETTING: Laboratory of Molecular Genetics - Institute of Biosciences - Universidade de São Paulo. PARTICIPANTS: 581 senior students from selected Jewish high schools. PROCEDURE: Molecular analysis of Tay-Sachs disease causing mutations by PCR amplification of genomic DNA, followed by restriction enzyme digestion. RESULTS: Among 581 students that attended educational classes, 404 (70% elected to be tested for Tay-Sachs disease mutations. Of these, approximately 65% were of Ashkenazi Jewish origin. Eight carriers were detected corresponding to a carrier frequency of 1 in every 33 individuals in the Ashkenazi Jewish fraction of the sample. CONCLUSION: The frequency of Tay-Sachs disease carriers among the Ashkenazi Jewish population of Brazil is similar to that of other countries where carrier screening programs have led to a significant decrease in disease incidence. Therefore, it is justifiable to implement a Tay-Sachs disease carrier screening program for the Brazilian Jewish population.

  14. The frequency of Tay-Sachs disease causing mutations in the Brazilian Jewish population justifies a carrier screening program.

    Science.gov (United States)

    Rozenberg, R; Pereira, L da V

    2001-07-05

    Tay-Sachs disease is an autosomal recessive disease characterized by progressive neurologic degeneration, fatal in early childhood. In the Ashkenazi Jewish population the disease incidence is about 1 in every 3,500 newborns and the carrier frequency is 1 in every 29 individuals. Carrier screening programs for Tay-Sachs disease have reduced disease incidence by 90% in high-risk populations in several countries. The Brazilian Jewish population is estimated at 90,000 individuals. Currently, there is no screening program for Tay-Sachs disease in this population. To evaluate the importance of a Tay-Sachs disease carrier screening program in the Brazilian Jewish population by determining the frequency of heterozygotes and the acceptance of the program by the community. Laboratory of Molecular Genetics--Institute of Biosciences--Universidade de São Paulo. 581 senior students from selected Jewish high schools. Molecular analysis of Tay-Sachs disease causing mutations by PCR amplification of genomic DNA, followed by restriction enzyme digestion. Among 581 students that attended educational classes, 404 (70%) elected to be tested for Tay-Sachs disease mutations. Of these, approximately 65% were of Ashkenazi Jewish origin. Eight carriers were detected corresponding to a carrier frequency of 1 in every 33 individuals in the Ashkenazi Jewish fraction of the sample. The frequency of Tay-Sachs disease carriers among the Ashkenazi Jewish population of Brazil is similar to that of other countries where carrier screening programs have led to a significant decrease in disease incidence. Therefore, it is justifiable to implement a Tay-Sachs disease carrier screening program for the Brazilian Jewish population.

  15. Metrics of quantum states

    International Nuclear Information System (INIS)

    Ma Zhihao; Chen Jingling

    2011-01-01

    In this work we study metrics of quantum states, which are natural generalizations of the usual trace metric and Bures metric. Some useful properties of the metrics are proved, such as the joint convexity and contractivity under quantum operations. Our result has a potential application in studying the geometry of quantum states as well as the entanglement detection.

  16. $\\eta$-metric structures

    OpenAIRE

    Gaba, Yaé Ulrich

    2017-01-01

    In this paper, we discuss recent results about generalized metric spaces and fixed point theory. We introduce the notion of $\\eta$-cone metric spaces, give some topological properties and prove some fixed point theorems for contractive type maps on these spaces. In particular we show that theses $\\eta$-cone metric spaces are natural generalizations of both cone metric spaces and metric type spaces.

  17. CMB anisotropies at all orders: the non-linear Sachs-Wolfe formula

    Energy Technology Data Exchange (ETDEWEB)

    Roldan, Omar, E-mail: oaroldan@if.ufrj.br [Instituto de Física, Universidade Federal do Rio de Janeiro, 21941-972, Rio de Janeiro, RJ (Brazil)

    2017-08-01

    We obtain the non-linear generalization of the Sachs-Wolfe + integrated Sachs-Wolfe (ISW) formula describing the CMB temperature anisotropies. Our formula is valid at all orders in perturbation theory, is also valid in all gauges and includes scalar, vector and tensor modes. A direct consequence of our results is that the maps of the logarithmic temperature anisotropies are much cleaner than the usual CMB maps, because they automatically remove many secondary anisotropies. This can for instance, facilitate the search for primordial non-Gaussianity in future works. It also disentangles the non-linear ISW from other effects. Finally, we provide a method which can iteratively be used to obtain the lensing solution at the desired order.

  18. CMB anisotropies at all orders: the non-linear Sachs-Wolfe formula

    International Nuclear Information System (INIS)

    Roldan, Omar

    2017-01-01

    We obtain the non-linear generalization of the Sachs-Wolfe + integrated Sachs-Wolfe (ISW) formula describing the CMB temperature anisotropies. Our formula is valid at all orders in perturbation theory, is also valid in all gauges and includes scalar, vector and tensor modes. A direct consequence of our results is that the maps of the logarithmic temperature anisotropies are much cleaner than the usual CMB maps, because they automatically remove many secondary anisotropies. This can for instance, facilitate the search for primordial non-Gaussianity in future works. It also disentangles the non-linear ISW from other effects. Finally, we provide a method which can iteratively be used to obtain the lensing solution at the desired order.

  19. Role of the physician in screening for carriers of Tay-Sachs disease.

    Science.gov (United States)

    Lowden, J. A.

    1978-01-01

    A screening test for carriers of Tay-Sachs disease has been available in Toronto for more than 6 years. In that time more than 11 000 Jewish residents have been tested. Most had requested testing after hearing about the screening program from friends or the media; few had been advised by their physicians to be tested. To sample the attitudes of physicians in Toronto towards carrier screening, we studied questionnaire responses of 42 physicians whose practices were composed largely of Jewish patients. Only 31% regularly advised their young adult Jewish patients to have a carrier screening test but 76% said they had patients who asked if they should be tested. Of the 14 (33%) who had had one or more patients with Tay-Sachs disease 6 did not advise carrier testing. There was a positive correlation between specialty training and support for the screening program. Methods for increasing physician advocacy of these programs are discussed. PMID:709448

  20. Neurocognitive testing in late-onset Tay-Sachs disease: a pilot study.

    Science.gov (United States)

    Elstein, D; Doniger, G M; Simon, E; Korn-Lubetzki, I; Navon, R; Zimran, A

    2008-08-01

    To test neurocognitive function in patients with late-onset Tay-Sachs disease (LOTS) using a computerized system to assess whether cognition is a clinically relevant outcome measure of possible therapeutic intervention in LOTS. Ten adults with Tay-Sachs disease were administered at least one battery of the Mindstreams Neurotrax system for evaluation of cognitive function. Six sub-scores and a Global Cognitive Score (GCS) were tabulated. A disease specific severity score was also devised with six domains. Despite identical genotypes, all patients but the two oldest had > or = 3/6 sub-scores one standard deviation below normal mean (100); verbal and executive functions were most affected. The severity score measured other functions. Because of provocative findings on re-testing in patients exposed to miglustat, and despite the very small cohort, cognitive function may be an appropriate and clinically relevant outcome measure for future therapeutic interventions in LOTS.

  1. Diagnosis and molecular characterization of non-classic forms of Tay-Sachs disease in Brazil.

    Science.gov (United States)

    Rozenberg, R; Kok, F; Burin, M G; Sá Miranda, M C; Vasques, C; Henriques-Souza, A M M; Giugliani, R; Vainzof, Mariz; Pereira, L V

    2006-06-01

    Molecular analysis of five Brazilian families, including eight patients presenting with nonclassic Tay-Sachs disease, was performed to identify frequent causative mutations and their correlation with clinical course. Three patients were affected by the B1 subacute variant and were shown to carry the R178H mutation (the DN allele), which is also common among Portuguese patients. Two of them were compound heterozygotes, whereas the third presented with the mutation in both alleles. Since Brazil was a Portuguese colony for over two centuries, common ancestry might be the probable explanation. The fourth patient presented with a juvenile phenotype and carries the R499H mutation, which has been reported only once worldwide and is associated with residual enzyme activity, responsible for a slower clinical course. The fifth family, of an Ashkenazi Jewish background, showed an extensive intrafamilial clinical variability among three affected sibs presenting with muscle atrophy, ataxia, and psychiatric symptoms. They were first diagnosed as having atypical spinal muscular atrophy and, subsequently, spinocerebellar ataxia, but, recently, the diagnosis of late-onset Tay-Sachs disease was confirmed based on reduced plasma hexosaminidase A activity and the G269S/InsTATC1278 genotype. It is therefore highly recommended to test patients with a similar clinical history for Tay-Sachs disease. In the same family, one first cousin committed suicide at the age of 24 years, presenting with a clinical phenotype that suggested an undiagnosed case and highlighting the effect of the intrafamilial clinical variability in delaying a prompt diagnosis. It is now recognized that his parents are, in fact, a carrier couple. Additionally, another relative had been previously identified as a heterozygote in a Tay-Sachs disease screening program, but the information was not shared among the family. Since this information might anticipate diagnosis and genetic counseling, it is advisable that

  2. Substrate reduction therapy in the infantile form of Tay-Sachs disease.

    Science.gov (United States)

    Bembi, B; Marchetti, F; Guerci, V I; Ciana, G; Addobbati, R; Grasso, D; Barone, R; Cariati, R; Fernandez-Guillen, L; Butters, T; Pittis, M G

    2006-01-24

    Substrate reduction therapy (SRT) with miglustat has been proposed for treatment of some lysosomal storage disorders. Based on the positive experience in Gaucher disease and experimental data in Tay-Sachs (TSD) and Sandhoff animal models, the authors investigated the clinical efficacy of SRT in two patients with infantile TSD. SRT could not arrest the patients' neurologic deterioration. However, a significant drug concentration in CSF as well as macrocephaly prevention were observed.

  3. ACOG committee opinion. Number 318, October 2005. Screening for Tay-Sachs disease.

    Science.gov (United States)

    2005-10-01

    Tay-Sachs disease (TSD) is a severe progressive neurologic disease that causes death in early childhood. Carrier screening, should be offered before pregnancy to individuals and couples at high-risk, including those of Ashkenazi Jewish, French-Canadian, or Cajun descent and those with a family history consistent with TSD. If both partners are determined to be carriers of TSD, genetic counseling and prenatal diagnosis should be offered.

  4. Correlation of augmented startle reflex with brainstem electrophysiological responses in Tay-Sachs disease.

    Science.gov (United States)

    Nakamura, Sadao; Saito, Yoshiaki; Ishiyama, Akihiko; Sugai, Kenji; Iso, Takashi; Inagaki, Masumi; Sasaki, Masayuki

    2015-01-01

    To clarify the evolution of an augmented startle reflex in Tay-Sachs disease and compare the temporal relationship between this reflex and brainstem evoked potentials. Clinical and electrophysiological data from 3 patients with Tay-Sachs disease were retrospectively collected. The augmented startle reflex appeared between the age of 3 and 17 months and disappeared between the age of 4 and 6 years. Analysis of brainstem auditory evoked potentials revealed that poor segregation of peak I, but not peak III, coincided with the disappearance of the augmented startle reflex. A blink reflex with markedly high amplitude was observed in a patient with an augmented startle reflex. The correlation between the augmented startle reflex and the preservation of peak I but not peak III supports the theory that the superior olivary nucleus is dispensable for this reflex. The blink reflex with high amplitudes may represent augmented excitability of reticular formation at the pontine tegmentum in Tay-Sachs disease, where the pattern generators for the augmented startle and blink reflexes may functionally overlap. Copyright © 2014 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  5. Lyso-GM2 ganglioside: a possible biomarker of Tay-Sachs disease and Sandhoff disease.

    Directory of Open Access Journals (Sweden)

    Takashi Kodama

    Full Text Available To find a new biomarker of Tay-Sachs disease and Sandhoff disease. The lyso-GM2 ganglioside (lyso-GM2 levels in the brain and plasma in Sandhoff mice were measured by means of high performance liquid chromatography and the effect of a modified hexosaminidase (Hex B exhibiting Hex A-like activity was examined. Then, the lyso-GM2 concentrations in human plasma samples were determined. The lyso-GM2 levels in the brain and plasma in Sandhoff mice were apparently increased compared with those in wild-type mice, and they decreased on intracerebroventricular administration of the modified Hex B. The lyso-GM2 levels in plasma of patients with Tay-Sachs disease and Sandhoff disease were increased, and the increase in lyso-GM2 was associated with a decrease in Hex A activity. Lyso-GM2 is expected to be a potential biomarker of Tay-Sachs disease and Sandhoff disease.

  6. Doença de Tay-Sachs em criança de raça negra Tay-Sachs disease in a Negro child

    Directory of Open Access Journals (Sweden)

    Sérgio Rosemberg

    1970-12-01

    Full Text Available É relatado um caso de doença de Tay-Sachs em criança da raça negra. O encontro desta entidade em indivíduos não judeus é bastante raro, sendo este o quinto caso descrito em criança negra. Os autores estudam alguns aspectos genéticos, chegando à conclusão de que o cálculo da freqüência do gene mutante é impossível devido ao pequeno número de casos e ao fato de que não há estimativas seguras quanto à taxa de nascimento de indivíduos negros na população, Em que pese o fato de o diagnóstico se assentar em bases puramente clínicas e anatômicas, prescindindo os autores de exames bioquímicos específicos, os dados apresentados são congruentes com os casos clássicos descritos na literatura.A case of Tay-Sachs diseases in a Negro child is reported. The occurrence of this disease in non-Jewish individuals is very rare and this is the fifth case of a Negro-child reported in the litterature. Some genetical aspects are studied and the authors conclude that the frequency calculation of the mutant gene is impossible due to the small number of cases and to the fact that there is reliable information regarding the rate of birth of Negro individuals in the general population. The diagnosis was based on ophtalmological and clinical data. No specific biochemical tests were performed but the data obtained are congruent with other cases reported in the litterature.

  7. METRIC context unit architecture

    Energy Technology Data Exchange (ETDEWEB)

    Simpson, R.O.

    1988-01-01

    METRIC is an architecture for a simple but powerful Reduced Instruction Set Computer (RISC). Its speed comes from the simultaneous processing of several instruction streams, with instructions from the various streams being dispatched into METRIC's execution pipeline as they become available for execution. The pipeline is thus kept full, with a mix of instructions for several contexts in execution at the same time. True parallel programming is supported within a single execution unit, the METRIC Context Unit. METRIC's architecture provides for expansion through the addition of multiple Context Units and of specialized Functional Units. The architecture thus spans a range of size and performance from a single-chip microcomputer up through large and powerful multiprocessors. This research concentrates on the specification of the METRIC Context Unit at the architectural level. Performance tradeoffs made during METRIC's design are discussed, and projections of METRIC's performance are made based on simulation studies.

  8. Preconception risk assessment for thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease.

    Science.gov (United States)

    Hussein, Norita; Weng, Stephen F; Kai, Joe; Kleijnen, Jos; Qureshi, Nadeem

    2018-03-14

    Globally, about five per cent of children are born with congenital or genetic disorders. The most common autosomal recessive conditions are thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease, with higher carrier rates in specific patient populations. Identifying and counselling couples at genetic risk of the conditions before pregnancy enables them to make fully informed reproductive decisions, with some of these choices not being available if genetic counselling is only offered in an antenatal setting. This is an update of a previously published review. To assess the effectiveness of systematic preconception genetic risk assessment to improve reproductive outcomes in women and their partners who are identified as carriers of thalassaemia, sickle cell disease, cystic fibrosis and Tay-Sachs disease in healthcare settings when compared to usual care. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Trials Registers. In addition, we searched for all relevant trials from 1970 (or the date at which the database was first available if after 1970) to date using electronic databases (MEDLINE, Embase, CINAHL, PsycINFO), clinical trial databases (National Institutes of Health, Clinical Trials Search portal of the World Health Organization, metaRegister of controlled clinical trials), and hand searching of key journals and conference abstract books from 1998 to date (European Journal of Human Genetics, Genetics in Medicine, Journal of Community Genetics). We also searched the reference lists of relevant articles, reviews and guidelines and also contacted subject experts in the field to request any unpublished or other published trials.Date of latest search of the registers: 20 June 2017.Date of latest search of all other sources: 16 November 2017. Any randomised or quasi-randomised controlled trials (published or unpublished) comparing reproductive outcomes of systematic preconception genetic risk assessment for thalassaemia, sickle

  9. Expanding the spectrum of HEXA mutations in Indian patients with Tay–Sachs disease

    Directory of Open Access Journals (Sweden)

    Jayesh Sheth

    2014-01-01

    Full Text Available Tay–Sachs disease is an autosomal recessive neurodegenerative disorder occurring due to impaired activity of β-hexosaminidase-A (EC 3.2.1.52, resulting from the mutation in HEXA gene. Very little is known about the molecular pathology of TSD in Indian children except for a few mutations identified by us. The present study is aimed to determine additional mutations leading to Tay–Sachs disease in nine patients confirmed by the deficiency of β-hexosaminidase-A (C (D175A and c.805G>C (p.G269R in one case; and one small 1 bp deletion c.426delT (p.F142LfsX57 and one splice site mutation c.459+4A>C in the other two cases respectively. None of these mutations were detected in 100 chromosomes from healthy individuals of the same ethnic group. Three previously reported missense mutations, (i c.532C>T (p.R178C, (ii c.964G>T (p.D322Y, and (iii c.1385A>T (p.E462V; two nonsense mutations (i c.709C>T (p.Q237X and (ii c.1528C>T (p.R510X, one 4 bp insertion c.1277_1278insTATC (p.Y427IfsX5 and one splice site mutation c.459+5G>A were also identified in six cases. We observe from this study that novel mutations are more frequently observed in Indian patients with Tay–Sachs disease with clustering of ~73% of disease causing mutations in exons 5 to 12. This database can be used for a carrier rate screening in the larger population of the country.

  10. Expanding the spectrum of HEXA mutations in Indian patients with Tay-Sachs disease.

    Science.gov (United States)

    Sheth, Jayesh; Mistri, Mehul; Datar, Chaitanya; Kalane, Umesh; Patil, Shekhar; Kamate, Mahesh; Shah, Harshuti; Nampoothiri, Sheela; Gupta, Sarita; Sheth, Frenny

    2014-01-01

    Tay-Sachs disease is an autosomal recessive neurodegenerative disorder occurring due to impaired activity of β-hexosaminidase-A (EC 3.2.1.52), resulting from the mutation in HEXA gene. Very little is known about the molecular pathology of TSD in Indian children except for a few mutations identified by us. The present study is aimed to determine additional mutations leading to Tay-Sachs disease in nine patients confirmed by the deficiency of β-hexosaminidase-A (C (D175A) and c.805G>C (p.G269R) in one case; and one small 1 bp deletion c.426delT (p.F142LfsX57) and one splice site mutation c.459+4A>C in the other two cases respectively. None of these mutations were detected in 100 chromosomes from healthy individuals of the same ethnic group. Three previously reported missense mutations, (i) c.532C>T (p.R178C), (ii) c.964G>T (p.D322Y), and (iii) c.1385A>T (p.E462V); two nonsense mutations (i) c.709C>T (p.Q237X) and (ii) c.1528C>T (p.R510X), one 4 bp insertion c.1277_1278insTATC (p.Y427IfsX5) and one splice site mutation c.459+5G>A were also identified in six cases. We observe from this study that novel mutations are more frequently observed in Indian patients with Tay-Sachs disease with clustering of ~ 73% of disease causing mutations in exons 5 to 12. This database can be used for a carrier rate screening in the larger population of the country.

  11. [Dr Mavro Sachs (1817-1888): the first lecturer of Zagreb University].

    Science.gov (United States)

    Dugacki, Vladimir

    2010-01-01

    Mavro Sachs (Jánosháza, Hungary, 1817 % emdash; Rijeka, 1888.) was a Zagreb student since 1828. In 1846 he graduated in medicine from the University of Vienna and returned to Zagreb to be the city physician. In 1849, he started to teach forensic medicine at the School of Law of the Royal Academy of Sciences in Zagreb and continued teaching the same subject at the Zagreb University Faculty of Law in the capacity of docent (corresponds to lecturer in the British system). He also taught medical law at pharmaceutical studies of Zagreb University. From 1855 to 1860, he presided over the Jewish Community of Zagreb.

  12. Mechanism of interrupted saccades in patients with late-onset Tay-Sachs disease.

    Science.gov (United States)

    Optican, Lance M; Rucker, Janet C; Keller, Edward L; Leigh, R John

    2008-01-01

    In late-onset Tay-Sachs disease (LOTS), saccades are interrupted by one or more transient decelerations. Some saccades reaccelerate and continue on before eye velocity reaches zero, even in darkness. Intervals between successive decelerations are not regularly spaced. Peak decelerations of horizontal and vertical components of oblique saccades in LOTS is more synchronous than those in control subjects. We hypothesize that these decelerations are caused by dysregulation of the fastigial nuclei (FN) of the cerebellum, which fire brain stem inhibitory burst neurons (IBNs).

  13. Metric diffusion along foliations

    CERN Document Server

    Walczak, Szymon M

    2017-01-01

    Up-to-date research in metric diffusion along compact foliations is presented in this book. Beginning with fundamentals from the optimal transportation theory and the theory of foliations; this book moves on to cover Wasserstein distance, Kantorovich Duality Theorem, and the metrization of the weak topology by the Wasserstein distance. Metric diffusion is defined, the topology of the metric space is studied and the limits of diffused metrics along compact foliations are discussed. Essentials on foliations, holonomy, heat diffusion, and compact foliations are detailed and vital technical lemmas are proved to aide understanding. Graduate students and researchers in geometry, topology and dynamics of foliations and laminations will find this supplement useful as it presents facts about the metric diffusion along non-compact foliation and provides a full description of the limit for metrics diffused along foliation with at least one compact leaf on the two dimensions.

  14. Metric modular spaces

    CERN Document Server

    Chistyakov, Vyacheslav

    2015-01-01

    Aimed toward researchers and graduate students familiar with elements of functional analysis, linear algebra, and general topology; this book contains a general study of modulars, modular spaces, and metric modular spaces. Modulars may be thought of as generalized velocity fields and serve two important purposes: generate metric spaces in a unified manner and provide a weaker convergence, the modular convergence, whose topology is non-metrizable in general. Metric modular spaces are extensions of metric spaces, metric linear spaces, and classical modular linear spaces. The topics covered include the classification of modulars, metrizability of modular spaces, modular transforms and duality between modular spaces, metric  and modular topologies. Applications illustrated in this book include: the description of superposition operators acting in modular spaces, the existence of regular selections of set-valued mappings, new interpretations of spaces of Lipschitzian and absolutely continuous mappings, the existe...

  15. Prognostic Performance Metrics

    Data.gov (United States)

    National Aeronautics and Space Administration — This chapter presents several performance metrics for offline evaluation of prognostics algorithms. A brief overview of different methods employed for performance...

  16. Overview of journal metrics

    Directory of Open Access Journals (Sweden)

    Kihong Kim

    2018-02-01

    Full Text Available Various kinds of metrics used for the quantitative evaluation of scholarly journals are reviewed. The impact factor and related metrics including the immediacy index and the aggregate impact factor, which are provided by the Journal Citation Reports, are explained in detail. The Eigenfactor score and the article influence score are also reviewed. In addition, journal metrics such as CiteScore, Source Normalized Impact per Paper, SCImago Journal Rank, h-index, and g-index are discussed. Limitations and problems that these metrics have are pointed out. We should be cautious to rely on those quantitative measures too much when we evaluate journals or researchers.

  17. Generalized Bondi-Sachs equations for characteristic formalism of numerical relativity

    Science.gov (United States)

    Cao, Zhoujian; He, Xiaokai

    2013-11-01

    The Cauchy formalism of numerical relativity has been successfully applied to simulate various dynamical spacetimes without any symmetry assumption. But discovering how to set a mathematically consistent and physically realistic boundary condition is still an open problem for Cauchy formalism. In addition, the numerical truncation error and finite region ambiguity affect the accuracy of gravitational wave form calculation. As to the finite region ambiguity issue, the characteristic extraction method helps much. But it does not solve all of the above issues. Besides the above problems for Cauchy formalism, the computational efficiency is another problem. Although characteristic formalism of numerical relativity suffers the difficulty from caustics in the inner near zone, it has advantages in relation to all of the issues listed above. Cauchy-characteristic matching (CCM) is a possible way to take advantage of characteristic formalism regarding these issues and treat the inner caustics at the same time. CCM has difficulty treating the gauge difference between the Cauchy part and the characteristic part. We propose generalized Bondi-Sachs equations for characteristic formalism for the Cauchy-characteristic matching end. Our proposal gives out a possible same numerical evolution scheme for both the Cauchy part and the characteristic part. And our generalized Bondi-Sachs equations have one adjustable gauge freedom which can be used to relate the gauge used in the Cauchy part. Then these equations can make the Cauchy part and the characteristic part share a consistent gauge condition. So our proposal gives a possible new starting point for Cauchy-characteristic matching.

  18. Integrated Sachs-Wolfe effect in a quintessence cosmological model: Including anisotropic stress of dark energy

    International Nuclear Information System (INIS)

    Wang, Y. T.; Xu, L. X.; Gui, Y. X.

    2010-01-01

    In this paper, we investigate the integrated Sachs-Wolfe effect in the quintessence cold dark matter model with constant equation of state and constant speed of sound in dark energy rest frame, including dark energy perturbation and its anisotropic stress. Comparing with the ΛCDM model, we find that the integrated Sachs-Wolfe (ISW)-power spectrums are affected by different background evolutions and dark energy perturbation. As we change the speed of sound from 1 to 0 in the quintessence cold dark matter model with given state parameters, it is found that the inclusion of dark energy anisotropic stress makes the variation of magnitude of the ISW source uncertain due to the anticorrelation between the speed of sound and the ratio of dark energy density perturbation contrast to dark matter density perturbation contrast in the ISW-source term. Thus, the magnitude of the ISW-source term is governed by the competition between the alterant multiple of (1+3/2xc-circumflex s 2 ) and that of δ de /δ m with the variation of c-circumflex s 2 .

  19. Characterization of two Turkish beta-hexosaminidase mutations causing Tay-Sachs disease.

    Science.gov (United States)

    Ozkara, Hatice Asuman; Sandhoff, Konrad

    2003-04-01

    Two homoallelic mutations have recently been identified in the alpha-subunit of hexosaminidase A (EC 3.2.1.52) causing the infantile form of Tay-Sachs disease in Turkish patients. Both of these mutations, a 12 bp deletion (1096-1107 or 1098-1108 or 1099-1109) in exon 10 and a point mutation (G1362 to A, Gly454 to Asp) in exon 12, are located in the catalytic domain of the hexosaminidase alpha-chain. In order to determine whether these mutations affect the function of the catalytic domain or result in an instable protein, both mutant cDNAs were overexpressed in COS-1 cells. As judged by Western blotting, transfections of wild-type cDNA produced pro-alpha-chain and mature alpha-chain in parallel with a fivefold increase in cellular hexosaminidase activity using the synthetic substrate 4-methylumbelliferyl beta-N-acetylglucosamine 6-sulfate (MUGS). However, both mutants produced only pro-alpha-chains, although no mature form or detectable hexosaminidase activity towards two different synthetic substrates was observed. These data are consistent with the biochemical phenotype of infantile Tay-Sachs disease. We conclude that the overexpressed mutant pro-alpha-chains were misfolded and could not undergo further proteolytic processing to the active form of the enzyme in the lysosome.

  20. Brand metrics that matter

    NARCIS (Netherlands)

    Muntinga, D.; Bernritter, S.

    2017-01-01

    Het merk staat steeds meer centraal in de organisatie. Het is daarom essentieel om de gezondheid, prestaties en ontwikkelingen van het merk te meten. Het is echter een uitdaging om de juiste brand metrics te selecteren. Een enorme hoeveelheid metrics vraagt de aandacht van merkbeheerders. Maar welke

  1. Privacy Metrics and Boundaries

    NARCIS (Netherlands)

    L-F. Pau (Louis-François)

    2005-01-01

    textabstractThis paper aims at defining a set of privacy metrics (quantitative and qualitative) in the case of the relation between a privacy protector ,and an information gatherer .The aims with such metrics are: -to allow to assess and compare different user scenarios and their differences; for

  2. "Cherry red spot" in a patient with Tay-Sachs disease: case report "Mácula em cereja" em paciente com doença de Tay-Sachs: relato de caso

    Directory of Open Access Journals (Sweden)

    Ricardo Evangelista Marrocos de Aragão

    2009-08-01

    Full Text Available Tay-Sachs disease is an autosomal recessive disorder of sphingolipid metabolism, caused by enzime hexosaminidase A deficiency that leads to an accumulation of GM2 in neurocytes which results in progressive loss of neurological function. The accumulation of lipid in retinal ganglion cells that leads to a chalk-white appearance of the fundus called "cherry red spot" is the hallmark of Tay-Sachs disease. It is also seen in others neurometabolic diseases as well as in central retinal artery occlusion. This case reports a child with Tay-Sachs disease in a family with four previous similar deaths without diagnostic.Tay-Sachs é uma doença autossômica recessiva, caracterizada pela deficiência da enzima hexosaminidase A levando ao acúmulo de esfingolipídios (GM2 em células neuronais que resulta em uma perda progressiva da função neurológica. O acúmulo de lipídios em células ganglionais da retina leva a uma aparência de mácula em cereja, característica do fundo de olho de pessoas acometidas. "Mácula em cereja" também pode ser vista em outras doenças neurometabólicas e em oclusão da artéria central da retina. Este trabalho relata o caso de um paciente com doença de Tay-Sachs em uma família com história de quatro óbitos por causas semelhantes sem diagnóstico.

  3. Regional neuro axonal injury detected by 1H 3 Tesla spectroscopic imaging in late onset Tay sachs

    International Nuclear Information System (INIS)

    Gagoski, Borjan Aleksandar; Eichler, Florian S.

    2010-01-01

    Late-onset Tay Sachs (LOTS) is a rare lysosomal storage disorder resulting from mutations of the subunit of the lysosomal enzyme β-hexosaminidase A, which catalyzes the degradation of GM2 ganglioside. We have applied the fast encoding spectroscopic imaging technique to LOTS patients to further investigate the neuro degenerative consequences of this disease.(Author)

  4. Late-onset Tay-Sachs disease: phenotypic characterization and genotypic correlations in 21 affected patients.

    Science.gov (United States)

    Neudorfer, Orit; Pastores, Gregory M; Zeng, Bai J; Gianutsos, John; Zaroff, Charles M; Kolodny, Edwin H

    2005-02-01

    The purpose of this study was to describe the phenotype (and corresponding genotype) of adult patients with late-onset Tay-Sachs disease, a clinical variant of the GM2-gangliosidoses. A comprehensive physical examination, including neurological assessments, was performed to establish the current disease pattern and severity. In addition, the patients' past medical histories were reviewed. The patients' alpha-subunit mutations (beta-Hexosaminidase A genotype) were determined and correlated with their corresponding clinical findings and disease course. Twenty-one patients (current mean age: 27.0 years; range: 14-47 years) were identified. The pedigree revealed a relative with the "classic" infantile or late-onset form of Tay-Sachs disease in four (out of 18) unrelated families. The patients were predominantly male (15/21 individuals) and of Ashkenazi Jewish ancestry (15/18 families). Mean age at onset was 18.1 years; balance problems and difficulty climbing stairs were the most frequent presenting complaints. In several cases, the diagnosis was delayed (mean age at diagnosis: 27.0 years). Analysis of the beta-hex A gene revealed the G269S mutation as the most common disease allele; found in homozygosity (N = 1) or heterozygosity (N = 18; including 2 sib pairs). Disease onset (age 36 years) was delayed and progression relatively slower in the homozygous G269S patient. Two siblings (ages 28 and 31 years), of non-Jewish ancestry, were compound heterozygotes (TATC1278/W474C); their clinical course is dominated by psychiatric problems. Brain imaging studies revealed marked cerebellar atrophy in all patients (N = 18) tested, regardless of disease stage. Late-onset Tay-Sachs disease is an infrequent disorder and the diagnosis is often missed or delayed (by approximately 8 years). Early on, the majority of patients develop signs of either cerebellar or anterior motor neuron involvement. Affected individuals may also develop psychotic episodes. In most cases, the later

  5. Holographic Spherically Symmetric Metrics

    Science.gov (United States)

    Petri, Michael

    The holographic principle (HP) conjectures, that the maximum number of degrees of freedom of any realistic physical system is proportional to the system's boundary area. The HP has its roots in the study of black holes. It has recently been applied to cosmological solutions. In this article we apply the HP to spherically symmetric static space-times. We find that any regular spherically symmetric object saturating the HP is subject to tight constraints on the (interior) metric, energy-density, temperature and entropy-density. Whenever gravity can be described by a metric theory, gravity is macroscopically scale invariant and the laws of thermodynamics hold locally and globally, the (interior) metric of a regular holographic object is uniquely determined up to a constant factor and the interior matter-state must follow well defined scaling relations. When the metric theory of gravity is general relativity, the interior matter has an overall string equation of state (EOS) and a unique total energy-density. Thus the holographic metric derived in this article can serve as simple interior 4D realization of Mathur's string fuzzball proposal. Some properties of the holographic metric and its possible experimental verification are discussed. The geodesics of the holographic metric describe an isotropically expanding (or contracting) universe with a nearly homogeneous matter-distribution within the local Hubble volume. Due to the overall string EOS the active gravitational mass-density is zero, resulting in a coasting expansion with Ht = 1, which is compatible with the recent GRB-data.

  6. General exact solution for homogeneous time-dependent self-gravitating perfect fluids

    International Nuclear Information System (INIS)

    Gaete, P.; Hojman, R.

    1988-01-01

    A procedure to obtain the general exact solution of Einstein equations for a self-gravitating spherically-symmetric static perfect fluid obeying an arbitrary equation of state, is applied to time-dependent Kantowsky-Sachs line elements (with spherical, planar and hyperbolic symmetry). As in the static case, the solution is generated by an arbitrary function of the independent variable and its first derivative. To illustrate the results, the whole family of (plane-symmetric) solutions with a ''gamma-law'' equation of state is explicity obtained in terms of simple known functions. It is also shown that, while in the static plane-symmtric line elements, every metric is in one to one correspondence with a ''partner-metric'' (both originated from the same generatrix function), in this case every generatrix function univocally determines one metric. (author) [pt

  7. Unverrichteter Sache

    Directory of Open Access Journals (Sweden)

    Jani Kirov

    2007-01-01

    Full Text Available Rezensiertes Werk: Adriaan Lanni, Law and Justice in the Courts of Classical Athens, Cambridge: Cambridge University Press 2006, 210 S., ISBN-13 978-0-521-85759-8; ISBN-10 0-521-85759-7

  8. Tay-Sachs disease: progression of changes on neuroimaging in four cases

    Energy Technology Data Exchange (ETDEWEB)

    Fukumizu, M; Yoshikawa, H; Kurokawa, T [National Center Hospital for Mental, Nervous, and Muscular Disorders, Tokyo (Japan). Div. of Child Neurology; Takashima, S [National Inst. of Neuroscience, National Center of Neurology and Psychiatry, Tokyo (Japan). Div. of Mental Retardation and Birth Defect Research; Sakuragawa, N [National Inst. of Neuroscience, National Center of Neurology and Psychiatry, Tokyo (Japan). Div. of Inherited Metabolic Disease

    1992-11-01

    The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter. (orig.).

  9. Optical structures, algebraically special spacetimes, and the Goldberg-Sachs theorem in five dimensions

    International Nuclear Information System (INIS)

    Taghavi-Chabert, Arman

    2011-01-01

    Optical (or Robinson) structures are one generalization of four-dimensional shearfree congruences of null geodesics to higher dimensions. They are Lorentzian analogues of complex and CR structures. In this context, we extend the Goldberg-Sachs theorem to five dimensions. To be precise, we find a new algebraic condition on the Weyl tensor, which generalizes the Petrov type II condition, in the sense that it ensures the existence of such congruences on a five-dimensional spacetime, vacuum or under weaker assumptions on the Ricci tensor. This results in a significant simplification of the field equations. We discuss possible degenerate cases, including a five-dimensional generalization of the Petrov type D condition. We also show that the vacuum black ring solution is endowed with optical structures, yet fails to be algebraically special with respect to them. We finally explain the generalization of these ideas to higher dimensions, which has been checked in six and seven dimensions.

  10. Tay-Sachs disease: progression of changes on neuroimaging in four cases

    International Nuclear Information System (INIS)

    Fukumizu, M.; Yoshikawa, H.; Kurokawa, T.; Takashima, S.; Sakuragawa, N.

    1992-01-01

    The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter. (orig.)

  11. Late-onset Tay-Sachs disease: the spectrum of peripheral neuropathy in 30 affected patients.

    Science.gov (United States)

    Shapiro, Barbara E; Logigian, Eric L; Kolodny, Edwin H; Pastores, Gregory M

    2008-08-01

    Late-onset Tay-Sachs (LOTS) disease is a chronic, progressive, lysosomal storage disorder caused by a partial deficiency of beta-hexosaminidase A (HEXA) activity. Deficient levels of HEXA result in the intracellular accumulation of GM2-ganglioside, resulting in toxicity to nerve cells. Clinical manifestations primarily involve the central nervous system (CNS) and lower motor neurons, and include ataxia, weakness, spasticity, dysarthria, dysphagia, dystonia, seizures, psychosis, mania, depression, and cognitive decline. The prevalence of peripheral nervous system (PNS) involvement in LOTS has not been well documented, but it has traditionally been thought to be very low. We examined a cohort of 30 patients with LOTS who underwent clinical and electrophysiologic examination, and found evidence of a predominantly axon loss polyneuropathy affecting distal nerve segments in the lower and upper extremities in eight patients (27%).

  12. Probabilistic metric spaces

    CERN Document Server

    Schweizer, B

    2005-01-01

    Topics include special classes of probabilistic metric spaces, topologies, and several related structures, such as probabilistic normed and inner-product spaces. 1983 edition, updated with 3 new appendixes. Includes 17 illustrations.

  13. Tracker Performance Metric

    National Research Council Canada - National Science Library

    Olson, Teresa; Lee, Harry; Sanders, Johnnie

    2002-01-01

    .... We have developed the Tracker Performance Metric (TPM) specifically for this purpose. It was designed to measure the output performance, on a frame-by-frame basis, using its output position and quality...

  14. Hill-Sachs lesion location: does it play a role in engagement?

    Energy Technology Data Exchange (ETDEWEB)

    Gyftopoulos, Soterios; Wang, Annie; Babb, James [NYU Langone Medical Center, Department of Radiology, New York, NY (United States)

    2015-08-15

    To see if there is an association between engagement on physical examination (PE) and the location of the Hill-Sachs lesion (HSL) as assessed by the modified biceps angle. Sixty-two patients with a history of anterior shoulder dislocation, who underwent preoperative MRI and arthroscopy at our institution and were tested for engagement on PE, were collected. Two musculoskeletal radiologists reviewed the MR studies, noting the presence of an HSL and documenting the location of the HSL with the modified biceps angle. Statistical analysis included the Mann-Whitney (MW) test and ROC (receiver-operating characteristic) curve. Of 62 patients, there were 58 males and 4 females with a mean age of 30 (range 18-59 years). Twenty patients demonstrated engagement on PE, while 42 did not. All patients had evidence of an HSL on MRI and arthroscopy. The mean biceps angle for the engaging group was 151.5 ± 13.9 , and 142.4 ± 17.3 for the non-engaging group. The biceps angle was statistically significantly higher among patients who had engagement compared to those who did not (p = 0.027). Overall, diagnostic accuracy was highest for a biceps angle >149 , which resulted in a sensitivity of 70 % and specificity of 67 %. The modified biceps angle, as measured on MRI, was significantly higher in patients who demonstrated engagement on physical examination than in those who did not. This supports the theory that the location of the Hill-Sachs lesion may play a role in engagement and may be its most important characteristic when determining its significance. (orig.)

  15. Neuro-ophthalmology of late-onset Tay-Sachs disease (LOTS).

    Science.gov (United States)

    Rucker, J C; Shapiro, B E; Han, Y H; Kumar, A N; Garbutt, S; Keller, E L; Leigh, R J

    2004-11-23

    Late-onset Tay-Sachs disease (LOTS) is an adult-onset, autosomal recessive, progressive variant of GM2 gangliosidosis, characterized by involvement of the cerebellum and anterior horn cells. To determine the range of visual and ocular motor abnormalities in LOTS, as a prelude to evaluating the effectiveness of novel therapies. Fourteen patients with biochemically confirmed LOTS (8 men; age range 24 to 53 years; disease duration 5 to 30 years) and 10 age-matched control subjects were studied. Snellen visual acuity, contrast sensitivity, color vision, stereopsis, and visual fields were measured, and optic fundi were photographed. Horizontal and vertical eye movements (search coil) were recorded, and saccades, pursuit, vestibulo-ocular reflex (VOR), vergence, and optokinetic (OK) responses were measured. All patients showed normal visual functions and optic fundi. The main eye movement abnormality concerned saccades, which were "multistep," consisting of a series of small saccades and larger movements that showed transient decelerations. Larger saccades ended earlier and more abruptly (greater peak deceleration) in LOTS patients than in control subjects; these changes can be attributed to premature termination of the saccadic pulse. Smooth-pursuit and slow-phase OK gains were reduced, but VOR, vergence, and gaze holding were normal. Patients with late-onset Tay-Sachs disease (LOTS) show characteristic abnormalities of saccades but normal afferent visual systems. Hypometria, transient decelerations, and premature termination of saccades suggest disruption of a "latch circuit" that normally inhibits pontine omnipause neurons, permitting burst neurons to discharge until the eye movement is completed. These measurable abnormalities of saccades provide a means to evaluate the effects of novel treatments for LOTS.

  16. IT Project Management Metrics

    Directory of Open Access Journals (Sweden)

    2007-01-01

    Full Text Available Many software and IT projects fail in completing theirs objectives because different causes of which the management of the projects has a high weight. In order to have successfully projects, lessons learned have to be used, historical data to be collected and metrics and indicators have to be computed and used to compare them with past projects and avoid failure to happen. This paper presents some metrics that can be used for the IT project management.

  17. Mass Customization Measurements Metrics

    DEFF Research Database (Denmark)

    Nielsen, Kjeld; Brunø, Thomas Ditlev; Jørgensen, Kaj Asbjørn

    2014-01-01

    A recent survey has indicated that 17 % of companies have ceased mass customizing less than 1 year after initiating the effort. This paper presents measurement for a company’s mass customization performance, utilizing metrics within the three fundamental capabilities: robust process design, choice...... navigation, and solution space development. A mass customizer when assessing performance with these metrics can identify within which areas improvement would increase competitiveness the most and enable more efficient transition to mass customization....

  18. Fault Management Metrics

    Science.gov (United States)

    Johnson, Stephen B.; Ghoshal, Sudipto; Haste, Deepak; Moore, Craig

    2017-01-01

    This paper describes the theory and considerations in the application of metrics to measure the effectiveness of fault management. Fault management refers here to the operational aspect of system health management, and as such is considered as a meta-control loop that operates to preserve or maximize the system's ability to achieve its goals in the face of current or prospective failure. As a suite of control loops, the metrics to estimate and measure the effectiveness of fault management are similar to those of classical control loops in being divided into two major classes: state estimation, and state control. State estimation metrics can be classified into lower-level subdivisions for detection coverage, detection effectiveness, fault isolation and fault identification (diagnostics), and failure prognosis. State control metrics can be classified into response determination effectiveness and response effectiveness. These metrics are applied to each and every fault management control loop in the system, for each failure to which they apply, and probabilistically summed to determine the effectiveness of these fault management control loops to preserve the relevant system goals that they are intended to protect.

  19. Deep Transfer Metric Learning.

    Science.gov (United States)

    Junlin Hu; Jiwen Lu; Yap-Peng Tan; Jie Zhou

    2016-12-01

    Conventional metric learning methods usually assume that the training and test samples are captured in similar scenarios so that their distributions are assumed to be the same. This assumption does not hold in many real visual recognition applications, especially when samples are captured across different data sets. In this paper, we propose a new deep transfer metric learning (DTML) method to learn a set of hierarchical nonlinear transformations for cross-domain visual recognition by transferring discriminative knowledge from the labeled source domain to the unlabeled target domain. Specifically, our DTML learns a deep metric network by maximizing the inter-class variations and minimizing the intra-class variations, and minimizing the distribution divergence between the source domain and the target domain at the top layer of the network. To better exploit the discriminative information from the source domain, we further develop a deeply supervised transfer metric learning (DSTML) method by including an additional objective on DTML, where the output of both the hidden layers and the top layer are optimized jointly. To preserve the local manifold of input data points in the metric space, we present two new methods, DTML with autoencoder regularization and DSTML with autoencoder regularization. Experimental results on face verification, person re-identification, and handwritten digit recognition validate the effectiveness of the proposed methods.

  20. Unilaterally and rapidly progressing white matter lesion and elevated cytokines in a patient with Tay-Sachs disease.

    Science.gov (United States)

    Hayase, Tomomi; Shimizu, Jun; Goto, Tamako; Nozaki, Yasuyuki; Mori, Masato; Takahashi, Naoto; Namba, Eiji; Yamagata, Takanori; Momoi, Mariko Y

    2010-03-01

    We report the case of a girl with Tay-Sachs disease who had convulsions and deteriorated rapidly after an upper respiratory infection at the age of 11 months. At the age of 16 months, her seizures became intractable and magnetic resonance imaging of the brain showed high signal intensity on T2-weighted images and marked swelling in the white matter and basal nucelei of the right hemisphere. Her seizures and right hemisphere lesion improved with glycerol and dexamethasone treatment. When dexamethasone was discontinued, her symptoms worsened and lesions later appeared in the left hemisphere. Her cerebrospinal fluid showed elevated levels of the cytokines TNF-alpha and IL-5. It is considered that inflammation contributes to disease progression in Tay-Sachs disease.

  1. İki Kardeşte Görülen Tay-Sachs Hastalığı

    OpenAIRE

    KÜÇÜKÖDÜK, Ş.

    2010-01-01

    In view of two cases of autosomal recessive Tay-Sachs disease which affected two siblings, genetic consultation has been once more emphasized. The clinical features of this entity has been reviewed under the information obtained from recent literature. Otozomal resesif geçiş gösteren ve iki kardeşide etkiliyen Tay-Sachs hastalığında, genetik danışmanın önemi bir kez daha vurgulandı. Bu hastalığın klinik özellikleri literatür bilgilerinin ışığı altında gözden geçirildi....

  2. Cyber threat metrics.

    Energy Technology Data Exchange (ETDEWEB)

    Frye, Jason Neal; Veitch, Cynthia K.; Mateski, Mark Elliot; Michalski, John T.; Harris, James Mark; Trevino, Cassandra M.; Maruoka, Scott

    2012-03-01

    Threats are generally much easier to list than to describe, and much easier to describe than to measure. As a result, many organizations list threats. Fewer describe them in useful terms, and still fewer measure them in meaningful ways. This is particularly true in the dynamic and nebulous domain of cyber threats - a domain that tends to resist easy measurement and, in some cases, appears to defy any measurement. We believe the problem is tractable. In this report we describe threat metrics and models for characterizing threats consistently and unambiguously. The purpose of this report is to support the Operational Threat Assessment (OTA) phase of risk and vulnerability assessment. To this end, we focus on the task of characterizing cyber threats using consistent threat metrics and models. In particular, we address threat metrics and models for describing malicious cyber threats to US FCEB agencies and systems.

  3. Adaptive metric kernel regression

    DEFF Research Database (Denmark)

    Goutte, Cyril; Larsen, Jan

    2000-01-01

    Kernel smoothing is a widely used non-parametric pattern recognition technique. By nature, it suffers from the curse of dimensionality and is usually difficult to apply to high input dimensions. In this contribution, we propose an algorithm that adapts the input metric used in multivariate...... regression by minimising a cross-validation estimate of the generalisation error. This allows to automatically adjust the importance of different dimensions. The improvement in terms of modelling performance is illustrated on a variable selection task where the adaptive metric kernel clearly outperforms...

  4. Adaptive Metric Kernel Regression

    DEFF Research Database (Denmark)

    Goutte, Cyril; Larsen, Jan

    1998-01-01

    Kernel smoothing is a widely used nonparametric pattern recognition technique. By nature, it suffers from the curse of dimensionality and is usually difficult to apply to high input dimensions. In this paper, we propose an algorithm that adapts the input metric used in multivariate regression...... by minimising a cross-validation estimate of the generalisation error. This allows one to automatically adjust the importance of different dimensions. The improvement in terms of modelling performance is illustrated on a variable selection task where the adaptive metric kernel clearly outperforms the standard...

  5. Targeted disruption of the Hexa gene results in mice with biochemical and pathologic features of Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Proia, R.L.; Yamanaka, S.; Johnson, M.D. [and others

    1994-09-01

    Tay-Sachs disease, the prototype of the G{sub M2} gangliosidoses, is a catastrophic neurodegenerative disorder of infancy. The disease is caused by mutations in the HEXA gene resulting in an absence of the lysosomal enzyme, {beta}-hexosaminidase A. As consequence of the enzyme deficiency, G{sub M2} ganglioside accumulates progressively, beginning early in fetal life, to excessive amounts in the central nervous system (CNS). Rapid mental and motor deterioration starting in the first year of life leads to death by 2 to 4 years of age. Through the targeted disruption of the Hexa gene in embryonic stem cells, we have produced mice with biochemical and neuropathologic features of Tay-Sachs disease. The mutant mice exhibited less than 1% of normal {beta}-hexosaminidase A activity and accumulated G{sub M2} ganglioside in their CNS in an age-dependent manner. The accumulated ganglioside was stored in neurons as membranous cytoplasmic bodies characteristically found in the neurons of Tay-Sachs disease patients. At three to five months of age the mutant mice showed no apparent defects in motor or memory function. These {beta}-hexosaminidase A deficient mice should be useful for devising strategies to introduce functional enzymes and genes into the CNS. This model may also be valuable for studying the biochemical and pathologic changes occurring during the course of the disease.

  6. GM2-Gangliosidosis (Sandhoff and Tay Sachs disease): Diagnosis and Neuroimaging Findings (An Iranian Pediatric Case Series).

    Science.gov (United States)

    Karimzadeh, Parvaneh; Jafari, Narjes; Nejad Biglari, Habibeh; Jabbeh Dari, Sayena; Ahmad Abadi, Farzad; Alaee, Mohammad-Reza; Nemati, Hamid; Saket, Sasan; Tonekaboni, Seyed Hasan; Taghdiri, Mohammad-Mahdi; Ghofrani, Mohammad

    2014-01-01

    GM2-Gangliosidosis disease is a rare autosomal recessive genetic disorder that includes two disorders (Tay-Sachs and Sandhoff disease).These disorders cause a progressive deterioration of nerve cells and inherited deficiency in creating hexosaminidases A, B, and AB. Patients who were diagnosed withGM2-Gangliosidosis in the Neurology Department of Mofid Children's Hospital in Tehran, Iran from October 2009 to February 2014were included in our study. The disorder was confirmed by neurometabolic and enzyme level detection of hexosaminidases A, B, and AB in reference to Wagnester Laboratory in Germany. We assessed age, gender, past medical history, developmental status, clinical manifestations, and neuroimaging findings of 9 patients with Sandhoff disease and 9 with Tay Sachs disease. 83% of our patients were the offspring of consanguineous marriages. All of them had a developmental disorder as a chief complaint. 38%of patients had a history of developmental delay or regression and 22% had seizures. The patients with Sandhoff and Tay Sachs disease were followed for approximately 5 years and the follow-up showed all patients were bedridden or had expired due to refractory seizures, pneumonia aspiration, or swallowing disorders. Neuro-imaging findings included bilateral thalamic involvement, brain atrophy, and hypo myelination in near half of our patients (48%). According to the results of this study, we suggest that cherry-red spots, hyperacusis, refractory seizures, and relative parents in children with developmental delay and/or regression should be considered for assessment of GM2-Gangliosidosis disease.

  7. On the structure and applications of the Bondi-Metzner-Sachs group

    Science.gov (United States)

    Alessio, Francesco; Esposito, Giampiero

    This work is a pedagogical review dedicated to a modern description of the Bondi-Metzner-Sachs (BMS) group. Minkowski space-time has an interesting and useful group of isometries, but, for a generic space-time, the isometry group is simply the identity and hence provides no significant informations. Yet symmetry groups have important role to play in physics; in particular, the Poincaré group describing the isometries of Minkowski space-time plays a role in the standard definitions of energy-momentum and angular-momentum. For this reason alone it would seem to be important to look for a generalization of the concept of isometry group that can apply in a useful way to suitable curved space-times. The curved space-times that will be taken into account are the ones that suitably approach, at infinity, Minkowski space-time. In particular we will focus on asymptotically flat space-times. In this work, the concept of asymptotic symmetry group of those space-times will be studied. In the first two sections we derive the asymptotic group following the classical approach which was basically developed by Bondi, van den Burg, Metzner and Sachs. This is essentially the group of transformations between coordinate systems of a certain type in asymptotically flat space-times. In the third section the conformal method and the notion of “asymptotic simplicity” are introduced, following mainly the works of Penrose. This section prepares us for another derivation of the BMS group which will involve the conformal structure, and is thus more geometrical and fundamental. In the subsequent sections we discuss the properties of the BMS group, e.g. its algebra and the possibility to obtain as its subgroup the Poincaré group, as we may expect. The paper ends with a review of the BMS invariance properties of classical gravitational scattering discovered by Strominger, that are finding application to black hole physics and quantum gravity in the literature.

  8. Identification of novel mutations in HEXA gene in children affected with Tay Sachs disease from India.

    Directory of Open Access Journals (Sweden)

    Mehul Mistri

    Full Text Available Tay Sachs disease (TSD is a neurodegenerative disorder due to β-hexosaminidase A deficiency caused by mutations in the HEXA gene. The mutations leading to Tay Sachs disease in India are yet unknown. We aimed to determine mutations leading to TSD in India by complete sequencing of the HEXA gene. The clinical inclusion criteria included neuroregression, seizures, exaggerated startle reflex, macrocephaly, cherry red spot on fundus examination and spasticity. Neuroimaging criteria included thalamic hyperdensities on CT scan/T1W images of MRI of the brain. Biochemical criteria included deficiency of hexosaminidase A (less than 2% of total hexosaminidase activity for infantile patients. Total leukocyte hexosaminidase activity was assayed by 4-methylumbelliferyl-N-acetyl-β-D-glucosamine lysis and hexosaminidase A activity was assayed by heat inactivation method and 4-methylumbelliferyl-N-acetyl-β-D-glucosamine-6-sulphate lysis method. The exons and exon-intron boundaries of the HEXA gene were bidirectionally sequenced using an automated sequencer. Mutations were confirmed in parents and looked up in public databases. In silico analysis for mutations was carried out using SIFT, Polyphen2, MutationT@ster and Accelrys Discovery Studio softwares. Fifteen families were included in the study. We identified six novel missense mutations, c.340 G>A (p.E114K, c.964 G>A (p.D322N, c.964 G>T (p.D322Y, c.1178C>G (p.R393P and c.1385A>T (p.E462V, c.1432 G>A (p.G478R and two previously reported mutations. c.1277_1278insTATC and c.508C>T (p.R170W. The mutation p.E462V was found in six unrelated families from Gujarat indicating a founder effect. A previously known splice site mutation c.805+1 G>C and another intronic mutation c.672+30 T>G of unknown significance were also identified. Mutations could not be identified in one family. We conclude that TSD patients from Gujarat should be screened for the common mutation p.E462V.

  9. Identification of novel mutations in HEXA gene in children affected with Tay Sachs disease from India.

    Science.gov (United States)

    Mistri, Mehul; Tamhankar, Parag M; Sheth, Frenny; Sanghavi, Daksha; Kondurkar, Pratima; Patil, Swapnil; Idicula-Thomas, Susan; Gupta, Sarita; Sheth, Jayesh

    2012-01-01

    Tay Sachs disease (TSD) is a neurodegenerative disorder due to β-hexosaminidase A deficiency caused by mutations in the HEXA gene. The mutations leading to Tay Sachs disease in India are yet unknown. We aimed to determine mutations leading to TSD in India by complete sequencing of the HEXA gene. The clinical inclusion criteria included neuroregression, seizures, exaggerated startle reflex, macrocephaly, cherry red spot on fundus examination and spasticity. Neuroimaging criteria included thalamic hyperdensities on CT scan/T1W images of MRI of the brain. Biochemical criteria included deficiency of hexosaminidase A (less than 2% of total hexosaminidase activity for infantile patients). Total leukocyte hexosaminidase activity was assayed by 4-methylumbelliferyl-N-acetyl-β-D-glucosamine lysis and hexosaminidase A activity was assayed by heat inactivation method and 4-methylumbelliferyl-N-acetyl-β-D-glucosamine-6-sulphate lysis method. The exons and exon-intron boundaries of the HEXA gene were bidirectionally sequenced using an automated sequencer. Mutations were confirmed in parents and looked up in public databases. In silico analysis for mutations was carried out using SIFT, Polyphen2, MutationT@ster and Accelrys Discovery Studio softwares. Fifteen families were included in the study. We identified six novel missense mutations, c.340 G>A (p.E114K), c.964 G>A (p.D322N), c.964 G>T (p.D322Y), c.1178C>G (p.R393P) and c.1385A>T (p.E462V), c.1432 G>A (p.G478R) and two previously reported mutations. c.1277_1278insTATC and c.508C>T (p.R170W). The mutation p.E462V was found in six unrelated families from Gujarat indicating a founder effect. A previously known splice site mutation c.805+1 G>C and another intronic mutation c.672+30 T>G of unknown significance were also identified. Mutations could not be identified in one family. We conclude that TSD patients from Gujarat should be screened for the common mutation p.E462V.

  10. Metrical Phonology and SLA.

    Science.gov (United States)

    Tice, Bradley S.

    Metrical phonology, a linguistic process of phonological stress assessment and diagrammatic simplification of sentence and word stress, is discussed as it is found in the English language with the intention that it may be used in second language instruction. Stress is defined by its physical and acoustical correlates, and the principles of…

  11. Engineering performance metrics

    Science.gov (United States)

    Delozier, R.; Snyder, N.

    1993-03-01

    Implementation of a Total Quality Management (TQM) approach to engineering work required the development of a system of metrics which would serve as a meaningful management tool for evaluating effectiveness in accomplishing project objectives and in achieving improved customer satisfaction. A team effort was chartered with the goal of developing a system of engineering performance metrics which would measure customer satisfaction, quality, cost effectiveness, and timeliness. The approach to developing this system involved normal systems design phases including, conceptual design, detailed design, implementation, and integration. The lessons teamed from this effort will be explored in this paper. These lessons learned may provide a starting point for other large engineering organizations seeking to institute a performance measurement system accomplishing project objectives and in achieving improved customer satisfaction. To facilitate this effort, a team was chartered to assist in the development of the metrics system. This team, consisting of customers and Engineering staff members, was utilized to ensure that the needs and views of the customers were considered in the development of performance measurements. The development of a system of metrics is no different than the development of any type of system. It includes the steps of defining performance measurement requirements, measurement process conceptual design, performance measurement and reporting system detailed design, and system implementation and integration.

  12. Metrics for Probabilistic Geometries

    DEFF Research Database (Denmark)

    Tosi, Alessandra; Hauberg, Søren; Vellido, Alfredo

    2014-01-01

    the distribution over mappings is given by a Gaussian process. We treat the corresponding latent variable model as a Riemannian manifold and we use the expectation of the metric under the Gaussian process prior to define interpolating paths and measure distance between latent points. We show how distances...

  13. Metrics for energy resilience

    International Nuclear Information System (INIS)

    Roege, Paul E.; Collier, Zachary A.; Mancillas, James; McDonagh, John A.; Linkov, Igor

    2014-01-01

    Energy lies at the backbone of any advanced society and constitutes an essential prerequisite for economic growth, social order and national defense. However there is an Achilles heel to today's energy and technology relationship; namely a precarious intimacy between energy and the fiscal, social, and technical systems it supports. Recently, widespread and persistent disruptions in energy systems have highlighted the extent of this dependence and the vulnerability of increasingly optimized systems to changing conditions. Resilience is an emerging concept that offers to reconcile considerations of performance under dynamic environments and across multiple time frames by supplementing traditionally static system performance measures to consider behaviors under changing conditions and complex interactions among physical, information and human domains. This paper identifies metrics useful to implement guidance for energy-related planning, design, investment, and operation. Recommendations are presented using a matrix format to provide a structured and comprehensive framework of metrics relevant to a system's energy resilience. The study synthesizes previously proposed metrics and emergent resilience literature to provide a multi-dimensional model intended for use by leaders and practitioners as they transform our energy posture from one of stasis and reaction to one that is proactive and which fosters sustainable growth. - Highlights: • Resilience is the ability of a system to recover from adversity. • There is a need for methods to quantify and measure system resilience. • We developed a matrix-based approach to generate energy resilience metrics. • These metrics can be used in energy planning, system design, and operations

  14. Software Quality Assurance Metrics

    Science.gov (United States)

    McRae, Kalindra A.

    2004-01-01

    Software Quality Assurance (SQA) is a planned and systematic set of activities that ensures conformance of software life cycle processes and products conform to requirements, standards and procedures. In software development, software quality means meeting requirements and a degree of excellence and refinement of a project or product. Software Quality is a set of attributes of a software product by which its quality is described and evaluated. The set of attributes includes functionality, reliability, usability, efficiency, maintainability, and portability. Software Metrics help us understand the technical process that is used to develop a product. The process is measured to improve it and the product is measured to increase quality throughout the life cycle of software. Software Metrics are measurements of the quality of software. Software is measured to indicate the quality of the product, to assess the productivity of the people who produce the product, to assess the benefits derived from new software engineering methods and tools, to form a baseline for estimation, and to help justify requests for new tools or additional training. Any part of the software development can be measured. If Software Metrics are implemented in software development, it can save time, money, and allow the organization to identify the caused of defects which have the greatest effect on software development. The summer of 2004, I worked with Cynthia Calhoun and Frank Robinson in the Software Assurance/Risk Management department. My task was to research and collect, compile, and analyze SQA Metrics that have been used in other projects that are not currently being used by the SA team and report them to the Software Assurance team to see if any metrics can be implemented in their software assurance life cycle process.

  15. Conformal invariant powers of the Laplacian, Fefferman-Graham ambient metric and Ricci gauging

    International Nuclear Information System (INIS)

    Manvelyan, Ruben; Mkrtchyan, Karapet; Mkrtchyan, Ruben

    2007-01-01

    The hierarchy of conformally invariant kth powers of the Laplacian acting on a scalar field with scaling dimensions Δ (k) =k-d/2, k=1,2,3, as obtained in the recent work [R. Manvelyan, D.H. Tchrakian, Phys. Lett. B 644 (2007) 370, (hep-th/0611077)] is rederived using the Fefferman-Graham (d+2)-dimensional ambient space approach. The corresponding mysterious 'holographic' structure of these operators is clarified. We explore also the (d+2)-dimensional ambient space origin of the Ricci gauging procedure proposed by A. Iorio, L. O'Raifeartaigh, I. Sachs and C. Wiesendanger as another method of constructing the Weyl invariant Lagrangians. The corresponding gauged ambient metric, Fefferman-Graham expansion and extended Penrose-Brown-Henneaux transformations are proposed and analyzed

  16. Three novel mutations in Iranian patients with Tay-Sachs disease.

    Science.gov (United States)

    Jamali, Solmaz; Eskandari, Nasim; Aryani, Omid; Salehpour, Shadab; Zaman, Talieh; Kamalidehghan, Behnam; Houshmand, Massoud

    2014-01-01

    Tay-Sachs disease (TSD), or GM2 gangliosidosis, is a lethal autosomal recessive neurodegenerative disorder, which is caused by a deficiency of beta-hexosaminidase A (HEXA), resulting in lysosomal accumulation of GM2 ganglioside. The aim of this study was to identify the TSD-causing mutations in an Iranian population. In this study, we examined 31 patients for TSD-causing mutations using PCR, followed by restriction enzyme digestion. Molecular genetics analysis of DNA from 23 patients of TSD revealed mutations that has been previously reported, including four-base duplications c.1274_1277dupTATC in exon 11 and IVS2+1G>A, deletion TTAGGCAAGGGC in exon 10 as well as a few novel mutations, including C331G, which altered Gln>Glu in HEXB, A>G, T>C, and p.R510X in exon 14, which predicted a termination codon or nonsense mutation. In conclusion, with the discovery of these novel mutations, the genotypic spectrum of Iranian patients with TSD disease has been extended and could facilitate definition of disease-related mutations.

  17. Adeno-Associated Virus Gene Therapy in a Sheep Model of Tay-Sachs Disease.

    Science.gov (United States)

    Gray-Edwards, Heather L; Randle, Ashley N; Maitland, Stacy A; Benatti, Hector R; Hubbard, Spencer M; Canning, Peter F; Vogel, Matthew B; Brunson, Brandon L; Hwang, Misako; Ellis, Lauren E; Bradbury, Allison M; Gentry, Atoska S; Taylor, Amanda R; Wooldridge, Anne A; Wilhite, Dewey R; Winter, Randolph L; Whitlock, Brian K; Johnson, Jacob A; Holland, Merilee; Salibi, Nouha; Beyers, Ronald J; Sartin, James L; Denney, Thomas S; Cox, Nancy R; Sena-Esteves, Miguel; Martin, Douglas R

    2018-03-01

    Tay-Sachs disease (TSD) is a fatal neurodegenerative disorder caused by a deficiency of the enzyme hexosaminidase A (HexA). TSD also occurs in sheep, the only experimental model of TSD that has clinical signs of disease. The natural history of sheep TSD was characterized using serial neurological evaluations, 7 Tesla magnetic resonance imaging, echocardiograms, electrodiagnostics, and cerebrospinal fluid biomarkers. Intracranial gene therapy was also tested using AAVrh8 monocistronic vectors encoding the α-subunit of Hex (TSD α) or a mixture of two vectors encoding both the α and β subunits separately (TSD α + β) injected at high (1.3 × 10 13 vector genomes) or low (4.2 × 10 12 vector genomes) dose. Delay of symptom onset and/or reduction of acquired symptoms were noted in all adeno-associated virus-treated sheep. Postmortem evaluation showed superior HexA and vector genome distribution in the brain of TSD α + β sheep compared to TSD α sheep, but spinal cord distribution was low in all groups. Isozyme analysis showed superior HexA formation after treatment with both vectors (TSD α + β), and ganglioside clearance was most widespread in the TSD α + β high-dose sheep. Microglial activation and proliferation in TSD sheep-most prominent in the cerebrum-were attenuated after gene therapy. This report demonstrates therapeutic efficacy for TSD in the sheep brain, which is on the same order of magnitude as a child's brain.

  18. Simultaneous preimplantation genetic diagnosis for Tay-Sachs and Gaucher disease.

    Science.gov (United States)

    Altarescu, Gheona; Brooks, Barry; Margalioth, Ehud; Eldar Geva, Talia; Levy-Lahad, Ephrat; Renbaum, Paul

    2007-07-01

    Preimplantation genetic diagnosis (PGD) for single gene defects is described for a family in which each parent is a carrier of both Tay-Sachs (TS) and Gaucher disease (GD). A multiplex fluorescent polymerase chain reaction protocol was developed that simultaneously amplified all four familial mutations and 10 informative microsatellite markers. In one PGD cycle, seven blastomeres were analysed, reaching a conclusive diagnosis in six out of seven embryos for TS and in five out of seven embryos for GD. Of the six diagnosed embryos, one was wild type for both TS and GD, and three were wild type for GD and carriers of TS. Two remaining embryos were compound heterozygotes for TS. Two transferable embryos developed into blastocysts (wt/wt and wt GD/carrier TS) and both were transferred on day 5. This single cycle of PGD resulted in a healthy live child. Allele drop-out (ADO) was observed in three of 34 reactions, yielding an 8% ADO rate. The occurrence of ADO in single cell analysis and undetected recombination events are primary causes of misdiagnosis in PGD and emphasize the need to use multiple polymorphic markers. So far as is known, this is the first report of concomitant PGD for two frequent Ashkenazi Jewish recessive disorders.

  19. Radioimmunoassay and heat denaturation enzyme assay for the detection of Tay-Sachs heterozygotes during pregnancy

    International Nuclear Information System (INIS)

    Nguyen, C.; Gold, R.J.M.; Mahuran, D.; Lowden, J.A.

    1981-01-01

    Tay-Sachs disease results from a loss of activity of hexosaminidase A (HEX A) in body tissues and fluids. Heterozygotes for the disease are usually identified by their relatively low ratio of heat-labile HEX A to total hexosaminidase. During pregnancy an intermediate isoenzyme (HEX I) increases in activity in serum and obscures the heterozygote status. HEX I does not increase in leucocytes, tears and other body tissues but because of technical difficulties in these assays the authors examined the feasibility of using a radioimmunoassay for HEX A. By univariate analysis, the heat denaturation assay gave a lower cost of misclassification for non-pregnant normals while RIA did so for pregnant normals. A combination of both tests led to reduced cost of misclassification compared to either alone. Bayesian analysis of bivariate gaussian density functions for heat denaturation and for radioimmunoassays of HEX isoenzymes was employed to calculate misclassification frequencies. Among the parameters examined, HEX A measured by RIA and % HEX A by heat-denaturation assay were the two having the best discriminatory power. (Auth.)

  20. High school Tay-Sachs disease carrier screening: 5 to 11-year follow-up.

    Science.gov (United States)

    Curd, Helen; Lewis, Sharon; Macciocca, Ivan; Sahhar, Margaret; Petrou, Vicki; Bankier, Agnes; Lieberman, Sari; Levy-Lahad, Ephrat; Delatycki, Martin B

    2014-04-01

    The Melbourne high school Tay-Sachs disease (TSD) carrier screening program began in 1997. The aim of this study was to assess the outcomes of this screening program among those who had testing more than 5 years ago, to evaluate the long-term impact of screening. A questionnaire was used for data collection and consisted of validated scales and purposively designed questions. Questionnaires were sent to all carriers and two non-carriers for each carrier who were screened in the program between 1999 and 2005. Twenty-four out of 69 (34.8 %) carriers and 30/138 (21.7 %) non-carriers completed the questionnaire. Most participants (82 %) retained good knowledge of TSD and there was no evidence of a difference in knowledge between carriers and non-carriers. Most participants (83 %) were happy with the timing and setting of screening and thought that education and screening for TSD should be offered during high school. There was no difference between carriers and non-carriers in mean scores for the State Trait Anxiety Inventory and Decision Regret Scale. This evaluation indicated that 5-11 years post high school screening, those who were screened are supportive of the program and that negative consequences are rare.

  1. The presence of two different infantile Tay-Sachs disease mutations in a Cajun population

    Energy Technology Data Exchange (ETDEWEB)

    McDowell, G.A.; Blitzer, M.G. (Univ. of Maryland School of Medicine, Baltimore, MD (United States)); Mules, E.H. (Kennedy Krieger Institute, Baltimore, MD (United States)); Fabacher, P. (Office of Public Health, Shreveport, LA (United States)); Shapira, E. (Tulane Univ. of School of Medicine, New Orleans, LA (United States))

    1992-11-01

    A study was undertaken to characterize the mutation(s) responsible for Tay-Sachs disease (TSD) in a Cajun population in southwest Louisiana and to identify the origins of these mutations. Eleven of 12 infantile TSD alleles examined in six families had the [beta]-hexosaminidase A (Hex A) [alpha]-subunit exon 11 insertion mutation that is present in approximately 70% of Ashkenazi Jewish TSD heterozygotes. The mutation in the remaining allele was a single-base transition in the donor splice site of the [alpha]-subunit intron 9. To determine the origins of these two mutations in the Cajun population, the TSD carrier status was enzymatically determined for 90 members of four of the six families, and extensive pedigrees were constructed for all carriers. A single ancestral couple from France was found to be common to most of the carriers of the exon 11 insertion. Pedigree data suggest that this mutation has been in the Cajun population since its founding over 2 centuries ago and that it may be widely distributed within the population. In contrast, the intron 9 mutation apparently was introduced within the last century and probably is limited to a few Louisiana families. 29 refs., 4 figs.

  2. Ashkenazi Jewish population screening for Tay-Sachs disease: the international and Australian experience.

    Science.gov (United States)

    Lew, Raelia M; Burnett, Leslie; Proos, Anné L; Barlow-Stewart, Kristine; Delatycki, Martin B; Bankier, Agnes; Aizenberg, Harry; Field, Michael J; Berman, Yemima; Fleischer, Ronald; Fietz, Michael

    2015-03-01

    Internationally, Tay-Sachs disease (TSD) preconception screening of Ashkenazi Jewish (AJ) individuals and couples has led to effective primary prevention of TSD. In Australia, adolescent preconception genetic screening programs operate mainly in Jewish community high schools. These existing programs offer an effective means of primary prevention of TSD, are cost effective and safe. However, in the broader Australian community TSD screening is not systematically performed and cases still occur in unscreened AJ individuals. In order to improve the effectiveness of Australian screening, there is a need for definitive guidelines for healthcare professionals to facilitate extension of the proven benefits of preconception TSD screening to all AJ individuals at risk. We performed a systematic review of the relevant literature relating to AJ pre-conception and antenatal screening for TSD. The evidence was assessed using an established National Health and Medical Research Council evidence grading system. Evaluations of efficacy of TSD screening programs design and execution, cost-benefit and cost-utility health economic evaluation, and population outcomes were undertaken. The results have been used to propose a model for universal AJ TSD preconception and antenatal screening for the primary care setting. © 2014 The Authors. Journal of Paediatrics and Child Health © 2014 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  3. Spontaneous appearance of Tay-Sachs disease in an animal model.

    Science.gov (United States)

    Zeng, B J; Torres, P A; Viner, T C; Wang, Z H; Raghavan, S S; Alroy, J; Pastores, G M; Kolodny, E H

    2008-01-01

    Tay-Sachs disease (TSD) is a progressive neurodegenerative disorder due to an autosomal recessively inherited deficiency of beta-hexosaminidase A (Hex A). Deficiency of Hex A in TSD is caused by a defect of the alpha-subunit resulting from mutations of the HEXA gene. To date, there is no effective treatment for TSD. Animal models of genetic diseases, similar to those known to exist in humans, are valuable and essential research tools for the study of potentially effective therapies. However, there is no ideal animal model of TSD available for use in therapeutic trials. In the present study, we report an animal model (American flamingo; Phoenicopterus ruber) of TSD with Hex A deficiency occurring spontaneously in nature, with accumulation of G(M2)-ganglioside, deficiency of Hex A enzymatic activity, and a homozygous P469L mutation in exon 12 of the hexa gene. In addition, we have isolated the full-length cDNA sequence of the flamingo, which consists of 1581 nucleotides encoding a protein of 527 amino acids. Its coding sequence indicates approximately 71% identity at the nucleotide level and about 72.5% identity at the amino acid level with the encoding region of the human HEXA gene. This animal model, with many of the same features as TSD in humans, could represent a valuable resource for investigating therapy of TSD.

  4. Integrated Sachs-Wolfe effect versus redshift test for the cosmological parameters

    Science.gov (United States)

    Kantowski, R.; Chen, B.; Dai, X.

    2015-04-01

    We describe a method using the integrated Sachs-Wolfe (ISW) effect caused by individual inhomogeneities to determine the cosmological parameters H0, Ωm , and ΩΛ, etc. This ISW-redshift test requires detailed knowledge of the internal kinematics of a set of individual density perturbations, e.g., galaxy clusters and/or cosmic voids, in particular their density and velocity profiles, and their mass accretion rates. It assumes the density perturbations are isolated and embedded (equivalently compensated) and makes use of the newly found relation between the ISW temperature perturbation of the cosmic microwave background (CMB) and the Fermat potential of the lens. Given measurements of the amplitudes of the temperature variations in the CMB caused by such clusters or voids at various redshifts and estimates of their angular sizes or masses, one can constrain the cosmological parameters. More realistically, the converse is more likely, i.e., if the background cosmology is sufficiently constrained, measurement of ISW profiles of clusters and voids (e.g., hot and cold spots and rings) can constrain dynamical properties of the dark matter, including accretion, associated with such lenses and thus constrain the evolution of these objects with redshift.

  5. Enterprise Sustainment Metrics

    Science.gov (United States)

    2015-06-19

    are negatively impacting KPIs” (Parmenter, 2010: 31). In the current state, the Air Force’s AA and PBL metrics are once again split . AA does...must have the authority to “take immediate action to rectify situations that are negatively impacting KPIs” (Parmenter, 2010: 31). 3. Measuring...highest profitability and shareholder value for each company” (2014: 273). By systematically diagraming a process, either through a swim lane flowchart

  6. Tay-Sachs and Sandhoff diseases: enzymatic diagnosis in dried blood spots on filter paper: retrospective diagnoses in newborn-screening cards.

    Science.gov (United States)

    Chamoles, Néstor A; Blanco, Mariana; Gaggioli, Daniela; Casentini, Carina

    2002-04-01

    Tay-Sachs disease (TSD), Sandhoff disease (SD) and variants are caused by deficient activity of the lysosomal enzymes hexosaminidase A (HA) and total hexosaminidase (TH) (hexosaminidase A plus B), respectively. For diagnosis, these enzymes are usually measured in plasma or extracts of leukocytes. We describe methods for the assay of hexosaminidase A and total hexosaminidase activities in dried blood spots (DBSs) on filter paper. We studied 163 healthy controls, 9 Tay-Sachs patients, 4 Sandhoff patients, 18 obligate carriers and the newborn-screening cards from two patients with Tay-Sachs and one patient with Sandhoff disease. To tubes containing a 3-mm-diameter blood spot, we added elution liquid and substrate solution. After incubation at 37 degrees C, the amount of hydrolyzed product was compared with a calibrator to allow the quantification of enzyme activity. The described methodology is useful to distinguish patients with Tay-Sachs disease or Sandhoff disease from carriers and controls using samples that are sufficiently stable to be transported to the testing laboratory by mail. The diagnosis of both diseases from a newborn-screening card (NSC) was clearly demonstrated, even after storage for up to 38 months at room temperature. The newborn-screening card has been added to the biological materials that allow the identification of patients with Tay-Sachs disease and Sandhoff disease.

  7. Symmetries of the dual metrics

    International Nuclear Information System (INIS)

    Baleanu, D.

    1998-01-01

    The geometric duality between the metric g μν and a Killing tensor K μν is studied. The conditions were found when the symmetries of the metric g μν and the dual metric K μν are the same. Dual spinning space was constructed without introduction of torsion. The general results are applied to the case of Kerr-Newmann metric

  8. Kerr metric in cosmological background

    Energy Technology Data Exchange (ETDEWEB)

    Vaidya, P C [Gujarat Univ., Ahmedabad (India). Dept. of Mathematics

    1977-06-01

    A metric satisfying Einstein's equation is given which in the vicinity of the source reduces to the well-known Kerr metric and which at large distances reduces to the Robertson-Walker metric of a nomogeneous cosmological model. The radius of the event horizon of the Kerr black hole in the cosmological background is found out.

  9. Learning Low-Dimensional Metrics

    OpenAIRE

    Jain, Lalit; Mason, Blake; Nowak, Robert

    2017-01-01

    This paper investigates the theoretical foundations of metric learning, focused on three key questions that are not fully addressed in prior work: 1) we consider learning general low-dimensional (low-rank) metrics as well as sparse metrics; 2) we develop upper and lower (minimax)bounds on the generalization error; 3) we quantify the sample complexity of metric learning in terms of the dimension of the feature space and the dimension/rank of the underlying metric;4) we also bound the accuracy ...

  10. Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population

    Directory of Open Access Journals (Sweden)

    Anaita Udwadia-Hegde MD, DCH, MRCPCH

    2017-01-01

    Full Text Available Background: Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. Patient: We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. Conclusion: This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

  11. Temporary Efficacy of Pyrimethamine in Juvenile-Onset Tay-Sachs Disease Caused by 2 Unreported HEXA Mutations in the Indian Population.

    Science.gov (United States)

    Udwadia-Hegde, Anaita; Hajirnis, Omkar

    2017-01-01

    Juvenile Tay-Sachs disease is rarer than other forms of Tay-Sachs disease and is usually seen in children between the age of 2 and 10 years. Pyrimethamine as a pharmacological chaperone was used to increase β-hexosaminidase A activity in this patient. We describe a patient with Tay-Sachs disease from the Indian population, a juvenile case who presented with developmental regression starting at the age of three, initially with motor followed by language regression. She is currently incapacitated with severe behavioral issues. This brief communication gives an insight into the efficacy of pharmacological chaperones. It also describes two unreported mutations in hexosaminidase A gene from the Indian population. After commencing Pyrimethamine, though initial benefits with increase in levels corresponded with briefly halting the motor regression, the observed increase was only transient and not associated with discernible beneficial neurological or psychiatric effects.

  12. The effect of a combined glenoid and Hill-Sachs defect on glenohumeral stability: a biomechanical cadaveric study using 3-dimensional modeling of 142 patients.

    Science.gov (United States)

    Arciero, Robert A; Parrino, Anthony; Bernhardson, Andrew S; Diaz-Doran, Vilmaris; Obopilwe, Elifho; Cote, Mark P; Golijanin, Petr; Mazzocca, Augustus D; Provencher, Matthew T

    2015-06-01

    Bone loss in anterior glenohumeral instability occurs on both the glenoid and the humerus; however, existing biomechanical studies have evaluated glenoid and humeral head defects in isolation. Thus, little is known about the combined effect of these bony lesions in a clinically relevant model on glenohumeral stability. The purpose of this study was to determine the biomechanical efficacy of a Bankart repair in the setting of bipolar (glenoid and humeral head) bone defects determined via computer-generated 3-dimensional (3D) modeling of 142 patients with recurrent anterior shoulder instability. The null hypothesis was that adding a bipolar bone defect will have no effect on glenohumeral stability after soft tissue Bankart repair. Controlled laboratory study. A total of 142 consecutive patients with recurrent anterior instability were analyzed with 3D computed tomography scans. Two Hill-Sachs lesions were selected on the basis of volumetric size representing the 25th percentile (0.87 cm(3); small) and 50th percentile (1.47 cm(3); medium) and printed in plastic resin with a 3D printer. A total of 21 cadaveric shoulders were evaluated on a custom shoulder-testing device permitting 6 degrees of freedom, and the force required to translate the humeral head anteriorly 10 mm at a rate of 2.0 mm/s with a compressive load of 50 N was determined at 60° of glenohumeral abduction and 60° of external rotation. All Bankart lesions were made sharply from the 2- to 6-o'clock positions for a right shoulder. Subsequent Bankart repair with transosseous tunnels using high-strength suture was performed. Hill-Sachs lesions were made in the cadaver utilizing a plastic mold from the exact replica off the 3D printer. Testing was conducted in the following sequence for each specimen: (1) intact, (2) posterior capsulotomy, (3) Bankart lesion, (4) Bankart repair, (5) Bankart lesion with 2-mm glenoid defect, (6) Bankart repair, (7) Bankart lesion with 2-mm glenoid defect and Hill-Sachs lesion

  13. Magnification-temperature correlation: The dark side of integrated Sachs-Wolfe measurements

    International Nuclear Information System (INIS)

    LoVerde, Marilena; Hui, Lam; Gaztanaga, Enrique

    2007-01-01

    Integrated Sachs-Wolfe (ISW) measurements, which involve cross-correlating the microwave background anisotropies with the foreground large-scale structure (e.g. traced by galaxies/quasars), have proven to be an interesting probe of dark energy. We show that magnification bias, which is the inevitable modulation of the foreground number counts by gravitational lensing, alters both the scale dependence and amplitude of the observed ISW signal. This is true especially at high redshifts because (1) the intrinsic galaxy-temperature signal diminishes greatly back in the matter-dominated era, (2) the lensing efficiency increases with redshift and (3) the number count slope generally steepens with redshift in a magnitude limited sample. At z > or approx. 2, the magnification-temperature correlation dominates over the intrinsic galaxy-temperature correlation and causes the observed ISW signal to increase with redshift, despite dark energy subdominance--a result of the fact that magnification probes structures all the way from the observer to the sources. Ignoring magnification bias therefore can lead to (significantly) erroneous conclusions about dark energy. While the lensing modulation opens up an interesting high z window for ISW measurements, high redshift measurements are not expected to add much new information to low redshift ones if dark energy is indeed the cosmological constant. This is because lensing introduces significant covariance across redshifts. The most compelling reasons for pursuing high redshift ISW measurements are to look for potential surprises such as early dark energy domination or signatures of modified gravity. We conclude with a discussion of existing measurements, the highest redshift of which is at the margin of being sensitive to the magnification effect. We also develop a formalism which might be of more general interest: to predict biases in estimating parameters when certain physical effects are ignored in interpreting observations

  14. Updated tomographic analysis of the integrated Sachs-Wolfe effect and implications for dark energy

    Science.gov (United States)

    Stölzner, Benjamin; Cuoco, Alessandro; Lesgourgues, Julien; Bilicki, Maciej

    2018-03-01

    We derive updated constraints on the integrated Sachs-Wolfe (ISW) effect through cross-correlation of the cosmic microwave background with galaxy surveys. We improve with respect to similar previous analyses in several ways. First, we use the most recent versions of extragalactic object catalogs, SDSS DR12 photometric redshift (photo-z ) and 2MASS Photo-z data sets, as well as those employed earlier for ISW, SDSS QSO photo-z and NVSS samples. Second, we use for the first time the WISE × SuperCOSMOS catalog, which allows us to perform an all-sky analysis of the ISW up to z ˜0.4 . Third, thanks to the use of photo-z s , we separate each data set into different redshift bins, deriving the cross-correlation in each bin. This last step leads to a significant improvement in sensitivity. We remove cross-correlation between catalogs using masks which mutually exclude common regions of the sky. We use two methods to quantify the significance of the ISW effect. In the first one, we fix the cosmological model, derive linear galaxy biases of the catalogs, and then evaluate the significance of the ISW using a single parameter. In the second approach we perform a global fit of the ISW and of the galaxy biases varying the cosmological model. We find significances of the ISW in the range 4.7 - 5.0 σ thus reaching, for the first time in such an analysis, the threshold of 5 σ . Without the redshift tomography we find a significance of ˜4.0 σ , which shows the importance of the binning method. Finally we use the ISW data to infer constraints on the dark energy redshift evolution and equation of state. We find that the redshift range covered by the catalogs is still not optimal to derive strong constraints, although this goal will be likely reached using future datasets such as from Euclid, LSST, and SKA.

  15. Serial CT scans and MRI scans of Tay-Sachs disease

    International Nuclear Information System (INIS)

    Shimoizumi, Hideo; Miyao, Masutomo; Ichihashi, Koh; Sawa, Rituko; Yamamoto, Yoshifumi; Tanaka, Osamu; Yanagisawa, Masayoshi; Kamoshita, Shigehiko.

    1986-01-01

    Serial X-ray CT and MRI were performed on 2 cases of Tay-Sachs disease. Case 1 : a 3-year-10-month-old girl. Her developmental milestones were normal until the age of 6 months. At the age of 10 months, hypotonia and unduly sensitiveness to sounds were noticed. She had cherry red spots in both fundi, and the serum hexosaminidase A activity was low. Significant clinical features during the next 3 years included regression of developmental milestones to the level of one month, and myoclonic and generalised seizures. After 3 years she showed megalencephaly, unstable body temperature, and respiratory disorders. Case 2 : a 2-year-6-month-old boy. His developmental milestones were normal until the age of 5 months. Then he manifested hyperreaction to noises and further developments ceased. He started to deteriorate from the age of 1 year and 2 months, and had no head control at the age of 1 year and 6 months. Cherry red spots were detected and the serum hexsosaminidase A activity was low, too. X-ray CT at the early stage of cases 1 and 2 showed mild cerebral atrophy and high density areas in bilateral thalami and basal ganglia. At the late stage of Case 1, high density areas appeared in occipital and frontal white matters. MRI in both patients were strikingly similar. In their grey matters, linear light areas were demonstrated on IR images. Their white matters showed extensive dark (long T 1 ) areas on IR images, and extensive light (long T 2 ) areas on SE images. Bilateral thalami and basal ganglia were light on IR images and dark on SE images. The findings of MRI in our patients seemed to correlate well with pathological and biochemical changes in their brains. (author)

  16. Characterization of inducible models of Tay-Sachs and related disease.

    Science.gov (United States)

    Sargeant, Timothy J; Drage, Deborah J; Wang, Susan; Apostolakis, Apostolos A; Cox, Timothy M; Cachón-González, M Begoña

    2012-09-01

    Tay-Sachs and Sandhoff diseases are lethal inborn errors of acid β-N-acetylhexosaminidase activity, characterized by lysosomal storage of GM2 ganglioside and related glycoconjugates in the nervous system. The molecular events that lead to irreversible neuronal injury accompanied by gliosis are unknown; but gene transfer, when undertaken before neurological signs are manifest, effectively rescues the acute neurodegenerative illness in Hexb-/- (Sandhoff) mice that lack β-hexosaminidases A and B. To define determinants of therapeutic efficacy and establish a dynamic experimental platform to systematically investigate cellular pathogenesis of GM2 gangliosidosis, we generated two inducible experimental models. Reversible transgenic expression of β-hexosaminidase directed by two promoters, mouse Hexb and human Synapsin 1 promoters, permitted progression of GM2 gangliosidosis in Sandhoff mice to be modified at pre-defined ages. A single auto-regulatory tetracycline-sensitive expression cassette controlled expression of transgenic Hexb in the brain of Hexb-/- mice and provided long-term rescue from the acute neuronopathic disorder, as well as the accompanying pathological storage of glycoconjugates and gliosis in most parts of the brain. Ultimately, late-onset brainstem and ventral spinal cord pathology occurred and was associated with increased tone in the limbs. Silencing transgenic Hexb expression in five-week-old mice induced stereotypic signs and progression of Sandhoff disease, including tremor, bradykinesia, and hind-limb paralysis. As in germline Hexb-/- mice, these neurodegenerative manifestations advanced rapidly, indicating that the pathogenesis and progression of GM2 gangliosidosis is not influenced by developmental events in the maturing nervous system.

  17. Transient HEXA expression in a transformed human fetal Tay-Sachs disease neuroglial cell line

    Energy Technology Data Exchange (ETDEWEB)

    Fernandes, M.J.; Hechtman, P.; Kaplan, F. [McGill Univ., Quebec (Canada)] [and others

    1994-09-01

    Tay-Sachs disease (TSD) is a severe neurodegenerative disorder characterized by the accumulation of GM{sub 2} ganglioside in the neurons of the central cortex. The recessively inherited disorder results from deficiency of hexosaminidase A (Hex A), a heterodimer of an {alpha} and {beta} subunit encoded by the HEXA and HEXB genes. Expression of HEXA mutations in COS cells has several disadvantages including high endogenous hexosaminidase activity. We report a new transient expression system with very low endogenous Hex A activity. An SV40-transformed fetal TSD neuroglial cell line was assessed for transient expression of the HEXA gene. pCMV{alpha}, a vector incorporating the cytomegalovirus promoter with the human {alpha}-subunit cDNA insert, proved to be the most efficient expression vector. Transfection of 4x10{sup 6} cells with 5-20 {mu}g of plasmid resulted in 100 to 500-fold Hex A activity (4MUGS hydrolysis) relative to mock-transfected cells. Use of pCMV{beta}-Gal as a control for transfection efficiency indicated that 10-20% of cells were transfected. Hex A specific activity increased for at least 72 h post-transfection. This new transient expression system should greatly improve the characterization of mutations in which low levels of HEXA expression result in milder clinical phenotypes and permit studies on enzymatic properties of mutant forms of Hex A. Since the cells used are of CNS origin and synthesize gangliosides, it should also be possible to study, in culture, the metabolic phenotype associated with TSD.

  18. Molecular characterization of both alleles in an unusual Tay-Sachs disease BI variant

    Energy Technology Data Exchange (ETDEWEB)

    Coulter-Mackie, M.B. (Univ. of Western Ontario, London (Canada) Child Health Research Institute, Children' s Hospital of Western Ontario, London (Canada) Child Parent Resource Institute, London, Ontario (Canada))

    1994-06-01

    In a recent report, the authors described an exon 6 mutation in a Tay-Sachs B1 variant patient, first reported by Gordon et al. (1988), who displayed a typical B1 variant biochemical phenotype - i.e., (a) significant levels of hexosaminidase A (Hex A) activity in an assay with a neutral synthetic substrate, 4-methylumbelliferyl-[beta]-N-acetylglucosamide, and (b) <2% of control Hex A in a test on the sulfated substrate, 4-methylumbelliferyl-[beta]-N-acetylglucosamide-6-sulfate. The patient was found to carry a double mutation (G[sub 574][yields]C [val[sub 192][yields]leu] and G[sub 598][yields]A [val[sub 200][yields]met]) inherited from her mother. Only the 574 mutation produced a deleterious effect on Hex A activity in transfected COS0-1 cells, producing a B1 variant biochemical phenotype. The paternal allele apparently caused decreased abundance of mRNA, since no candidate paternal mutations were found in cloned reverse transcription-PCR (RT-PCR) products in the reported study. The biochemical phenotype of the original patient and the properties of the cDNA carrying the G[sub 574] [yields] C mutation in transient expression studies were compatible with a B1 variant mutation. The possibility remained that there might be some contribution from the paternal allele to the patient's phenotype. However, the paternal allele produces relatively low yields of a largely mis-spliced mRNA whose product would not be functional. Therefore, the G[sub 574] [yields] C (val[yields]leu) mutation in the maternal allele is clearly confirmed as a B1 variant mutation with all the ramifications for the substrate binding site and/or catalytic center that this implies.

  19. Tay Sachs disease in Australia: reduced disease incidence despite stable carrier frequency in Australian Jews.

    Science.gov (United States)

    Lew, Raelia M; Proos, Anne L; Burnett, Leslie; Delatycki, Martin; Bankier, Agnes; Fietz, Michael J

    2012-12-10

    To evaluate the outcomes of preconception screening of Jewish Australians for Tay Sachs disease (TSD) carrier status on Jewish TSD-affected births. Epidemiological observational study involving a complete retrospective audit of infantile and intermediate TSD cases diagnosed in Sydney and Melbourne between 1 January 1995 and 31 December 2011 (Royal Children's Hospital Melbourne; Pacific Laboratory Medicine Services, Pathology North, NSW Health Pathology, Sydney; Victorian Clinical Genetics Services, Melbourne; and SA Pathology, Adelaide), and carrier frequency among Jewish high school students attending schools participating in TSD screening programs over the same period. Jewish TSD carrier frequency; and expected versus observed Jewish TSD-affected births. The 2006 Census indicated that most of the total 88,826 Jewish Australians live in Melbourne (46%) and Sydney (40%). The 7,756 Jewish high school students screened for TSD in Sydney and Melbourne during the study period had a carrier frequency of one in 31 (3.26%; 95% CI, 2.89%-3.68%).The estimated expected number of TSD-affected births in Melbourne and Sydney in 1995-2011 was 4.1 for Jewish births and 7.4 for other births (a ratio of Jewish to non-Jewish births of 1:2). The actual number was 12 (four in Sydney and eight in Melbourne), of which two were Jewish (a ratio of Jewish to non-Jewish births of 1:5). This finding of fewer than expected Jewish TSD cases coincided with a period during which screening programs were operating. There have been no Jewish TSD-affected children born to parents who were screened previously. Community education, appreciation of autosomal recessive inheritance and genetic carrier screening before pregnancy are the likely factors in our finding of fewer than expected Jewish babies with TSD. Ongoing outcome monitoring must continue.

  20. Characterization of inducible models of Tay-Sachs and related disease.

    Directory of Open Access Journals (Sweden)

    Timothy J Sargeant

    2012-09-01

    Full Text Available Tay-Sachs and Sandhoff diseases are lethal inborn errors of acid β-N-acetylhexosaminidase activity, characterized by lysosomal storage of GM2 ganglioside and related glycoconjugates in the nervous system. The molecular events that lead to irreversible neuronal injury accompanied by gliosis are unknown; but gene transfer, when undertaken before neurological signs are manifest, effectively rescues the acute neurodegenerative illness in Hexb-/- (Sandhoff mice that lack β-hexosaminidases A and B. To define determinants of therapeutic efficacy and establish a dynamic experimental platform to systematically investigate cellular pathogenesis of GM2 gangliosidosis, we generated two inducible experimental models. Reversible transgenic expression of β-hexosaminidase directed by two promoters, mouse Hexb and human Synapsin 1 promoters, permitted progression of GM2 gangliosidosis in Sandhoff mice to be modified at pre-defined ages. A single auto-regulatory tetracycline-sensitive expression cassette controlled expression of transgenic Hexb in the brain of Hexb-/- mice and provided long-term rescue from the acute neuronopathic disorder, as well as the accompanying pathological storage of glycoconjugates and gliosis in most parts of the brain. Ultimately, late-onset brainstem and ventral spinal cord pathology occurred and was associated with increased tone in the limbs. Silencing transgenic Hexb expression in five-week-old mice induced stereotypic signs and progression of Sandhoff disease, including tremor, bradykinesia, and hind-limb paralysis. As in germline Hexb-/- mice, these neurodegenerative manifestations advanced rapidly, indicating that the pathogenesis and progression of GM2 gangliosidosis is not influenced by developmental events in the maturing nervous system.

  1. Probing the integrated Sachs-Wolfe effect using embedded lens models

    Science.gov (United States)

    Chen, B.; Kantowski, R.

    2015-04-01

    The photometry profile of the integrated Sachs-Wolfe (ISW) effect, recently obtained by the Planck consortium by stacking patches of cosmic microwave background (CMB) sky maps around a large number of cosmic voids, contains a cold ring at about half the void's effective radius surrounded by a hot ring near the void's boundary. The source of the temperature structure is assumed to be the ISW effect but the exact cause of the ringed structure is not currently well understood, particularly the outer hot ring. Numerical simulations have suggested that hot/cold ring structures can be produced by motions associated with nonlinear growths of cosmic structures whose gravitational potentials produce the ISW effect. We have recently developed the embedded lens theory and the Fermat potential formalism which can be used to model the ISW effect caused by intervening individual lens inhomogeneities evolving arbitrarily. This theory only requires knowledge of the void's projected mass profile as a function of the passing CMB photons' impact radius and the rate of change of that mass distribution at passage. We present two simple embedded void lens models with evolving mass densities and investigate the ISW effect caused by these lenses. Both models possess expanding mass shells which produce hot rings around central cold regions, consistent with the recent observations. By adding a small overdensity at the void's center we can produce the slight positive temperature excess hinted at in Planck's photometric results. We conclude that the embedded lens theory and the Fermat potential formalism is well suited for modeling the ISW effect.

  2. Embedded Lensing Time Delays, the Fermat Potential, and the Integrated Sachs-Wolfe Effect

    Science.gov (United States)

    Chen, Bin; Kantowski, Ronald; Dai, Xinyu

    2015-05-01

    We derive the Fermat potential for a spherically symmetric lens embedded in a Friedman-Lemaître-Robertson-Walker cosmology and use it to investigate the late-time integrated Sachs-Wolfe (ISW) effect, i.e., secondary temperature fluctuations in the cosmic microwave background (CMB) caused by individual large-scale clusters and voids. We present a simple analytical expression for the temperature fluctuation in the CMB across such a lens as a derivative of the lens’ Fermat potential. This formalism is applicable to both linear and nonlinear density evolution scenarios, to arbitrarily large density contrasts, and to all open and closed background cosmologies. It is much simpler to use and makes the same predictions as conventional approaches. In this approach the total temperature fluctuation can be split into a time-delay part and an evolutionary part. Both parts must be included for cosmic structures that evolve and both can be equally important. We present very simple ISW models for cosmic voids and galaxy clusters to illustrate the ease of use of our formalism. We use the Fermat potentials of simple cosmic void models to compare predicted ISW effects with those recently extracted from WMAP and Planck data by stacking large cosmic voids using the aperture photometry method. If voids in the local universe with large density contrasts are no longer evolving we find that the time delay contribution alone predicts values consistent with the measurements. However, we find that for voids still evolving linearly, the evolutionary contribution cancels a significant part of the time delay contribution and results in predicted signals that are much smaller than recently observed.

  3. Pathological alterations typical of human Tay-Sachs disease, in the retina of a deep-sea fish

    Science.gov (United States)

    Fishelson, L.; Delarea, Yacov; Galil, Bella S.

    Micrographs of retinas from the deep-sea fish Cataetyx laticeps revealed visual cells containing membranous whorls in the ellipsoids of the inner segments resulting from stretching and modifications of the mitochondria membranes and their cristae. These pathological structures seem to be homologous to the whorls observed in retinas of human carriers of Tay-Sachs disease. This disease, a genetic disorder, is found in humans and some mammals. Our findings in fish suggest that the gene responsible can be found throughout the vertebrate evolutionary tree, possibly dormant in most taxa.

  4. Identification of two HEXA mutations causing infantile-onset Tay-Sachs disease in the Persian population.

    Science.gov (United States)

    Haghighi, Alireza; Rezazadeh, Jamileh; Shadmehri, Azam Ahmadi; Haghighi, Amirreza; Kornreich, Ruth; Desnick, Robert J

    2011-09-01

    The β-hexosaminidase A (HEXA) mutations in the first reported cases of infantile Tay-Sachs disease in the Persian population were identified in two unrelated consanguineous families. The clinical diagnoses of the affected infants were confirmed by their markedly deficient levels of HEXA activity in plasma or peripheral leukocytes. The specific causative mutation in each family was determined by sequencing the HEXA alleles in both sets of related parents. Two mutations were identified: c.1A>G (p.MIV), which obliterated the initiating methionine in codon 1, and c.1177C>T (p.R393X), which predicted a termination codon or nonsense mutation.

  5. Metrics with vanishing quantum corrections

    International Nuclear Information System (INIS)

    Coley, A A; Hervik, S; Gibbons, G W; Pope, C N

    2008-01-01

    We investigate solutions of the classical Einstein or supergravity equations that solve any set of quantum corrected Einstein equations in which the Einstein tensor plus a multiple of the metric is equated to a symmetric conserved tensor T μν (g αβ , ∂ τ g αβ , ∂ τ ∂ σ g αβ , ...,) constructed from sums of terms, the involving contractions of the metric and powers of arbitrary covariant derivatives of the curvature tensor. A classical solution, such as an Einstein metric, is called universal if, when evaluated on that Einstein metric, T μν is a multiple of the metric. A Ricci flat classical solution is called strongly universal if, when evaluated on that Ricci flat metric, T μν vanishes. It is well known that pp-waves in four spacetime dimensions are strongly universal. We focus attention on a natural generalization; Einstein metrics with holonomy Sim(n - 2) in which all scalar invariants are zero or constant. In four dimensions we demonstrate that the generalized Ghanam-Thompson metric is weakly universal and that the Goldberg-Kerr metric is strongly universal; indeed, we show that universality extends to all four-dimensional Sim(2) Einstein metrics. We also discuss generalizations to higher dimensions

  6. Sharp metric obstructions for quasi-Einstein metrics

    Science.gov (United States)

    Case, Jeffrey S.

    2013-02-01

    Using the tractor calculus to study smooth metric measure spaces, we adapt results of Gover and Nurowski to give sharp metric obstructions to the existence of quasi-Einstein metrics on suitably generic manifolds. We do this by introducing an analogue of the Weyl tractor W to the setting of smooth metric measure spaces. The obstructions we obtain can be realized as tensorial invariants which are polynomial in the Riemann curvature tensor and its divergence. By taking suitable limits of their tensorial forms, we then find obstructions to the existence of static potentials, generalizing to higher dimensions a result of Bartnik and Tod, and to the existence of potentials for gradient Ricci solitons.

  7. Completion of a Dislocated Metric Space

    Directory of Open Access Journals (Sweden)

    P. Sumati Kumari

    2015-01-01

    Full Text Available We provide a construction for the completion of a dislocated metric space (abbreviated d-metric space; we also prove that the completion of the metric associated with a d-metric coincides with the metric associated with the completion of the d-metric.

  8. Towards N = 2 SUSY homogeneous quantum cosmology; Einstein-Yang-Mills systems

    International Nuclear Information System (INIS)

    Donets, E.E.; Tentyukov, M.N.; Tsulaya, M.M.

    1998-01-01

    The application of N = 2 supersymmetric Quantum Mechanics for the quantization of homogeneous systems coupled with gravity is discussed. Starting with the superfield formulation of N = 2 SUSY sigma-model, Hermitian self-adjoint expressions for quantum Hamiltonians and Lagrangians for any signature of a sigma-model metric are obtained. This approach is then applied to coupled SU (2) Einstein-Yang-Mills (EYM) systems in axially-symmetric Bianchi - I,II,VIII, IX, Kantowski-Sachs and closed Friedmann-Robertson-Walker cosmological models. It is shown that all these models admit the embedding into N = 2 SUSY sigma-model with the explicit expressions for superpotentials, being direct sums of gravitational and Yang-Mills (YM) parts. In addition, YM parts of superpotentials exactly coincide with the corresponding Chern-Simons terms. The spontaneous SUSY breaking, caused by YM instantons in EYM systems is discussed in a number of examples

  9. Metric adjusted skew information

    DEFF Research Database (Denmark)

    Hansen, Frank

    2008-01-01

    ) that vanishes for observables commuting with the state. We show that the skew information is a convex function on the manifold of states. It also satisfies other requirements, proposed by Wigner and Yanase, for an effective measure-of-information content of a state relative to a conserved observable. We...... establish a connection between the geometrical formulation of quantum statistics as proposed by Chentsov and Morozova and measures of quantum information as introduced by Wigner and Yanase and extended in this article. We show that the set of normalized Morozova-Chentsov functions describing the possible......We extend the concept of Wigner-Yanase-Dyson skew information to something we call "metric adjusted skew information" (of a state with respect to a conserved observable). This "skew information" is intended to be a non-negative quantity bounded by the variance (of an observable in a state...

  10. Serial MR imaging and 1H-MR spectroscopy in monozygotic twins with Tay-Sachs disease.

    Science.gov (United States)

    Imamura, A; Miyajima, H; Ito, R; Orii, K O

    2008-10-01

    Four-year-old monozygotic female twins with early onset Tay-Sachs disease are described. The sisters showed similar slowly progressive clinical symptoms and deterioration, however the younger sister also demonstrated intractable myoclonus in the right leg. The serial MR images and (1)H-MR spectroscopy of the brain were obtained in both twins. MR images showed high intensity on T (2)-weighted image in the bilateral white matter, however there were no signal changes in the basal ganglia and thalamus during any of the phases. The ratio of N-acetylaspartate (NAA)/creatine (Cr) was decreased in the both white matter lesions and the corpus striatum, and that of myoinositol (mI)/Cr was increased in the damaged white matter on MR spectroscopy. The elevation of the lactate peak was clearly demonstrated in the left basal ganglia of the younger sister; however it was not shown in cerebral lesions of the elder sister. Changes in metabolites on MR spectroscopy were closely linked to the respective clinical features of each twin. Follow-up examination by (1)H-MR spectroscopy is useful for the evaluation of neuronal changes in children with Tay-Sachs disease.

  11. Haematopoietic Stem Cell Transplantation Arrests the Progression of Neurodegenerative Disease in Late-Onset Tay-Sachs Disease.

    Science.gov (United States)

    Stepien, Karolina M; Lum, Su Han; Wraith, J Edmond; Hendriksz, Christian J; Church, Heather J; Priestman, David; Platt, Frances M; Jones, Simon; Jovanovic, Ana; Wynn, Robert

    2017-12-07

    Tay-Sachs disease is a rare metabolic disease caused by a deficiency of hexosaminidase A that leads to accumulation of GM2 gangliosides predominantly in neural tissue. Late-onset Tay-Sachs disease variant is associated with a higher level of residual HexA activity. Treatment options are limited, and there are a few described cases who have undergone haematopoietic stem cell transplantation (HSCT) with variable outcome.We describe a case of a 23-year-old male patient who presented with a long-standing tremor since 7 years of age. He had gait ataxia, a speech stammer and swallowing problems. His condition had had a static course apart from his tremor that had been gradually deteriorating. Because of the deterioration in his neurological function, the patient had an uneventful, matched-sibling donor bone marrow transplant at the age of 15 years. Eight years post-HSCT, at the age of 23, he retains full donor engraftment, and his white cell beta-HexA of 191 nmol/mg/h is comparable to normal controls (in-assay control = 187). He continues to experience some intentional tremor that is tolerable for daily life and nonprogressive since HSCT. HSCT is a potential treatment option which might arrest neurodegeneration in patients with LOTS.

  12. Crystal Structure of Human [Beta]-Hexosaminidase B: Understanding the Molecular Basis of Sandhoff and Tay-Sachs Disease

    Energy Technology Data Exchange (ETDEWEB)

    Mark, Brian L.; Mahuran, Don J.; Cherney, Maia M.; Zhao, Dalian; Knapp, Spencer; James, Michael N.G.

    2010-12-01

    In humans, two major {beta}-hexosaminidase isoenzymes exist: Hex A and Hex B. Hex A is a heterodimer of subunits {alpha} and {beta} (60% identity), whereas Hex B is a homodimer of {beta}-subunits. Interest in human {beta}-hexosaminidase stems from its association with Tay-Sachs and Sandhoff disease; these are prototypical lysosomal storage disorders resulting from the abnormal accumulation of G{sub M2}-ganglioside (G{sub M2}). Hex A degrades G{sub M2} by removing a terminal N-acetyl-D-galactosamine ({beta}-GalNAc) residue, and this activity requires the G{sub M2}-activator, a protein which solubilizes the ganglioside for presentation to Hex A. We present here the crystal structure of human Hex B, alone (2.4 {angstrom}) and in complex with the mechanistic inhibitors GalNAc-isofagomine (2.2 {angstrom}) or NAG-thiazoline (2.5 {angstrom}). From these, and the known X-ray structure of the G{sub M2}-activator, we have modeled Hex A in complex with the activator and ganglioside. Together, our crystallographic and modeling data demonstrate how {alpha} and {beta}-subunits dimerize to form either Hex A or Hex B, how these isoenzymes hydrolyze diverse substrates, and how many documented point mutations cause Sandhoff disease ({beta}-subunit mutations) and Tay-Sachs disease ({alpha}-subunit mutations).

  13. Influence of defect size and localization on the engagement of reverse Hill-Sachs lesions.

    Science.gov (United States)

    Moroder, Philipp; Runer, Armin; Kraemer, Manuel; Fierlbeck, Johann; Niederberger, Alfred; Cotofana, Sebastian; Vasvari, Imre; Hettegger, Bernhard; Tauber, Mark; Hurschler, Christof; Resch, Herbert

    2015-03-01

    Reverse Hill-Sachs (RHS) lesions can cause recurrent posterior shoulder instability because of engagement with the posterior glenoid rim; however, the effect of defect size and localization have yet to be determined. Both size and localization are critical for the engagement of an RHS defect with the posterior glenoid rim. Controlled laboratory study. Ten RHS defects with predefined extent and localization were created through an anterolateral rotator cuff sparing approach in 10 fresh-frozen cadaveric shoulder specimens using a custom-made saw guide. Computed tomography scans of all specimens were completed, and standardized measurements were performed to determine the size (alpha angle) and localization (beta angle) of the defect as well as a combination of both parameters (gamma angle). Internal rotation motions were imposed on the shoulder joint in different arm positions and with varying amount of posterior translation by means of a robot-assisted shoulder simulator. The association between engagement of the defects and the defined parameters (alpha, beta, and gamma angles) was analyzed. In 0° of abduction, a cutoff value between engaging and nonengaging defects of 37.5° for the alpha angle (100% sensitivity; 75% specificity; area under the curve [AUC], 0.875; P = .055) and 36.5° for the beta angle (100% sensitivity; 25% specificity; AUC, 0.708; P = .286) was determined. The gamma angle showed the highest discriminatory power (AUC, 0.938; P = .025) with a cutoff value of 85.5° rendering 100% sensitivity and 75% specificity in the prediction of engagement. An increase in the applied posterior translation force decreased the degrees of internal rotation necessary before engagement occurred. No engagement occurred during internal rotation with the arm in 60° of abduction or 60° of flexion. The size and localization of RHS defects are both critical factors for engagement. The combination of both parameters in terms of the gamma angle measurement might be a

  14. The metric system: An introduction

    Energy Technology Data Exchange (ETDEWEB)

    Lumley, S.M.

    1995-05-01

    On July 13, 1992, Deputy Director Duane Sewell restated the Laboratory`s policy on conversion to the metric system which was established in 1974. Sewell`s memo announced the Laboratory`s intention to continue metric conversion on a reasonable and cost effective basis. Copies of the 1974 and 1992 Administrative Memos are contained in the Appendix. There are three primary reasons behind the Laboratory`s conversion to the metric system. First, Public Law 100-418, passed in 1988, states that by the end of fiscal year 1992 the Federal Government must begin using metric units in grants, procurements, and other business transactions. Second, on July 25, 1991, President George Bush signed Executive Order 12770 which urged Federal agencies to expedite conversion to metric units. Third, the contract between the University of California and the Department of Energy calls for the Laboratory to convert to the metric system. Thus, conversion to the metric system is a legal requirement and a contractual mandate with the University of California. Public Law 100-418 and Executive Order 12770 are discussed in more detail later in this section, but first they examine the reasons behind the nation`s conversion to the metric system. The second part of this report is on applying the metric system.

  15. Attack-Resistant Trust Metrics

    Science.gov (United States)

    Levien, Raph

    The Internet is an amazingly powerful tool for connecting people together, unmatched in human history. Yet, with that power comes great potential for spam and abuse. Trust metrics are an attempt to compute the set of which people are trustworthy and which are likely attackers. This chapter presents two specific trust metrics developed and deployed on the Advogato Website, which is a community blog for free software developers. This real-world experience demonstrates that the trust metrics fulfilled their goals, but that for good results, it is important to match the assumptions of the abstract trust metric computation to the real-world implementation.

  16. The metric system: An introduction

    Science.gov (United States)

    Lumley, Susan M.

    On 13 Jul. 1992, Deputy Director Duane Sewell restated the Laboratory's policy on conversion to the metric system which was established in 1974. Sewell's memo announced the Laboratory's intention to continue metric conversion on a reasonable and cost effective basis. Copies of the 1974 and 1992 Administrative Memos are contained in the Appendix. There are three primary reasons behind the Laboratory's conversion to the metric system. First, Public Law 100-418, passed in 1988, states that by the end of fiscal year 1992 the Federal Government must begin using metric units in grants, procurements, and other business transactions. Second, on 25 Jul. 1991, President George Bush signed Executive Order 12770 which urged Federal agencies to expedite conversion to metric units. Third, the contract between the University of California and the Department of Energy calls for the Laboratory to convert to the metric system. Thus, conversion to the metric system is a legal requirement and a contractual mandate with the University of California. Public Law 100-418 and Executive Order 12770 are discussed in more detail later in this section, but first they examine the reasons behind the nation's conversion to the metric system. The second part of this report is on applying the metric system.

  17. Metric-adjusted skew information

    DEFF Research Database (Denmark)

    Liang, Cai; Hansen, Frank

    2010-01-01

    on a bipartite system and proved superadditivity of the Wigner-Yanase-Dyson skew informations for such states. We extend this result to the general metric-adjusted skew information. We finally show that a recently introduced extension to parameter values 1 ...We give a truly elementary proof of the convexity of metric-adjusted skew information following an idea of Effros. We extend earlier results of weak forms of superadditivity to general metric-adjusted skew information. Recently, Luo and Zhang introduced the notion of semi-quantum states...... of (unbounded) metric-adjusted skew information....

  18. Two classes of metric spaces

    Directory of Open Access Journals (Sweden)

    Isabel Garrido

    2016-04-01

    Full Text Available The class of metric spaces (X,d known as small-determined spaces, introduced by Garrido and Jaramillo, are properly defined by means of some type of real-valued Lipschitz functions on X. On the other hand, B-simple metric spaces introduced by Hejcman are defined in terms of some kind of bornologies of bounded subsets of X. In this note we present a common framework where both classes of metric spaces can be studied which allows us to see not only the relationships between them but also to obtain new internal characterizations of these metric properties.

  19. Software metrics: Software quality metrics for distributed systems. [reliability engineering

    Science.gov (United States)

    Post, J. V.

    1981-01-01

    Software quality metrics was extended to cover distributed computer systems. Emphasis is placed on studying embedded computer systems and on viewing them within a system life cycle. The hierarchy of quality factors, criteria, and metrics was maintained. New software quality factors were added, including survivability, expandability, and evolvability.

  20. Multimedia messages in genetics: design, development, and evaluation of a computer-based instructional resource for secondary school students in a Tay Sachs disease carrier screening program.

    Science.gov (United States)

    Gason, Alexandra A; Aitken, MaryAnne; Delatycki, Martin B; Sheffield, Edith; Metcalfe, Sylvia A

    2004-01-01

    Tay Sachs disease is a recessively inherited neurodegenerative disorder, for which carrier screening programs exist worldwide. Education for those offered a screening test is essential in facilitating informed decision-making. In Melbourne, Australia, we have designed, developed, and evaluated a computer-based instructional resource for use in the Tay Sachs disease carrier screening program for secondary school students attending Jewish schools. The resource entitled "Genetics in the Community: Tay Sachs disease" was designed on a platform of educational learning theory. The development of the resource included formative evaluation using qualitative data analysis supported by descriptive quantitative data. The final resource was evaluated within the screening program and compared with the standard oral presentation using a questionnaire. Knowledge outcomes were measured both before and after either of the educational formats. Data from the formative evaluation were used to refine the content and functionality of the final resource. The questionnaire evaluation of 302 students over two years showed the multimedia resource to be equally effective as an oral educational presentation in facilitating participants' knowledge construction. The resource offers a large number of potential benefits, which are not limited to the Tay Sachs disease carrier screening program setting, such as delivery of a consistent educational message, short delivery time, and minimum financial and resource commitment. This article outlines the value of considering educational theory and describes the process of multimedia development providing a framework that may be of value when designing genetics multimedia resources in general.

  1. Multimetric indices: How many metrics?

    Science.gov (United States)

    Multimetric indices (MMI’s) often include 5 to 15 metrics, each representing a different attribute of assemblage condition, such as species diversity, tolerant taxa, and nonnative taxa. Is there an optimal number of metrics for MMIs? To explore this question, I created 1000 9-met...

  2. Metrical Phonology: German Sound System.

    Science.gov (United States)

    Tice, Bradley S.

    Metrical phonology, a linguistic process of phonological stress assessment and diagrammatic simplification of sentence and word stress, is discussed as it is found in the English and German languages. The objective is to promote use of metrical phonology as a tool for enhancing instruction in stress patterns in words and sentences, particularly in…

  3. Extending cosmology: the metric approach

    OpenAIRE

    Mendoza, S.

    2012-01-01

    Comment: 2012, Extending Cosmology: The Metric Approach, Open Questions in Cosmology; Review article for an Intech "Open questions in cosmology" book chapter (19 pages, 3 figures). Available from: http://www.intechopen.com/books/open-questions-in-cosmology/extending-cosmology-the-metric-approach

  4. Numerical Calabi-Yau metrics

    International Nuclear Information System (INIS)

    Douglas, Michael R.; Karp, Robert L.; Lukic, Sergio; Reinbacher, Rene

    2008-01-01

    We develop numerical methods for approximating Ricci flat metrics on Calabi-Yau hypersurfaces in projective spaces. Our approach is based on finding balanced metrics and builds on recent theoretical work by Donaldson. We illustrate our methods in detail for a one parameter family of quintics. We also suggest several ways to extend our results

  5. High resolution metric imaging payload

    Science.gov (United States)

    Delclaud, Y.

    2017-11-01

    Alcatel Space Industries has become Europe's leader in the field of high and very high resolution optical payloads, in the frame work of earth observation system able to provide military government with metric images from space. This leadership allowed ALCATEL to propose for the export market, within a French collaboration frame, a complete space based system for metric observation.

  6. Weyl metrics and wormholes

    Energy Technology Data Exchange (ETDEWEB)

    Gibbons, Gary W. [DAMTP, University of Cambridge, Wilberforce Road, Cambridge, CB3 0WA U.K. (United Kingdom); Volkov, Mikhail S., E-mail: gwg1@cam.ac.uk, E-mail: volkov@lmpt.univ-tours.fr [Laboratoire de Mathématiques et Physique Théorique, LMPT CNRS—UMR 7350, Université de Tours, Parc de Grandmont, Tours, 37200 France (France)

    2017-05-01

    We study solutions obtained via applying dualities and complexifications to the vacuum Weyl metrics generated by massive rods and by point masses. Rescaling them and extending to complex parameter values yields axially symmetric vacuum solutions containing singularities along circles that can be viewed as singular matter sources. These solutions have wormhole topology with several asymptotic regions interconnected by throats and their sources can be viewed as thin rings of negative tension encircling the throats. For a particular value of the ring tension the geometry becomes exactly flat although the topology remains non-trivial, so that the rings literally produce holes in flat space. To create a single ring wormhole of one metre radius one needs a negative energy equivalent to the mass of Jupiter. Further duality transformations dress the rings with the scalar field, either conventional or phantom. This gives rise to large classes of static, axially symmetric solutions, presumably including all previously known solutions for a gravity-coupled massless scalar field, as for example the spherically symmetric Bronnikov-Ellis wormholes with phantom scalar. The multi-wormholes contain infinite struts everywhere at the symmetry axes, apart from solutions with locally flat geometry.

  7. Metrics for image segmentation

    Science.gov (United States)

    Rees, Gareth; Greenway, Phil; Morray, Denise

    1998-07-01

    An important challenge in mapping image-processing techniques onto applications is the lack of quantitative performance measures. From a systems engineering perspective these are essential if system level requirements are to be decomposed into sub-system requirements which can be understood in terms of algorithm selection and performance optimization. Nowhere in computer vision is this more evident than in the area of image segmentation. This is a vigorous and innovative research activity, but even after nearly two decades of progress, it remains almost impossible to answer the question 'what would the performance of this segmentation algorithm be under these new conditions?' To begin to address this shortcoming, we have devised a well-principled metric for assessing the relative performance of two segmentation algorithms. This allows meaningful objective comparisons to be made between their outputs. It also estimates the absolute performance of an algorithm given ground truth. Our approach is an information theoretic one. In this paper, we describe the theory and motivation of our method, and present practical results obtained from a range of state of the art segmentation methods. We demonstrate that it is possible to measure the objective performance of these algorithms, and to use the information so gained to provide clues about how their performance might be improved.

  8. Metric regularity and subdifferential calculus

    International Nuclear Information System (INIS)

    Ioffe, A D

    2000-01-01

    The theory of metric regularity is an extension of two classical results: the Lyusternik tangent space theorem and the Graves surjection theorem. Developments in non-smooth analysis in the 1980s and 1990s paved the way for a number of far-reaching extensions of these results. It was also well understood that the phenomena behind the results are of metric origin, not connected with any linear structure. At the same time it became clear that some basic hypotheses of the subdifferential calculus are closely connected with the metric regularity of certain set-valued maps. The survey is devoted to the metric theory of metric regularity and its connection with subdifferential calculus in Banach spaces

  9. Late onset Tay-Sachs disease in mice with targeted disruption of the Hexa gene: behavioral changes and pathology of the central nervous system.

    Science.gov (United States)

    Miklyaeva, Elena I; Dong, Weijia; Bureau, Alexandre; Fattahie, Roya; Xu, Yongqin; Su, Meng; Fick, Gordon H; Huang, Jing-Qi; Igdoura, Suleiman; Hanai, Nobuo; Gravel, Roy A

    2004-03-19

    Tay-Sachs disease is an autosomal recessive neurodegenerative disease resulting from a block in the hydrolysis of GM2 ganglioside, an intermediate in ganglioside catabolism. The mouse model of Tay-Sachs disease (Hexa -/-) has been described as behaviorally indistinguishable from wild type until at least 1 year of age due to a sialidase-mediated bypass of the metabolic defect that reduces the rate of GM2 ganglioside accumulation. In this study, we have followed our mouse model to over 2 years of age and have documented a significant disease phenotype that is reminiscent of the late onset, chronic form of human Tay-Sachs disease. Onset occurs at 11-12 months of age and progresses slowly, in parallel with increasing storage of GM2 ganglioside. The disease is characterized by hind limb spasticity, weight loss, tremors, abnormal posture with lordosis, possible visual impairment, and, late in the disease, muscle weakness, clasping of the limbs, and myoclonic twitches of the head. Immunodetection of GM2 ganglioside showed that storage varies widely in different regions, but is most intense in pyriform cortex, hippocampus (CA3 field, subiculum), amygdala, hypothalamus (paraventricular supraoptic, ventromedial and arcuate nuclei, and mammilary body), and the somatosensory cortex (layer V) in 1- to 2-year-old mutant mice. We suggest that the Tay-Sachs mouse model is a phenotypically valid model of disease and may provide for a reliable indicator of the impact of therapeutic strategies, in particular geared to the late onset, chronic form of human Tay-Sachs disease.

  10. Splice junction mutation in some Ashkenazi Jews with Tay-Sachs disease: Evidence against a single defect within this ethnic group

    Energy Technology Data Exchange (ETDEWEB)

    Myerowitz, R. (National Institute of Diabetes, Digestive and Kidney Diseases, Bethesda, MD (USA))

    1988-06-01

    Tay-Sachs disease is an inherited disorder in which the {alpha} chain of the lysosomal enzyme {beta}-N-acetylhexosaminidase A bears the mutation. Ashkenazi Jews are found to be carriers for a severe type of Tay-Sachs disease, the classic form, 10 times more frequently than the general population. Ashkenazi Jewish patients with classic Tay-Sachs disease have appeared to be clinically and biochemically identical, and the usual assumption has been that they harbor the same {alpha}-chain mutation. The author has isolated the {alpha}-chain gene from an Ashkenazi Jewish patient, GM2968, with classic Tay-Sachs disease and compared its nucleotide sequences with that of the normal {alpha}-chain gene in the promoter region, exon and splice junction regions, and polyadenylylation signal area. Only one difference was observed between these sequences. The alteration is presumed to be functionally significant and to result in aberrant mRNA splicing. Utilizing the polymerase chain reaction to amplify the region encompassing the mutation, the author developed an assay to screen patients and heterozygote carriers for this mutation. Surprisingly, in each of two Ashkenazi patients, only one {alpha}-chain allele harbored the splice junction mutation. Only one parent of each of these patients was positive for the defect. Another Ashkenazi patient did not bear this mutation at all nor did either of the subject's parents. The data are consistent with the presence of more than one mutation underlying the classic form of Tay-Sachs disease in the Ashkenazi Jewish population.

  11. Splice junction mutation in some Ashkenazi Jews with Tay-Sachs disease: Evidence against a single defect within this ethnic group

    International Nuclear Information System (INIS)

    Myerowitz, R.

    1988-01-01

    Tay-Sachs disease is an inherited disorder in which the α chain of the lysosomal enzyme β-N-acetylhexosaminidase A bears the mutation. Ashkenazi Jews are found to be carriers for a severe type of Tay-Sachs disease, the classic form, 10 times more frequently than the general population. Ashkenazi Jewish patients with classic Tay-Sachs disease have appeared to be clinically and biochemically identical, and the usual assumption has been that they harbor the same α-chain mutation. The author has isolated the α-chain gene from an Ashkenazi Jewish patient, GM2968, with classic Tay-Sachs disease and compared its nucleotide sequences with that of the normal α-chain gene in the promoter region, exon and splice junction regions, and polyadenylylation signal area. Only one difference was observed between these sequences. The alteration is presumed to be functionally significant and to result in aberrant mRNA splicing. Utilizing the polymerase chain reaction to amplify the region encompassing the mutation, the author developed an assay to screen patients and heterozygote carriers for this mutation. Surprisingly, in each of two Ashkenazi patients, only one α-chain allele harbored the splice junction mutation. Only one parent of each of these patients was positive for the defect. Another Ashkenazi patient did not bear this mutation at all nor did either of the subject's parents. The data are consistent with the presence of more than one mutation underlying the classic form of Tay-Sachs disease in the Ashkenazi Jewish population

  12. Impact of gene patents and licensing practices on access to genetic testing and carrier screening for Tay-Sachs and Canavan disease.

    Science.gov (United States)

    Colaianni, Alessandra; Chandrasekharan, Subhashini; Cook-Deegan, Robert

    2010-04-01

    Genetic testing for Tay-Sachs and Canavan disease is particularly important for Ashkenazi Jews, because both conditions are more frequent in that population. This comparative case study was possible because of different patenting and licensing practices. The role of DNA testing differs between Tay-Sachs and Canavan diseases. The first-line screening test for Tay-Sachs remains an enzyme activity test rather than genotyping. Genotyping is used for preimplantation diagnosis and confirmatory testing. In contrast, DNA-based testing is the basis for Canavan screening and diagnosis. The HEXA gene for Tay-Sachs was cloned at the National Institutes of Health, and the gene was patented but has not been licensed. The ASPA gene for Canavan disease was cloned and patented by Miami Children's Hospital. Miami Children's Hospital did not inform family members and patient groups that had contributed to the gene discovery that it was applying for a patent, and pursued restrictive licensing practices when a patent issued in 1997. This led to intense controversy, litigation, and a sealed, nonpublic 2003 settlement that apparently allowed for nonexclusive licensing. A survey of laboratories revealed a possible price premium for ASPA testing, with per-unit costs higher than for other genetic tests in the Secretary's Advisory Committee on Genetics, Health, and Society case studies. The main conclusion from comparing genetic testing for Tay-Sachs and Canavan diseases, however, is that patenting and licensing conducted without communication with patients and advocates cause mistrust and can lead to controversy and litigation, a negative model to contrast with the positive model of patenting and licensing for genetic testing of cystic fibrosis.

  13. On extracting physical content from asymptotically flat spacetime metrics

    International Nuclear Information System (INIS)

    Kozameh, C; Newman, E T; Silva-Ortigoza, G

    2008-01-01

    A major issue in general relativity, from its earliest days to the present, is how to extract physical information from any solution or class of solutions to the Einstein equations. Though certain information can be obtained for arbitrary solutions, e.g., via geodesic deviation, in general, because of the coordinate freedom, it is often hard or impossible to do. Most of the time information is found from special conditions, e.g. degenerate principle null vectors, weak fields close to Minkowski space (using coordinates close to Minkowski coordinates), or from solutions that have symmetries or approximate symmetries. In the present work, we will be concerned with asymptotically flat spacetimes where the approximate symmetry is the Bondi-Metzner-Sachs group. For these spaces the Bondi 4-momentum vector and its evolution, found from the Weyl tensor at infinity, describes the total energy-momentum of the interior source and the energy-momentum radiated. By generalizing the structures (shear-free null geodesic congruences) associated with the algebraically special metrics to asymptotically shear-free null geodesic congruences, which are available in all asymptotically flat spacetimes, we give kinematic meaning to the Bondi 4-momentum. In other words, we describe the Bondi vector and its evolution in terms of a center of mass position vector, its velocity and a spin vector, all having clear geometric meaning. Among other items, from dynamic arguments, we define a unique (at our level of approximation) total angular momentum and extract its evolution equation in the form of a conservation law with an angular momentum flux

  14. METRICS DEVELOPMENT FOR PATENTS.

    Science.gov (United States)

    Veiga, Daniela Francescato; Ferreira, Lydia Masako

    2015-01-01

    To develop a proposal for metrics for patents to be applied in assessing the postgraduate programs of Medicine III - Capes. From the reading and analysis of the 2013 area documents of all the 48 areas of Capes, a proposal for metrics for patents was developed to be applied in Medicine III programs. Except for the areas Biotechnology, Food Science, Biological Sciences III, Physical Education, Engineering I, III and IV and Interdisciplinary, most areas do not adopt a scoring system for patents. The proposal developed was based on the criteria of Biotechnology, with adaptations. In general, it will be valued, in ascending order, the deposit, the granting and licensing/production. It will also be assigned higher scores to patents registered abroad and whenever there is a participation of students. This proposal can be applied to the item Intellectual Production of the evaluation form, in subsection Technical Production/Patents. The percentage of 10% for academic programs and 40% for Masters Professionals should be maintained. The program will be scored as Very Good when it reaches 400 points or over; Good, between 200 and 399 points; Regular, between 71 and 199 points; Weak up to 70 points; Insufficient, no punctuation. Desenvolver uma proposta de métricas para patentes a serem aplicadas na avaliação dos Programas de Pós-Graduação da Área Medicina III - Capes. A partir da leitura e análise dos documentos de área de 2013 de todas as 48 Áreas da Capes, desenvolveu-se uma proposta de métricas para patentes, a ser aplicada na avaliação dos programas da área. Constatou-se que, com exceção das áreas Biotecnologia, Ciência de Alimentos, Ciências Biológicas III, Educação Física, Engenharias I, III e IV e Interdisciplinar, a maioria não adota sistema de pontuação para patentes. A proposta desenvolvida baseou-se nos critérios da Biotecnologia, com adaptações. De uma forma geral, foi valorizado, em ordem crescente, o depósito, a concessão e o

  15. A Metric for Heterotic Moduli

    Science.gov (United States)

    Candelas, Philip; de la Ossa, Xenia; McOrist, Jock

    2017-12-01

    Heterotic vacua of string theory are realised, at large radius, by a compact threefold with vanishing first Chern class together with a choice of stable holomorphic vector bundle. These form a wide class of potentially realistic four-dimensional vacua of string theory. Despite all their phenomenological promise, there is little understanding of the metric on the moduli space of these. What is sought is the analogue of special geometry for these vacua. The metric on the moduli space is important in phenomenology as it normalises D-terms and Yukawa couplings. It is also of interest in mathematics, since it generalises the metric, first found by Kobayashi, on the space of gauge field connections, to a more general context. Here we construct this metric, correct to first order in {α^{\\backprime}}, in two ways: first by postulating a metric that is invariant under background gauge transformations of the gauge field, and also by dimensionally reducing heterotic supergravity. These methods agree and the resulting metric is Kähler, as is required by supersymmetry. Checking the metric is Kähler is intricate and the anomaly cancellation equation for the H field plays an essential role. The Kähler potential nevertheless takes a remarkably simple form: it is the Kähler potential of special geometry with the Kähler form replaced by the {α^{\\backprime}}-corrected hermitian form.

  16. Implications of Metric Choice for Common Applications of Readmission Metrics

    OpenAIRE

    Davies, Sheryl; Saynina, Olga; Schultz, Ellen; McDonald, Kathryn M; Baker, Laurence C

    2013-01-01

    Objective. To quantify the differential impact on hospital performance of three readmission metrics: all-cause readmission (ACR), 3M Potential Preventable Readmission (PPR), and Centers for Medicare and Medicaid 30-day readmission (CMS).

  17. Generation of HEXA-deficient hiPSCs from fibroblasts of a Tay-Sachs disease patient.

    Science.gov (United States)

    Liu, Zhong; Zhao, Rui

    2016-09-01

    Human iPSC line TSD-01-hiPSC was generated from fibroblasts of a patient with infantile Tay-Sachs disease (TSD). The patient is compound heterozygous at the HEXA gene by carrying a 1278insTATC allele and an IVS12+1G>C allele. STEMCCA lentivirus, which expresses OCT4, SOX2, KLF4, and c-MYC from a polycistronic transcript, were used for reprogramming. TSD-01-hiPSC express pluripotency markers such as OCT4, SOX2, NANOG, Tra-1-60, and alkaline phosphatase, and can differentiate into tissues from all the three embryonic germ layers. This TSD patient-derived hiPSC line may serve as a valuable in vitro tool for disease modeling and drug test. Copyright © 2016 The Authors. Published by Elsevier B.V. All rights reserved.

  18. Generation of HEXA-deficient hiPSCs from fibroblasts of a Tay-Sachs disease patient

    Directory of Open Access Journals (Sweden)

    Zhong Liu

    2016-09-01

    Full Text Available Human iPSC line TSD-01-hiPSC was generated from fibroblasts of a patient with infantile Tay-Sachs disease (TSD. The patient is compound heterozygous at the HEXA gene by carrying a 1278insTATC allele and an IVS12+1G>C allele. STEMCCA lentivirus, which expresses OCT4, SOX2, KLF4, and c-MYC from a polycistronic transcript, were used for reprogramming. TSD-01-hiPSC express pluripotency markers such as OCT4, SOX2, NANOG, Tra-1-60, and alkaline phosphatase, and can differentiate into tissues from all the three embryonic germ layers. This TSD patient-derived hiPSC line may serve as a valuable in vitro tool for disease modeling and drug test.

  19. Issues in Benchmark Metric Selection

    Science.gov (United States)

    Crolotte, Alain

    It is true that a metric can influence a benchmark but will esoteric metrics create more problems than they will solve? We answer this question affirmatively by examining the case of the TPC-D metric which used the much debated geometric mean for the single-stream test. We will show how a simple choice influenced the benchmark and its conduct and, to some extent, DBMS development. After examining other alternatives our conclusion is that the “real” measure for a decision-support benchmark is the arithmetic mean.

  20. Background metric in supergravity theories

    International Nuclear Information System (INIS)

    Yoneya, T.

    1978-01-01

    In supergravity theories, we investigate the conformal anomaly of the path-integral determinant and the problem of fermion zero modes in the presence of a nontrivial background metric. Except in SO(3) -invariant supergravity, there are nonvanishing conformal anomalies. As a consequence, amplitudes around the nontrivial background metric contain unpredictable arbitrariness. The fermion zero modes which are explicitly constructed for the Euclidean Schwarzschild metric are interpreted as an indication of the supersymmetric multiplet structure of a black hole. The degree of degeneracy of a black hole is 2/sup 4n/ in SO(n) supergravity

  1. Generalized Painleve-Gullstrand metrics

    Energy Technology Data Exchange (ETDEWEB)

    Lin Chunyu [Department of Physics, National Cheng Kung University, Tainan 70101, Taiwan (China)], E-mail: l2891112@mail.ncku.edu.tw; Soo Chopin [Department of Physics, National Cheng Kung University, Tainan 70101, Taiwan (China)], E-mail: cpsoo@mail.ncku.edu.tw

    2009-02-02

    An obstruction to the implementation of spatially flat Painleve-Gullstrand (PG) slicings is demonstrated, and explicitly discussed for Reissner-Nordstroem and Schwarzschild-anti-deSitter spacetimes. Generalizations of PG slicings which are not spatially flat but which remain regular at the horizons are introduced. These metrics can be obtained from standard spherically symmetric metrics by physical Lorentz boosts. With these generalized PG metrics, problematic contributions to the imaginary part of the action in the Parikh-Wilczek derivation of Hawking radiation due to the obstruction can be avoided.

  2. Daylight metrics and energy savings

    Energy Technology Data Exchange (ETDEWEB)

    Mardaljevic, John; Heschong, Lisa; Lee, Eleanor

    2009-12-31

    The drive towards sustainable, low-energy buildings has increased the need for simple, yet accurate methods to evaluate whether a daylit building meets minimum standards for energy and human comfort performance. Current metrics do not account for the temporal and spatial aspects of daylight, nor of occupants comfort or interventions. This paper reviews the historical basis of current compliance methods for achieving daylit buildings, proposes a technical basis for development of better metrics, and provides two case study examples to stimulate dialogue on how metrics can be applied in a practical, real-world context.

  3. Murine Sialidase Neu3 facilitates GM2 degradation and bypass in mouse model of Tay-Sachs disease.

    Science.gov (United States)

    Seyrantepe, Volkan; Demir, Secil Akyildiz; Timur, Zehra Kevser; Von Gerichten, Johanna; Marsching, Christian; Erdemli, Esra; Oztas, Emin; Takahashi, Kohta; Yamaguchi, Kazunori; Ates, Nurselin; Dönmez Demir, Buket; Dalkara, Turgay; Erich, Katrin; Hopf, Carsten; Sandhoff, Roger; Miyagi, Taeko

    2018-01-01

    Tay-Sachs disease is a severe lysosomal storage disorder caused by mutations in Hexa, the gene that encodes for the α subunit of lysosomal β-hexosaminidase A (HEXA), which converts GM2 to GM3 ganglioside. Unexpectedly, Hexa -/- mice have a normal lifespan and show no obvious neurological impairment until at least one year of age. These mice catabolize stored GM2 ganglioside using sialidase(s) to remove sialic acid and form the glycolipid GA2, which is further processed by β-hexosaminidase B. Therefore, the presence of the sialidase (s) allows the consequences of the Hexa defect to be bypassed. To determine if the sialidase NEU3 contributes to GM2 ganglioside degradation, we generated a mouse model with combined deficiencies of HEXA and NEU3. The Hexa -/- Neu3 -/- mice were healthy at birth, but died at 1.5 to 4.5months of age. Thin-layer chromatography and mass spectrometric analysis of the brains of Hexa -/- Neu3 -/- mice revealed the abnormal accumulation of GM2 ganglioside. Histological and immunohistochemical analysis demonstrated cytoplasmic vacuolation in the neurons. Electron microscopic examination of the brain, kidneys and testes revealed pleomorphic inclusions of many small vesicles and complex lamellar structures. The Hexa -/- Neu3 -/- mice exhibited progressive neurodegeneration with neuronal loss, Purkinje cell depletion, and astrogliosis. Slow movement, ataxia, and tremors were the prominent neurological abnormalities observed in these mice. Furthermore, radiographs revealed abnormalities in the skeletal bones of the Hexa -/- Neu3 -/- mice. Thus, the Hexa -/- Neu3 -/- mice mimic the neuropathological and clinical abnormalities of the classical early-onset Tay-Sachs patients, and provide a suitable model for the future pre-clinical testing of potential treatments for this condition. Copyright © 2017 Elsevier Inc. All rights reserved.

  4. Next-Generation Metrics: Responsible Metrics & Evaluation for Open Science

    Energy Technology Data Exchange (ETDEWEB)

    Wilsdon, J.; Bar-Ilan, J.; Peters, I.; Wouters, P.

    2016-07-01

    Metrics evoke a mixed reaction from the research community. A commitment to using data to inform decisions makes some enthusiastic about the prospect of granular, real-time analysis o of research and its wider impacts. Yet we only have to look at the blunt use of metrics such as journal impact factors, h-indices and grant income targets, to be reminded of the pitfalls. Some of the most precious qualities of academic culture resist simple quantification, and individual indicators often struggle to do justice to the richness and plurality of research. Too often, poorly designed evaluation criteria are “dominating minds, distorting behaviour and determining careers (Lawrence, 2007).” Metrics hold real power: they are constitutive of values, identities and livelihoods. How to exercise that power to more positive ends has been the focus of several recent and complementary initiatives, including the San Francisco Declaration on Research Assessment (DORA1), the Leiden Manifesto2 and The Metric Tide3 (a UK government review of the role of metrics in research management and assessment). Building on these initiatives, the European Commission, under its new Open Science Policy Platform4, is now looking to develop a framework for responsible metrics for research management and evaluation, which can be incorporated into the successor framework to Horizon 2020. (Author)

  5. Let's Make Metric Ice Cream

    Science.gov (United States)

    Zimmerman, Marianna

    1975-01-01

    Describes a classroom activity which involved sixth grade students in a learning situation including making ice cream, safety procedures in a science laboratory, calibrating a thermometer, using metric units of volume and mass. (EB)

  6. Experiential space is hardly metric

    Czech Academy of Sciences Publication Activity Database

    Šikl, Radovan; Šimeček, Michal; Lukavský, Jiří

    2008-01-01

    Roč. 2008, č. 37 (2008), s. 58-58 ISSN 0301-0066. [European Conference on Visual Perception. 24.08-28.08.2008, Utrecht] R&D Projects: GA ČR GA406/07/1676 Institutional research plan: CEZ:AV0Z70250504 Keywords : visual space perception * metric and non-metric perceptual judgments * ecological validity Subject RIV: AN - Psychology

  7. Coverage Metrics for Model Checking

    Science.gov (United States)

    Penix, John; Visser, Willem; Norvig, Peter (Technical Monitor)

    2001-01-01

    When using model checking to verify programs in practice, it is not usually possible to achieve complete coverage of the system. In this position paper we describe ongoing research within the Automated Software Engineering group at NASA Ames on the use of test coverage metrics to measure partial coverage and provide heuristic guidance for program model checking. We are specifically interested in applying and developing coverage metrics for concurrent programs that might be used to support certification of next generation avionics software.

  8. Phantom metrics with Killing spinors

    Directory of Open Access Journals (Sweden)

    W.A. Sabra

    2015-11-01

    Full Text Available We study metric solutions of Einstein–anti-Maxwell theory admitting Killing spinors. The analogue of the IWP metric which admits a space-like Killing vector is found and is expressed in terms of a complex function satisfying the wave equation in flat (2+1-dimensional space–time. As examples, electric and magnetic Kasner spaces are constructed by allowing the solution to depend only on the time coordinate. Euclidean solutions are also presented.

  9. Scalar-metric and scalar-metric-torsion gravitational theories

    International Nuclear Information System (INIS)

    Aldersley, S.J.

    1977-01-01

    The techniques of dimensional analysis and of the theory of tensorial concomitants are employed to study field equations in gravitational theories which incorporate scalar fields of the Brans-Dicke type. Within the context of scalar-metric gravitational theories, a uniqueness theorem for the geometric (or gravitational) part of the field equations is proven and a Lagrangian is determined which is uniquely specified by dimensional analysis. Within the context of scalar-metric-torsion gravitational theories a uniqueness theorem for field Lagrangians is presented and the corresponding Euler-Lagrange equations are given. Finally, an example of a scalar-metric-torsion theory is presented which is similar in many respects to the Brans-Dicke theory and the Einstein-Cartan theory

  10. Molecular characterization of a novel HEXA mutation at the +3 position of intron 8 in a Tay-Sachs disease patient

    Energy Technology Data Exchange (ETDEWEB)

    Richard, M.; Triggs-Raine, B. [Univ. of Manitoba, Winnipeg (Canada); Natowicz, M. [E.K. Shriver Center, Waltham, MA (United States)

    1994-09-01

    Tay-Sachs disease is an autosomal recessive lysosomal storage disorder resulting from mutations in the HEXA gene that cause a deficiency in the activity of that enzyme {beta}-hexosaminidase A (Hex A). This deficiency leads to the build-up of G{sub M2} ganglioside, resulting in neurodegeneration and death. Biochemical analysis of a non-Jewish patient with a late-infantile form of Tay-Sachs disease revealed a substantial level of Hex A activity (38.4%) when 4-MUG was used as the substrate. However, when a substrate (4-MUGS) specific for the {alpha}-subunit of Hex A ({alpha}{beta}) was used, almost no activity was detected in the HEXA gene of the patient using SSCP analysis followed by sequencing. The first mutation, a G533A substitution in exon 5, is previously described and associated with the B1 form of Tay-Sachs disease. The second mutation is a novel a-to-g base change at the +3 position of intron 8. This was confirmed using the AIRS method, whereby a MaeIII site was created in the presence of the mutation. Normal and patient mRNA was reverse transcribed and exons 7 to 9 were PCR-amplified from the cDNA. An abnormally sized amplification product detected only in the patient cDNA was sequenced; exon 8 had been deleted and exons 7 and 9 were spliced together. A substantial level of normally-sized PCR product was also detected in the patient`s cDNA. Experiments are in progress to determine if this is produced from the allele harboring the G533A mutation. Given that previous mutations of this type have been associated with 97-100% abnormal splicing, this mutation is likely to be the cause, together with the G533A mutation, of Tay-Sachs disease in this patient.

  11. Area density of localization-entropy II: double cone-localization and quantum origin of the Bondi-Metzner-Sachs symmetry

    Energy Technology Data Exchange (ETDEWEB)

    Schroer, Bert

    2006-04-15

    The holographic encoding is generalized to subalgebras of QFT localized in double cones. It is shown that as a result of this radically different spacetime encoding the modular group acts geometrically on the holographic image. As a result we obtain a formula for localization entropy which is identical to the previously derived formula for the wedge-localized subalgebra. The symmetry group in the holographic encoding turns out to be the Bondi-Metzner-Sachs group. (author)

  12. Regge calculus from discontinuous metrics

    International Nuclear Information System (INIS)

    Khatsymovsky, V.M.

    2003-01-01

    Regge calculus is considered as a particular case of the more general system where the linklengths of any two neighbouring 4-tetrahedra do not necessarily coincide on their common face. This system is treated as that one described by metric discontinuous on the faces. In the superspace of all discontinuous metrics the Regge calculus metrics form some hypersurface defined by continuity conditions. Quantum theory of the discontinuous metric system is assumed to be fixed somehow in the form of quantum measure on (the space of functionals on) the superspace. The problem of reducing this measure to the Regge hypersurface is addressed. The quantum Regge calculus measure is defined from a discontinuous metric measure by inserting the δ-function-like phase factor. The requirement that continuity conditions be imposed in a 'face-independent' way fixes this factor uniquely. The term 'face-independent' means that this factor depends only on the (hyper)plane spanned by the face, not on it's form and size. This requirement seems to be natural from the viewpoint of existence of the well-defined continuum limit maximally free of lattice artefacts

  13. Symmetries of Taub-NUT dual metrics

    International Nuclear Information System (INIS)

    Baleanu, D.; Codoban, S.

    1998-01-01

    Recently geometric duality was analyzed for a metric which admits Killing tensors. An interesting example arises when the manifold has Killing-Yano tensors. The symmetries of the dual metrics in the case of Taub-NUT metric are investigated. Generic and non-generic symmetries of dual Taub-NUT metric are analyzed

  14. A Kerr-NUT metric

    International Nuclear Information System (INIS)

    Vaidya, P.C.; Patel, L.K.; Bhatt, P.V.

    1976-01-01

    Using Galilean time and retarded distance as coordinates the usual Kerr metric is expressed in form similar to the Newman-Unti-Tamburino (NUT) metric. The combined Kerr-NUT metric is then investigated. In addition to the Kerr and NUT solutions of Einstein's equations, three other types of solutions are derived. These are (i) the radiating Kerr solution, (ii) the radiating NUT solution satisfying Rsub(ik) = sigmaxisub(i)xisub(k), xisub(i)xisup(i) = 0, and (iii) the associated Kerr solution satisfying Rsub(ik) = 0. Solution (i) is distinct from and simpler than the one reported earlier by Vaidya and Patel (Phys. Rev.; D7:3590 (1973)). Solutions (ii) and (iii) gave line elements which have the axis of symmetry as a singular line. (author)

  15. Complexity Metrics for Workflow Nets

    DEFF Research Database (Denmark)

    Lassen, Kristian Bisgaard; van der Aalst, Wil M.P.

    2009-01-01

    analysts have difficulties grasping the dynamics implied by a process model. Recent empirical studies show that people make numerous errors when modeling complex business processes, e.g., about 20 percent of the EPCs in the SAP reference model have design flaws resulting in potential deadlocks, livelocks......, etc. It seems obvious that the complexity of the model contributes to design errors and a lack of understanding. It is not easy to measure complexity, however. This paper presents three complexity metrics that have been implemented in the process analysis tool ProM. The metrics are defined...... for a subclass of Petri nets named Workflow nets, but the results can easily be applied to other languages. To demonstrate the applicability of these metrics, we have applied our approach and tool to 262 relatively complex Protos models made in the context of various student projects. This allows us to validate...

  16. The uniqueness of the Fisher metric as information metric

    Czech Academy of Sciences Publication Activity Database

    Le, Hong-Van

    2017-01-01

    Roč. 69, č. 4 (2017), s. 879-896 ISSN 0020-3157 Institutional support: RVO:67985840 Keywords : Chentsov’s theorem * mixed topology * monotonicity of the Fisher metric Subject RIV: BA - General Mathematics OBOR OECD: Pure mathematics Impact factor: 1.049, year: 2016 https://link.springer.com/article/10.1007%2Fs10463-016-0562-0

  17. Thermodynamic metrics and optimal paths.

    Science.gov (United States)

    Sivak, David A; Crooks, Gavin E

    2012-05-11

    A fundamental problem in modern thermodynamics is how a molecular-scale machine performs useful work, while operating away from thermal equilibrium without excessive dissipation. To this end, we derive a friction tensor that induces a Riemannian manifold on the space of thermodynamic states. Within the linear-response regime, this metric structure controls the dissipation of finite-time transformations, and bestows optimal protocols with many useful properties. We discuss the connection to the existing thermodynamic length formalism, and demonstrate the utility of this metric by solving for optimal control parameter protocols in a simple nonequilibrium model.

  18. Invariant metrics for Hamiltonian systems

    International Nuclear Information System (INIS)

    Rangarajan, G.; Dragt, A.J.; Neri, F.

    1991-05-01

    In this paper, invariant metrics are constructed for Hamiltonian systems. These metrics give rise to norms on the space of homeogeneous polynomials of phase-space variables. For an accelerator lattice described by a Hamiltonian, these norms characterize the nonlinear content of the lattice. Therefore, the performance of the lattice can be improved by minimizing the norm as a function of parameters describing the beam-line elements in the lattice. A four-fold increase in the dynamic aperture of a model FODO cell is obtained using this procedure. 7 refs

  19. Generalization of Vaidya's radiation metric

    Energy Technology Data Exchange (ETDEWEB)

    Gleiser, R J; Kozameh, C N [Universidad Nacional de Cordoba (Argentina). Instituto de Matematica, Astronomia y Fisica

    1981-11-01

    In this paper it is shown that if Vaidya's radiation metric is considered from the point of view of kinetic theory in general relativity, the corresponding phase space distribution function can be generalized in a particular way. The new family of spherically symmetric radiation metrics obtained contains Vaidya's as a limiting situation. The Einstein field equations are solved in a ''comoving'' coordinate system. Two arbitrary functions of a single variable are introduced in the process of solving these equations. Particular examples considered are a stationary solution, a nonvacuum solution depending on a single parameter, and several limiting situations.

  20. Technical Privacy Metrics: a Systematic Survey

    OpenAIRE

    Wagner, Isabel; Eckhoff, David

    2018-01-01

    The file attached to this record is the author's final peer reviewed version The goal of privacy metrics is to measure the degree of privacy enjoyed by users in a system and the amount of protection offered by privacy-enhancing technologies. In this way, privacy metrics contribute to improving user privacy in the digital world. The diversity and complexity of privacy metrics in the literature makes an informed choice of metrics challenging. As a result, instead of using existing metrics, n...

  1. Remarks on G-Metric Spaces

    Directory of Open Access Journals (Sweden)

    Bessem Samet

    2013-01-01

    Full Text Available In 2005, Mustafa and Sims (2006 introduced and studied a new class of generalized metric spaces, which are called G-metric spaces, as a generalization of metric spaces. We establish some useful propositions to show that many fixed point theorems on (nonsymmetric G-metric spaces given recently by many authors follow directly from well-known theorems on metric spaces. Our technique can be easily extended to other results as shown in application.

  2. DLA Energy Biofuel Feedstock Metrics Study

    Science.gov (United States)

    2012-12-11

    moderately/highly in- vasive  Metric 2: Genetically modified organism ( GMO ) hazard, Yes/No and Hazard Category  Metric 3: Species hybridization...4– biofuel distribution Stage # 5– biofuel use Metric 1: State inva- siveness ranking Yes Minimal Minimal No No Metric 2: GMO hazard Yes...may utilize GMO microbial or microalgae species across the applicable biofuel life cycles (stages 1–3). The following consequence Metrics 4–6 then

  3. Separable metrics and radiating stars

    Indian Academy of Sciences (India)

    We study the junction condition relating the pressure to heat flux at the boundary of an accelerating and expanding spherically symmetric radiating star. We transform the junction condition to an ordinary differential equation by making a separability assumption on the metric functions in the space–time variables.

  4. Socio-technical security metrics

    NARCIS (Netherlands)

    Gollmann, D.; Herley, C.; Koenig, V.; Pieters, W.; Sasse, M.A.

    2015-01-01

    Report from Dagstuhl seminar 14491. This report documents the program and the outcomes of Dagstuhl Seminar 14491 “Socio-Technical Security Metrics”. In the domain of safety, metrics inform many decisions, from the height of new dikes to the design of nuclear plants. We can state, for example, that

  5. Leading Gainful Employment Metric Reporting

    Science.gov (United States)

    Powers, Kristina; MacPherson, Derek

    2016-01-01

    This chapter will address the importance of intercampus involvement in reporting of gainful employment student-level data that will be used in the calculation of gainful employment metrics by the U.S. Department of Education. The authors will discuss why building relationships within the institution is critical for effective gainful employment…

  6. Memory effects of transformation textures in steel and its prediction by the double Kurdjumov–Sachs relation

    International Nuclear Information System (INIS)

    Tomida, T.; Wakita, M.; Yasuyama, M.; Sugaya, S.; Tomota, Y.; Vogel, S.C.

    2013-01-01

    The phenomenon that the transformation texture near the initial texture reproduces after the phase transformation cycle such as ferrite (α, body-centered cubic) → austenite (γ, face-centered cubic) → α is called a texture memory. In this study, the texture change in a 0.1% C–1% Mn hot-rolled steel sheet during the α → γ → α transformation cycle was studied via neutron diffraction and the transformation texture prediction based on a variant selection rule that we call the double Kurdjumov–Sachs (K–S) relation. The texture change observed by neutron diffraction, which clearly showed the texture memory, could be quantitatively reproduced by the proposed variant selection rule adopted into the calculation method based on the spherical harmonics expansion of orientation distribution functions. Therefore, it is most likely that the texture memory in steel is caused by the preferential selection of those K–S variants that reduce the interfacial energy between a precipitate and two adjoining parent phase grains at the same time, which we call the double K–S relation

  7. A direct gene transfer strategy via brain internal capsule reverses the biochemical defect in Tay-Sachs disease.

    Science.gov (United States)

    Martino, S; Marconi, P; Tancini, B; Dolcetta, D; De Angelis, M G Cusella; Montanucci, P; Bregola, G; Sandhoff, K; Bordignon, C; Emiliani, C; Manservigi, R; Orlacchio, A

    2005-08-01

    Therapy for neurodegenerative lysosomal Tay-Sachs (TS) disease requires active hexosaminidase (Hex) A production in the central nervous system and an efficient therapeutic approach that can act faster than human disease progression. We combined the efficacy of a non-replicating Herpes simplex vector encoding for the Hex A alpha-subunit (HSV-T0alphaHex) and the anatomic structure of the brain internal capsule to distribute the missing enzyme optimally. With this gene transfer strategy, for the first time, we re-established the Hex A activity and totally removed the GM2 ganglioside storage in both injected and controlateral hemispheres, in the cerebellum and spinal cord of TS animal model in the span of one month's treatment. In our studies, no adverse effects were observed due to the viral vector, injection site or gene expression and on the basis of these results, we feel confident that the same approach could be applied to similar diseases involving an enzyme defect.

  8. Tay–Sachs disease mutations in HEXA target the α chain of hexosaminidase A to endoplasmic reticulum–associated degradation

    Science.gov (United States)

    Dersh, Devin; Iwamoto, Yuichiro; Argon, Yair

    2016-01-01

    Loss of function of the enzyme β-hexosaminidase A (HexA) causes the lysosomal storage disorder Tay–Sachs disease (TSD). It has been proposed that mutations in the α chain of HexA can impair folding, enzyme assembly, and/or trafficking, yet there is surprisingly little known about the mechanisms of these potential routes of pathogenesis. We therefore investigated the biosynthesis and trafficking of TSD-associated HexA α mutants, seeking to identify relevant cellular quality control mechanisms. The α mutants E482K and G269S are defective in enzymatic activity, unprocessed by lysosomal proteases, and exhibit altered folding pathways compared with wild-type α. E482K is more severely misfolded than G269S, as observed by its aggregation and inability to associate with the HexA β chain. Importantly, both mutants are retrotranslocated from the endoplasmic reticulum (ER) to the cytosol and are degraded by the proteasome, indicating that they are cleared via ER-associated degradation (ERAD). Leveraging these discoveries, we observed that manipulating the cellular folding environment or ERAD pathways can alter the kinetics of mutant α degradation. Additionally, growth of patient fibroblasts at a permissive temperature or with chemical chaperones increases cellular Hex activity by improving mutant α folding. Therefore modulation of the ER quality control systems may be a potential therapeutic route for improving some forms of TSD. PMID:27682588

  9. Tay-Sachs Carrier Screening by Enzyme and Molecular Analyses in the New York City Minority Population.

    Science.gov (United States)

    Mehta, Nikita; Lazarin, Gabriel A; Spiegel, Erica; Berentsen, Kathleen; Brennan, Kelly; Giordano, Jessica; Haque, Imran S; Wapner, Ronald

    2016-09-01

    Carrier screening for Tay-Sachs disease is performed by sequence analysis of the HEXA gene and/or hexosaminidase A enzymatic activity testing. Enzymatic analysis (EA) has been suggested as the optimal carrier screening method, especially in non-Ashkenazi Jewish (non-AJ) individuals, but its utilization and efficacy have not been fully evaluated in the general population. This study assesses the reliability of EA in comparison with HEXA sequence analysis in non-AJ populations. Five hundred eight Hispanic and African American patients (516 samples) had EA of their leukocytes performed and 12 of these patients who tested positive by EA ("carriers") had subsequent HEXA gene sequencing performed. Of the 508 patients, 25 (4.9%) were EA positive and 40 (7.9%) were inconclusive. Of the 12 patients who were sequenced, 11 did not carry a pathogenic variant and one carried a likely deleterious mutation (NM_000520.4(HEXA):c.1510C>T). High inconclusive rates and poor correlation between positive/inconclusive enzyme results and identification of pathogenic mutations suggest that ethnic-specific recalibration of reference ranges for EA may be necessary. Alternatively, HEXA gene sequencing could be performed.

  10. Mortality incidence estimation using federal death certificate and natality data with an application to Tay-Sachs disease.

    Science.gov (United States)

    Jalal, Kabir; Carter, Randy L

    2015-09-01

    For confidentiality reasons, US federal death certificate data are incomplete with regards to the dates of birth and death for the decedents, making calculation of total lifetime of a decedent impossible and thus estimation of mortality incidence difficult. This paper proposes the use of natality data and an imputation-based method to estimate age-specific mortality incidence rates in the face of this missing information. By utilizing previously determined probabilities of birth, a birth date and death date are imputed for every decedent in the dataset. Thus, the birth cohort of each individual is imputed, and the total on-study time can be calculated. This idea is implemented in two approaches for estimation of mortality incidence rates. The first is an extension of a person-time approach, while the second is an extension of a life table approach. Monte Carlo simulations showed that both approaches perform well in comparison to the ideal complete data methods, but that the person-time method is preferred. An application to Tay-Sachs disease is demonstrated. It is concluded that the imputation methods proposed provide valid estimates of the incidence of death from death certificate data without the need for additional assumptions under which usual mortality rates provide valid estimates. © 2015 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

  11. Assessing the potential success of cystic fibrosis carrier screening: lessons learned from Tay-Sachs disease and beta-thalassemia.

    Science.gov (United States)

    Laberge, A-M; Watts, C; Porter, K; Burke, W

    2010-01-01

    The objective of this study was to identify factors involved in the success of 2 well-established population-based carrier screening programs - Tay-Sachs disease (TSD) in Ashkenazi Jews and beta-thalassemia in Sardinia and Cyprus - and to assess the potential for success of a population-based cystic fibrosis (CF) carrier screening strategy using these factors. We performed a literature review and key informant interviews. Factors involved in the success of TSD and beta-thalassemia carrier screening programs include disease characteristics (well-defined population at risk, severe disease with predictable course, availability of effective treatment), test characteristics (high sensitivity, straightforward interpretation of results), and community characteristics (involvement of community, support of families and advocacy groups, consensus in favor of avoiding affected births). Current CF screening strategies include few of the factors listed above. Unlike TSD and beta-thalassemia, the purpose of current CF carrier screening strategies is informed reproductive decision-making, without an explicit goal of reducing disease incidence. When compared to TSD and beta-thalassemia, CF is a less favorable candidate for population-based carrier screening. Because of its different purpose, CF carrier screening will require different measures of success than those used for TSD and beta-thalassemia carrier screening, and a consensus on the value or success of CF carrier screening may be difficult to achieve.

  12. Group covariance and metrical theory

    International Nuclear Information System (INIS)

    Halpern, L.

    1983-01-01

    The a priori introduction of a Lie group of transformations into a physical theory has often proved to be useful; it usually serves to describe special simplified conditions before a general theory can be worked out. Newton's assumptions of absolute space and time are examples where the Euclidian group and translation group have been introduced. These groups were extended to the Galilei group and modified in the special theory of relativity to the Poincare group to describe physics under the given conditions covariantly in the simplest way. The criticism of the a priori character leads to the formulation of the general theory of relativity. The general metric theory does not really give preference to a particular invariance group - even the principle of equivalence can be adapted to a whole family of groups. The physical laws covariantly inserted into the metric space are however adapted to the Poincare group. 8 references

  13. General relativity: An erfc metric

    Science.gov (United States)

    Plamondon, Réjean

    2018-06-01

    This paper proposes an erfc potential to incorporate in a symmetric metric. One key feature of this model is that it relies on the existence of an intrinsic physical constant σ, a star-specific proper length that scales all its surroundings. Based thereon, the new metric is used to study the space-time geometry of a static symmetric massive object, as seen from its interior. The analytical solutions to the Einstein equation are presented, highlighting the absence of singularities and discontinuities in such a model. The geodesics are derived in their second- and first-order differential formats. Recalling the slight impact of the new model on the classical general relativity tests in the solar system, a number of facts and open problems are briefly revisited on the basis of a heuristic definition of σ. A special attention is given to gravitational collapses and non-singular black holes.

  14. hdm: High-dimensional metrics

    OpenAIRE

    Chernozhukov, Victor; Hansen, Christian; Spindler, Martin

    2016-01-01

    In this article the package High-dimensional Metrics (\\texttt{hdm}) is introduced. It is a collection of statistical methods for estimation and quantification of uncertainty in high-dimensional approximately sparse models. It focuses on providing confidence intervals and significance testing for (possibly many) low-dimensional subcomponents of the high-dimensional parameter vector. Efficient estimators and uniformly valid confidence intervals for regression coefficients on target variables (e...

  15. Multi-Metric Sustainability Analysis

    Energy Technology Data Exchange (ETDEWEB)

    Cowlin, Shannon [National Renewable Energy Lab. (NREL), Golden, CO (United States); Heimiller, Donna [National Renewable Energy Lab. (NREL), Golden, CO (United States); Macknick, Jordan [National Renewable Energy Lab. (NREL), Golden, CO (United States); Mann, Margaret [National Renewable Energy Lab. (NREL), Golden, CO (United States); Pless, Jacquelyn [National Renewable Energy Lab. (NREL), Golden, CO (United States); Munoz, David [Colorado School of Mines, Golden, CO (United States)

    2014-12-01

    A readily accessible framework that allows for evaluating impacts and comparing tradeoffs among factors in energy policy, expansion planning, and investment decision making is lacking. Recognizing this, the Joint Institute for Strategic Energy Analysis (JISEA) funded an exploration of multi-metric sustainability analysis (MMSA) to provide energy decision makers with a means to make more comprehensive comparisons of energy technologies. The resulting MMSA tool lets decision makers simultaneously compare technologies and potential deployment locations.

  16. Sensory Metrics of Neuromechanical Trust.

    Science.gov (United States)

    Softky, William; Benford, Criscillia

    2017-09-01

    Today digital sources supply a historically unprecedented component of human sensorimotor data, the consumption of which is correlated with poorly understood maladies such as Internet addiction disorder and Internet gaming disorder. Because both natural and digital sensorimotor data share common mathematical descriptions, one can quantify our informational sensorimotor needs using the signal processing metrics of entropy, noise, dimensionality, continuity, latency, and bandwidth. Such metrics describe in neutral terms the informational diet human brains require to self-calibrate, allowing individuals to maintain trusting relationships. With these metrics, we define the trust humans experience using the mathematical language of computational models, that is, as a primitive statistical algorithm processing finely grained sensorimotor data from neuromechanical interaction. This definition of neuromechanical trust implies that artificial sensorimotor inputs and interactions that attract low-level attention through frequent discontinuities and enhanced coherence will decalibrate a brain's representation of its world over the long term by violating the implicit statistical contract for which self-calibration evolved. Our hypersimplified mathematical understanding of human sensorimotor processing as multiscale, continuous-time vibratory interaction allows equally broad-brush descriptions of failure modes and solutions. For example, we model addiction in general as the result of homeostatic regulation gone awry in novel environments (sign reversal) and digital dependency as a sub-case in which the decalibration caused by digital sensorimotor data spurs yet more consumption of them. We predict that institutions can use these sensorimotor metrics to quantify media richness to improve employee well-being; that dyads and family-size groups will bond and heal best through low-latency, high-resolution multisensory interaction such as shared meals and reciprocated touch; and

  17. Metric reconstruction from Weyl scalars

    Energy Technology Data Exchange (ETDEWEB)

    Whiting, Bernard F; Price, Larry R [Department of Physics, PO Box 118440, University of Florida, Gainesville, FL 32611 (United States)

    2005-08-07

    The Kerr geometry has remained an elusive world in which to explore physics and delve into the more esoteric implications of general relativity. Following the discovery, by Kerr in 1963, of the metric for a rotating black hole, the most major advance has been an understanding of its Weyl curvature perturbations based on Teukolsky's discovery of separable wave equations some ten years later. In the current research climate, where experiments across the globe are preparing for the first detection of gravitational waves, a more complete understanding than concerns just the Weyl curvature is now called for. To understand precisely how comparatively small masses move in response to the gravitational waves they emit, a formalism has been developed based on a description of the whole spacetime metric perturbation in the neighbourhood of the emission region. Presently, such a description is not available for the Kerr geometry. While there does exist a prescription for obtaining metric perturbations once curvature perturbations are known, it has become apparent that there are gaps in that formalism which are still waiting to be filled. The most serious gaps include gauge inflexibility, the inability to include sources-which are essential when the emitting masses are considered-and the failure to describe the l = 0 and 1 perturbation properties. Among these latter properties of the perturbed spacetime, arising from a point mass in orbit, are the perturbed mass and axial component of angular momentum, as well as the very elusive Carter constant for non-axial angular momentum. A status report is given on recent work which begins to repair these deficiencies in our current incomplete description of Kerr metric perturbations.

  18. Metric reconstruction from Weyl scalars

    International Nuclear Information System (INIS)

    Whiting, Bernard F; Price, Larry R

    2005-01-01

    The Kerr geometry has remained an elusive world in which to explore physics and delve into the more esoteric implications of general relativity. Following the discovery, by Kerr in 1963, of the metric for a rotating black hole, the most major advance has been an understanding of its Weyl curvature perturbations based on Teukolsky's discovery of separable wave equations some ten years later. In the current research climate, where experiments across the globe are preparing for the first detection of gravitational waves, a more complete understanding than concerns just the Weyl curvature is now called for. To understand precisely how comparatively small masses move in response to the gravitational waves they emit, a formalism has been developed based on a description of the whole spacetime metric perturbation in the neighbourhood of the emission region. Presently, such a description is not available for the Kerr geometry. While there does exist a prescription for obtaining metric perturbations once curvature perturbations are known, it has become apparent that there are gaps in that formalism which are still waiting to be filled. The most serious gaps include gauge inflexibility, the inability to include sources-which are essential when the emitting masses are considered-and the failure to describe the l = 0 and 1 perturbation properties. Among these latter properties of the perturbed spacetime, arising from a point mass in orbit, are the perturbed mass and axial component of angular momentum, as well as the very elusive Carter constant for non-axial angular momentum. A status report is given on recent work which begins to repair these deficiencies in our current incomplete description of Kerr metric perturbations

  19. Sustainability Metrics: The San Luis Basin Project

    Science.gov (United States)

    Sustainability is about promoting humanly desirable dynamic regimes of the environment. Metrics: ecological footprint, net regional product, exergy, emergy, and Fisher Information. Adaptive management: (1) metrics assess problem, (2) specific problem identified, and (3) managemen...

  20. Crowdsourcing metrics of digital collections

    Directory of Open Access Journals (Sweden)

    Tuula Pääkkönen

    2015-12-01

    Full Text Available In the National Library of Finland (NLF there are millions of digitized newspaper and journal pages, which are openly available via the public website  http://digi.kansalliskirjasto.fi. To serve users better, last year the front end was completely overhauled with its main aim in crowdsourcing features, e.g., by giving end-users the opportunity to create digital clippings and a personal scrapbook from the digital collections. But how can you know whether crowdsourcing has had an impact? How much crowdsourcing functionalities have been used so far? Did crowdsourcing work? In this paper the statistics and metrics of a recent crowdsourcing effort are analysed across the different digitized material types (newspapers, journals, ephemera. The subjects, categories and keywords given by the users are analysed to see which topics are the most appealing. Some notable public uses of the crowdsourced article clippings are highlighted. These metrics give us indications on how the end-users, based on their own interests, are investigating and using the digital collections. Therefore, the suggested metrics illustrate the versatility of the information needs of the users, varying from citizen science to research purposes. By analysing the user patterns, we can respond to the new needs of the users by making minor changes to accommodate the most active participants, while still making the service more approachable for those who are trying out the functionalities for the first time. Participation in the clippings and annotations can enrich the materials in unexpected ways and can possibly pave the way for opportunities of using crowdsourcing more also in research contexts. This creates more opportunities for the goals of open science since source data becomes ­available, making it possible for researchers to reach out to the general public for help. In the long term, utilizing, for example, text mining methods can allow these different end-user segments to

  1. A family of metric gravities

    Science.gov (United States)

    Shuler, Robert

    2018-04-01

    The goal of this paper is to take a completely fresh approach to metric gravity, in which the metric principle is strictly adhered to but its properties in local space-time are derived from conservation principles, not inferred from a global field equation. The global field strength variation then gains some flexibility, but only in the regime of very strong fields (2nd-order terms) whose measurement is now being contemplated. So doing provides a family of similar gravities, differing only in strong fields, which could be developed into meaningful verification targets for strong fields after the manner in which far-field variations were used in the 20th century. General Relativity (GR) is shown to be a member of the family and this is demonstrated by deriving the Schwarzschild metric exactly from a suitable field strength assumption. The method of doing so is interesting in itself because it involves only one differential equation rather than the usual four. Exact static symmetric field solutions are also given for one pedagogical alternative based on potential, and one theoretical alternative based on inertia, and the prospects of experimentally differentiating these are analyzed. Whether the method overturns the conventional wisdom that GR is the only metric theory of gravity and that alternatives must introduce additional interactions and fields is somewhat semantical, depending on whether one views the field strength assumption as a field and whether the assumption that produces GR is considered unique in some way. It is of course possible to have other fields, and the local space-time principle can be applied to field gravities which usually are weak-field approximations having only time dilation, giving them the spatial factor and promoting them to full metric theories. Though usually pedagogical, some of them are interesting from a quantum gravity perspective. Cases are noted where mass measurement errors, or distributions of dark matter, can cause one

  2. Hybrid metric-Palatini stars

    Science.gov (United States)

    Danilǎ, Bogdan; Harko, Tiberiu; Lobo, Francisco S. N.; Mak, M. K.

    2017-02-01

    We consider the internal structure and the physical properties of specific classes of neutron, quark and Bose-Einstein condensate stars in the recently proposed hybrid metric-Palatini gravity theory, which is a combination of the metric and Palatini f (R ) formalisms. It turns out that the theory is very successful in accounting for the observed phenomenology, since it unifies local constraints at the Solar System level and the late-time cosmic acceleration, even if the scalar field is very light. In this paper, we derive the equilibrium equations for a spherically symmetric configuration (mass continuity and Tolman-Oppenheimer-Volkoff) in the framework of the scalar-tensor representation of the hybrid metric-Palatini theory, and we investigate their solutions numerically for different equations of state of neutron and quark matter, by adopting for the scalar field potential a Higgs-type form. It turns out that the scalar-tensor definition of the potential can be represented as an Clairaut differential equation, and provides an explicit form for f (R ) given by f (R )˜R +Λeff, where Λeff is an effective cosmological constant. Furthermore, stellar models, described by the stiff fluid, radiation-like, bag model and the Bose-Einstein condensate equations of state are explicitly constructed in both general relativity and hybrid metric-Palatini gravity, thus allowing an in-depth comparison between the predictions of these two gravitational theories. As a general result it turns out that for all the considered equations of state, hybrid gravity stars are more massive than their general relativistic counterparts. Furthermore, two classes of stellar models corresponding to two particular choices of the functional form of the scalar field (constant value, and logarithmic form, respectively) are also investigated. Interestingly enough, in the case of a constant scalar field the equation of state of the matter takes the form of the bag model equation of state describing

  3. Metrics for Evaluation of Student Models

    Science.gov (United States)

    Pelanek, Radek

    2015-01-01

    Researchers use many different metrics for evaluation of performance of student models. The aim of this paper is to provide an overview of commonly used metrics, to discuss properties, advantages, and disadvantages of different metrics, to summarize current practice in educational data mining, and to provide guidance for evaluation of student…

  4. Context-dependent ATC complexity metric

    NARCIS (Netherlands)

    Mercado Velasco, G.A.; Borst, C.

    2015-01-01

    Several studies have investigated Air Traffic Control (ATC) complexity metrics in a search for a metric that could best capture workload. These studies have shown how daunting the search for a universal workload metric (one that could be applied in different contexts: sectors, traffic patterns,

  5. Properties of C-metric spaces

    Science.gov (United States)

    Croitoru, Anca; Apreutesei, Gabriela; Mastorakis, Nikos E.

    2017-09-01

    The subject of this paper belongs to the theory of approximate metrics [23]. An approximate metric on X is a real application defined on X × X that satisfies only a part of the metric axioms. In a recent paper [23], we introduced a new type of approximate metric, named C-metric, that is an application which satisfies only two metric axioms: symmetry and triangular inequality. The remarkable fact in a C-metric space is that a topological structure induced by the C-metric can be defined. The innovative idea of this paper is that we obtain some convergence properties of a C-metric space in the absence of a metric. In this paper we investigate C-metric spaces. The paper is divided into four sections. Section 1 is for Introduction. In Section 2 we recall some concepts and preliminary results. In Section 3 we present some properties of C-metric spaces, such as convergence properties, a canonical decomposition and a C-fixed point theorem. Finally, in Section 4 some conclusions are highlighted.

  6. Tay-Sachs disease mutations in HEXA target the α chain of hexosaminidase A to endoplasmic reticulum-associated degradation.

    Science.gov (United States)

    Dersh, Devin; Iwamoto, Yuichiro; Argon, Yair

    2016-12-01

    Loss of function of the enzyme β-hexosaminidase A (HexA) causes the lysosomal storage disorder Tay-Sachs disease (TSD). It has been proposed that mutations in the α chain of HexA can impair folding, enzyme assembly, and/or trafficking, yet there is surprisingly little known about the mechanisms of these potential routes of pathogenesis. We therefore investigated the biosynthesis and trafficking of TSD-associated HexA α mutants, seeking to identify relevant cellular quality control mechanisms. The α mutants E482K and G269S are defective in enzymatic activity, unprocessed by lysosomal proteases, and exhibit altered folding pathways compared with wild-type α. E482K is more severely misfolded than G269S, as observed by its aggregation and inability to associate with the HexA β chain. Importantly, both mutants are retrotranslocated from the endoplasmic reticulum (ER) to the cytosol and are degraded by the proteasome, indicating that they are cleared via ER-associated degradation (ERAD). Leveraging these discoveries, we observed that manipulating the cellular folding environment or ERAD pathways can alter the kinetics of mutant α degradation. Additionally, growth of patient fibroblasts at a permissive temperature or with chemical chaperones increases cellular Hex activity by improving mutant α folding. Therefore modulation of the ER quality control systems may be a potential therapeutic route for improving some forms of TSD. © 2016 Dersh et al. This article is distributed by The American Society for Cell Biology under license from the author(s). Two months after publication it is available to the public under an Attribution–Noncommercial–Share Alike 3.0 Unported Creative Commons License (http://creativecommons.org/licenses/by-nc-sa/3.0).

  7. Effect of cyclic, low dose pyrimethamine treatment in patients with Late Onset Tay Sachs: an open label, extended pilot study.

    Science.gov (United States)

    Osher, Etty; Fattal-Valevski, Aviva; Sagie, Liora; Urshanski, Nataly; Sagiv, Nadav; Peleg, Leah; Lerman-Sagie, Tally; Zimran, Ari; Elstein, Deborah; Navon, Ruth; Valevski, Avi; Stern, Naftali

    2015-04-17

    Late Onset Tay- Sachs disease (LOTS) is a rare neurodegenerative lysosomal storage disease which results from mutations in the gene encoding the α subunit (HEXA) of β-hexosaminidase enzyme (HexA). At the present time, no effective treatment exists for LOTS and other neurodegenerative diseases involving the central nerve system (CNS). Pyrimethamine (PMT) was previously shown to act as a HexA chaperone in human fibroblasts in vitro carrying some (e.g., αG269S), but not all LOTS-related mutations. The present study assessed the effect of cyclic, low dose and long term pyrimethamine treatment on HexA in subjects with LOTS. In an open label trial in 4 LOTS patients, PMT was initiated at an average daily dose of ~2.7 mg and administered cyclically guided by blood lymphocyte HexA activity for a mean duration of 82.8 (±22.5; SD) weeks (~1.5 year). HexA activity rose in all subjects, with a mean peak increase of 2.24 folds (±0.52; SD) over baseline activity (range 1.87-3). The mean treatment time required to attain this peak was of 15.7 (±4.8; SD) weeks. Following increase in activity, HexA gradually declined with the continued use of PMT, which was then stopped, resulting in the return of HexA activity to baseline. A second cycle of PMT treatment was then initiated, resulting again in an increase in HexA activity. Three of the patients experienced a measurable neuropsychiatric deterioration whereas one subject remained entirely stable. Cyclic low dose of PMT can increase HexA activity in LOTS patients. However, the observed increase is repeatedly transient and not associated with discernible beneficial neurological or psychiatric effects.

  8. A Drosophila protein family implicated in pheromone perception is related to Tay-Sachs GM2-activator protein.

    Science.gov (United States)

    Starostina, Elena; Xu, Aiguo; Lin, Heping; Pikielny, Claudio W

    2009-01-02

    Low volatility, lipid-like cuticular hydrocarbon pheromones produced by Drosophila melanogaster females play an essential role in triggering and modulating mating behavior, but the chemosensory mechanisms involved remain poorly understood. Recently, we showed that the CheB42a protein, which is expressed in only 10 pheromone-sensing taste hairs on the front legs of males, modulates progression to late stages of male courtship behavior in response to female-specific cuticular hydrocarbons. Here we report that expression of all 12 genes in the CheB gene family is predominantly or exclusively gustatory-specific, and occurs in many different, often non-overlapping patterns. Only the Gr family of gustatory receptor genes displays a comparable variety of gustatory-specific expression patterns. Unlike Grs, however, expression of all but one CheB gene is sexually dimorphic. Like CheB42a, other CheBs may therefore function specifically in gustatory perception of pheromones. We also show that CheBs belong to the ML superfamily of lipid-binding proteins, and are most similar to human GM2-activator protein (GM2-AP). In particular, GM2-AP residues involved in ligand binding are conserved in CheBs but not in other ML proteins. Finally, CheB42a is specifically secreted into the inner lumen of pheromone-sensing taste hairs, where pheromones interact with membrane-bound receptors. We propose that CheB proteins interact directly with lipid-like Drosophila pheromones and modulate their detection by the gustatory signal transduction machinery. Furthermore, as loss of GM2-AP in Tay-Sachs disease prevents degradation of GM2 gangliosides and results in neurodegeneration, the function of CheBs in pheromone response may involve biochemical mechanisms critical for lipid metabolism in human neurons.

  9. A Drosophila Protein Family Implicated in Pheromone Perception Is Related to Tay-Sachs GM2-Activator Protein*

    Science.gov (United States)

    Starostina, Elena; Xu, Aiguo; Lin, Heping; Pikielny, Claudio W.

    2009-01-01

    Low volatility, lipid-like cuticular hydrocarbon pheromones produced by Drosophila melanogaster females play an essential role in triggering and modulating mating behavior, but the chemosensory mechanisms involved remain poorly understood. Recently, we showed that the CheB42a protein, which is expressed in only 10 pheromone-sensing taste hairs on the front legs of males, modulates progression to late stages of male courtship behavior in response to female-specific cuticular hydrocarbons. Here we report that expression of all 12 genes in the CheB gene family is predominantly or exclusively gustatory-specific, and occurs in many different, often non-overlapping patterns. Only the Gr family of gustatory receptor genes displays a comparable variety of gustatory-specific expression patterns. Unlike Grs, however, expression of all but one CheB gene is sexually dimorphic. Like CheB42a, other CheBs may therefore function specifically in gustatory perception of pheromones. We also show that CheBs belong to the ML superfamily of lipid-binding proteins, and are most similar to human GM2-activator protein (GM2-AP). In particular, GM2-AP residues involved in ligand binding are conserved in CheBs but not in other ML proteins. Finally, CheB42a is specifically secreted into the inner lumen of pheromone-sensing taste hairs, where pheromones interact with membrane-bound receptors. We propose that CheB proteins interact directly with lipid-like Drosophila pheromones and modulate their detection by the gustatory signal transduction machinery. Furthermore, as loss of GM2-AP in Tay-Sachs disease prevents degradation of GM2 gangliosides and results in neurodegeneration, the function of CheBs in pheromone response may involve biochemical mechanisms critical for lipid metabolism in human neurons. PMID:18952610

  10. Evaluation of the risk for Tay-Sachs disease in individuals of French Canadian ancestry living in new England.

    Science.gov (United States)

    Martin, Dianna C; Mark, Brian L; Triggs-Raine, Barbara L; Natowicz, Marvin R

    2007-03-01

    The assessment of risk for Tay-Sachs disease (TSD) in individuals of French Canadian background living in New England is an important health issue. In preliminary studies of the enzyme-defined carrier frequency for TSD among Franco-Americans in New England, we found frequencies (1:53) higher than predicted from the incidence of infantile TSD in this region. We have now further evaluated the risk for TSD in the Franco-American population of New England. Using a fluorescence-based assay for beta-hexosaminidase activity, we determined the carrier frequencies for TSD in 2783 Franco-Americans. DNA analysis was used to identify mutations causing enzyme deficiency in TSD carriers. We determined the enzyme-defined carrier frequency for TSD as 1:65 (95% confidence interval 1:49 to 1:90). DNA-based analysis of 24 of the enzyme-defined carriers revealed 21 with sequence changes: 9 disease-causing, 4 benign, and 8 of unknown significance. Six of the unknowns were identified as c.748G>A p.G250S, a mutation we show by expression analysis to behave similarly to the previously described c.805G>A p.G269S adult-onset TSD mutation. This putative adult-onset TSD c.748G>A p.G250S mutation has a population frequency similar to the common 7.6 kb deletion mutation that occurs in persons of French Canadian ancestry. We estimate the frequency of deleterious TSD alleles in Franco-Americans to be 1:73 (95% confidence interval 1:55 to 1:107). These data provide a more complete data base from which to formulate policy recommendations regarding TSD heterozygosity screening in individuals of French Canadian background.

  11. Fermat Potentials of Embedded Lensing, the Integrated Sachs-Wolfe Effect, and Weak-Lensing of CMB by Cosmic Voids

    Science.gov (United States)

    Chen, Bin; Kantowski, R.; Dai, X.

    2014-01-01

    We have developed an accurate gravitational lens theory for an inhomogeneity embedded in an otherwise homogeneous universe, which to the lowest order is applicable to any mass distribution. We derive the Fermat potential for a spherically symmetric lens embedded in a FLRW cosmology and use it to investigate the late-time integrated Sachs-Wolfe effect (ISW) caused by individual large scale inhomogeneities, in particular, cosmic voids. We present a simple analytical expression for the CMB temperature fluctuation across such a lens as the derivative of the lens Fermat potential. Our formalism is applicable to both linear and nonlinear density evolution scenarios, to arbitrarily large density contrasts, and to all open and closed background cosmologies. Our results are particularly useful for modeling ISW effects extracted through stacking large numbers of cosmic voids and clusters (that is, the aperture photometry method). For structures co-expanding with the background cosmology, i.e., for time-independent density contrasts, we find that the gravitational lensing time delay alone can produce fluctuations of the order of seen in recent observations by WMAP and Planck. We revisit the possibility of explaining the non-Gaussian cold spot on the south hemisphere via the Rees-Sciama effect of a large cosmic void using constraints obtained from the most recent void catalogs and our new void-lensing formalism, and compare it with other explanations such as a collapsing cosmic texture. We also study the remapping of primordial CMB anisotropies, the weak-lensing shear, and magnification caused by void lensing.

  12. Traumatic glenohumeral bone defects and their relationship to failure of arthroscopic Bankart repairs: significance of the inverted-pear glenoid and the humeral engaging Hill-Sachs lesion.

    Science.gov (United States)

    Burkhart, S S; De Beer, J F

    2000-10-01

    Our goal was to analyze the results of 194 consecutive arthroscopic Bankart repairs (performed by 2 surgeons with an identical suture anchor technique) in order to identify specific factors related to recurrence of instability. Case series. We analyzed 194 consecutive arthroscopic Bankart repairs by suture anchor technique performed for traumatic anterior-inferior instability. The average follow-up was 27 months (range, 14 to 79 months). There were 101 contact athletes (96 South African rugby players and 5 American football players). We identified significant bone defects on either the humerus or the glenoid as (1) "inverted-pear" glenoid, in which the normally pear-shaped glenoid had lost enough anterior-inferior bone to assume the shape of an inverted pear; or (2) "engaging" Hill-Sachs lesion of the humerus, in which the orientation of the Hill-Sachs lesion was such that it engaged the anterior glenoid with the shoulder in abduction and external rotation. There were 21 recurrent dislocations and subluxations (14 dislocations, 7 subluxations). Of those 21 shoulders with recurrent instability, 14 had significant bone defects (3 engaging Hill-Sachs and 11 inverted-pear Bankart lesions). For the group of patients without significant bone defects (173 shoulders), there were 7 recurrences (4% recurrence rate). For the group with significant bone defects (21 patients), there were 14 recurrences (67% recurrence rate). For contact athletes without significant bone defects, there was a 6.5% recurrence rate, whereas for contact athletes with significant bone defects, there was an 89% recurrence rate. (1) Arthroscopic Bankart repairs give results equal to open Bankart repairs if there are no significant structural bone deficits (engaging Hill-Sachs or inverted-pear Bankart lesions). (2) Patients with significant bone deficits as defined in this study are not candidates for arthroscopic Bankart repair. (3) Contact athletes without structural bone deficits may be treated by

  13. On characterizations of quasi-metric completeness

    Energy Technology Data Exchange (ETDEWEB)

    Dag, H.; Romaguera, S.; Tirado, P.

    2017-07-01

    Hu proved in [4] that a metric space (X, d) is complete if and only if for any closed subspace C of (X, d), every Banach contraction on C has fixed point. Since then several authors have investigated the problem of characterizing the metric completeness by means of fixed point theorems. Recently this problem has been studied in the more general context of quasi-metric spaces for different notions of completeness. Here we present a characterization of a kind of completeness for quasi-metric spaces by means of a quasi-metric versions of Hu’s theorem. (Author)

  14. The Metric of Colour Space

    DEFF Research Database (Denmark)

    Gravesen, Jens

    2015-01-01

    and found the MacAdam ellipses which are often interpreted as defining the metric tensor at their centres. An important question is whether it is possible to define colour coordinates such that the Euclidean distance in these coordinates correspond to human perception. Using cubic splines to represent......The space of colours is a fascinating space. It is a real vector space, but no matter what inner product you put on the space the resulting Euclidean distance does not correspond to human perception of difference between colours. In 1942 MacAdam performed the first experiments on colour matching...

  15. Product Operations Status Summary Metrics

    Science.gov (United States)

    Takagi, Atsuya; Toole, Nicholas

    2010-01-01

    The Product Operations Status Summary Metrics (POSSUM) computer program provides a readable view into the state of the Phoenix Operations Product Generation Subsystem (OPGS) data pipeline. POSSUM provides a user interface that can search the data store, collect product metadata, and display the results in an easily-readable layout. It was designed with flexibility in mind for support in future missions. Flexibility over various data store hierarchies is provided through the disk-searching facilities of Marsviewer. This is a proven program that has been in operational use since the first day of the Phoenix mission.

  16. Web metrics for library and information professionals

    CERN Document Server

    Stuart, David

    2014-01-01

    This is a practical guide to using web metrics to measure impact and demonstrate value. The web provides an opportunity to collect a host of different metrics, from those associated with social media accounts and websites to more traditional research outputs. This book is a clear guide for library and information professionals as to what web metrics are available and how to assess and use them to make informed decisions and demonstrate value. As individuals and organizations increasingly use the web in addition to traditional publishing avenues and formats, this book provides the tools to unlock web metrics and evaluate the impact of this content. The key topics covered include: bibliometrics, webometrics and web metrics; data collection tools; evaluating impact on the web; evaluating social media impact; investigating relationships between actors; exploring traditional publications in a new environment; web metrics and the web of data; the future of web metrics and the library and information professional.Th...

  17. Metrics for building performance assurance

    Energy Technology Data Exchange (ETDEWEB)

    Koles, G.; Hitchcock, R.; Sherman, M.

    1996-07-01

    This report documents part of the work performed in phase I of a Laboratory Directors Research and Development (LDRD) funded project entitled Building Performance Assurances (BPA). The focus of the BPA effort is to transform the way buildings are built and operated in order to improve building performance by facilitating or providing tools, infrastructure, and information. The efforts described herein focus on the development of metrics with which to evaluate building performance and for which information and optimization tools need to be developed. The classes of building performance metrics reviewed are (1) Building Services (2) First Costs, (3) Operating Costs, (4) Maintenance Costs, and (5) Energy and Environmental Factors. The first category defines the direct benefits associated with buildings; the next three are different kinds of costs associated with providing those benefits; the last category includes concerns that are broader than direct costs and benefits to the building owner and building occupants. The level of detail of the various issues reflect the current state of knowledge in those scientific areas and the ability of the to determine that state of knowledge, rather than directly reflecting the importance of these issues; it intentionally does not specifically focus on energy issues. The report describes work in progress and is intended as a resource and can be used to indicate the areas needing more investigation. Other reports on BPA activities are also available.

  18. Miglustat in late-onset Tay-Sachs disease: a 12-month, randomized, controlled clinical study with 24 months of extended treatment.

    Science.gov (United States)

    Shapiro, Barbara E; Pastores, Gregory M; Gianutsos, John; Luzy, Cécile; Kolodny, Edwin H

    2009-06-01

    To evaluate the safety and efficacy of miglustat in patients with GM2 gangliosidosis. A randomized, multicenter, open-label, 12-month study involving patients aged 18 years or older, randomized 2:1 to miglustat (200 mg TID) or "no miglustat treatment." This study was followed by 24 months of extended treatment during which all patients received miglustat. Primary efficacy endpoints were change in eight measures of isometric muscle strength in the limbs and isometric grip strength, evaluated at baseline, and months 12 and 36. Secondary efficacy endpoints included gait, balance, disability, and other neurological assessments. Safety evaluations included adverse event reporting. Thirty patients (67% male, age range 18-56 years) with late-onset Tay-Sachs disease were enrolled; 20 were randomized to miglustat and 10 to "no miglustat treatment." Muscle and grip strength generally decreased over the study period. No differences were observed between the two groups in any efficacy measure, either during the 12-month randomized phase or the full 36 months. The most common treatment-related adverse events were decrease in weight and diarrhea. Miglustat treatment was not shown to lead to measurable benefits in this cohort of patients with late-onset Tay-Sachs disease. The observed safety profile was consistent with that of the approved dose (100 mg TID) in type 1 Gaucher disease.

  19. In silico analyses of essential interactions of iminosugars with the Hex A active site and evaluation of their pharmacological chaperone effects for Tay-Sachs disease.

    Science.gov (United States)

    Kato, Atsushi; Nakagome, Izumi; Nakagawa, Shinpei; Kinami, Kyoko; Adachi, Isao; Jenkinson, Sarah F; Désiré, Jérôme; Blériot, Yves; Nash, Robert J; Fleet, George W J; Hirono, Shuichi

    2017-11-15

    The affinity of a series of iminosugar-based inhibitors exhibiting various ring sizes toward Hex A and their essential interactions with the enzyme active site were investigated. All the Hex A-inhibiting iminosugars tested formed hydrogen bonds with Arg178, Asp322, Tyr421 and Glu462 and had the favorable cation-π interaction with Trp460. Among them, DMDP amide (6) proved to be the most potent competitive inhibitor with a K i value of 0.041 μM. We analyzed the dynamic properties of both DMDP amide (6) and DNJNAc (1) in aqueous solution using molecular dynamics (MD) calculations; the distance of the interaction between Asp322 and 3-OH and Glu323 and 6-OH was important for stable interactions with Hex A, reducing fluctuations in the plasticity of the active site. DMDP amide (6) dose-dependently increased intracellular Hex A activity in the G269S mutant cells and restored Hex A activity up to approximately 43% of the wild type level; this effect clearly exceeded the border line treatment for Tay-Sachs disease, which is regarded as 10-15% of the wild type level. This is a significantly greater effect than that of pyrimethamine, which is currently in Phase 2 clinical trials. DMDP amide (6), therefore, represents a new promising pharmacological chaperone candidate for the treatment of Tay-Sachs disease.

  20. Metric approach to quantum constraints

    International Nuclear Information System (INIS)

    Brody, Dorje C; Hughston, Lane P; Gustavsson, Anna C T

    2009-01-01

    A framework for deriving equations of motion for constrained quantum systems is introduced and a procedure for its implementation is outlined. In special cases, the proposed new method, which takes advantage of the fact that the space of pure states in quantum mechanics has both a symplectic structure and a metric structure, reduces to a quantum analogue of the Dirac theory of constraints in classical mechanics. Explicit examples involving spin-1/2 particles are worked out in detail: in the first example, our approach coincides with a quantum version of the Dirac formalism, while the second example illustrates how a situation that cannot be treated by Dirac's approach can nevertheless be dealt with in the present scheme.

  1. Metrics for Business Process Models

    Science.gov (United States)

    Mendling, Jan

    Up until now, there has been little research on why people introduce errors in real-world business process models. In a more general context, Simon [404] points to the limitations of cognitive capabilities and concludes that humans act rationally only to a certain extent. Concerning modeling errors, this argument would imply that human modelers lose track of the interrelations of large and complex models due to their limited cognitive capabilities and introduce errors that they would not insert in a small model. A recent study by Mendling et al. [275] explores in how far certain complexity metrics of business process models have the potential to serve as error determinants. The authors conclude that complexity indeed appears to have an impact on error probability. Before we can test such a hypothesis in a more general setting, we have to establish an understanding of how we can define determinants that drive error probability and how we can measure them.

  2. Active Metric Learning for Supervised Classification

    OpenAIRE

    Kumaran, Krishnan; Papageorgiou, Dimitri; Chang, Yutong; Li, Minhan; Takáč, Martin

    2018-01-01

    Clustering and classification critically rely on distance metrics that provide meaningful comparisons between data points. We present mixed-integer optimization approaches to find optimal distance metrics that generalize the Mahalanobis metric extensively studied in the literature. Additionally, we generalize and improve upon leading methods by removing reliance on pre-designated "target neighbors," "triplets," and "similarity pairs." Another salient feature of our method is its ability to en...

  3. On Nakhleh's metric for reduced phylogenetic networks

    OpenAIRE

    Cardona, Gabriel; Llabrés, Mercè; Rosselló, Francesc; Valiente Feruglio, Gabriel Alejandro

    2009-01-01

    We prove that Nakhleh’s metric for reduced phylogenetic networks is also a metric on the classes of tree-child phylogenetic networks, semibinary tree-sibling time consistent phylogenetic networks, and multilabeled phylogenetic trees. We also prove that it separates distinguishable phylogenetic networks. In this way, it becomes the strongest dissimilarity measure for phylogenetic networks available so far. Furthermore, we propose a generalization of that metric that separates arbitrary phyl...

  4. Generalized tolerance sensitivity and DEA metric sensitivity

    OpenAIRE

    Neralić, Luka; E. Wendell, Richard

    2015-01-01

    This paper considers the relationship between Tolerance sensitivity analysis in optimization and metric sensitivity analysis in Data Envelopment Analysis (DEA). Herein, we extend the results on the generalized Tolerance framework proposed by Wendell and Chen and show how this framework includes DEA metric sensitivity as a special case. Further, we note how recent results in Tolerance sensitivity suggest some possible extensions of the results in DEA metric sensitivity.

  5. The definitive guide to IT service metrics

    CERN Document Server

    McWhirter, Kurt

    2012-01-01

    Used just as they are, the metrics in this book will bring many benefits to both the IT department and the business as a whole. Details of the attributes of each metric are given, enabling you to make the right choices for your business. You may prefer and are encouraged to design and create your own metrics to bring even more value to your business - this book will show you how to do this, too.

  6. Generalized tolerance sensitivity and DEA metric sensitivity

    Directory of Open Access Journals (Sweden)

    Luka Neralić

    2015-03-01

    Full Text Available This paper considers the relationship between Tolerance sensitivity analysis in optimization and metric sensitivity analysis in Data Envelopment Analysis (DEA. Herein, we extend the results on the generalized Tolerance framework proposed by Wendell and Chen and show how this framework includes DEA metric sensitivity as a special case. Further, we note how recent results in Tolerance sensitivity suggest some possible extensions of the results in DEA metric sensitivity.

  7. Common Metrics for Human-Robot Interaction

    Science.gov (United States)

    Steinfeld, Aaron; Lewis, Michael; Fong, Terrence; Scholtz, Jean; Schultz, Alan; Kaber, David; Goodrich, Michael

    2006-01-01

    This paper describes an effort to identify common metrics for task-oriented human-robot interaction (HRI). We begin by discussing the need for a toolkit of HRI metrics. We then describe the framework of our work and identify important biasing factors that must be taken into consideration. Finally, we present suggested common metrics for standardization and a case study. Preparation of a larger, more detailed toolkit is in progress.

  8. Chaotic inflation with metric and matter perturbations

    International Nuclear Information System (INIS)

    Feldman, H.A.; Brandenberger, R.H.

    1989-01-01

    A perturbative scheme to analyze the evolution of both metric and scalar field perturbations in an expanding universe is developed. The scheme is applied to study chaotic inflation with initial metric and scalar field perturbations present. It is shown that initial gravitational perturbations with wavelength smaller than the Hubble radius rapidly decay. The metric simultaneously picks up small perturbations determined by the matter inhomogeneities. Both are frozen in once the wavelength exceeds the Hubble radius. (orig.)

  9. Gravitational lensing in metric theories of gravity

    International Nuclear Information System (INIS)

    Sereno, Mauro

    2003-01-01

    Gravitational lensing in metric theories of gravity is discussed. I introduce a generalized approximate metric element, inclusive of both post-post-Newtonian contributions and a gravitomagnetic field. Following Fermat's principle and standard hypotheses, I derive the time delay function and deflection angle caused by an isolated mass distribution. Several astrophysical systems are considered. In most of the cases, the gravitomagnetic correction offers the best perspectives for an observational detection. Actual measurements distinguish only marginally different metric theories from each other

  10. About the possibility of a generalized metric

    International Nuclear Information System (INIS)

    Lukacs, B.; Ladik, J.

    1991-10-01

    The metric (the structure of the space-time) may be dependent on the properties of the object measuring it. The case of size dependence of the metric was examined. For this dependence the simplest possible form of the metric tensor has been constructed which fulfils the following requirements: there be two extremal characteristic scales; the metric be unique and the usual between them; the change be sudden in the neighbourhood of these scales; the size of the human body appear as a parameter (postulated on the basis of some philosophical arguments). Estimates have been made for the two extremal length scales according to existing observations. (author) 19 refs

  11. Enhancing Authentication Models Characteristic Metrics via ...

    African Journals Online (AJOL)

    In this work, we derive the universal characteristic metrics set for authentication models based on security, usability and design issues. We then compute the probability of the occurrence of each characteristic metrics in some single factor and multifactor authentication models in order to determine the effectiveness of these ...

  12. Gravitational Metric Tensor Exterior to Rotating Homogeneous ...

    African Journals Online (AJOL)

    The covariant and contravariant metric tensors exterior to a homogeneous spherical body rotating uniformly about a common φ axis with constant angular velocity ω is constructed. The constructed metric tensors in this gravitational field have seven non-zero distinct components.The Lagrangian for this gravitational field is ...

  13. Invariant metric for nonlinear symplectic maps

    Indian Academy of Sciences (India)

    In this paper, we construct an invariant metric in the space of homogeneous polynomials of a given degree (≥ 3). The homogeneous polynomials specify a nonlinear symplectic map which in turn represents a Hamiltonian system. By minimizing the norm constructed out of this metric as a function of system parameters, we ...

  14. Finite Metric Spaces of Strictly negative Type

    DEFF Research Database (Denmark)

    Hjorth, Poul G.

    If a finite metric space is of strictly negative type then its transfinite diameter is uniquely realized by an infinite extent (“load vector''). Finite metric spaces that have this property include all trees, and all finite subspaces of Euclidean and Hyperbolic spaces. We prove that if the distance...

  15. Fixed point theory in metric type spaces

    CERN Document Server

    Agarwal, Ravi P; O’Regan, Donal; Roldán-López-de-Hierro, Antonio Francisco

    2015-01-01

    Written by a team of leading experts in the field, this volume presents a self-contained account of the theory, techniques and results in metric type spaces (in particular in G-metric spaces); that is, the text approaches this important area of fixed point analysis beginning from the basic ideas of metric space topology. The text is structured so that it leads the reader from preliminaries and historical notes on metric spaces (in particular G-metric spaces) and on mappings, to Banach type contraction theorems in metric type spaces, fixed point theory in partially ordered G-metric spaces, fixed point theory for expansive mappings in metric type spaces, generalizations, present results and techniques in a very general abstract setting and framework. Fixed point theory is one of the major research areas in nonlinear analysis. This is partly due to the fact that in many real world problems fixed point theory is the basic mathematical tool used to establish the existence of solutions to problems which arise natur...

  16. Metric solution of a spinning mass

    International Nuclear Information System (INIS)

    Sato, H.

    1982-01-01

    Studies on a particular class of asymptotically flat and stationary metric solutions called the Kerr-Tomimatsu-Sato class are reviewed about its derivation and properties. For a further study, an almost complete list of the papers worked on the Tomimatsu-Sato metrics is given. (Auth.)

  17. On Information Metrics for Spatial Coding.

    Science.gov (United States)

    Souza, Bryan C; Pavão, Rodrigo; Belchior, Hindiael; Tort, Adriano B L

    2018-04-01

    The hippocampal formation is involved in navigation, and its neuronal activity exhibits a variety of spatial correlates (e.g., place cells, grid cells). The quantification of the information encoded by spikes has been standard procedure to identify which cells have spatial correlates. For place cells, most of the established metrics derive from Shannon's mutual information (Shannon, 1948), and convey information rate in bits/s or bits/spike (Skaggs et al., 1993, 1996). Despite their widespread use, the performance of these metrics in relation to the original mutual information metric has never been investigated. In this work, using simulated and real data, we find that the current information metrics correlate less with the accuracy of spatial decoding than the original mutual information metric. We also find that the top informative cells may differ among metrics, and show a surrogate-based normalization that yields comparable spatial information estimates. Since different information metrics may identify different neuronal populations, we discuss current and alternative definitions of spatially informative cells, which affect the metric choice. Copyright © 2018 IBRO. Published by Elsevier Ltd. All rights reserved.

  18. Validation of Metrics for Collaborative Systems

    Directory of Open Access Journals (Sweden)

    Ion IVAN

    2008-01-01

    Full Text Available This paper describe the new concepts of collaborative systems metrics validation. The paper define the quality characteristics of collaborative systems. There are proposed a metric to estimate the quality level of collaborative systems. There are performed measurements of collaborative systems quality using a specially designed software.

  19. Validation of Metrics for Collaborative Systems

    OpenAIRE

    Ion IVAN; Cristian CIUREA

    2008-01-01

    This paper describe the new concepts of collaborative systems metrics validation. The paper define the quality characteristics of collaborative systems. There are proposed a metric to estimate the quality level of collaborative systems. There are performed measurements of collaborative systems quality using a specially designed software.

  20. Software Power Metric Model: An Implementation | Akwukwuma ...

    African Journals Online (AJOL)

    ... and the execution time (TIME) in each case was recorded. We then obtain the application functions point count. Our result shows that the proposed metric is computable, consistent in its use of unit, and is programming language independent. Keywords: Software attributes, Software power, measurement, Software metric, ...

  1. Tay-Sachs Disease

    Science.gov (United States)

    ... better understanding of how neurological deficits arise in lipid storage diseases and on the development of new treatments targeting disease mechanisms. Specific research on the gangliodisoses including expanding the use of ...

  2. Tay-Sachs Disease

    Science.gov (United States)

    ... During the next months — or even years — the baby will progressively lose the ability to see, hear, and move. A red spot will develop in the back of the child's eyes. The child will stop smiling, crawling, turning over, and reaching out for things. By ...

  3. Metrics for border management systems.

    Energy Technology Data Exchange (ETDEWEB)

    Duggan, Ruth Ann

    2009-07-01

    There are as many unique and disparate manifestations of border systems as there are borders to protect. Border Security is a highly complex system analysis problem with global, regional, national, sector, and border element dimensions for land, water, and air domains. The complexity increases with the multiple, and sometimes conflicting, missions for regulating the flow of people and goods across borders, while securing them for national security. These systems include frontier border surveillance, immigration management and customs functions that must operate in a variety of weather, terrain, operational conditions, cultural constraints, and geopolitical contexts. As part of a Laboratory Directed Research and Development Project 08-684 (Year 1), the team developed a reference framework to decompose this complex system into international/regional, national, and border elements levels covering customs, immigration, and border policing functions. This generalized architecture is relevant to both domestic and international borders. As part of year two of this project (09-1204), the team determined relevant relative measures to better understand border management performance. This paper describes those relative metrics and how they can be used to improve border management systems.

  4. The metrics of science and technology

    CERN Document Server

    Geisler, Eliezer

    2000-01-01

    Dr. Geisler's far-reaching, unique book provides an encyclopedic compilation of the key metrics to measure and evaluate the impact of science and technology on academia, industry, and government. Focusing on such items as economic measures, patents, peer review, and other criteria, and supported by an extensive review of the literature, Dr. Geisler gives a thorough analysis of the strengths and weaknesses inherent in metric design, and in the use of the specific metrics he cites. His book has already received prepublication attention, and will prove especially valuable for academics in technology management, engineering, and science policy; industrial R&D executives and policymakers; government science and technology policymakers; and scientists and managers in government research and technology institutions. Geisler maintains that the application of metrics to evaluate science and technology at all levels illustrates the variety of tools we currently possess. Each metric has its own unique strengths and...

  5. Smart Grid Status and Metrics Report Appendices

    Energy Technology Data Exchange (ETDEWEB)

    Balducci, Patrick J. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Antonopoulos, Chrissi A. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Clements, Samuel L. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Gorrissen, Willy J. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Kirkham, Harold [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Ruiz, Kathleen A. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Smith, David L. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Weimar, Mark R. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Gardner, Chris [APQC, Houston, TX (United States); Varney, Jeff [APQC, Houston, TX (United States)

    2014-07-01

    A smart grid uses digital power control and communication technology to improve the reliability, security, flexibility, and efficiency of the electric system, from large generation through the delivery systems to electricity consumers and a growing number of distributed generation and storage resources. To convey progress made in achieving the vision of a smart grid, this report uses a set of six characteristics derived from the National Energy Technology Laboratory Modern Grid Strategy. The Smart Grid Status and Metrics Report defines and examines 21 metrics that collectively provide insight into the grid’s capacity to embody these characteristics. This appendix presents papers covering each of the 21 metrics identified in Section 2.1 of the Smart Grid Status and Metrics Report. These metric papers were prepared in advance of the main body of the report and collectively form its informational backbone.

  6. Metrics for Polyphonic Sound Event Detection

    Directory of Open Access Journals (Sweden)

    Annamaria Mesaros

    2016-05-01

    Full Text Available This paper presents and discusses various metrics proposed for evaluation of polyphonic sound event detection systems used in realistic situations where there are typically multiple sound sources active simultaneously. The system output in this case contains overlapping events, marked as multiple sounds detected as being active at the same time. The polyphonic system output requires a suitable procedure for evaluation against a reference. Metrics from neighboring fields such as speech recognition and speaker diarization can be used, but they need to be partially redefined to deal with the overlapping events. We present a review of the most common metrics in the field and the way they are adapted and interpreted in the polyphonic case. We discuss segment-based and event-based definitions of each metric and explain the consequences of instance-based and class-based averaging using a case study. In parallel, we provide a toolbox containing implementations of presented metrics.

  7. Orientation relationship in various 9% Cr ferritic/martensitic steels-EBSD comparison between Nishiyama-Wassermann, Kurdjumov-Sachs and Greninger-Troiano

    International Nuclear Information System (INIS)

    Barcelo, F.; Bechade, J. L.; Fournier, B.

    2010-01-01

    EBSD measurements were carried out on four different martensitic steels (T91, P92, EM10 and Eurofer) in various metallurgical conditions (nine different microstructural states). The usual orientation relationships (ORs) between the parent austenitic phase and the resulting martensite in martensitic steels are those of Nishiyama-Wassermann (NW) and Kurjumov-Sachs (KS). The present study first proposes a methodology based on the combined analysis of the misorientation distribution, the pole figures (PFs) and the angle/axis pairs. This methodology leads to the conclusion that neither NW nor KS relationships are able to account for all the features observed whatever the material under study. A third OR proposed by Greninger and Troiano (GT) proves to describe the relationship between austenite and ferrite in all four different martensitic steels much more accurately. (authors)

  8. Crystallographic Structure of Human β-Hexosaminidase A: Interpretation of Tay-Sachs Mutations and Loss of GM2 Ganglioside Hydrolysis

    Science.gov (United States)

    Lemieux, M. Joanne; Mark, Brian L.; Cherney, Maia M.; Withers, Stephen G.; Mahuran, Don J.; James, Michael N. G.

    2010-01-01

    Lysosomal β-hexosaminidase A (Hex A) is essential for the degradation of GM2 gangliosides in the central and peripheral nervous system. Accumulation of GM2 leads to severely debilitating neurodegeneration associated with Tay-Sachs disease (TSD), Sandoff disease (SD) and AB variant. Here, we present the X-ray crystallographic structure of Hex A to 2.8 Å resolution and the structure of Hex A in complex with NAG-thiazoline, (NGT) to 3.25 Å resolution. NGT, a mechanism-based inhibitor, has been shown to act as a chemical chaperone that, to some extent, prevents misfolding of a Hex A mutant associated with adult onset Tay Sachs disease and, as a result, increases the residual activity of Hex A to a level above the critical threshold for disease. The crystal structure of Hex A reveals an αβ heterodimer, with each subunit having a functional active site. Only the α-subunit active site can hydrolyze GM2 gangliosides due to a flexible loop structure that is removed post-translationally from β, and to the presence of αAsn423 and αArg424. The loop structure is involved in binding the GM2 activator protein, while αArg424 is critical for binding the carboxylate group of the N-acetyl-neuraminic acid residue of GM2. The β-subunit lacks these key residues and has βAsp452 and βLeu453 in their place; the β-subunit therefore cleaves only neutral substrates efficiently. Mutations in the α-subunit, associated with TSD, and those in the β-subunit, associated with SD are discussed. The effect of NGT binding in the active site of a mutant Hex A and its effect on protein function is discussed. PMID:16698036

  9. GM2 gangliosidoses in Spain: analysis of the HEXA and HEXB genes in 34 Tay-Sachs and 14 Sandhoff patients.

    Science.gov (United States)

    Gort, Laura; de Olano, Natalia; Macías-Vidal, Judit; Coll, M A Josep

    2012-09-10

    The GM2 gangliosidoses are autosomal recessive lysosomal storage diseases caused by a deficiency of the β-hexosaminidase A enzyme. This enzyme is composed of two polypeptide chains designated the α- and β- subunits and it interacts with the GM2 activator protein. The HEXA and HEXB genes encode the α-subunit and the β-subunit, respectively. Mutations in these genes are causative of Tay-Sachs disease (HEXA) and Sandhoff disease (HEXB). We analyzed the complete HEXA gene in 34 Spanish patients with Tay-Sachs disease and the HEXB gene in 14 Spanish patients with Sandhoff disease. We identified 27 different mutations, 14 of which were novel, in the HEXA gene and 14 different mutations, 8 of which unreported until now, in the HEXB gene, and we attempted to correlate these mutations with the clinical presentation of the patients. We found a high frequency of c.459+5G>A (IVS4+5G>A) mutation in HEXA affected patients, 22 of 68 alleles, which represent the 32.4%. This is the highest percentage found of this mutation in a population. All patients homozygous for mutation c.459+5G>A presented with the infantile form of the disease and, as previously reported, patients carrying mutation p.R178H in at least one of the alleles presented with a milder form. In HEXB affected patients, the novel deletion c.171delG accounts for 21.4% of the mutant alleles (6/28). All patients with this deletion showed the infantile form of the disease. The Spanish GM2 gangliosidoses affected patients show a great mutational heterogeneity as seen in other inherited lisosomal diseases in this country. Copyright © 2012. Published by Elsevier B.V.

  10. Design, synthesis, and biological evaluation of enantiomeric beta-N-acetylhexosaminidase inhibitors LABNAc and DABNAc as potential agents against Tay-Sachs and Sandhoff disease.

    Science.gov (United States)

    Rountree, J S Shane; Butters, Terry D; Wormald, Mark R; Boomkamp, Stephanie D; Dwek, Raymond A; Asano, Naoki; Ikeda, Kyoko; Evinson, Emma L; Nash, Robert J; Fleet, George W J

    2009-03-01

    N-Acetylhexosaminidases are of considerable importance in mammals and are involved in various significant biological processes. In humans, deficiencies of these enzymes in the lysosome, resulting from inherited genetic defects, cause the glycolipid storage disorders Tay-Sachs and Sandhoff diseases. One promising therapy for these diseases involves the use of beta-N-acetylhexosaminidase inhibitors as chemical chaperones to enhance the enzyme activity above sub-critical levels. Herein we describe the synthesis and biological evaluation of a potent inhibitor, 2-acetamido-1,4-imino-1,2,4-trideoxy-L-arabinitol (LABNAc), in a high-yielding 11-step procedure from D-lyxonolactone. The N-benzyl and N-butyl analogues were also prepared and found to be potent inhibitors. The enantiomers DABNAc and NBn-DABNAc were synthesised from L-lyxonolactone, and were also evaluated. The L-iminosugar LABNAc and its derivatives were found to be potent noncompetitive inhibitors of some beta-N-acetylhexosaminidases, while the D-iminosugar DABNAc and its derivatives were found to be weaker competitive inhibitors. These results support previous work postulating that D-iminosugar mimics inhibit D-glycohydrolases competitively, and that their corresponding L-enantiomers show noncompetitive inhibition of these enzymes. Molecular modelling studies confirm that the spatial organisation in enantiomeric inhibitors leads to a different overlay with the monosaccharide substrate. Initial cell-based studies suggest that NBn-LABNAc can act as a chemical chaperone to enhance the deficient enzyme's activity to levels that may cause a positive pharmacological effect. LABNAc, NBn-LABNAc, and NBu-LABNAc are potent and selective inhibitors of beta-N-acetylhexosaminidase and may be useful as therapeutic agents for treating adult Tay-Sachs and Sandhoff diseases.

  11. Robustness Metrics: Consolidating the multiple approaches to quantify Robustness

    DEFF Research Database (Denmark)

    Göhler, Simon Moritz; Eifler, Tobias; Howard, Thomas J.

    2016-01-01

    robustness metrics; 3) Functional expectancy and dispersion robustness metrics; and 4) Probability of conformance robustness metrics. The goal was to give a comprehensive overview of robustness metrics and guidance to scholars and practitioners to understand the different types of robustness metrics...

  12. Partial rectangular metric spaces and fixed point theorems.

    Science.gov (United States)

    Shukla, Satish

    2014-01-01

    The purpose of this paper is to introduce the concept of partial rectangular metric spaces as a generalization of rectangular metric and partial metric spaces. Some properties of partial rectangular metric spaces and some fixed point results for quasitype contraction in partial rectangular metric spaces are proved. Some examples are given to illustrate the observed results.

  13. Measuring Information Security: Guidelines to Build Metrics

    Science.gov (United States)

    von Faber, Eberhard

    Measuring information security is a genuine interest of security managers. With metrics they can develop their security organization's visibility and standing within the enterprise or public authority as a whole. Organizations using information technology need to use security metrics. Despite the clear demands and advantages, security metrics are often poorly developed or ineffective parameters are collected and analysed. This paper describes best practices for the development of security metrics. First attention is drawn to motivation showing both requirements and benefits. The main body of this paper lists things which need to be observed (characteristic of metrics), things which can be measured (how measurements can be conducted) and steps for the development and implementation of metrics (procedures and planning). Analysis and communication is also key when using security metrics. Examples are also given in order to develop a better understanding. The author wants to resume, continue and develop the discussion about a topic which is or increasingly will be a critical factor of success for any security managers in larger organizations.

  14. Characterising risk - aggregated metrics: radiation and noise

    International Nuclear Information System (INIS)

    Passchier, W.

    1998-01-01

    The characterisation of risk is an important phase in the risk assessment - risk management process. From the multitude of risk attributes a few have to be selected to obtain a risk characteristic or profile that is useful for risk management decisions and implementation of protective measures. One way to reduce the number of attributes is aggregation. In the field of radiation protection such an aggregated metric is firmly established: effective dose. For protection against environmental noise the Health Council of the Netherlands recently proposed a set of aggregated metrics for noise annoyance and sleep disturbance. The presentation will discuss similarities and differences between these two metrics and practical limitations. The effective dose has proven its usefulness in designing radiation protection measures, which are related to the level of risk associated with the radiation practice in question, given that implicit judgements on radiation induced health effects are accepted. However, as the metric does not take into account the nature of radiation practice, it is less useful in policy discussions on the benefits and harm of radiation practices. With respect to the noise exposure metric, only one effect is targeted (annoyance), and the differences between sources are explicitly taken into account. This should make the metric useful in policy discussions with respect to physical planning and siting problems. The metric proposed has only significance on a population level, and can not be used as a predictor for individual risk. (author)

  15. Energy functionals for Calabi-Yau metrics

    International Nuclear Information System (INIS)

    Headrick, M; Nassar, A

    2013-01-01

    We identify a set of ''energy'' functionals on the space of metrics in a given Kähler class on a Calabi-Yau manifold, which are bounded below and minimized uniquely on the Ricci-flat metric in that class. Using these functionals, we recast the problem of numerically solving the Einstein equation as an optimization problem. We apply this strategy, using the ''algebraic'' metrics (metrics for which the Kähler potential is given in terms of a polynomial in the projective coordinates), to the Fermat quartic and to a one-parameter family of quintics that includes the Fermat and conifold quintics. We show that this method yields approximations to the Ricci-flat metric that are exponentially accurate in the degree of the polynomial (except at the conifold point, where the convergence is polynomial), and therefore orders of magnitude more accurate than the balanced metrics, previously studied as approximations to the Ricci-flat metric. The method is relatively fast and easy to implement. On the theoretical side, we also show that the functionals can be used to give a heuristic proof of Yau's theorem

  16. Metrics Are Needed for Collaborative Software Development

    Directory of Open Access Journals (Sweden)

    Mojgan Mohtashami

    2011-10-01

    Full Text Available There is a need for metrics for inter-organizational collaborative software development projects, encompassing management and technical concerns. In particular, metrics are needed that are aimed at the collaborative aspect itself, such as readiness for collaboration, the quality and/or the costs and benefits of collaboration in a specific ongoing project. We suggest questions and directions for such metrics, spanning the full lifespan of a collaborative project, from considering the suitability of collaboration through evaluating ongoing projects to final evaluation of the collaboration.

  17. Indefinite metric fields and the renormalization group

    International Nuclear Information System (INIS)

    Sherry, T.N.

    1976-11-01

    The renormalization group equations are derived for the Green functions of an indefinite metric field theory. In these equations one retains the mass dependence of the coefficient functions, since in the indefinite metric theories the masses cannot be neglected. The behavior of the effective coupling constant in the asymptotic and infrared limits is analyzed. The analysis is illustrated by means of a simple model incorporating indefinite metric fields. The model scales at first order, and at this order also the effective coupling constant has both ultra-violet and infra-red fixed points, the former being the bare coupling constant

  18. Metric learning for DNA microarray data analysis

    International Nuclear Information System (INIS)

    Takeuchi, Ichiro; Nakagawa, Masao; Seto, Masao

    2009-01-01

    In many microarray studies, gene set selection is an important preliminary step for subsequent main task such as tumor classification, cancer subtype identification, etc. In this paper, we investigate the possibility of using metric learning as an alternative to gene set selection. We develop a simple metric learning algorithm aiming to use it for microarray data analysis. Exploiting a property of the algorithm, we introduce a novel approach for extending the metric learning to be adaptive. We apply the algorithm to previously studied microarray data on malignant lymphoma subtype identification.

  19. Software metrics a rigorous and practical approach

    CERN Document Server

    Fenton, Norman

    2014-01-01

    A Framework for Managing, Measuring, and Predicting Attributes of Software Development Products and ProcessesReflecting the immense progress in the development and use of software metrics in the past decades, Software Metrics: A Rigorous and Practical Approach, Third Edition provides an up-to-date, accessible, and comprehensive introduction to software metrics. Like its popular predecessors, this third edition discusses important issues, explains essential concepts, and offers new approaches for tackling long-standing problems.New to the Third EditionThis edition contains new material relevant

  20. Metrics, Media and Advertisers: Discussing Relationship

    Directory of Open Access Journals (Sweden)

    Marco Aurelio de Souza Rodrigues

    2014-11-01

    Full Text Available This study investigates how Brazilian advertisers are adapting to new media and its attention metrics. In-depth interviews were conducted with advertisers in 2009 and 2011. In 2009, new media and its metrics were celebrated as innovations that would increase advertising campaigns overall efficiency. In 2011, this perception has changed: New media’s profusion of metrics, once seen as an advantage, started to compromise its ease of use and adoption. Among its findings, this study argues that there is an opportunity for media groups willing to shift from a product-focused strategy towards a customer-centric one, through the creation of new, simple and integrative metrics

  1. Networks and centroid metrics for understanding football

    African Journals Online (AJOL)

    Gonçalo Dias

    games. However, it seems that the centroid metric, supported only by the position of players in the field ...... the strategy adopted by the coach (Gama et al., 2014). ... centroid distance as measures of team's tactical performance in youth football.

  2. Clean Cities Annual Metrics Report 2009 (Revised)

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, C.

    2011-08-01

    Document provides Clean Cities coalition metrics about the use of alternative fuels; the deployment of alternative fuel vehicles, hybrid electric vehicles (HEVs), and idle reduction initiatives; fuel economy activities; and programs to reduce vehicle miles driven.

  3. Metric Guidelines Inservice and/or Preservice

    Science.gov (United States)

    Granito, Dolores

    1978-01-01

    Guidelines are given for designing teacher training for going metric. The guidelines were developed from existing guidelines, journal articles, a survey of colleges, and the detailed reactions of a panel. (MN)

  4. Science and Technology Metrics and Other Thoughts

    National Research Council Canada - National Science Library

    Harman, Wayne; Staton, Robin

    2006-01-01

    This report explores the subject of science and technology metrics and other topics to begin to provide Navy managers, as well as scientists and engineers, additional tools and concepts with which to...

  5. Using Activity Metrics for DEVS Simulation Profiling

    Directory of Open Access Journals (Sweden)

    Muzy A.

    2014-01-01

    Full Text Available Activity metrics can be used to profile DEVS models before and during the simulation. It is critical to get good activity metrics of models before and during their simulation. Having a means to compute a-priori activity of components (analytic activity may be worth when simulating a model (or parts of it for the first time. After, during the simulation, analytic activity can be corrected using dynamic one. In this paper, we introduce McCabe cyclomatic complexity metric (MCA to compute analytic activity. Both static and simulation activity metrics have been implemented through a plug-in of the DEVSimPy (DEVS Simulator in Python language environment and applied to DEVS models.

  6. Evaluating and Estimating the WCET Criticality Metric

    DEFF Research Database (Denmark)

    Jordan, Alexander

    2014-01-01

    a programmer (or compiler) from targeting optimizations the right way. A possible resort is to use a metric that targets WCET and which can be efficiently computed for all code parts of a program. Similar to dynamic profiling techniques, which execute code with input that is typically expected...... for the application, based on WCET analysis we can indicate how critical a code fragment is, in relation to the worst-case bound. Computing such a metric on top of static analysis, incurs a certain overhead though, which increases with the complexity of the underlying WCET analysis. We present our approach...... to estimate the Criticality metric, by relaxing the precision of WCET analysis. Through this, we can reduce analysis time by orders of magnitude, while only introducing minor error. To evaluate our estimation approach and share our garnered experience using the metric, we evaluate real-time programs, which...

  7. 16 CFR 1511.8 - Metric references.

    Science.gov (United States)

    2010-01-01

    ... 16 Commercial Practices 2 2010-01-01 2010-01-01 false Metric references. 1511.8 Section 1511.8 Commercial Practices CONSUMER PRODUCT SAFETY COMMISSION FEDERAL HAZARDOUS SUBSTANCES ACT REGULATIONS... parentheses for convenience and information only. ...

  8. Flight Crew State Monitoring Metrics, Phase I

    Data.gov (United States)

    National Aeronautics and Space Administration — eSky will develop specific crew state metrics based on the timeliness, tempo and accuracy of pilot inputs required by the H-mode Flight Control System (HFCS)....

  9. Supplier selection using different metric functions

    Directory of Open Access Journals (Sweden)

    Omosigho S.E.

    2015-01-01

    Full Text Available Supplier selection is an important component of supply chain management in today’s global competitive environment. Hence, the evaluation and selection of suppliers have received considerable attention in the literature. Many attributes of suppliers, other than cost, are considered in the evaluation and selection process. Therefore, the process of evaluation and selection of suppliers is a multi-criteria decision making process. The methodology adopted to solve the supplier selection problem is intuitionistic fuzzy TOPSIS (Technique for Order Preference by Similarity to the Ideal Solution. Generally, TOPSIS is based on the concept of minimum distance from the positive ideal solution and maximum distance from the negative ideal solution. We examine the deficiencies of using only one metric function in TOPSIS and propose the use of spherical metric function in addition to the commonly used metric functions. For empirical supplier selection problems, more than one metric function should be used.

  10. Classroom reconstruction of the Schwarzschild metric

    OpenAIRE

    Kassner, Klaus

    2015-01-01

    A promising way to introduce general relativity in the classroom is to study the physical implications of certain given metrics, such as the Schwarzschild one. This involves lower mathematical expenditure than an approach focusing on differential geometry in its full glory and permits to emphasize physical aspects before attacking the field equations. Even so, in terms of motivation, lacking justification of the metric employed may pose an obstacle. The paper discusses how to establish the we...

  11. Marketing communication metrics for social media

    OpenAIRE

    Töllinen, Aarne; Karjaluoto, Heikki

    2011-01-01

    The objective of this paper is to develop a conceptual framework for measuring the effectiveness of social media marketing communications. Specifically, we study whether the existing marketing communications performance metrics are still valid in the changing digitalised communications landscape, or whether it is time to rethink them, or even to devise entirely new metrics. Recent advances in information technology and marketing bring a need to re-examine measurement models. We combine two im...

  12. Some observations on a fuzzy metric space

    Energy Technology Data Exchange (ETDEWEB)

    Gregori, V.

    2017-07-01

    Let $(X,d)$ be a metric space. In this paper we provide some observations about the fuzzy metric space in the sense of Kramosil and Michalek $(Y,N,/wedge)$, where $Y$ is the set of non-negative real numbers $[0,/infty[$ and $N(x,y,t)=1$ if $d(x,y)/leq t$ and $N(x,y,t)=0$ if $d(x,y)/geq t$. (Author)

  13. Area Regge calculus and discontinuous metrics

    International Nuclear Information System (INIS)

    Wainwright, Chris; Williams, Ruth M

    2004-01-01

    Taking the triangle areas as independent variables in the theory of Regge calculus can lead to ambiguities in the edge lengths, which can be interpreted as discontinuities in the metric. We construct solutions to area Regge calculus using a triangulated lattice and find that on a spacelike or timelike hypersurface no such discontinuity can arise. On a null hypersurface however, we can have such a situation and the resulting metric can be interpreted as a so-called refractive wave

  14. Relaxed metrics and indistinguishability operators: the relationship

    Energy Technology Data Exchange (ETDEWEB)

    Martin, J.

    2017-07-01

    In 1982, the notion of indistinguishability operator was introduced by E. Trillas in order to fuzzify the crisp notion of equivalence relation (/cite{Trillas}). In the study of such a class of operators, an outstanding property must be pointed out. Concretely, there exists a duality relationship between indistinguishability operators and metrics. The aforesaid relationship was deeply studied by several authors that introduced a few techniques to generate metrics from indistinguishability operators and vice-versa (see, for instance, /cite{BaetsMesiar,BaetsMesiar2}). In the last years a new generalization of the metric notion has been introduced in the literature with the purpose of developing mathematical tools for quantitative models in Computer Science and Artificial Intelligence (/cite{BKMatthews,Ma}). The aforementioned generalized metrics are known as relaxed metrics. The main target of this talk is to present a study of the duality relationship between indistinguishability operators and relaxed metrics in such a way that the aforementioned classical techniques to generate both concepts, one from the other, can be extended to the new framework. (Author)

  15. Baby universe metric equivalent to an interior black-hole metric

    International Nuclear Information System (INIS)

    Gonzalez-Diaz, P.F.

    1991-01-01

    It is shown that the maximally extended metric corresponding to a large wormhole is the unique possible wormhole metric whose baby universe sector is conformally equivalent ot the maximal inextendible Kruskal metric corresponding to the interior region of a Schwarzschild black hole whose gravitational radius is half the wormhole neck radius. The physical implications of this result in the black hole evaporation process are discussed. (orig.)

  16. The bystander effect model of Brenner and Sachs fitted to lung cancer data in 11 cohorts of underground miners, and equivalence of fit of a linear relative risk model with adjustment for attained age and age at exposure

    International Nuclear Information System (INIS)

    Little, M P

    2004-01-01

    Bystander effects following exposure to α-particles have been observed in many experimental systems, and imply that linearly extrapolating low dose risks from high dose data might materially underestimate risk. Brenner and Sachs (2002 Int. J. Radiat. Biol. 78 593-604; 2003 Health Phys. 85 103-8) have recently proposed a model of the bystander effect which they use to explain the inverse dose rate effect observed for lung cancer in underground miners exposed to radon daughters. In this paper we fit the model of the bystander effect proposed by Brenner and Sachs to 11 cohorts of underground miners, taking account of the covariance structure of the data and the period of latency between the development of the first pre-malignant cell and clinically overt cancer. We also fitted a simple linear relative risk model, with adjustment for age at exposure and attained age. The methods that we use for fitting both models are different from those used by Brenner and Sachs, in particular taking account of the covariance structure, which they did not, and omitting certain unjustifiable adjustments to the miner data. The fit of the original model of Brenner and Sachs (with 0 y period of latency) is generally poor, although it is much improved by assuming a 5 or 6 y period of latency from the first appearance of a pre-malignant cell to cancer. The fit of this latter model is equivalent to that of a linear relative risk model with adjustment for age at exposure and attained age. In particular, both models are capable of describing the observed inverse dose rate effect in this data set

  17. The dynamics of metric-affine gravity

    International Nuclear Information System (INIS)

    Vitagliano, Vincenzo; Sotiriou, Thomas P.; Liberati, Stefano

    2011-01-01

    Highlights: → The role and the dynamics of the connection in metric-affine theories is explored. → The most general second order action does not lead to a dynamical connection. → Including higher order invariants excites new degrees of freedom in the connection. → f(R) actions are also discussed and shown to be a non- representative class. - Abstract: Metric-affine theories of gravity provide an interesting alternative to general relativity: in such an approach, the metric and the affine (not necessarily symmetric) connection are independent quantities. Furthermore, the action should include covariant derivatives of the matter fields, with the covariant derivative naturally defined using the independent connection. As a result, in metric-affine theories a direct coupling involving matter and connection is also present. The role and the dynamics of the connection in such theories is explored. We employ power counting in order to construct the action and search for the minimal requirements it should satisfy for the connection to be dynamical. We find that for the most general action containing lower order invariants of the curvature and the torsion the independent connection does not carry any dynamics. It actually reduces to the role of an auxiliary field and can be completely eliminated algebraically in favour of the metric and the matter field, introducing extra interactions with respect to general relativity. However, we also show that including higher order terms in the action radically changes this picture and excites new degrees of freedom in the connection, making it (or parts of it) dynamical. Constructing actions that constitute exceptions to this rule requires significant fine tuned and/or extra a priori constraints on the connection. We also consider f(R) actions as a particular example in order to show that they constitute a distinct class of metric-affine theories with special properties, and as such they cannot be used as representative toy

  18. Evaluation metrics for biostatistical and epidemiological collaborations.

    Science.gov (United States)

    Rubio, Doris McGartland; Del Junco, Deborah J; Bhore, Rafia; Lindsell, Christopher J; Oster, Robert A; Wittkowski, Knut M; Welty, Leah J; Li, Yi-Ju; Demets, Dave

    2011-10-15

    Increasing demands for evidence-based medicine and for the translation of biomedical research into individual and public health benefit have been accompanied by the proliferation of special units that offer expertise in biostatistics, epidemiology, and research design (BERD) within academic health centers. Objective metrics that can be used to evaluate, track, and improve the performance of these BERD units are critical to their successful establishment and sustainable future. To develop a set of reliable but versatile metrics that can be adapted easily to different environments and evolving needs, we consulted with members of BERD units from the consortium of academic health centers funded by the Clinical and Translational Science Award Program of the National Institutes of Health. Through a systematic process of consensus building and document drafting, we formulated metrics that covered the three identified domains of BERD practices: the development and maintenance of collaborations with clinical and translational science investigators, the application of BERD-related methods to clinical and translational research, and the discovery of novel BERD-related methodologies. In this article, we describe the set of metrics and advocate their use for evaluating BERD practices. The routine application, comparison of findings across diverse BERD units, and ongoing refinement of the metrics will identify trends, facilitate meaningful changes, and ultimately enhance the contribution of BERD activities to biomedical research. Copyright © 2011 John Wiley & Sons, Ltd.

  19. A Metric on Phylogenetic Tree Shapes.

    Science.gov (United States)

    Colijn, C; Plazzotta, G

    2018-01-01

    The shapes of evolutionary trees are influenced by the nature of the evolutionary process but comparisons of trees from different processes are hindered by the challenge of completely describing tree shape. We present a full characterization of the shapes of rooted branching trees in a form that lends itself to natural tree comparisons. We use this characterization to define a metric, in the sense of a true distance function, on tree shapes. The metric distinguishes trees from random models known to produce different tree shapes. It separates trees derived from tropical versus USA influenza A sequences, which reflect the differing epidemiology of tropical and seasonal flu. We describe several metrics based on the same core characterization, and illustrate how to extend the metric to incorporate trees' branch lengths or other features such as overall imbalance. Our approach allows us to construct addition and multiplication on trees, and to create a convex metric on tree shapes which formally allows computation of average tree shapes. © The Author(s) 2017. Published by Oxford University Press, on behalf of the Society of Systematic Biologists.

  20. Future of the PCI Readmission Metric.

    Science.gov (United States)

    Wasfy, Jason H; Yeh, Robert W

    2016-03-01

    Between 2013 and 2014, the Centers for Medicare and Medicaid Services and the National Cardiovascular Data Registry publically reported risk-adjusted 30-day readmission rates after percutaneous coronary intervention (PCI) as a pilot project. A key strength of this public reporting effort included risk adjustment with clinical rather than administrative data. Furthermore, because readmission after PCI is common, expensive, and preventable, this metric has substantial potential to improve quality and value in American cardiology care. Despite this, concerns about the metric exist. For example, few PCI readmissions are caused by procedural complications, limiting the extent to which improved procedural technique can reduce readmissions. Also, similar to other readmission measures, PCI readmission is associated with socioeconomic status and race. Accordingly, the metric may unfairly penalize hospitals that care for underserved patients. Perhaps in the context of these limitations, Centers for Medicare and Medicaid Services has not yet included PCI readmission among metrics that determine Medicare financial penalties. Nevertheless, provider organizations may still wish to focus on this metric to improve value for cardiology patients. PCI readmission is associated with low-risk chest discomfort and patient anxiety. Therefore, patient education, improved triage mechanisms, and improved care coordination offer opportunities to minimize PCI readmissions. Because PCI readmission is common and costly, reducing PCI readmission offers provider organizations a compelling target to improve the quality of care, and also performance in contracts involve shared financial risk. © 2016 American Heart Association, Inc.

  1. g-Weak Contraction in Ordered Cone Rectangular Metric Spaces

    Directory of Open Access Journals (Sweden)

    S. K. Malhotra

    2013-01-01

    Full Text Available We prove some common fixed-point theorems for the ordered g-weak contractions in cone rectangular metric spaces without assuming the normality of cone. Our results generalize some recent results from cone metric and cone rectangular metric spaces into ordered cone rectangular metric spaces. Examples are provided which illustrate the results.

  2. Defining a Progress Metric for CERT RMM Improvement

    Science.gov (United States)

    2017-09-14

    REV-03.18.2016.0 Defining a Progress Metric for CERT-RMM Improvement Gregory Crabb Nader Mehravari David Tobar September 2017 TECHNICAL ...fendable resource allocation decisions. Technical metrics measure aspects of controls implemented through technology (systems, soft- ware, hardware...implementation metric would be the percentage of users who have received anti-phishing training . • Effectiveness/efficiency metrics measure whether

  3. NASA education briefs for the classroom. Metrics in space

    Science.gov (United States)

    The use of metric measurement in space is summarized for classroom use. Advantages of the metric system over the English measurement system are described. Some common metric units are defined, as are special units for astronomical study. International system unit prefixes and a conversion table of metric/English units are presented. Questions and activities for the classroom are recommended.

  4. SOCIAL METRICS APPLIED TO SMART TOURISM

    Directory of Open Access Journals (Sweden)

    O. Cervantes

    2016-09-01

    Full Text Available We present a strategy to make productive use of semantically-related social data, from a user-centered semantic network, in order to help users (tourists and citizens in general to discover cultural heritage, points of interest and available services in a smart city. This data can be used to personalize recommendations in a smart tourism application. Our approach is based on flow centrality metrics typically used in social network analysis: flow betweenness, flow closeness and eccentricity. These metrics are useful to discover relevant nodes within the network yielding nodes that can be interpreted as suggestions (venues or services to users. We describe the semantic network built on graph model, as well as social metrics algorithms used to produce recommendations. We also present challenges and results from a prototypical implementation applied to the case study of the City of Puebla, Mexico.

  5. Landscape pattern metrics and regional assessment

    Science.gov (United States)

    O'Neill, R. V.; Riitters, K.H.; Wickham, J.D.; Jones, K.B.

    1999-01-01

    The combination of remote imagery data, geographic information systems software, and landscape ecology theory provides a unique basis for monitoring and assessing large-scale ecological systems. The unique feature of the work has been the need to develop and interpret quantitative measures of spatial pattern-the landscape indices. This article reviews what is known about the statistical properties of these pattern metrics and suggests some additional metrics based on island biogeography, percolation theory, hierarchy theory, and economic geography. Assessment applications of this approach have required interpreting the pattern metrics in terms of specific environmental endpoints, such as wildlife and water quality, and research into how to represent synergystic effects of many overlapping sources of stress.

  6. A bi-metric theory of gravitation

    International Nuclear Information System (INIS)

    Rosen, N.

    1975-01-01

    The bi-metric theory of gravitation proposed previously is simplified in that the auxiliary conditions are discarded, the two metric tensors being tied together only by means of the boundary conditions. Some of the properties of the field of a particle are investigated; there is no black hole, and it appears that no gravitational collapse can take place. Although the proposed theory and general relativity are at present observationally indistinguishable, some differences are pointed out which may some day be susceptible of observation. An alternative bi-metric theory is considered which gives for the precession of the perihelion 5/6 of the value given by general relativity; it seems less satisfactory than the present theory from the aesthetic point of view. (author)

  7. Steiner trees for fixed orientation metrics

    DEFF Research Database (Denmark)

    Brazil, Marcus; Zachariasen, Martin

    2009-01-01

    We consider the problem of constructing Steiner minimum trees for a metric defined by a polygonal unit circle (corresponding to s = 2 weighted legal orientations in the plane). A linear-time algorithm to enumerate all angle configurations for degree three Steiner points is given. We provide...... a simple proof that the angle configuration for a Steiner point extends to all Steiner points in a full Steiner minimum tree, such that at most six orientations suffice for edges in a full Steiner minimum tree. We show that the concept of canonical forms originally introduced for the uniform orientation...... metric generalises to the fixed orientation metric. Finally, we give an O(s n) time algorithm to compute a Steiner minimum tree for a given full Steiner topology with n terminal leaves....

  8. Metrical and dynamical aspects in complex analysis

    CERN Document Server

    2017-01-01

    The central theme of this reference book is the metric geometry of complex analysis in several variables. Bridging a gap in the current literature, the text focuses on the fine behavior of the Kobayashi metric of complex manifolds and its relationships to dynamical systems, hyperbolicity in the sense of Gromov and operator theory, all very active areas of research. The modern points of view expressed in these notes, collected here for the first time, will be of interest to academics working in the fields of several complex variables and metric geometry. The different topics are treated coherently and include expository presentations of the relevant tools, techniques and objects, which will be particularly useful for graduate and PhD students specializing in the area.

  9. Social Metrics Applied to Smart Tourism

    Science.gov (United States)

    Cervantes, O.; Gutiérrez, E.; Gutiérrez, F.; Sánchez, J. A.

    2016-09-01

    We present a strategy to make productive use of semantically-related social data, from a user-centered semantic network, in order to help users (tourists and citizens in general) to discover cultural heritage, points of interest and available services in a smart city. This data can be used to personalize recommendations in a smart tourism application. Our approach is based on flow centrality metrics typically used in social network analysis: flow betweenness, flow closeness and eccentricity. These metrics are useful to discover relevant nodes within the network yielding nodes that can be interpreted as suggestions (venues or services) to users. We describe the semantic network built on graph model, as well as social metrics algorithms used to produce recommendations. We also present challenges and results from a prototypical implementation applied to the case study of the City of Puebla, Mexico.

  10. Validation of Metrics as Error Predictors

    Science.gov (United States)

    Mendling, Jan

    In this chapter, we test the validity of metrics that were defined in the previous chapter for predicting errors in EPC business process models. In Section 5.1, we provide an overview of how the analysis data is generated. Section 5.2 describes the sample of EPCs from practice that we use for the analysis. Here we discuss a disaggregation by the EPC model group and by error as well as a correlation analysis between metrics and error. Based on this sample, we calculate a logistic regression model for predicting error probability with the metrics as input variables in Section 5.3. In Section 5.4, we then test the regression function for an independent sample of EPC models from textbooks as a cross-validation. Section 5.5 summarizes the findings.

  11. Metric Learning for Hyperspectral Image Segmentation

    Science.gov (United States)

    Bue, Brian D.; Thompson, David R.; Gilmore, Martha S.; Castano, Rebecca

    2011-01-01

    We present a metric learning approach to improve the performance of unsupervised hyperspectral image segmentation. Unsupervised spatial segmentation can assist both user visualization and automatic recognition of surface features. Analysts can use spatially-continuous segments to decrease noise levels and/or localize feature boundaries. However, existing segmentation methods use tasks-agnostic measures of similarity. Here we learn task-specific similarity measures from training data, improving segment fidelity to classes of interest. Multiclass Linear Discriminate Analysis produces a linear transform that optimally separates a labeled set of training classes. The defines a distance metric that generalized to a new scenes, enabling graph-based segmentation that emphasizes key spectral features. We describe tests based on data from the Compact Reconnaissance Imaging Spectrometer (CRISM) in which learned metrics improve segment homogeneity with respect to mineralogical classes.

  12. Kerr metric in the deSitter background

    International Nuclear Information System (INIS)

    Vaidya, P.C.

    1984-01-01

    In addition to the Kerr metric with cosmological constant Λ several other metrics are presented giving a Kerr-like solution of Einstein's equations in the background of deSitter universe. A new metric of what may be termed as rotating deSitter space-time devoid of matter but containing null fluid with twisting null rays, has been presented. This metric reduces to the standard deSitter metric when the twist in the rays vanishes. Kerr metric in this background is the immediate generalization of Schwarzschild's exterior metric with cosmological constant. (author)

  13. Active Metric Learning from Relative Comparisons

    OpenAIRE

    Xiong, Sicheng; Rosales, Rómer; Pei, Yuanli; Fern, Xiaoli Z.

    2014-01-01

    This work focuses on active learning of distance metrics from relative comparison information. A relative comparison specifies, for a data point triplet $(x_i,x_j,x_k)$, that instance $x_i$ is more similar to $x_j$ than to $x_k$. Such constraints, when available, have been shown to be useful toward defining appropriate distance metrics. In real-world applications, acquiring constraints often require considerable human effort. This motivates us to study how to select and query the most useful ...

  14. Heuristic extension of the Schwarzschild metric

    International Nuclear Information System (INIS)

    Espinosa, J.M.

    1982-01-01

    The Schwarzschild solution of Einstein's equations of gravitation has several singularities. It is known that the singularity at r = 2Gm/c 2 is only apparent, a result of the coordinates in which the solution was found. Paradoxical results occuring near the singularity show the system of coordinates is incomplete. We introduce a simple, two-dimensional metric with an apparent singularity that makes it incomplete. By a straightforward, heuristic procedure we extend and complete this simple metric. We then use the same procedure to give a heuristic derivation of the Kruskal system of coordinates, which is known to extend the Schwarzschild manifold past its apparent singularity and produce a complete manifold

  15. Metric inhomogeneous Diophantine approximation in positive characteristic

    DEFF Research Database (Denmark)

    Kristensen, Simon

    2011-01-01

    We obtain asymptotic formulae for the number of solutions to systems of inhomogeneous linear Diophantine inequalities over the field of formal Laurent series with coefficients from a finite fields, which are valid for almost every such system. Here `almost every' is with respect to Haar measure...... of the coefficients of the homogeneous part when the number of variables is at least two (singly metric case), and with respect to the Haar measure of all coefficients for any number of variables (doubly metric case). As consequences, we derive zero-one laws in the spirit of the Khintchine-Groshev Theorem and zero...

  16. Metric inhomogeneous Diophantine approximation in positive characteristic

    DEFF Research Database (Denmark)

    Kristensen, S.

    We obtain asymptotic formulae for the number of solutions to systems of inhomogeneous linear Diophantine inequalities over the field of formal Laurent series with coefficients from a finite fields, which are valid for almost every such system. Here 'almost every' is with respect to Haar measure...... of the coefficients of the homogeneous part when the number of variables is at least two (singly metric case), and with respect to the Haar measure of all coefficients for any number of variables (doubly metric case). As consequences, we derive zero-one laws in the spirit of the Khintchine--Groshev Theorem and zero...

  17. Jacobi-Maupertuis metric and Kepler equation

    Science.gov (United States)

    Chanda, Sumanto; Gibbons, Gary William; Guha, Partha

    This paper studies the application of the Jacobi-Eisenhart lift, Jacobi metric and Maupertuis transformation to the Kepler system. We start by reviewing fundamentals and the Jacobi metric. Then we study various ways to apply the lift to Kepler-related systems: first as conformal description and Bohlin transformation of Hooke’s oscillator, second in contact geometry and third in Houri’s transformation [T. Houri, Liouville integrability of Hamiltonian systems and spacetime symmetry (2016), www.geocities.jp/football_physician/publication.html], coupled with Milnor’s construction [J. Milnor, On the geometry of the Kepler problem, Am. Math. Mon. 90 (1983) 353-365] with eccentric anomaly.

  18. Noncommutative black holes

    Energy Technology Data Exchange (ETDEWEB)

    Lopez-DomInguez, J C [Instituto de Fisica de la Universidad de Guanajuato PO Box E-143, 37150 Leoen Gto. (Mexico); Obregon, O [Instituto de Fisica de la Universidad de Guanajuato PO Box E-143, 37150 Leoen Gto. (Mexico); RamIrez, C [Facultad de Ciencias FIsico Matematicas, Universidad Autonoma de Puebla, PO Box 1364, 72000 Puebla (Mexico); Sabido, M [Instituto de Fisica de la Universidad de Guanajuato PO Box E-143, 37150 Leoen Gto. (Mexico)

    2007-11-15

    We study noncommutative black holes, by using a diffeomorphism between the Schwarzschild black hole and the Kantowski-Sachs cosmological model, which is generalized to noncommutative minisuperspace. Through the use of the Feynman-Hibbs procedure we are able to study the thermodynamics of the black hole, in particular, we calculate Hawking's temperature and entropy for the 'noncommutative' Schwarzschild black hole.

  19. Prader-Willi syndrome and Tay-Sachs disease in association with mixed maternal uniparental isodisomy and heterodisomy 15 in a girl who also had isochromosome Xq.

    Science.gov (United States)

    Zeesman, Susan; McCready, Elizabeth; Sadikovic, Bekim; Nowaczyk, Małgorzata Jm

    2015-01-01

    Malsegregation of chromosomes during reproduction can result in uniparental disomy when associated with trisomy rescue, monosomy rescue or gamete complementation. Pathogenicity stemming from uniparental disomy in liveborns results from imprinting disorders or autozygosity for autosomal recessive disorders. We report on a girl with Prader-Willi syndrome and Tay-Sachs disease resulting from maternal uniparental disomy of chromosome 15. The child also had an isochromosome Xq. To further characterize the etiology of the aberrant chromosome 15 and the isochromosome Xq, SNP loci from both chromosomes were assessed in the proband and parents, and genome-wide DNA methylation analysis was performed. SNP and DNA methylation analysis confirmed maternal uniparental heterodisomy around the Prader-Willi locus, while the region around the HEXA locus showed maternal uniparental isodisomy. This result is consistent with trisomy rescue of a maternal meiosis l error in a chromosome 15 with two meiotic recombinations. SNP analysis of the X chromosomes is consistent with a maternal origin for the isochromosome. © 2014 Wiley Periodicals, Inc.

  20. Molecular analysis of HEXA gene in Argentinean patients affected with Tay-Sachs disease: possible common origin of the prevalent c.459+5A>G mutation.

    Science.gov (United States)

    Zampieri, Stefania; Montalvo, Annalisa; Blanco, Mariana; Zanin, Irene; Amartino, Hernan; Vlahovicek, Kristian; Szlago, Marina; Schenone, Andrea; Pittis, Gabriela; Bembi, Bruno; Dardis, Andrea

    2012-05-15

    Tay-Sachs disease (TSD) is a recessively inherited disorder caused by the deficient activity of hexosaminidase A due to mutations in the HEXA gene. Up to date there is no information regarding the molecular genetics of TSD in Argentinean patients. In the present study we have studied 17 Argentinean families affected by TSD, including 20 patients with the acute infantile form and 3 with the sub-acute form. Overall, we identified 14 different mutations accounting for 100% of the studied alleles. Eight mutations were novel: 5 were single base changes leading to drastic residue changes or truncated proteins, 2 were small deletions and one was an intronic mutation that may cause a splicing defect. Although the spectrum of mutations was highly heterogeneous, a high frequency of the c.459+5G>A mutation, previously described in different populations was found among the studied cohort. Haplotype analysis suggested that in these families the c.459+5G>A mutation might have arisen by a single mutational event. Copyright © 2012 Elsevier B.V. All rights reserved.

  1. Quantitative properties of the Schwarzschild metric

    Czech Academy of Sciences Publication Activity Database

    Křížek, Michal; Křížek, Filip

    2018-01-01

    Roč. 2018, č. 1 (2018), s. 1-10 Institutional support: RVO:67985840 Keywords : exterior and interior Schwarzschild metric * proper radius * coordinate radius Subject RIV: BA - General Mathematics OBOR OECD: Applied mathematics http://astro.shu-bg.net/pasb/index_files/Papers/2018/SCHWARZ8.pdf

  2. Strong Ideal Convergence in Probabilistic Metric Spaces

    Indian Academy of Sciences (India)

    In the present paper we introduce the concepts of strongly ideal convergent sequence and strong ideal Cauchy sequence in a probabilistic metric (PM) space endowed with the strong topology, and establish some basic facts. Next, we define the strong ideal limit points and the strong ideal cluster points of a sequence in this ...

  3. lakemorpho: Calculating lake morphometry metrics in R.

    Science.gov (United States)

    Hollister, Jeffrey; Stachelek, Joseph

    2017-01-01

    Metrics describing the shape and size of lakes, known as lake morphometry metrics, are important for any limnological study. In cases where a lake has long been the subject of study these data are often already collected and are openly available. Many other lakes have these data collected, but access is challenging as it is often stored on individual computers (or worse, in filing cabinets) and is available only to the primary investigators. The vast majority of lakes fall into a third category in which the data are not available. This makes broad scale modelling of lake ecology a challenge as some of the key information about in-lake processes are unavailable. While this valuable in situ information may be difficult to obtain, several national datasets exist that may be used to model and estimate lake morphometry. In particular, digital elevation models and hydrography have been shown to be predictive of several lake morphometry metrics. The R package lakemorpho has been developed to utilize these data and estimate the following morphometry metrics: surface area, shoreline length, major axis length, minor axis length, major and minor axis length ratio, shoreline development, maximum depth, mean depth, volume, maximum lake length, mean lake width, maximum lake width, and fetch. In this software tool article we describe the motivation behind developing lakemorpho , discuss the implementation in R, and describe the use of lakemorpho with an example of a typical use case.

  4. Contraction theorems in fuzzy metric space

    International Nuclear Information System (INIS)

    Farnoosh, R.; Aghajani, A.; Azhdari, P.

    2009-01-01

    In this paper, the results on fuzzy contractive mapping proposed by Dorel Mihet will be proved for B-contraction and C-contraction in the case of George and Veeramani fuzzy metric space. The existence of fixed point with weaker conditions will be proved; that is, instead of the convergence of subsequence, p-convergence of subsequence is used.

  5. Inferring feature relevances from metric learning

    DEFF Research Database (Denmark)

    Schulz, Alexander; Mokbel, Bassam; Biehl, Michael

    2015-01-01

    Powerful metric learning algorithms have been proposed in the last years which do not only greatly enhance the accuracy of distance-based classifiers and nearest neighbor database retrieval, but which also enable the interpretability of these operations by assigning explicit relevance weights...

  6. DIGITAL MARKETING: SUCCESS METRICS, FUTURE TRENDS

    OpenAIRE

    Preeti Kaushik

    2017-01-01

    Abstract – Business Marketing is one of the prospective which has been tremendously affected by digital world in last few years. Digital marketing refers to doing advertising through digital channels. This paper provides detailed study of metrics to measure success of digital marketing platform and glimpse of future of technologies by 2020.

  7. Assessing Software Quality Through Visualised Cohesion Metrics

    Directory of Open Access Journals (Sweden)

    Timothy Shih

    2001-05-01

    Full Text Available Cohesion is one of the most important factors for software quality as well as maintainability, reliability and reusability. Module cohesion is defined as a quality attribute that seeks for measuring the singleness of the purpose of a module. The module of poor quality can be a serious obstacle to the system quality. In order to design a good software quality, software managers and engineers need to introduce cohesion metrics to measure and produce desirable software. A highly cohesion software is thought to be a desirable constructing. In this paper, we propose a function-oriented cohesion metrics based on the analysis of live variables, live span and the visualization of processing element dependency graph. We give six typical cohesion examples to be measured as our experiments and justification. Therefore, a well-defined, well-normalized, well-visualized and well-experimented cohesion metrics is proposed to indicate and thus enhance software cohesion strength. Furthermore, this cohesion metrics can be easily incorporated with software CASE tool to help software engineers to improve software quality.

  8. Metric propositional neighborhood logics on natural numbers

    DEFF Research Database (Denmark)

    Bresolin, Davide; Della Monica, Dario; Goranko, Valentin

    2013-01-01

    Metric Propositional Neighborhood Logic (MPNL) over natural numbers. MPNL features two modalities referring, respectively, to an interval that is “met by” the current one and to an interval that “meets” the current one, plus an infinite set of length constraints, regarded as atomic propositions...

  9. Calabi–Yau metrics and string compactification

    Directory of Open Access Journals (Sweden)

    Michael R. Douglas

    2015-09-01

    Full Text Available Yau proved an existence theorem for Ricci-flat Kähler metrics in the 1970s, but we still have no closed form expressions for them. Nevertheless there are several ways to get approximate expressions, both numerical and analytical. We survey some of this work and explain how it can be used to obtain physical predictions from superstring theory.

  10. Goedel-type metrics in various dimensions

    International Nuclear Information System (INIS)

    Guerses, Metin; Karasu, Atalay; Sarioglu, Oezguer

    2005-01-01

    Goedel-type metrics are introduced and used in producing charged dust solutions in various dimensions. The key ingredient is a (D - 1)-dimensional Riemannian geometry which is then employed in constructing solutions to the Einstein-Maxwell field equations with a dust distribution in D dimensions. The only essential field equation in the procedure turns out to be the source-free Maxwell's equation in the relevant background. Similarly the geodesics of this type of metric are described by the Lorentz force equation for a charged particle in the lower dimensional geometry. It is explicitly shown with several examples that Goedel-type metrics can be used in obtaining exact solutions to various supergravity theories and in constructing spacetimes that contain both closed timelike and closed null curves and that contain neither of these. Among the solutions that can be established using non-flat backgrounds, such as the Tangherlini metrics in (D - 1)-dimensions, there exists a class which can be interpreted as describing black-hole-type objects in a Goedel-like universe

  11. Strong Statistical Convergence in Probabilistic Metric Spaces

    OpenAIRE

    Şençimen, Celaleddin; Pehlivan, Serpil

    2008-01-01

    In this article, we introduce the concepts of strongly statistically convergent sequence and strong statistically Cauchy sequence in a probabilistic metric (PM) space endowed with the strong topology, and establish some basic facts. Next, we define the strong statistical limit points and the strong statistical cluster points of a sequence in this space and investigate the relations between these concepts.

  12. Language Games: University Responses to Ranking Metrics

    Science.gov (United States)

    Heffernan, Troy A.; Heffernan, Amanda

    2018-01-01

    League tables of universities that measure performance in various ways are now commonplace, with numerous bodies providing their own rankings of how institutions throughout the world are seen to be performing on a range of metrics. This paper uses Lyotard's notion of language games to theorise that universities are regaining some power over being…

  13. A new universal colour image fidelity metric

    NARCIS (Netherlands)

    Toet, A.; Lucassen, M.P.

    2003-01-01

    We extend a recently introduced universal grayscale image quality index to a newly developed perceptually decorrelated colour space. The resulting colour image fidelity metric quantifies the distortion of a processed colour image relative to its original version. We evaluated the new colour image

  14. Standardised metrics for global surgical surveillance.

    Science.gov (United States)

    Weiser, Thomas G; Makary, Martin A; Haynes, Alex B; Dziekan, Gerald; Berry, William R; Gawande, Atul A

    2009-09-26

    Public health surveillance relies on standardised metrics to evaluate disease burden and health system performance. Such metrics have not been developed for surgical services despite increasing volume, substantial cost, and high rates of death and disability associated with surgery. The Safe Surgery Saves Lives initiative of WHO's Patient Safety Programme has developed standardised public health metrics for surgical care that are applicable worldwide. We assembled an international panel of experts to develop and define metrics for measuring the magnitude and effect of surgical care in a population, while taking into account economic feasibility and practicability. This panel recommended six measures for assessing surgical services at a national level: number of operating rooms, number of operations, number of accredited surgeons, number of accredited anaesthesia professionals, day-of-surgery death ratio, and postoperative in-hospital death ratio. We assessed the feasibility of gathering such statistics at eight diverse hospitals in eight countries and incorporated them into the WHO Guidelines for Safe Surgery, in which methods for data collection, analysis, and reporting are outlined.

  15. A Lagrangian-dependent metric space

    International Nuclear Information System (INIS)

    El-Tahir, A.

    1989-08-01

    A generalized Lagrangian-dependent metric of the static isotropic spacetime is derived. Its behaviour should be governed by imposing physical constraints allowing to avert the pathological features of gravity at the strong field domain. This would restrict the choice of the Lagrangian form. (author). 10 refs

  16. Clean Cities 2011 Annual Metrics Report

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, C.

    2012-12-01

    This report details the petroleum savings and vehicle emissions reductions achieved by the U.S. Department of Energy's Clean Cities program in 2011. The report also details other performance metrics, including the number of stakeholders in Clean Cities coalitions, outreach activities by coalitions and national laboratories, and alternative fuel vehicles deployed.

  17. Clean Cities 2010 Annual Metrics Report

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, C.

    2012-10-01

    This report details the petroleum savings and vehicle emissions reductions achieved by the U.S. Department of Energy's Clean Cities program in 2010. The report also details other performance metrics, including the number of stakeholders in Clean Cities coalitions, outreach activities by coalitions and national laboratories, and alternative fuel vehicles deployed.

  18. Genetic basis of a cognitive complexity metric

    NARCIS (Netherlands)

    Hansell, Narelle K; Halford, Graeme S; Andrews, Glenda; Shum, David H K; Harris, Sarah E; Davies, Gail; Franic, Sanja; Christoforou, Andrea; Zietsch, Brendan; Painter, Jodie; Medland, Sarah E; Ehli, Erik A; Davies, Gareth E; Steen, Vidar M; Lundervold, Astri J; Reinvang, Ivar; Montgomery, Grant W; Espeseth, Thomas; Hulshoff Pol, Hilleke E; Starr, John M; Martin, Nicholas G; Le Hellard, Stephanie; Boomsma, Dorret I; Deary, Ian J; Wright, Margaret J

    2015-01-01

    Relational complexity (RC) is a metric reflecting capacity limitation in relational processing. It plays a crucial role in higher cognitive processes and is an endophenotype for several disorders. However, the genetic underpinnings of complex relational processing have not been investigated. Using

  19. Genetic Basis of a Cognitive Complexity Metric

    NARCIS (Netherlands)

    Hansell, N.K.; Halford, G.S.; Andrews, G.; Shum, D.H.K.; Harris, S.E.; Davies, G.; Franic, S.; Christoforou, A.; Zietsch, B.; Painter, J.; Medland, S.E.; Ehli, E.A.; Davies, G.E.; Steen, V.M.; Lundervold, A.J.; Reinvang, I.; Montgomery, G.W.; Espeseth, T.; Hulshoff Pol, H.E.; Starr, J.M.; Martin, N.G.; Le Hellard, S.; Boomsma, D.I.; Deary, I.J.; Wright, M.J.

    2015-01-01

    Relational complexity (RC) is a metric reflecting capacity limitation in relational processing. It plays a crucial role in higher cognitive processes and is an endophenotype for several disorders. However, the genetic underpinnings of complex relational processing have not been investigated. Using

  20. Business model metrics : An open repository

    NARCIS (Netherlands)

    Heikkila, M.; Bouwman, W.A.G.A.; Heikkila, J.; Solaimani, S.; Janssen, W.

    2015-01-01

    Development of successful business models has become a necessity in turbulent business environments, but compared to research on business modeling tools, attention to the role of metrics in designing business models in literature is limited. Building on existing approaches to business models and

  1. Software quality metrics aggregation in industry

    NARCIS (Netherlands)

    Mordal, K.; Anquetil, N.; Laval, J.; Serebrenik, A.; Vasilescu, B.N.; Ducasse, S.

    2013-01-01

    With the growing need for quality assessment of entire software systems in the industry, new issues are emerging. First, because most software quality metrics are defined at the level of individual software components, there is a need for aggregation methods to summarize the results at the system

  2. Invariance group of the Finster metric function

    International Nuclear Information System (INIS)

    Asanov, G.S.

    1985-01-01

    An invariance group of the Finsler metric function is introduced and studied that directly generalized the respective concept (a group of Euclidean rolations) of the Rieman geometry. A sequential description of the isotopic invariance of physical fields on the base of the Finsler geometry is possible in terms of this group

  3. Sigma Routing Metric for RPL Protocol

    Directory of Open Access Journals (Sweden)

    Paul Sanmartin

    2018-04-01

    Full Text Available This paper presents the adaptation of a specific metric for the RPL protocol in the objective function MRHOF. Among the functions standardized by IETF, we find OF0, which is based on the minimum hop count, as well as MRHOF, which is based on the Expected Transmission Count (ETX. However, when the network becomes denser or the number of nodes increases, both OF0 and MRHOF introduce long hops, which can generate a bottleneck that restricts the network. The adaptation is proposed to optimize both OFs through a new routing metric. To solve the above problem, the metrics of the minimum number of hops and the ETX are combined by designing a new routing metric called SIGMA-ETX, in which the best route is calculated using the standard deviation of ETX values between each node, as opposed to working with the ETX average along the route. This method ensures a better routing performance in dense sensor networks. The simulations are done through the Cooja simulator, based on the Contiki operating system. The simulations showed that the proposed optimization outperforms at a high margin in both OF0 and MRHOF, in terms of network latency, packet delivery ratio, lifetime, and power consumption.

  4. Observable traces of non-metricity: New constraints on metric-affine gravity

    Science.gov (United States)

    Delhom-Latorre, Adrià; Olmo, Gonzalo J.; Ronco, Michele

    2018-05-01

    Relaxing the Riemannian condition to incorporate geometric quantities such as torsion and non-metricity may allow to explore new physics associated with defects in a hypothetical space-time microstructure. Here we show that non-metricity produces observable effects in quantum fields in the form of 4-fermion contact interactions, thereby allowing us to constrain the scale of non-metricity to be greater than 1 TeV by using results on Bahbah scattering. Our analysis is carried out in the framework of a wide class of theories of gravity in the metric-affine approach. The bound obtained represents an improvement of several orders of magnitude to previous experimental constraints.

  5. Conformal and related changes of metric on the product of two almost contact metric manifolds.

    OpenAIRE

    Blair, D. E.

    1990-01-01

    This paper studies conformal and related changes of the product metric on the product of two almost contact metric manifolds. It is shown that if one factor is Sasakian, the other is not, but that locally the second factor is of the type studied by Kenmotsu. The results are more general and given in terms of trans-Sasakian, α-Sasakian and β-Kenmotsu structures.

  6. Metrics for measuring distances in configuration spaces

    International Nuclear Information System (INIS)

    Sadeghi, Ali; Ghasemi, S. Alireza; Schaefer, Bastian; Mohr, Stephan; Goedecker, Stefan; Lill, Markus A.

    2013-01-01

    In order to characterize molecular structures we introduce configurational fingerprint vectors which are counterparts of quantities used experimentally to identify structures. The Euclidean distance between the configurational fingerprint vectors satisfies the properties of a metric and can therefore safely be used to measure dissimilarities between configurations in the high dimensional configuration space. In particular we show that these metrics are a perfect and computationally cheap replacement for the root-mean-square distance (RMSD) when one has to decide whether two noise contaminated configurations are identical or not. We introduce a Monte Carlo approach to obtain the global minimum of the RMSD between configurations, which is obtained from a global minimization over all translations, rotations, and permutations of atomic indices

  7. A perceptual metric for photo retouching.

    Science.gov (United States)

    Kee, Eric; Farid, Hany

    2011-12-13

    In recent years, advertisers and magazine editors have been widely criticized for taking digital photo retouching to an extreme. Impossibly thin, tall, and wrinkle- and blemish-free models are routinely splashed onto billboards, advertisements, and magazine covers. The ubiquity of these unrealistic and highly idealized images has been linked to eating disorders and body image dissatisfaction in men, women, and children. In response, several countries have considered legislating the labeling of retouched photos. We describe a quantitative and perceptually meaningful metric of photo retouching. Photographs are rated on the degree to which they have been digitally altered by explicitly modeling and estimating geometric and photometric changes. This metric correlates well with perceptual judgments of photo retouching and can be used to objectively judge by how much a retouched photo has strayed from reality.

  8. Metric-Aware Secure Service Orchestration

    Directory of Open Access Journals (Sweden)

    Gabriele Costa

    2012-12-01

    Full Text Available Secure orchestration is an important concern in the internet of service. Next to providing the required functionality the composite services must also provide a reasonable level of security in order to protect sensitive data. Thus, the orchestrator has a need to check whether the complex service is able to satisfy certain properties. Some properties are expressed with metrics for precise definition of requirements. Thus, the problem is to analyse the values of metrics for a complex business process. In this paper we extend our previous work on analysis of secure orchestration with quantifiable properties. We show how to define, verify and enforce quantitative security requirements in one framework with other security properties. The proposed approach should help to select the most suitable service architecture and guarantee fulfilment of the declared security requirements.

  9. Machine Learning for ATLAS DDM Network Metrics

    CERN Document Server

    Lassnig, Mario; The ATLAS collaboration; Vamosi, Ralf

    2016-01-01

    The increasing volume of physics data is posing a critical challenge to the ATLAS experiment. In anticipation of high luminosity physics, automation of everyday data management tasks has become necessary. Previously many of these tasks required human decision-making and operation. Recent advances in hardware and software have made it possible to entrust more complicated duties to automated systems using models trained by machine learning algorithms. In this contribution we show results from our ongoing automation efforts. First, we describe our framework for distributed data management and network metrics, automatically extract and aggregate data, train models with various machine learning algorithms, and eventually score the resulting models and parameters. Second, we use these models to forecast metrics relevant for network-aware job scheduling and data brokering. We show the characteristics of the data and evaluate the forecasting accuracy of our models.

  10. Beyond Lovelock gravity: Higher derivative metric theories

    Science.gov (United States)

    Crisostomi, M.; Noui, K.; Charmousis, C.; Langlois, D.

    2018-02-01

    We consider theories describing the dynamics of a four-dimensional metric, whose Lagrangian is diffeomorphism invariant and depends at most on second derivatives of the metric. Imposing degeneracy conditions we find a set of Lagrangians that, apart form the Einstein-Hilbert one, are either trivial or contain more than 2 degrees of freedom. Among the partially degenerate theories, we recover Chern-Simons gravity, endowed with constraints whose structure suggests the presence of instabilities. Then, we enlarge the class of parity violating theories of gravity by introducing new "chiral scalar-tensor theories." Although they all raise the same concern as Chern-Simons gravity, they can nevertheless make sense as low energy effective field theories or, by restricting them to the unitary gauge (where the scalar field is uniform), as Lorentz breaking theories with a parity violating sector.

  11. High-Dimensional Metrics in R

    OpenAIRE

    Chernozhukov, Victor; Hansen, Chris; Spindler, Martin

    2016-01-01

    The package High-dimensional Metrics (\\Rpackage{hdm}) is an evolving collection of statistical methods for estimation and quantification of uncertainty in high-dimensional approximately sparse models. It focuses on providing confidence intervals and significance testing for (possibly many) low-dimensional subcomponents of the high-dimensional parameter vector. Efficient estimators and uniformly valid confidence intervals for regression coefficients on target variables (e.g., treatment or poli...

  12. Interiors of Vaidya's radiating metric: Gravitational collapse

    International Nuclear Information System (INIS)

    Fayos, F.; Jaen, X.; Llanta, E.; Senovilla, J.M.M.

    1992-01-01

    Using the Darmois junction conditions, we give the necessary and sufficient conditions for the matching of a general spherically symmetric metric to a Vaidya radiating solution. We present also these conditions in terms of the physical quantities of the corresponding energy-momentum tensors. The physical interpretation of the results and their possible applications are studied, and we also perform a detailed analysis of previous work on the subject by other authors

  13. Anisotropic rectangular metric for polygonal surface remeshing

    KAUST Repository

    Pellenard, Bertrand

    2013-06-18

    We propose a new method for anisotropic polygonal surface remeshing. Our algorithm takes as input a surface triangle mesh. An anisotropic rectangular metric, defined at each triangle facet of the input mesh, is derived from both a user-specified normal-based tolerance error and the requirement to favor rectangle-shaped polygons. Our algorithm uses a greedy optimization procedure that adds, deletes and relocates generators so as to match two criteria related to partitioning and conformity.

  14. A Metrics Approach for Collaborative Systems

    Directory of Open Access Journals (Sweden)

    Cristian CIUREA

    2009-01-01

    Full Text Available This article presents different types of collaborative systems, their structure and classification. This paper defines the concept of virtual campus as a collaborative system. It builds architecture for virtual campus oriented on collaborative training processes. It analyses the quality characteristics of collaborative systems and propose techniques for metrics construction and validation in order to evaluate them. The article analyzes different ways to increase the efficiency and the performance level in collaborative banking systems.

  15. Preserved Network Metrics across Translated Texts

    Science.gov (United States)

    Cabatbat, Josephine Jill T.; Monsanto, Jica P.; Tapang, Giovanni A.

    2014-09-01

    Co-occurrence language networks based on Bible translations and the Universal Declaration of Human Rights (UDHR) translations in different languages were constructed and compared with random text networks. Among the considered network metrics, the network size, N, the normalized betweenness centrality (BC), and the average k-nearest neighbors, knn, were found to be the most preserved across translations. Moreover, similar frequency distributions of co-occurring network motifs were observed for translated texts networks.

  16. Anisotropic rectangular metric for polygonal surface remeshing

    KAUST Repository

    Pellenard, Bertrand; Morvan, Jean-Marie; Alliez, Pierre

    2013-01-01

    We propose a new method for anisotropic polygonal surface remeshing. Our algorithm takes as input a surface triangle mesh. An anisotropic rectangular metric, defined at each triangle facet of the input mesh, is derived from both a user-specified normal-based tolerance error and the requirement to favor rectangle-shaped polygons. Our algorithm uses a greedy optimization procedure that adds, deletes and relocates generators so as to match two criteria related to partitioning and conformity.

  17. Smart Grid Status and Metrics Report

    Energy Technology Data Exchange (ETDEWEB)

    Balducci, Patrick J. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Weimar, Mark R. [Pacific Northwest National Lab. (PNNL), Richland, WA (United States); Kirkham, Harold [Pacific Northwest National Lab. (PNNL), Richland, WA (United States)

    2014-07-01

    To convey progress made in achieving the vision of a smart grid, this report uses a set of six characteristics derived from the National Energy Technology Laboratory Modern Grid Strategy. It measures 21 metrics to provide insight into the grid’s capacity to embody these characteristics. This report looks across a spectrum of smart grid concerns to measure the status of smart grid deployment and impacts.

  18. Metrics in Keplerian orbits quotient spaces

    Science.gov (United States)

    Milanov, Danila V.

    2018-03-01

    Quotient spaces of Keplerian orbits are important instruments for the modelling of orbit samples of celestial bodies on a large time span. We suppose that variations of the orbital eccentricities, inclinations and semi-major axes remain sufficiently small, while arbitrary perturbations are allowed for the arguments of pericentres or longitudes of the nodes, or both. The distance between orbits or their images in quotient spaces serves as a numerical criterion for such problems of Celestial Mechanics as search for common origin of meteoroid streams, comets, and asteroids, asteroid families identification, and others. In this paper, we consider quotient sets of the non-rectilinear Keplerian orbits space H. Their elements are identified irrespective of the values of pericentre arguments or node longitudes. We prove that distance functions on the quotient sets, introduced in Kholshevnikov et al. (Mon Not R Astron Soc 462:2275-2283, 2016), satisfy metric space axioms and discuss theoretical and practical importance of this result. Isometric embeddings of the quotient spaces into R^n, and a space of compact subsets of H with Hausdorff metric are constructed. The Euclidean representations of the orbits spaces find its applications in a problem of orbit averaging and computational algorithms specific to Euclidean space. We also explore completions of H and its quotient spaces with respect to corresponding metrics and establish a relation between elements of the extended spaces and rectilinear trajectories. Distance between an orbit and subsets of elliptic and hyperbolic orbits is calculated. This quantity provides an upper bound for the metric value in a problem of close orbits identification. Finally the invariance of the equivalence relations in H under coordinates change is discussed.

  19. The Planck Vacuum and the Schwarzschild Metrics

    Directory of Open Access Journals (Sweden)

    Daywitt W. C.

    2009-07-01

    Full Text Available The Planck vacuum (PV is assumed to be the source of the visible universe. So under conditions of sufficient stress, there must exist a pathway through which energy from the PV can travel into this universe. Conversely, the passage of energy from the visible universe to the PV must also exist under the same stressful conditions. The following examines two versions of the Schwarzschild metric equation for compatability with this open-pathway idea.

  20. Metrics and Its Function in Poetry

    Institute of Scientific and Technical Information of China (English)

    XIAO Zhong-qiong; CHEN Min-jie

    2013-01-01

    Poetry is a special combination of musical and linguistic qualities-of sounds both regarded as pure sound and as mean-ingful speech. Part of the pleasure of poetry lies in its relationship with music. Metrics, including rhythm and meter, is an impor-tant method for poetry to express poetic sentiment. Through the introduction of poetic language and typical examples, the writer of this paper tries to discuss the relationship between sound and meaning.

  1. Quantum cosmology of a Bianchi III LRS geometry coupled to a source free electromagnetic field

    Science.gov (United States)

    Karagiorgos, A.; Pailas, T.; Dimakis, N.; Terzis, Petros A.; Christodoulakis, T.

    2018-03-01

    We consider a Bianchi type III axisymmetric geometry in the presence of an electromagnetic field. A first result at the classical level is that the symmetry of the geometry need not be applied on the electromagnetic tensor Fμν the algebraic restrictions, implied by the Einstein field equations to the stress energy tensor Tμν, suffice to reduce the general Fμν to the appropriate form. The classical solution thus found contains a time dependent electric and a constant magnetic charge. The solution is also reachable from the corresponding mini-superspace action, which is strikingly similar to the Reissner-Nordstr{öm one. This points to a connection between the black hole geometry and the cosmological solution here found, which is the analog of the known correlation between the Schwarzschild and the Kantowski-Sachs metrics. The configuration space is drastically modified by the presence of the magnetic charge from a 3D flat to a 3D pp wave geometry. We map the emerging linear and quadratic classical integrals of motion, to quantum observables. Along with the Wheeler-DeWitt equation these observables provide unique, up to constants, wave functions. The employment of a Bohmian interpretation of these quantum states results in deterministic (semi-classical) geometries most of which are singularity free.

  2. Image characterization metrics for muon tomography

    Science.gov (United States)

    Luo, Weidong; Lehovich, Andre; Anashkin, Edward; Bai, Chuanyong; Kindem, Joel; Sossong, Michael; Steiger, Matt

    2014-05-01

    Muon tomography uses naturally occurring cosmic rays to detect nuclear threats in containers. Currently there are no systematic image characterization metrics for muon tomography. We propose a set of image characterization methods to quantify the imaging performance of muon tomography. These methods include tests of spatial resolution, uniformity, contrast, signal to noise ratio (SNR) and vertical smearing. Simulated phantom data and analysis methods were developed to evaluate metric applicability. Spatial resolution was determined as the FWHM of the point spread functions in X, Y and Z axis for 2.5cm tungsten cubes. Uniformity was measured by drawing a volume of interest (VOI) within a large water phantom and defined as the standard deviation of voxel values divided by the mean voxel value. Contrast was defined as the peak signals of a set of tungsten cubes divided by the mean voxel value of the water background. SNR was defined as the peak signals of cubes divided by the standard deviation (noise) of the water background. Vertical smearing, i.e. vertical thickness blurring along the zenith axis for a set of 2 cm thick tungsten plates, was defined as the FWHM of vertical spread function for the plate. These image metrics provided a useful tool to quantify the basic imaging properties for muon tomography.

  3. Common HEXB polymorphisms reduce serum HexA and HexB enzymatic activities, potentially masking Tay-Sachs disease carrier identification.

    Science.gov (United States)

    Vallance, Hilary; Morris, Tara J; Coulter-Mackie, Marion; Lim-Steele, Joyce; Kaback, Michael

    2006-02-01

    A DNA-proven Tay-Sachs disease (TSD) carrier and his brother were found to have serum percent Hexosaminidase A (%HexA) enzymatic activities in the non-carrier range, while the leukocyte %HexA profiles clearly identified them as TSD heterozygotes. Both their serum HexA and HexB enzymatic activities were below reference range, suggesting inheritance of mutations in both the HEXA (alpha-subunit) and HEXB (beta-subunit) genes. DNA sequencing revealed that both individuals, carried the common HEXA 1277_1278insTATC mutation, and two common HEXB polymorphisms: [619A>G (+) delTG]. To determine if these HEXB polymorphisms reduce HexA and HexB enzymatic activities, 69 DNA samples from subjects previously screened enzymatically in both serum and leukocytes for TSD carrier status were selected for either high, mid-range or low serum Total Hex (defined as the sum of HexA and HexB) activities and were tested for the HEXB mutations. Further, three additional TSD carriers ascertained by the atypical pattern of normal serum %HexA but carrier leukocyte %HexA, were found to have the [delTG (+) 619A>G] genotype. In addition, the frequency of the [delTG (+) 619A>G] genotype was significantly higher (P G] haplotype in the Ashkenazi Jewish population (approximately 10%), up to 10% of TSD carriers may have normal serum %HexA values with low total Hex. Accordingly, serum %HexA should not be the sole criterion used for carrier status determination. Where total Hex activity is reduced, further testing with leukocyte Hex profiles is indicated.

  4. Population-based Tay-Sachs screening among Ashkenazi Jewish young adults in the 21st century: Hexosaminidase A enzyme assay is essential for accurate testing.

    Science.gov (United States)

    Schneider, Adele; Nakagawa, Sachiko; Keep, Rosanne; Dorsainville, Darnelle; Charrow, Joel; Aleck, Kirk; Hoffman, Jodi; Minkoff, Sherman; Finegold, David; Sun, Wei; Spencer, Andrew; Lebow, Johannah; Zhan, Jie; Apfelroth, Stephen; Schreiber-Agus, Nicole; Gross, Susan

    2009-11-01

    Tay-Sachs disease (TSD) carrier screening, initiated in the 1970s, has reduced the birth-rate of Ashkenazi Jews with TSD worldwide by 90%. Recently, several nationwide programs have been established that provide carrier screening for the updated panel of Jewish genetic diseases on college campuses and in Jewish community settings. The goals of this study were to determine the performance characteristics of clinical TSD testing in college- and community-based screening programs and to determine if molecular testing alone is adequate in those settings. Clinical data for TSD testing were retrospectively anonymized and subsequently analyzed for 1,036 individuals who participated in these programs. The performance characteristics of the serum and the platelet Hexosaminidase assays were compared, and also correlated with the results of targeted DNA analysis. The serum assay identified 29 carriers and the platelet assay identified 35 carriers for carrier rates of 1/36 and 1/29, respectively. One hundred sixty-nine samples (16.3%) were inconclusive by serum assay in marked contrast to four inconclusive samples (0.4%) by the platelet assay. Molecular analysis alone would have missed four of the 35 carriers detected by the platelet assay, yielding a false negative rate of 11.4% with a sensitivity of 88.6%. Based on the results of this study, platelet assay was superior to serum with a minimal inconclusive rate. Due to changing demographics of the Ashkenazi Jewish population, molecular testing alone in the setting of broad-based population screening programs is not sufficient, and biochemical analysis should be the assay of choice. Copyright 2009 Wiley-Liss, Inc.

  5. Origin and spread of the 1278insTATC mutation causing Tay-Sachs disease in Ashkenazi Jews: genetic drift as a robust and parsimonious hypothesis.

    Science.gov (United States)

    Frisch, Amos; Colombo, Roberto; Michaelovsky, Elena; Karpati, Mazal; Goldman, Boleslaw; Peleg, Leah

    2004-03-01

    The 1278insTATC is the most prevalent beta-hexosaminidase A ( HEXA) gene mutation causing Tay-Sachs disease (TSD), one of the four lysosomal storage diseases (LSDs) occurring at elevated frequencies among Ashkenazi Jews (AJs). To investigate the genetic history of this mutation in the AJ population, a conserved haplotype (D15S981:175-D15S131:240-D15S1050:284-D15S197:144-D15S188:418) was identified in 1278insTATC chromosomes from 55 unrelated AJ individuals (15 homozygotes and 40 heterozygotes for the TSD mutation), suggesting the occurrence of a common founder. When two methods were used for analysis of linkage disequilibrium (LD) between flanking polymorphic markers and the disease locus and for the study of the decay of LD over time, the estimated age of the insertion was found to be 40+/-12 generations (95% confidence interval: 30-50 generations), so that the most recent common ancestor of the mutation-bearing chromosomes would date to the 8th-9th century. This corresponds with the demographic expansion of AJs in central Europe, following the founding of the Ashkenaz settlement in the early Middle Ages. The results are consistent with the geographic distribution of the main TSD mutation, 1278insTATC being more common in central Europe, and with the coalescent times of mutations causing two other LSDs, Gaucher disease and mucolipidosis type IV. Evidence for the absence of a determinant positive selection (heterozygote advantage) over the mutation is provided by a comparison between the estimated age of 1278insTATC and the probability of the current AJ frequency of the mutant allele as a function of its age, calculated by use of a branching-process model. Therefore, the founder effect in a rapidly expanding population arising from a bottleneck provides a robust parsimonious hypothesis explaining the spread of 1278insTATC-linked TSD in AJ individuals.

  6. Two novel exonic point mutations in HEXA identified in a juvenile Tay-Sachs patient: role of alternative splicing and nonsense-mediated mRNA decay.

    Science.gov (United States)

    Levit, A; Nutman, D; Osher, E; Kamhi, E; Navon, R

    2010-06-01

    We have identified three mutations in the beta-hexoseaminidase A (HEXA) gene in a juvenile Tay-Sachs disease (TSD) patient, which exhibited a reduced level of HEXA mRNA. Two mutations are novel, c.814G>A (p.Gly272Arg) and c.1305C>T (p.=), located in exon 8 and in exon 11, respectively. The third mutation, c.1195A>G (p.Asn399Asp) in exon 11, has been previously characterized as a common polymorphism in African-Americans. Hex A activity measured in TSD Glial cells, transfected with HEXA cDNA constructs bearing these mutations, was unaltered from the activity level measured in normal HEXA cDNA. Analysis of RT-PCR products revealed three aberrant transcripts in the patient, one where exon 8 was absent, one where exon 11 was absent and a third lacking both exons 10 and 11. All three novel transcripts contain frameshifts resulting in premature termination codons (PTCs). Transfection of mini-gene constructs carrying the c.814G>A and c.1305C>T mutations proved that the two mutations result in exon skipping. mRNAs that harbor a PTC are detected and degraded by the nonsense-mediated mRNA decay (NMD) pathway to prevent synthesis of abnormal proteins. However, although NMD is functional in the patient's fibroblasts, aberrant transcripts are still present. We suggest that the level of correctly spliced transcripts as well as the efficiency in which NMD degrade the PTC-containing transcripts, apparently plays an important role in the phenotype severity of the unique patient and thus should be considered as a potential target for drug therapy.

  7. Neuraminidase-1 contributes significantly to the degradation of neuronal B-series gangliosides but not to the bypass of the catabolic block in Tay–Sachs mouse models

    Directory of Open Access Journals (Sweden)

    Z.K. Timur

    2015-09-01

    Full Text Available Tay–Sachs disease is a severe lysosomal storage disorder caused by mutations in the HEXA gene coding for α subunit of lysosomal β-Hexosaminidase A enzyme, which converts GM2 to GM3 ganglioside. HexA−/− mice, depleted of the β-Hexosaminidase A iso-enzyme, remain asymptomatic up to 1 year of age because of a metabolic bypass by neuraminidase(s. These enzymes remove a sialic acid residue converting GM2 to GA2, which is further degraded by the still intact β-Hexosaminidase B iso-enzyme into lactosylceramide. A previously identified ganglioside metabolizing neuraminidase, Neu4, is abundantly expressed in the mouse brain and has activity against gangliosides like GM2 in vitro. Neu4−/− mice showed increased GD1a and decreased GM1 ganglioside in the brain suggesting the importance of the Neu4 in ganglioside catabolism. Mice with targeted disruption of both HexA and Neu4 genes showed accumulating GM2 ganglioside and epileptic seizures with 40% penetrance, indicating that the neuraminidase Neu4 is a modulatory gene, but may not be the only neuraminidase contributing to the metabolic bypass in HexA−/− mice. Therefore, we elucidated the biological role of neuraminidase-1 in ganglioside degradation in mouse. Analysis of HexA−/−Neu1−/− and HexA−/−Neu4−/−Neu1−/− mice models showed significant contribution of neuraminidase-1 on B-series ganglioside degradation in the brain. Therefore, we speculate that other neuraminidase/neuraminidases such as Neu2 and/or Neu3 might be also involved in the ganglioside degradation pathway in HexA−/− mice.

  8. A double mutation in exon 6 of the [beta]-hexosaminidase [alpha] subunit in a patient with the B1 variant of Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Ainsworth, P.J. (Univ. of Western Ontario, Ontario (Canada) Child Health Research Institute, London, Ontario (Canada)); Coulter-Mackie, M.B. (Univ. of Western Ontario, Ontario (Canada) Child Health Research Institute, London, Ontario (Canada) Children' s Psychiatric Research Institute, London, Ontario (Canada))

    1992-10-01

    The B1 variant form of Tay-Sachs disease is enzymologically unique in that the causative mutation(s) appear to affect the active site in the [alpha] subunit of [beta]-hexosaminidase A without altering its ability to associate with the [beta] subunit. Most previously reported B1 variant mutations were found in exon 5 within codon 178. The coding sequence of the [alpha] subunit gene of a patient with the B1 variant form was examined with a combination of reverse transcription of mRNA to cDNA, PCR, and dideoxy sequencing. A double mutation in exon 6 has been identified: a G[sub 574][yields]C transversion causing a val[sub 192][yields]leu change and a G[sub 598][yields] A transition resulting in a val[sub 200][yields]met alteration. The amplified cDNAs were otherwise normal throughout their sequence. The 574 and 598 alterations have been confirmed by amplification directly from genomic DNA from the patient and her mother. Transient-expression studies of the two exon 6 mutations (singly or together) in COS-1 cells show that the G[sub 574][yields]C change is sufficient to cause the loss of enzyme activity. The biochemical phenotype of the 574 alteration in transfection studies is consistent with that expected for a B1 variant mutation. As such, this mutation differs from previously reported B1 variant mutations, all of which occur in exon 5. 31 refs., 2 figs., 2 tabs.

  9. A Fundamental Metric for Metal Recycling Applied to Coated Magnesium

    NARCIS (Netherlands)

    Meskers, C.E.M.; Reuter, M.A.; Boin, U.; Kvithyld, A.

    2008-01-01

    A fundamental metric for the assessment of the recyclability and, hence, the sustainability of coated magnesium scrap is presented; this metric combines kinetics and thermodynamics. The recycling process, consisting of thermal decoating and remelting, was studied by thermogravimetry and differential

  10. Ideal Based Cyber Security Technical Metrics for Control Systems

    Energy Technology Data Exchange (ETDEWEB)

    W. F. Boyer; M. A. McQueen

    2007-10-01

    Much of the world's critical infrastructure is at risk from attack through electronic networks connected to control systems. Security metrics are important because they provide the basis for management decisions that affect the protection of the infrastructure. A cyber security technical metric is the security relevant output from an explicit mathematical model that makes use of objective measurements of a technical object. A specific set of technical security metrics are proposed for use by the operators of control systems. Our proposed metrics are based on seven security ideals associated with seven corresponding abstract dimensions of security. We have defined at least one metric for each of the seven ideals. Each metric is a measure of how nearly the associated ideal has been achieved. These seven ideals provide a useful structure for further metrics development. A case study shows how the proposed metrics can be applied to an operational control system.

  11. 43 CFR 12.915 - Metric system of measurement.

    Science.gov (United States)

    2010-10-01

    ... procurements, grants, and other business-related activities. Metric implementation may take longer where the... recipient, such as when foreign competitors are producing competing products in non-metric units. (End of...

  12. The Jacobi metric for timelike geodesics in static spacetimes

    Science.gov (United States)

    Gibbons, G. W.

    2016-01-01

    It is shown that the free motion of massive particles moving in static spacetimes is given by the geodesics of an energy-dependent Riemannian metric on the spatial sections analogous to Jacobi's metric in classical dynamics. In the massless limit Jacobi's metric coincides with the energy independent Fermat or optical metric. For stationary metrics, it is known that the motion of massless particles is given by the geodesics of an energy independent Finslerian metric of Randers type. The motion of massive particles is governed by neither a Riemannian nor a Finslerian metric. The properies of the Jacobi metric for massive particles moving outside the horizon of a Schwarschild black hole are described. By constrast with the massless case, the Gaussian curvature of the equatorial sections is not always negative.

  13. Active packaging for fresh-cut broccoli using 1-methylcyclopropene in biodegradable sachet/ Embalagem ativa para brócolis minimamente processado utilizando 1-metilciclopropeno em sachê biodegradável

    Directory of Open Access Journals (Sweden)

    Marta de Toledo Benassi

    2006-07-01

    Full Text Available Fresh-cut broccoli florets were packed in polypropylene pots containing a sachet with 1- methylcyclopropene (1-MCP and sealed with biodegradable starch-based film. Broccoli was stored for 8 days at 12oC and after this time the color and the texture of the product were similar of the fresh broccoli, with no off-flavor development or decay. Active packaging with 1-MCP in a sachet was efficient to extent shelf life of broccoli florets retarding yellowing and vitamin C losses. It is an alternative of 1-MCP treatment for fresh-cut products and besides, the sachet can absorbs condensed water reducing spoilage and off-odor development.Brócolis minimamente processados foram embalados em bandejas de polipropileno com sachê contendo 1-metilciclopropeno (1-MCP e selado com filme biodegradável de amido. Os brócolis foram armazenados por 8 dias a 12oC e após este tempo a cor e a textura mantiveram-se similares às do produto fresco, sem desenvolvimento de odor não característico ou podridão. A embalagem ativa contendo 1-MCP na forma de sachê foi eficiente no aumento da vida de prateleira de brócolis, retardando o amarelecimento e a perda de vitamina C. Esta é uma alternativa para o tratamento com 1-MCP para produtos minimamente processados e além disso, o sachê pode absorver a água condensada, reduzindo a deterioração e o desenvolvimento de odor não característico.

  14. The Val{sup 192}Leu mutation in the {alpha}-subunit of {beta}-hexosaminidase A is not associated with the B1-variant form of Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Hou, Y.; Vavougios, G.; Hinek, A. [Univ. of Toronto (Canada)] [and others

    1996-07-01

    Substitution mutations adversely affecting the {alpha}-subunit of {beta}-hexosaminidase A ({alpha}{beta}) (EC 3.2.1.52) result in Tay-Sachs disease. The majority affect the initial folding of the pro-{alpha} chain in the endoplasmic reticulum, resulting in its retention and degradation. A much less common occurrence is a mutation that specifically affects an {open_quotes}active-site{close_quotes} residue necessary for substrate binding and/or catalysis. In this case, hexosaminidase A is present in the lysosome, but it lacks all {alpha}-specific activity. This biochemical phenotype is referred to as the {open_quotes}B1-variant form{close_quotes} of Tay-Sachs disease. Kinetic analysis of suspected B1-variant mutations is complex because hexosaminidase A is heterodimeric and both subunits possess similar active sites. In this report, we examine a previously identified B1-variant mutation, {alpha}-Val{sup 192}Leu. Chinese hamster ovary cells were permanently cotransfected with an {alpha}-cDNA-construct encoding the substitution and a mutant {beta}-cDNA ({beta}-Arg{sup 211}Lys), encoding a {beta}-subunit that is inactive but normal in all other respects. We were surprised to find that the Val{sup 192}Leu substitution produced a pro-{alpha} chain that did not form {alpha}-{beta} dimers and was not transported to the lysosome. Finally, we reexamined the hexosaminidase activity and protein levels in the fibroblasts from the original patient. These data were also not consistent with the biochemical phenotype of the B1 variant of Tay-Sachs disease previously reported to be present. Thus, we conclude that the Val{sup 192}Leu substitution does not specifically affect the {alpha}-active site. 23 refs., 4 figs., 2 tabs.

  15. Factor structure of the Tomimatsu-Sato metrics

    International Nuclear Information System (INIS)

    Perjes, Z.

    1989-02-01

    Based on an earlier result stating that δ = 3 Tomimatsu-Sato (TS) metrics can be factored over the field of integers, an analogous representation for higher TS metrics was sought. It is shown that the factoring property of TS metrics follows from the structure of special Hankel determinants. A set of linear algebraic equations determining the factors was defined, and the factors of the first five TS metrics were tabulated, together with their primitive factors. (R.P.) 4 refs.; 2 tabs

  16. What can article-level metrics do for you?

    Science.gov (United States)

    Fenner, Martin

    2013-10-01

    Article-level metrics (ALMs) provide a wide range of metrics about the uptake of an individual journal article by the scientific community after publication. They include citations, usage statistics, discussions in online comments and social media, social bookmarking, and recommendations. In this essay, we describe why article-level metrics are an important extension of traditional citation-based journal metrics and provide a number of example from ALM data collected for PLOS Biology.

  17. Tay-Sachs disease in an Arab family due to c.78G>A HEXA nonsense mutation encoding a p.W26X early truncation enzyme peptide.

    Science.gov (United States)

    Haghighi, Alireza; Masri, Amira; Kornreich, Ruth; Desnick, Robert J

    2011-12-01

    Tay-Sachs disease (TSD), a pan-ethnic, autosomal recessive, neurodegenerative, lysosomal disease, results from deficient β-hexosaminidase A activity due to β-hexosaminidase α-subunit (HEXA) mutations. Prenatal/premarital carrier screening programs in the Ashkenazi Jewish community have markedly reduced disease occurrence. We report the first Jordanian Arab TSD patient diagnosed by deficient β-hexosaminidase A activity. HEXA mutation analysis revealed homozygosity for a nonsense mutation, c.78G>A (p.W26X). Previously reported in Arab patients, this mutation is a candidate for TSD screening in Arab populations. Copyright © 2011 Elsevier Inc. All rights reserved.

  18. A convergence theory for probabilistic metric spaces | Jäger ...

    African Journals Online (AJOL)

    We develop a theory of probabilistic convergence spaces based on Tardiff's neighbourhood systems for probabilistic metric spaces. We show that the resulting category is a topological universe and we characterize a subcategory that is isomorphic to the category of probabilistic metric spaces. Keywords: Probabilistic metric ...

  19. Understanding Acceptance of Software Metrics--A Developer Perspective

    Science.gov (United States)

    Umarji, Medha

    2009-01-01

    Software metrics are measures of software products and processes. Metrics are widely used by software organizations to help manage projects, improve product quality and increase efficiency of the software development process. However, metrics programs tend to have a high failure rate in organizations, and developer pushback is one of the sources…

  20. Modified intuitionistic fuzzy metric spaces and some fixed point theorems

    International Nuclear Information System (INIS)

    Saadati, R.; Sedghi, S.; Shobe, N.

    2008-01-01

    Since the intuitionistic fuzzy metric space has extra conditions (see [Gregori V, Romaguera S, Veereamani P. A note on intuitionistic fuzzy metric spaces. Chaos, Solitons and Fractals 2006;28:902-5]). In this paper, we consider modified intuitionistic fuzzy metric spaces and prove some fixed point theorems in these spaces. All the results presented in this paper are new

  1. Tide or Tsunami? The Impact of Metrics on Scholarly Research

    Science.gov (United States)

    Bonnell, Andrew G.

    2016-01-01

    Australian universities are increasingly resorting to the use of journal metrics such as impact factors and ranking lists in appraisal and promotion processes, and are starting to set quantitative "performance expectations" which make use of such journal-based metrics. The widespread use and misuse of research metrics is leading to…

  2. Robustness of climate metrics under climate policy ambiguity

    International Nuclear Information System (INIS)

    Ekholm, Tommi; Lindroos, Tomi J.; Savolainen, Ilkka

    2013-01-01

    Highlights: • We assess the economic impacts of using different climate metrics. • The setting is cost-efficient scenarios for three interpretations of the 2C target. • With each target setting, the optimal metric is different. • Therefore policy ambiguity prevents the selection of an optimal metric. • Robust metric values that perform well with multiple policy targets however exist. -- Abstract: A wide array of alternatives has been proposed as the common metrics with which to compare the climate impacts of different emission types. Different physical and economic metrics and their parameterizations give diverse weights between e.g. CH 4 and CO 2 , and fixing the metric from one perspective makes it sub-optimal from another. As the aims of global climate policy involve some degree of ambiguity, it is not possible to determine a metric that would be optimal and consistent with all policy aims. This paper evaluates the cost implications of using predetermined metrics in cost-efficient mitigation scenarios. Three formulations of the 2 °C target, including both deterministic and stochastic approaches, shared a wide range of metric values for CH 4 with which the mitigation costs are only slightly above the cost-optimal levels. Therefore, although ambiguity in current policy might prevent us from selecting an optimal metric, it can be possible to select robust metric values that perform well with multiple policy targets

  3. Graev metrics on free products and HNN extensions

    DEFF Research Database (Denmark)

    Slutsky, Konstantin

    2014-01-01

    We give a construction of two-sided invariant metrics on free products (possibly with amalgamation) of groups with two-sided invariant metrics and, under certain conditions, on HNN extensions of such groups. Our approach is similar to the Graev's construction of metrics on free groups over pointed...

  4. The universal connection and metrics on moduli spaces

    International Nuclear Information System (INIS)

    Massamba, Fortune; Thompson, George

    2003-11-01

    We introduce a class of metrics on gauge theoretic moduli spaces. These metrics are made out of the universal matrix that appears in the universal connection construction of M. S. Narasimhan and S. Ramanan. As an example we construct metrics on the c 2 = 1 SU(2) moduli space of instantons on R 4 for various universal matrices. (author)

  5. ST-intuitionistic fuzzy metric space with properties

    Science.gov (United States)

    Arora, Sahil; Kumar, Tanuj

    2017-07-01

    In this paper, we define ST-intuitionistic fuzzy metric space and the notion of convergence and completeness properties of cauchy sequences is studied. Further, we prove some properties of ST-intuitionistic fuzzy metric space. Finally, we introduce the concept of symmetric ST Intuitionistic Fuzzy metric space.

  6. Term Based Comparison Metrics for Controlled and Uncontrolled Indexing Languages

    Science.gov (United States)

    Good, B. M.; Tennis, J. T.

    2009-01-01

    Introduction: We define a collection of metrics for describing and comparing sets of terms in controlled and uncontrolled indexing languages and then show how these metrics can be used to characterize a set of languages spanning folksonomies, ontologies and thesauri. Method: Metrics for term set characterization and comparison were identified and…

  7. Software architecture analysis tool : software architecture metrics collection

    NARCIS (Netherlands)

    Muskens, J.; Chaudron, M.R.V.; Westgeest, R.

    2002-01-01

    The Software Engineering discipline lacks the ability to evaluate software architectures. Here we describe a tool for software architecture analysis that is based on metrics. Metrics can be used to detect possible problems and bottlenecks in software architectures. Even though metrics do not give a

  8. Otherwise Engaged : Social Media from Vanity Metrics to Critical Analytics

    NARCIS (Netherlands)

    Rogers, R.

    2018-01-01

    Vanity metrics is a term that captures the measurement and display of how well one is doing in the “success theater” of social media. The notion of vanity metrics implies a critique of metrics concerning both the object of measurement as well as their capacity to measure unobtrusively or only to

  9. Meter Detection in Symbolic Music Using Inner Metric Analysis

    NARCIS (Netherlands)

    de Haas, W.B.; Volk, A.

    2016-01-01

    In this paper we present PRIMA: a new model tailored to symbolic music that detects the meter and the first downbeat position of a piece. Given onset data, the metrical structure of a piece is interpreted using the Inner Metric Analysis (IMA) model. IMA identifies the strong and weak metrical

  10. Regional Sustainability: The San Luis Basin Metrics Project

    Science.gov (United States)

    There are a number of established, scientifically supported metrics of sustainability. Many of the metrics are data intensive and require extensive effort to collect data and compute. Moreover, individual metrics may not capture all aspects of a system that are relevant to sust...

  11. Extremal limits of the C metric: Nariai, Bertotti-Robinson, and anti-Nariai C metrics

    International Nuclear Information System (INIS)

    Dias, Oscar J.C.; Lemos, Jose P.S.

    2003-01-01

    In two previous papers we have analyzed the C metric in a background with a cosmological constant Λ, namely, the de-Sitter (dS) C metric (Λ>0), and the anti-de Sitter (AdS) C metric (Λ 0, Λ=0, and Λ 2 xS-tilde 2 ) to each point in the deformed two-sphere S-tilde 2 corresponds a dS 2 spacetime, except for one point which corresponds to a dS 2 spacetime with an infinite straight strut or string. There are other important new features that appear. One expects that the solutions found in this paper are unstable and decay into a slightly nonextreme black hole pair accelerated by a strut or by strings. Moreover, the Euclidean version of these solutions mediate the quantum process of black hole pair creation that accompanies the decay of the dS and AdS spaces

  12. Massless and massive quanta resulting from a mediumlike metric tensor

    International Nuclear Information System (INIS)

    Soln, J.

    1985-01-01

    A simple model of the ''primordial'' scalar field theory is presented in which the metric tensor is a generalization of the metric tensor from electrodynamics in a medium. The radiation signal corresponding to the scalar field propagates with a velocity that is generally less than c. This signal can be associated simultaneously with imaginary and real effective (momentum-dependent) masses. The requirement that the imaginary effective mass vanishes, which we take to be the prerequisite for the vacuumlike signal propagation, leads to the ''spontaneous'' splitting of the metric tensor into two distinct metric tensors: one metric tensor gives rise to masslesslike radiation and the other to a massive particle. (author)

  13. Principle of space existence and De Sitter metric

    International Nuclear Information System (INIS)

    Mal'tsev, V.K.

    1990-01-01

    The selection principle for the solutions of the Einstein equations suggested in a series of papers implies the existence of space (g ik ≠ 0) only in the presence of matter (T ik ≠0). This selection principle (principle of space existence, in the Markov terminology) implies, in the general case, the absence of the cosmological solution with the De Sitter metric. On the other hand, the De Sitter metric is necessary for describing both inflation and deflation periods of the Universe. It is shown that the De Sitter metric is also allowed by the selection principle under discussion if the metric experiences the evolution into the Friedmann metric

  14. Pragmatic security metrics applying metametrics to information security

    CERN Document Server

    Brotby, W Krag

    2013-01-01

    Other books on information security metrics discuss number theory and statistics in academic terms. Light on mathematics and heavy on utility, PRAGMATIC Security Metrics: Applying Metametrics to Information Security breaks the mold. This is the ultimate how-to-do-it guide for security metrics.Packed with time-saving tips, the book offers easy-to-follow guidance for those struggling with security metrics. Step by step, it clearly explains how to specify, develop, use, and maintain an information security measurement system (a comprehensive suite of metrics) to

  15. Classification in medical images using adaptive metric k-NN

    Science.gov (United States)

    Chen, C.; Chernoff, K.; Karemore, G.; Lo, P.; Nielsen, M.; Lauze, F.

    2010-03-01

    The performance of the k-nearest neighborhoods (k-NN) classifier is highly dependent on the distance metric used to identify the k nearest neighbors of the query points. The standard Euclidean distance is commonly used in practice. This paper investigates the performance of k-NN classifier with respect to different adaptive metrics in the context of medical imaging. We propose using adaptive metrics such that the structure of the data is better described, introducing some unsupervised learning knowledge in k-NN. We investigated four different metrics are estimated: a theoretical metric based on the assumption that images are drawn from Brownian Image Model (BIM), the normalized metric based on variance of the data, the empirical metric is based on the empirical covariance matrix of the unlabeled data, and an optimized metric obtained by minimizing the classification error. The spectral structure of the empirical covariance also leads to Principal Component Analysis (PCA) performed on it which results the subspace metrics. The metrics are evaluated on two data sets: lateral X-rays of the lumbar aortic/spine region, where we use k-NN for performing abdominal aorta calcification detection; and mammograms, where we use k-NN for breast cancer risk assessment. The results show that appropriate choice of metric can improve classification.

  16. THE ROLE OF ARTICLE LEVEL METRICS IN SCIENTIFIC PUBLISHING

    Directory of Open Access Journals (Sweden)

    Vladimir TRAJKOVSKI

    2016-04-01

    Full Text Available Emerging metrics based on article-level does not exclude traditional metrics based on citations to the journal, but complements them. Article-level metrics (ALMs provide a wide range of metrics about the uptake of an individual journal article by the scientific community after publication. They include citations, statistics of usage, discussions in online comments and social media, social bookmarking, and recommendations. In this editorial, the role of article level metrics in publishing scientific papers has been described. Article-Level Metrics (ALMs are rapidly emerging as important tools to quantify how individual articles are being discussed, shared, and used. Data sources depend on the tool, but they include classic metrics indicators depending on citations, academic social networks (Mendeley, CiteULike, Delicious and social media (Facebook, Twitter, blogs, and Youtube. The most popular tools used to apply this new metrics are: Public Library of Science - Article-Level Metrics, Altmetric, Impactstory and Plum Analytics. Journal Impact Factor (JIF does not consider impact or influence beyond citations count as this count reflected only through Thomson Reuters’ Web of Science® database. JIF provides indicator related to the journal, but not related to a published paper. Thus, altmetrics now becomes an alternative metrics for performance assessment of individual scientists and their contributed scholarly publications. Macedonian scholarly publishers have to work on implementing of article level metrics in their e-journals. It is the way to increase their visibility and impact in the world of science.

  17. Outsourced Similarity Search on Metric Data Assets

    DEFF Research Database (Denmark)

    Yiu, Man Lung; Assent, Ira; Jensen, Christian S.

    2012-01-01

    . Outsourcing offers the data owner scalability and a low initial investment. The need for privacy may be due to the data being sensitive (e.g., in medicine), valuable (e.g., in astronomy), or otherwise confidential. Given this setting, the paper presents techniques that transform the data prior to supplying......This paper considers a cloud computing setting in which similarity querying of metric data is outsourced to a service provider. The data is to be revealed only to trusted users, not to the service provider or anyone else. Users query the server for the most similar data objects to a query example...

  18. New Metrics from a Fractional Gravitational Field

    International Nuclear Information System (INIS)

    El-Nabulsi, Rami Ahmad

    2017-01-01

    Agop et al. proved in Commun. Theor. Phys. (2008) that, a Reissner–Nordstrom type metric is obtained, if gauge gravitational field in a fractal spacetime is constructed by means of concepts of scale relativity. We prove in this short communication that similar result is obtained if gravity in D-spacetime dimensions is fractionalized by means of the Glaeske–Kilbas–Saigo fractional. Besides, non-singular gravitational fields are obtained without using extra-dimensions. We present few examples to show that these gravitational fields hold a number of motivating features in spacetime physics. (paper)

  19. Energy Metrics for State Government Buildings

    Science.gov (United States)

    Michael, Trevor

    Measuring true progress towards energy conservation goals requires the accurate reporting and accounting of energy consumption. An accurate energy metrics framework is also a critical element for verifiable Greenhouse Gas Inventories. Energy conservation in government can reduce expenditures on energy costs leaving more funds available for public services. In addition to monetary savings, conserving energy can help to promote energy security, air quality, and a reduction of carbon footprint. With energy consumption/GHG inventories recently produced at the Federal level, state and local governments are beginning to also produce their own energy metrics systems. In recent years, many states have passed laws and executive orders which require their agencies to reduce energy consumption. In June 2008, SC state government established a law to achieve a 20% energy usage reduction in state buildings by 2020. This study examines case studies from other states who have established similar goals to uncover the methods used to establish an energy metrics system. Direct energy consumption in state government primarily comes from buildings and mobile sources. This study will focus exclusively on measuring energy consumption in state buildings. The case studies reveal that many states including SC are having issues gathering the data needed to accurately measure energy consumption across all state buildings. Common problems found include a lack of enforcement and incentives that encourage state agencies to participate in any reporting system. The case studies are aimed at finding the leverage used to gather the needed data. The various approaches at coercing participation will hopefully reveal methods that SC can use to establish the accurate metrics system needed to measure progress towards its 20% by 2020 energy reduction goal. Among the strongest incentives found in the case studies is the potential for monetary savings through energy efficiency. Framing energy conservation

  20. Multi-Robot Assembly Strategies and Metrics

    Science.gov (United States)

    MARVEL, JEREMY A.; BOSTELMAN, ROGER; FALCO, JOE

    2018-01-01

    We present a survey of multi-robot assembly applications and methods and describe trends and general insights into the multi-robot assembly problem for industrial applications. We focus on fixtureless assembly strategies featuring two or more robotic systems. Such robotic systems include industrial robot arms, dexterous robotic hands, and autonomous mobile platforms, such as automated guided vehicles. In this survey, we identify the types of assemblies that are enabled by utilizing multiple robots, the algorithms that synchronize the motions of the robots to complete the assembly operations, and the metrics used to assess the quality and performance of the assemblies. PMID:29497234

  1. Metric preheating and limitations of linearized gravity

    International Nuclear Information System (INIS)

    Bassett, Bruce A.; Tamburini, Fabrizio; Kaiser, David I.; Maartens, Roy

    1999-01-01

    During the preheating era after inflation, resonant amplification of quantum field fluctuations takes place. Recently it has become clear that this must be accompanied by resonant amplification of scalar metric fluctuations, since the two are united by Einstein's equations. Furthermore, this 'metric preheating' enhances particle production, and leads to gravitational rescattering effects even at linear order. In multi-field models with strong preheating (q>>1), metric perturbations are driven non-linear, with the strongest amplification typically on super-Hubble scales (k→0). This amplification is causal, being due to the super-Hubble coherence of the inflaton condensate, and is accompanied by resonant growth of entropy perturbations. The amplification invalidates the use of the linearized Einstein field equations, irrespective of the amount of fine-tuning of the initial conditions. This has serious implications on all scales - from large-angle cosmic microwave background (CMB) anisotropies to primordial black holes. We investigate the (q,k) parameter space in a two-field model, and introduce the time to non-linearity, t nl , as the timescale for the breakdown of the linearized Einstein equations. t nl is a robust indicator of resonance behavior, showing the fine structure in q and k that one expects from a quasi-Floquet system, and we argue that t nl is a suitable generalization of the static Floquet index in an expanding universe. Backreaction effects are expected to shut down the linear resonances, but cannot remove the existing amplification, which threatens the viability of strong preheating when confronted with the CMB. Mode-mode coupling and turbulence tend to re-establish scale invariance, but this process is limited by causality and for small k the primordial scale invariance of the spectrum may be destroyed. We discuss ways to escape the above conclusions, including secondary phases of inflation and preheating solely to fermions. The exclusion principle

  2. Alternative kinetic energy metrics for Lagrangian systems

    Science.gov (United States)

    Sarlet, W.; Prince, G.

    2010-11-01

    We examine Lagrangian systems on \\ {R}^n with standard kinetic energy terms for the possibility of additional, alternative Lagrangians with kinetic energy metrics different to the Euclidean one. Using the techniques of the inverse problem in the calculus of variations we find necessary and sufficient conditions for the existence of such Lagrangians. We illustrate the problem in two and three dimensions with quadratic and cubic potentials. As an aside we show that the well-known anomalous Lagrangians for the Coulomb problem can be removed by switching on a magnetic field, providing an appealing resolution of the ambiguous quantizations of the hydrogen atom.

  3. Differential geometry bundles, connections, metrics and curvature

    CERN Document Server

    Taubes, Clifford Henry

    2011-01-01

    Bundles, connections, metrics and curvature are the 'lingua franca' of modern differential geometry and theoretical physics. This book will supply a graduate student in mathematics or theoretical physics with the fundamentals of these objects. Many of the tools used in differential topology are introduced and the basic results about differentiable manifolds, smooth maps, differential forms, vector fields, Lie groups, and Grassmanians are all presented here. Other material covered includes the basic theorems about geodesics and Jacobi fields, the classification theorem for flat connections, the

  4. Multi-Robot Assembly Strategies and Metrics.

    Science.gov (United States)

    Marvel, Jeremy A; Bostelman, Roger; Falco, Joe

    2018-02-01

    We present a survey of multi-robot assembly applications and methods and describe trends and general insights into the multi-robot assembly problem for industrial applications. We focus on fixtureless assembly strategies featuring two or more robotic systems. Such robotic systems include industrial robot arms, dexterous robotic hands, and autonomous mobile platforms, such as automated guided vehicles. In this survey, we identify the types of assemblies that are enabled by utilizing multiple robots, the algorithms that synchronize the motions of the robots to complete the assembly operations, and the metrics used to assess the quality and performance of the assemblies.

  5. Indefinite metric and regularization of electrodynamics

    International Nuclear Information System (INIS)

    Gaudin, M.

    1984-06-01

    The invariant regularization of Pauli and Villars in quantum electrodynamics can be considered as deriving from a local and causal lagrangian theory for spin 1/2 bosons, by introducing an indefinite metric and a condition on the allowed states similar to the Lorentz condition. The consequences are the asymptotic freedom of the photon's propagator. We present a calcultion of the effective charge to the fourth order in the coupling as a function of the auxiliary masses, the theory avoiding all mass divergencies to this order [fr

  6. Metrics for comparing plasma mass filters

    Energy Technology Data Exchange (ETDEWEB)

    Fetterman, Abraham J.; Fisch, Nathaniel J. [Department of Astrophysical Sciences, Princeton University, Princeton, New Jersey 08540 (United States)

    2011-10-15

    High-throughput mass separation of nuclear waste may be useful for optimal storage, disposal, or environmental remediation. The most dangerous part of nuclear waste is the fission product, which produces most of the heat and medium-term radiation. Plasmas are well-suited to separating nuclear waste because they can separate many different species in a single step. A number of plasma devices have been designed for such mass separation, but there has been no standardized comparison between these devices. We define a standard metric, the separative power per unit volume, and derive it for three different plasma mass filters: the plasma centrifuge, Ohkawa filter, and the magnetic centrifugal mass filter.

  7. Metrics for comparing plasma mass filters

    International Nuclear Information System (INIS)

    Fetterman, Abraham J.; Fisch, Nathaniel J.

    2011-01-01

    High-throughput mass separation of nuclear waste may be useful for optimal storage, disposal, or environmental remediation. The most dangerous part of nuclear waste is the fission product, which produces most of the heat and medium-term radiation. Plasmas are well-suited to separating nuclear waste because they can separate many different species in a single step. A number of plasma devices have been designed for such mass separation, but there has been no standardized comparison between these devices. We define a standard metric, the separative power per unit volume, and derive it for three different plasma mass filters: the plasma centrifuge, Ohkawa filter, and the magnetic centrifugal mass filter.

  8. Decision Analysis for Metric Selection on a Clinical Quality Scorecard.

    Science.gov (United States)

    Guth, Rebecca M; Storey, Patricia E; Vitale, Michael; Markan-Aurora, Sumita; Gordon, Randolph; Prevost, Traci Q; Dunagan, Wm Claiborne; Woeltje, Keith F

    2016-09-01

    Clinical quality scorecards are used by health care institutions to monitor clinical performance and drive quality improvement. Because of the rapid proliferation of quality metrics in health care, BJC HealthCare found it increasingly difficult to select the most impactful scorecard metrics while still monitoring metrics for regulatory purposes. A 7-step measure selection process was implemented incorporating Kepner-Tregoe Decision Analysis, which is a systematic process that considers key criteria that must be satisfied in order to make the best decision. The decision analysis process evaluates what metrics will most appropriately fulfill these criteria, as well as identifies potential risks associated with a particular metric in order to identify threats to its implementation. Using this process, a list of 750 potential metrics was narrowed to 25 that were selected for scorecard inclusion. This decision analysis process created a more transparent, reproducible approach for selecting quality metrics for clinical quality scorecards. © The Author(s) 2015.

  9. Balanced metrics for vector bundles and polarised manifolds

    DEFF Research Database (Denmark)

    Garcia Fernandez, Mario; Ross, Julius

    2012-01-01

    leads to a Hermitian-Einstein metric on E and a constant scalar curvature Kähler metric in c_1(L). For special values of α, limits of balanced metrics are solutions of a system of coupled equations relating a Hermitian-Einstein metric on E and a Kähler metric in c1(L). For this, we compute the top two......We consider a notion of balanced metrics for triples (X, L, E) which depend on a parameter α, where X is smooth complex manifold with an ample line bundle L and E is a holomorphic vector bundle over X. For generic choice of α, we prove that the limit of a convergent sequence of balanced metrics...

  10. Construction of Einstein-Sasaki metrics in D≥7

    International Nuclear Information System (INIS)

    Lue, H.; Pope, C. N.; Vazquez-Poritz, J. F.

    2007-01-01

    We construct explicit Einstein-Kaehler metrics in all even dimensions D=2n+4≥6, in terms of a 2n-dimensional Einstein-Kaehler base metric. These are cohomogeneity 2 metrics which have the new feature of including a NUT-type parameter, or gravomagnetic charge, in addition to..' in addition to mass and rotation parameters. Using a canonical construction, these metrics all yield Einstein-Sasaki metrics in dimensions D=2n+5≥7. As is commonly the case in this type of construction, for suitable choices of the free parameters the Einstein-Sasaki metrics can extend smoothly onto complete and nonsingular manifolds, even though the underlying Einstein-Kaehler metric has conical singularities. We discuss some explicit examples in the case of seven-dimensional Einstein-Sasaki spaces. These new spaces can provide supersymmetric backgrounds in M theory, which play a role in the AdS 4 /CFT 3 correspondence

  11. National Metrical Types in Nineteenth Century Art Song

    Directory of Open Access Journals (Sweden)

    Leigh VanHandel

    2010-01-01

    Full Text Available William Rothstein’s article “National metrical types in music of the eighteenth and early nineteenth centuries” (2008 proposes a distinction between the metrical habits of 18th and early 19th century German music and those of Italian and French music of that period. Based on theoretical treatises and compositional practice, he outlines these national metrical types and discusses the characteristics of each type. This paper presents the results of a study designed to determine whether, and to what degree, Rothstein’s characterizations of national metrical types are present in 19th century French and German art song. Studying metrical habits in this genre may provide a lens into changing metrical conceptions of 19th century theorists and composers, as well as to the metrical habits and compositional style of individual 19th century French and German art song composers.

  12. Metrication: An economic wake-up call for US industry

    Science.gov (United States)

    Carver, G. P.

    1993-03-01

    As the international standard of measurement, the metric system is one key to success in the global marketplace. International standards have become an important factor in international economic competition. Non-metric products are becoming increasingly unacceptable in world markets that favor metric products. Procurement is the primary federal tool for encouraging and helping U.S. industry to convert voluntarily to the metric system. Besides the perceived unwillingness of the customer, certain regulatory language, and certain legal definitions in some states, there are no major impediments to conversion of the remaining non-metric industries to metric usage. Instead, there are good reasons for changing, including an opportunity to rethink many industry standards and to take advantage of size standardization. Also, when the remaining industries adopt the metric system, they will come into conformance with federal agencies engaged in similar activities.

  13. The first family with Tay-Sachs disease in Cyprus: Genetic analysis reveals a nonsense (c.78G>A) and a silent (c.1305C>T) mutation and allows preimplantation genetic diagnosis.

    Science.gov (United States)

    Georgiou, Theodoros; Christopoulos, George; Anastasiadou, Violetta; Hadjiloizou, Stavros; Cregeen, David; Jackson, Marie; Mavrikiou, Gavriella; Kleanthous, Marina; Drousiotou, Anthi

    2014-12-01

    Tay-Sachs disease (TSD) is a recessively inherited neurodegenerative disorder caused by mutations in the HEXA gene resulting in β-hexosaminidase A (HEX A) deficiency and neuronal accumulation of GM2 ganglioside. We describe the first patient with Tay-Sachs disease in the Cypriot population, a juvenile case which presented with developmental regression at the age of five. The diagnosis was confirmed by measurement of HEXA activity in plasma, peripheral leucocytes and fibroblasts. Sequencing the HEXA gene resulted in the identification of two previously described mutations: the nonsense mutation c.78G>A (p.Trp26X) and the silent mutation c.1305C>T (p.=). The silent mutation was reported once before in a juvenile TSD patient of West Indian origin with an unusually mild phenotype. The presence of this mutation in another juvenile TSD patient provides further evidence that it is a disease-causing mutation. Successful preimplantation genetic diagnosis (PGD) and prenatal follow-up were provided to the couple.

  14. Fanpage metrics analysis. "Study on content engagement"

    Science.gov (United States)

    Rahman, Zoha; Suberamanian, Kumaran; Zanuddin, Hasmah Binti; Moghavvemi, Sedigheh; Nasir, Mohd Hairul Nizam Bin Md

    2016-08-01

    Social Media is now determined as an excellent communicative tool to connect directly with consumers. One of the most significant ways to connect with the consumers through these Social Networking Sites (SNS) is to create a facebook fanpage with brand contents and to place different posts periodically on these fanpages. In measuring social networking sites' effectiveness, corporate houses are now analyzing metrics in terms of calculating engagement rate, number of comments/share and likings in fanpages. So now, it is very important for the marketers to know the effectiveness of different contents or posts of fanpages in order to increase the fan responsiveness and engagement rate in the fan pages. In the study the authors have analyzed total 1834 brand posts from 17 international brands of Electronics companies. Data of 9 months (From December 2014 to August 2015) have been collected for analyses, which were available online in the Brand' fan pages. An econometrics analysis is conducted using Eviews 9, to determine the impact of different contents on fanpage engagement. The study picked the four most frequently posted content to determine their impact on PTA (people Talking About) metrics and Fanpage engagement activities.

  15. Network Community Detection on Metric Space

    Directory of Open Access Journals (Sweden)

    Suman Saha

    2015-08-01

    Full Text Available Community detection in a complex network is an important problem of much interest in recent years. In general, a community detection algorithm chooses an objective function and captures the communities of the network by optimizing the objective function, and then, one uses various heuristics to solve the optimization problem to extract the interesting communities for the user. In this article, we demonstrate the procedure to transform a graph into points of a metric space and develop the methods of community detection with the help of a metric defined for a pair of points. We have also studied and analyzed the community structure of the network therein. The results obtained with our approach are very competitive with most of the well-known algorithms in the literature, and this is justified over the large collection of datasets. On the other hand, it can be observed that time taken by our algorithm is quite less compared to other methods and justifies the theoretical findings.

  16. Value of the Company and Marketing Metrics

    Directory of Open Access Journals (Sweden)

    André Luiz Ramos

    2013-12-01

    Full Text Available Thinking marketing strategies from a resource-based perspective (Barney, 1991, proposing assets as either tangible, organizational and human, and from Constantin and Luch’s vision (1994, where strategic resources can be tanbigle or intangible, internal or external to the firm, raises a research approach on Marketing and Finance. According to Srivastava, Shervani and Fahey (1998 there are 3 market assets types, which generate firm value. Firm value can be measured by discounted cashflow, compromising marketing activities with value generation forcasts (Anderson, 1982; Day, Fahey, 1988; Doyle, 2000; Rust et al., 2004a. The economic value of marketing strategies and marketing metrics are calling strategy researchers’ and marketing managers’ attention, making clear the need for building a bridge able to articulate marketing and finance form a strategic perspective. This article proposes an analytical framework based on different scientific approaches envolving risk and return promoted by marketing strategies and points out advances concerning both methodological approaches and marketing strategies and its impact on firm metrics and value, usgin Srinivasan and Hanssens (2009 as a start point.

  17. Defining a standard metric for electricity savings

    International Nuclear Information System (INIS)

    Koomey, Jonathan; Akbari, Hashem; Blumstein, Carl; Brown, Marilyn; Brown, Richard; Calwell, Chris; Carter, Sheryl; Cavanagh, Ralph; Chang, Audrey; Claridge, David; Craig, Paul; Diamond, Rick; Eto, Joseph H; Fulkerson, William; Gadgil, Ashok; Geller, Howard; Goldemberg, Jose; Goldman, Chuck; Goldstein, David B; Greenberg, Steve

    2010-01-01

    The growing investment by governments and electric utilities in energy efficiency programs highlights the need for simple tools to help assess and explain the size of the potential resource. One technique that is commonly used in this effort is to characterize electricity savings in terms of avoided power plants, because it is easier for people to visualize a power plant than it is to understand an abstraction such as billions of kilowatt-hours. Unfortunately, there is no standardization around the characteristics of such power plants. In this letter we define parameters for a standard avoided power plant that have physical meaning and intuitive plausibility, for use in back-of-the-envelope calculations. For the prototypical plant this article settles on a 500 MW existing coal plant operating at a 70% capacity factor with 7% T and D losses. Displacing such a plant for one year would save 3 billion kWh/year at the meter and reduce emissions by 3 million metric tons of CO 2 per year. The proposed name for this metric is the Rosenfeld, in keeping with the tradition among scientists of naming units in honor of the person most responsible for the discovery and widespread adoption of the underlying scientific principle in question-Dr Arthur H Rosenfeld.

  18. Defining a standard metric for electricity savings

    Energy Technology Data Exchange (ETDEWEB)

    Koomey, Jonathan [Lawrence Berkeley National Laboratory and Stanford University, PO Box 20313, Oakland, CA 94620-0313 (United States); Akbari, Hashem; Blumstein, Carl; Brown, Marilyn; Brown, Richard; Calwell, Chris; Carter, Sheryl; Cavanagh, Ralph; Chang, Audrey; Claridge, David; Craig, Paul; Diamond, Rick; Eto, Joseph H; Fulkerson, William; Gadgil, Ashok; Geller, Howard; Goldemberg, Jose; Goldman, Chuck; Goldstein, David B; Greenberg, Steve, E-mail: JGKoomey@stanford.ed

    2010-01-15

    The growing investment by governments and electric utilities in energy efficiency programs highlights the need for simple tools to help assess and explain the size of the potential resource. One technique that is commonly used in this effort is to characterize electricity savings in terms of avoided power plants, because it is easier for people to visualize a power plant than it is to understand an abstraction such as billions of kilowatt-hours. Unfortunately, there is no standardization around the characteristics of such power plants. In this letter we define parameters for a standard avoided power plant that have physical meaning and intuitive plausibility, for use in back-of-the-envelope calculations. For the prototypical plant this article settles on a 500 MW existing coal plant operating at a 70% capacity factor with 7% T and D losses. Displacing such a plant for one year would save 3 billion kWh/year at the meter and reduce emissions by 3 million metric tons of CO{sub 2} per year. The proposed name for this metric is the Rosenfeld, in keeping with the tradition among scientists of naming units in honor of the person most responsible for the discovery and widespread adoption of the underlying scientific principle in question-Dr Arthur H Rosenfeld.

  19. Covariant electrodynamics in linear media: Optical metric

    Science.gov (United States)

    Thompson, Robert T.

    2018-03-01

    While the postulate of covariance of Maxwell's equations for all inertial observers led Einstein to special relativity, it was the further demand of general covariance—form invariance under general coordinate transformations, including between accelerating frames—that led to general relativity. Several lines of inquiry over the past two decades, notably the development of metamaterial-based transformation optics, has spurred a greater interest in the role of geometry and space-time covariance for electrodynamics in ponderable media. I develop a generally covariant, coordinate-free framework for electrodynamics in general dielectric media residing in curved background space-times. In particular, I derive a relation for the spatial medium parameters measured by an arbitrary timelike observer. In terms of those medium parameters I derive an explicit expression for the pseudo-Finslerian optical metric of birefringent media and show how it reduces to a pseudo-Riemannian optical metric for nonbirefringent media. This formulation provides a basis for a unified approach to ray and congruence tracing through media in curved space-times that may smoothly vary among positively refracting, negatively refracting, and vacuum.

  20. Axisymmetric plasma equilibria in a Kerr metric

    Science.gov (United States)

    Elsässer, Klaus

    2001-10-01

    Plasma equilibria near a rotating black hole are considered within the multifluid description. An isothermal two-component plasma with electrons and positrons or ions is determined by four structure functions and the boundary conditions. These structure functions are the Bernoulli function and the toroidal canonical momentum per mass for each species. The quasi-neutrality assumption (no charge density, no toroidal current) allows to solve Maxwell's equations analytically for any axisymmetric stationary metric, and to reduce the fluid equations to one single scalar equation for the stream function \\chi of the positrons or ions, respectively. The basic smallness parameter is the ratio of the skin depth of electrons to the scale length of the metric and fluid quantities, and, in the case of an electron-ion plasma, the mass ratio m_e/m_i. The \\chi-equation can be solved by standard methods, and simple solutions for a Kerr geometry are available; they show characteristic flow patterns, depending on the structure functions and the boundary conditions.

  1. Defining a Standard Metric for Electricity Savings

    Energy Technology Data Exchange (ETDEWEB)

    Brown, Marilyn; Akbari, Hashem; Blumstein, Carl; Koomey, Jonathan; Brown, Richard; Calwell, Chris; Carter, Sheryl; Cavanagh, Ralph; Chang, Audrey; Claridge, David; Craig, Paul; Diamond, Rick; Eto, Joseph H.; Fulkerson, William; Gadgil, Ashok; Geller, Howard; Goldemberg, Jose; Goldman, Chuck; Goldstein, David B.; Greenberg, Steve; Hafemeister, David; Harris, Jeff; Harvey, Hal; Heitz, Eric; Hirst, Eric; Hummel, Holmes; Kammen, Dan; Kelly, Henry; Laitner, Skip; Levine, Mark; Lovins, Amory; Masters, Gil; McMahon, James E.; Meier, Alan; Messenger, Michael; Millhone, John; Mills, Evan; Nadel, Steve; Nordman, Bruce; Price, Lynn; Romm, Joe; Ross, Marc; Rufo, Michael; Sathaye, Jayant; Schipper, Lee; Schneider, Stephen H; Sweeney, James L; Verdict, Malcolm; Vorsatz, Diana; Wang, Devra; Weinberg, Carl; Wilk, Richard; Wilson, John; Worrell, Ernst

    2009-03-01

    The growing investment by governments and electric utilities in energy efficiency programs highlights the need for simple tools to help assess and explain the size of the potential resource. One technique that is commonly used in this effort is to characterize electricity savings in terms of avoided power plants, because it is easier for people to visualize a power plant than it is to understand an abstraction such as billions of kilowatt-hours. Unfortunately, there is no standardization around the characteristics of such power plants. In this letter we define parameters for a standard avoided power plant that have physical meaning and intuitive plausibility, for use in back-of-the-envelope calculations. For the prototypical plant this article settles on a 500 MW existing coal plant operating at a 70percent capacity factor with 7percent T&D losses. Displacing such a plant for one year would save 3 billion kW h per year at the meter and reduce emissions by 3 million metric tons of CO2 per year. The proposed name for this metric is the Rosenfeld, in keeping with the tradition among scientists of naming units in honor of the person most responsible for the discovery and widespread adoption of the underlying scientific principle in question--Dr. Arthur H. Rosenfeld.

  2. Novel Vector Design and Hexosaminidase Variant Enabling Self-Complementary Adeno-Associated Virus for the Treatment of Tay-Sachs Disease.

    Science.gov (United States)

    Karumuthil-Melethil, Subha; Nagabhushan Kalburgi, Sahana; Thompson, Patrick; Tropak, Michael; Kaytor, Michael D; Keimel, John G; Mark, Brian L; Mahuran, Don; Walia, Jagdeep S; Gray, Steven J

    2016-07-01

    GM2 gangliosidosis is a family of three genetic neurodegenerative disorders caused by the accumulation of GM2 ganglioside (GM2) in neuronal tissue. Two of these are due to the deficiency of the heterodimeric (α-β), "A" isoenzyme of lysosomal β-hexosaminidase (HexA). Mutations in the α-subunit (encoded by HEXA) lead to Tay-Sachs disease (TSD), whereas mutations in the β-subunit (encoded by HEXB) lead to Sandhoff disease (SD). The third form results from a deficiency of the GM2 activator protein (GM2AP), a substrate-specific cofactor for HexA. In their infantile, acute forms, these diseases rapidly progress with mental and psychomotor deterioration resulting in death by approximately 4 years of age. After gene transfer that overexpresses one of the deficient subunits, the amount of HexA heterodimer formed would empirically be limited by the availability of the other endogenous Hex subunit. The present study used a new variant of the human HexA α-subunit, μ, incorporating critical sequences from the β-subunit that produce a stable homodimer (HexM) and promote functional interactions with the GM2AP- GM2 complex. We report the design of a compact adeno-associated viral (AAV) genome using a synthetic promoter-intron combination to allow self-complementary (sc) packaging of the HEXM gene. Also, a previously published capsid mutant, AAV9.47, was used to deliver the gene to brain and spinal cord while having restricted biodistribution to the liver. The novel capsid and cassette design combination was characterized in vivo in TSD mice for its ability to efficiently transduce cells in the central nervous system when delivered intravenously in both adult and neonatal mice. This study demonstrates that the modified HexM is capable of degrading long-standing GM2 storage in mice, and it further demonstrates the potential of this novel scAAV vector design to facilitate widespread distribution of the HEXM gene or potentially other similar-sized genes to the nervous system.

  3. Comprehensive Metric Education Project: Implementing Metrics at a District Level Administrative Guide.

    Science.gov (United States)

    Borelli, Michael L.

    This document details the administrative issues associated with guiding a school district through its metrication efforts. Issues regarding staff development, curriculum development, and the acquisition of instructional resources are considered. Alternative solutions are offered. Finally, an overall implementation strategy is discussed with…

  4. Social Media Metrics Importance and Usage Frequency in Latvia

    Directory of Open Access Journals (Sweden)

    Ronalds Skulme

    2017-12-01

    Full Text Available Purpose of the article: The purpose of this paper was to explore which social media marketing metrics are most often used and are most important for marketing experts in Latvia and can be used to evaluate marketing campaign effectiveness. Methodology/methods: In order to achieve the aim of this paper several theoretical and practical research methods were used, such as theoretical literature analysis, surveying and grouping. First of all, theoretical research about social media metrics was conducted. Authors collected information about social media metric grouping methods and the most frequently mentioned social media metrics in the literature. The collected information was used as the foundation for the expert surveys. The expert surveys were used to collect information from Latvian marketing professionals to determine which social media metrics are used most often and which social media metrics are most important in Latvia. Scientific aim: The scientific aim of this paper was to identify if social media metrics importance varies depending on the consumer purchase decision stage. Findings: Information about the most important and most often used social media marketing metrics in Latvia was collected. A new social media grouping framework is proposed. Conclusions: The main conclusion is that the importance and the usage frequency of the social media metrics is changing depending of consumer purchase decisions stage the metric is used to evaluate.

  5. Measurable Control System Security through Ideal Driven Technical Metrics

    Energy Technology Data Exchange (ETDEWEB)

    Miles McQueen; Wayne Boyer; Sean McBride; Marie Farrar; Zachary Tudor

    2008-01-01

    The Department of Homeland Security National Cyber Security Division supported development of a small set of security ideals as a framework to establish measurable control systems security. Based on these ideals, a draft set of proposed technical metrics was developed to allow control systems owner-operators to track improvements or degradations in their individual control systems security posture. The technical metrics development effort included review and evaluation of over thirty metrics-related documents. On the bases of complexity, ambiguity, or misleading and distorting effects the metrics identified during the reviews were determined to be weaker than necessary to aid defense against the myriad threats posed by cyber-terrorism to human safety, as well as to economic prosperity. Using the results of our metrics review and the set of security ideals as a starting point for metrics development, we identified thirteen potential technical metrics - with at least one metric supporting each ideal. Two case study applications of the ideals and thirteen metrics to control systems were then performed to establish potential difficulties in applying both the ideals and the metrics. The case studies resulted in no changes to the ideals, and only a few deletions and refinements to the thirteen potential metrics. This led to a final proposed set of ten core technical metrics. To further validate the security ideals, the modifications made to the original thirteen potential metrics, and the final proposed set of ten core metrics, seven separate control systems security assessments performed over the past three years were reviewed for findings and recommended mitigations. These findings and mitigations were then mapped to the security ideals and metrics to assess gaps in their coverage. The mappings indicated that there are no gaps in the security ideals and that the ten core technical metrics provide significant coverage of standard security issues with 87% coverage. Based

  6. Comparison of luminance based metrics in different lighting conditions

    DEFF Research Database (Denmark)

    Wienold, J.; Kuhn, T.E.; Christoffersen, J.

    In this study, we evaluate established and newly developed metrics for predicting glare using data from three different research studies. The evaluation covers two different targets: 1. How well the user’s perception of glare magnitude correlates to the prediction of the glare metrics? 2. How well...... do the glare metrics describe the subjects’ disturbance by glare? We applied Spearman correlations, logistic regressions and an accuracy evaluation, based on an ROC-analysis. The results show that five of the twelve investigated metrics are failing at least one of the statistical tests. The other...... seven metrics CGI, modified DGI, DGP, Ev, average Luminance of the image Lavg, UGP and UGR are passing all statistical tests. DGP, CGI, DGI_mod and UGP have largest AUC and might be slightly more robust. The accuracy of the predictions of afore mentioned seven metrics for the disturbance by glare lies...

  7. Performance metrics for the evaluation of hyperspectral chemical identification systems

    Science.gov (United States)

    Truslow, Eric; Golowich, Steven; Manolakis, Dimitris; Ingle, Vinay

    2016-02-01

    Remote sensing of chemical vapor plumes is a difficult but important task for many military and civilian applications. Hyperspectral sensors operating in the long-wave infrared regime have well-demonstrated detection capabilities. However, the identification of a plume's chemical constituents, based on a chemical library, is a multiple hypothesis testing problem which standard detection metrics do not fully describe. We propose using an additional performance metric for identification based on the so-called Dice index. Our approach partitions and weights a confusion matrix to develop both the standard detection metrics and identification metric. Using the proposed metrics, we demonstrate that the intuitive system design of a detector bank followed by an identifier is indeed justified when incorporating performance information beyond the standard detection metrics.

  8. Metrics correlation and analysis service (MCAS)

    International Nuclear Information System (INIS)

    Baranovski, Andrew; Dykstra, Dave; Garzoglio, Gabriele; Hesselroth, Ted; Mhashilkar, Parag; Levshina, Tanya

    2010-01-01

    The complexity of Grid workflow activities and their associated software stacks inevitably involves multiple organizations, ownership, and deployment domains. In this setting, important and common tasks such as the correlation and display of metrics and debugging information (fundamental ingredients of troubleshooting) are challenged by the informational entropy inherent to independently maintained and operated software components. Because such an information pool is disorganized, it is a difficult environment for business intelligence analysis i.e. troubleshooting, incident investigation, and trend spotting. The mission of the MCAS project is to deliver a software solution to help with adaptation, retrieval, correlation, and display of workflow-driven data and of type-agnostic events, generated by loosely coupled or fully decoupled middleware.

  9. Metrics correlation and analysis service (MCAS)

    International Nuclear Information System (INIS)

    Baranovski, Andrew; Dykstra, Dave; Garzoglio, Gabriele; Hesselroth, Ted; Mhashilkar, Parag; Levshina, Tanya

    2009-01-01

    The complexity of Grid workflow activities and their associated software stacks inevitably involves multiple organizations, ownership, and deployment domains. In this setting, important and common tasks such as the correlation and display of metrics and debugging information (fundamental ingredients of troubleshooting) are challenged by the informational entropy inherent to independently maintained and operated software components. Because such an information 'pond' is disorganized, it a difficult environment for business intelligence analysis i.e. troubleshooting, incident investigation and trend spotting. The mission of the MCAS project is to deliver a software solution to help with adaptation, retrieval, correlation, and display of workflow-driven data and of type-agnostic events, generated by disjoint middleware.

  10. Development of Technology Transfer Economic Growth Metrics

    Science.gov (United States)

    Mastrangelo, Christina M.

    1998-01-01

    The primary objective of this project is to determine the feasibility of producing technology transfer metrics that answer the question: Do NASA/MSFC technical assistance activities impact economic growth? The data for this project resides in a 7800-record database maintained by Tec-Masters, Incorporated. The technology assistance data results from survey responses from companies and individuals who have interacted with NASA via a Technology Transfer Agreement, or TTA. The goal of this project was to determine if the existing data could provide indications of increased wealth. This work demonstrates that there is evidence that companies that used NASA technology transfer have a higher job growth rate than the rest of the economy. It also shows that the jobs being supported are jobs in higher wage SIC codes, and this indicates improvements in personal wealth. Finally, this work suggests that with correct data, the wealth issue may be addressed.

  11. MESUR metrics from scholarly usage of resources

    CERN Document Server

    CERN. Geneva; Van de Sompel, Herbert

    2007-01-01

    Usage data is increasingly regarded as a valuable resource in the assessment of scholarly communication items. However, the development of quantitative, usage-based indicators of scholarly impact is still in its infancy. The Digital Library Research & Prototyping Team at the Los Alamos National Laboratory's Research library has therefore started a program to expand the set of usage-based tools for the assessment of scholarly communication items. The two-year MESUR project, funded by the Andrew W. Mellon Foundation, aims to define and validate a range of usage-based impact metrics, and issue guidelines with regards to their characteristics and proper application. The MESUR project is constructing a large-scale semantic model of the scholarly community that seamlessly integrates a wide range of bibliographic, citation and usage data. Functioning as a reference data set, this model is analyzed to characterize the intricate networks of typed relationships that exist in the scholarly community. The resulting c...

  12. Einstein metrics and Brans-Dicke superfields

    International Nuclear Information System (INIS)

    Marques, S.

    1988-01-01

    It is obtained here a space conformal to the Einstein space-time, making the transition from an internal bosonic space, constructed with the Majorana constant spinors in the Majorana representation, to a bosonic ''superspace,'' through the use of Einstein vierbeins. These spaces are related to a Grassmann space constructed with the Majorana spinors referred to above, where the ''metric'' is a function of internal bosonic coordinates. The conformal function is a scale factor in the zone of gravitational radiation. A conformal function dependent on space-time coordinates can be constructed in that region when we introduce Majorana spinors which are functions of those coordinates. With this we obtain a scalar field of Brans-Dicke type. 11 refs

  13. Advanced reactors: the case for metric design

    International Nuclear Information System (INIS)

    Ruby, L.

    1986-01-01

    The author argues that DOE should insist that all design specifications for advanced reactors be in the International System of Units (SI) in accordance with the Metric Conversion Act of 1975. Despite a lack of leadership from the federal government, industry has had to move toward conversion in order to compete on world markets. The US is the only major country without a scheduled conversion program. SI avoids the disadvantages of ambiguous names, non-coherent units, multiple units for the same quantity, multiple definitions, as well as barriers to international exchange and marketing and problems in comparing safety and code parameters. With a first step by DOE, the Nuclear Regulatory Commission should add the same requirements to reactor licensing guidelines. 4 references

  14. Analytical Cost Metrics : Days of Future Past

    Energy Technology Data Exchange (ETDEWEB)

    Prajapati, Nirmal [Colorado State Univ., Fort Collins, CO (United States); Rajopadhye, Sanjay [Colorado State Univ., Fort Collins, CO (United States); Djidjev, Hristo Nikolov [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2018-02-20

    As we move towards the exascale era, the new architectures must be capable of running the massive computational problems efficiently. Scientists and researchers are continuously investing in tuning the performance of extreme-scale computational problems. These problems arise in almost all areas of computing, ranging from big data analytics, artificial intelligence, search, machine learning, virtual/augmented reality, computer vision, image/signal processing to computational science and bioinformatics. With Moore’s law driving the evolution of hardware platforms towards exascale, the dominant performance metric (time efficiency) has now expanded to also incorporate power/energy efficiency. Therefore the major challenge that we face in computing systems research is: “how to solve massive-scale computational problems in the most time/power/energy efficient manner?”

  15. Clean Cities 2013 Annual Metrics Report

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, C.; Singer, M.

    2014-10-01

    Each year, the U.S. Department of Energy asks its Clean Cities program coordinators to submit annual reports of their activities and accomplishments for the previous calendar year. Data and information are submitted via an online database that is maintained as part of the Alternative Fuels Data Center (AFDC) at the National Renewable Energy Laboratory (NREL). Coordinators submit a range of data that characterize the membership, funding, projects, and activities of their coalitions. They also submit data about sales of alternative fuels, deployment of alternative fuel vehicles (AFVs) and hybrid electric vehicles (HEVs), idle-reduction (IR) initiatives, fuel economy activities, and programs to reduce vehicle miles traveled (VMT). NREL analyzes the data and translates them into petroleum-use reduction impacts, which are summarized in this 2013 Annual Metrics Report.

  16. Clean Cities 2014 Annual Metrics Report

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, Caley [National Renewable Energy Lab. (NREL), Golden, CO (United States); Singer, Mark [National Renewable Energy Lab. (NREL), Golden, CO (United States)

    2015-12-22

    Each year, the U.S. Department of Energy asks its Clean Cities program coordinators to submit annual reports of their activities and accomplishments for the previous calendar year. Data and information are submitted via an online database that is maintained as part of the Alternative Fuels Data Center (AFDC) at the National Renewable Energy Laboratory (NREL). Coordinators submit a range of data that characterize the membership, funding, projects, and activities of their coalitions. They also submit data about sales of alternative fuels, deployment of alternative fuel vehicles (AFVs) and hybrid electric vehicles (HEVs), idle-reduction (IR) initiatives, fuel economy activities, and programs to reduce vehicle miles traveled (VMT). NREL analyzes the data and translates them into petroleum-use reduction impacts, which are summarized in this 2014 Annual Metrics Report.

  17. Outsourced similarity search on metric data assets

    KAUST Repository

    Yiu, Man Lung

    2012-02-01

    This paper considers a cloud computing setting in which similarity querying of metric data is outsourced to a service provider. The data is to be revealed only to trusted users, not to the service provider or anyone else. Users query the server for the most similar data objects to a query example. Outsourcing offers the data owner scalability and a low-initial investment. The need for privacy may be due to the data being sensitive (e.g., in medicine), valuable (e.g., in astronomy), or otherwise confidential. Given this setting, the paper presents techniques that transform the data prior to supplying it to the service provider for similarity queries on the transformed data. Our techniques provide interesting trade-offs between query cost and accuracy. They are then further extended to offer an intuitive privacy guarantee. Empirical studies with real data demonstrate that the techniques are capable of offering privacy while enabling efficient and accurate processing of similarity queries.

  18. Special metrics and group actions in geometry

    CERN Document Server

    Fino, Anna; Musso, Emilio; Podestà, Fabio; Vezzoni, Luigi

    2017-01-01

    The volume is a follow-up to the INdAM meeting “Special metrics and quaternionic geometry” held in Rome in November 2015. It offers a panoramic view of a selection of cutting-edge topics in differential geometry, including 4-manifolds, quaternionic and octonionic geometry, twistor spaces, harmonic maps, spinors, complex and conformal geometry, homogeneous spaces and nilmanifolds, special geometries in dimensions 5–8, gauge theory, symplectic and toric manifolds, exceptional holonomy and integrable systems. The workshop was held in honor of Simon Salamon, a leading international scholar at the forefront of academic research who has made significant contributions to all these subjects. The articles published here represent a compelling testimony to Salamon’s profound and longstanding impact on the mathematical community. Target readership includes graduate students and researchers working in Riemannian and complex geometry, Lie theory and mathematical physics.

  19. Quasi-metrics, midpoints and applications

    Energy Technology Data Exchange (ETDEWEB)

    Valero, O.

    2017-07-01

    In applied sciences, the scientific community uses simultaneously different kinds of information coming from several sources in order to infer a conclusion or working decision. In the literature there are many techniques for merging the information and providing, hence, a meaningful fused data. In mostpractical cases such fusion methods are based on aggregation operators on somenumerical values, i.e. the aim of the fusion process is to obtain arepresentative number from a finite sequence of numerical data. In the aforementioned cases, the input data presents some kind of imprecision and for thisreason it is represented as fuzzy sets. Moreover, in such problems the comparisons between the numerical values that represent the information described by the fuzzy sets become necessary. The aforementioned comparisons are made by means of a distance defined on fuzzy sets. Thus, the numerical operators aggregating distances between fuzzy sets as incoming data play a central role in applied problems. Recently, J.J. Nieto and A. Torres gave some applications of the aggregation of distances on fuzzy sets to the study of real medical data in /cite{Nieto}. These applications are based on the notion of segment joining two given fuzzy sets and on the notion of set of midpoints between fuzzy sets. A few results obtained by Nieto and Torres have been generalized in turn by Casasnovas and Rossell/'{o} in /cite{Casas,Casas2}. Nowadays, quasi-metrics provide efficient tools in some fields of computer science and in bioinformatics. Motivated by the exposed facts, a study of segments joining two fuzzy sets and of midpoints between fuzzy sets when the measure, used for comparisons, is a quasi-metric has been made in /cite{Casas3, SebVal2013,TiradoValero}. (Author)

  20. Analytic convergence of harmonic metrics for parabolic Higgs bundles

    Science.gov (United States)

    Kim, Semin; Wilkin, Graeme

    2018-04-01

    In this paper we investigate the moduli space of parabolic Higgs bundles over a punctured Riemann surface with varying weights at the punctures. We show that the harmonic metric depends analytically on the weights and the stable Higgs bundle. This gives a Higgs bundle generalisation of a theorem of McOwen on the existence of hyperbolic cone metrics on a punctured surface within a given conformal class, and a generalisation of a theorem of Judge on the analytic parametrisation of these metrics.

  1. Exact solutions of strong gravity in generalized metrics

    International Nuclear Information System (INIS)

    Hojman, R.; Smailagic, A.

    1981-05-01

    We consider classical solutions for the strong gravity theory of Salam and Strathdee in a wider class of metrics with positive, zero and negative curvature. It turns out that such solutions exist and their relevance for quark confinement is explored. Only metrics with positive curvature (spherical symmetry) give a confining potential in a simple picture of the scalar hadron. This supports the idea of describing the hadron as a closed microuniverse of the strong metric. (author)

  2. An accurate metric for the spacetime around neutron stars

    OpenAIRE

    Pappas, George

    2016-01-01

    The problem of having an accurate description of the spacetime around neutron stars is of great astrophysical interest. For astrophysical applications, one needs to have a metric that captures all the properties of the spacetime around a neutron star. Furthermore, an accurate appropriately parameterised metric, i.e., a metric that is given in terms of parameters that are directly related to the physical structure of the neutron star, could be used to solve the inverse problem, which is to inf...

  3. Problems in Systematic Application of Software Metrics and Possible Solution

    OpenAIRE

    Rakic, Gordana; Budimac, Zoran

    2013-01-01

    Systematic application of software metric techniques can lead to significant improvements of the quality of a final software product. However, there is still the evident lack of wider utilization of software metrics techniques and tools due to many reasons. In this paper we investigate some limitations of contemporary software metrics tools and then propose construction of a new tool that would solve some of the problems. We describe the promising prototype, its internal structure, and then f...

  4. Two-dimensional manifolds with metrics of revolution

    International Nuclear Information System (INIS)

    Sabitov, I Kh

    2000-01-01

    This is a study of the topological and metric structure of two-dimensional manifolds with a metric that is locally a metric of revolution. In the case of compact manifolds this problem can be thoroughly investigated, and in particular it is explained why there are no closed analytic surfaces of revolution in R 3 other than a sphere and a torus (moreover, in the smoothness class C ∞ such surfaces, understood in a certain generalized sense, exist in any topological class)

  5. A software quality model and metrics for risk assessment

    Science.gov (United States)

    Hyatt, L.; Rosenberg, L.

    1996-01-01

    A software quality model and its associated attributes are defined and used as the model for the basis for a discussion on risk. Specific quality goals and attributes are selected based on their importance to a software development project and their ability to be quantified. Risks that can be determined by the model's metrics are identified. A core set of metrics relating to the software development process and its products is defined. Measurements for each metric and their usability and applicability are discussed.

  6. Chaos of discrete dynamical systems in complete metric spaces

    International Nuclear Information System (INIS)

    Shi Yuming; Chen Guanrong

    2004-01-01

    This paper is concerned with chaos of discrete dynamical systems in complete metric spaces. Discrete dynamical systems governed by continuous maps in general complete metric spaces are first discussed, and two criteria of chaos are then established. As a special case, two corresponding criteria of chaos for discrete dynamical systems in compact subsets of metric spaces are obtained. These results have extended and improved the existing relevant results of chaos in finite-dimensional Euclidean spaces

  7. Kaubandussanktsioonide otstarbekusest / Jeffrey D. Sachs

    Index Scriptorium Estoniae

    Sachs, Jeffrey D.

    2003-01-01

    Autor leiab, et kaubandus- ja finantssanktsioonide rakendamine diktaatorlikele režiimidele on vale lähenemine, kuna rahvusvaheline marginaalitsemine haavab tavalisi kodanikke enam kui despootlikke süsteeme.

  8. Development of quality metrics for ambulatory pediatric cardiology: Infection prevention.

    Science.gov (United States)

    Johnson, Jonathan N; Barrett, Cindy S; Franklin, Wayne H; Graham, Eric M; Halnon, Nancy J; Hattendorf, Brandy A; Krawczeski, Catherine D; McGovern, James J; O'Connor, Matthew J; Schultz, Amy H; Vinocur, Jeffrey M; Chowdhury, Devyani; Anderson, Jeffrey B

    2017-12-01

    In 2012, the American College of Cardiology's (ACC) Adult Congenital and Pediatric Cardiology Council established a program to develop quality metrics to guide ambulatory practices for pediatric cardiology. The council chose five areas on which to focus their efforts; chest pain, Kawasaki Disease, tetralogy of Fallot, transposition of the great arteries after arterial switch, and infection prevention. Here, we sought to describe the process, evaluation, and results of the Infection Prevention Committee's metric design process. The infection prevention metrics team consisted of 12 members from 11 institutions in North America. The group agreed to work on specific infection prevention topics including antibiotic prophylaxis for endocarditis, rheumatic fever, and asplenia/hyposplenism; influenza vaccination and respiratory syncytial virus prophylaxis (palivizumab); preoperative methods to reduce intraoperative infections; vaccinations after cardiopulmonary bypass; hand hygiene; and testing to identify splenic function in patients with heterotaxy. An extensive literature review was performed. When available, previously published guidelines were used fully in determining metrics. The committee chose eight metrics to submit to the ACC Quality Metric Expert Panel for review. Ultimately, metrics regarding hand hygiene and influenza vaccination recommendation for patients did not pass the RAND analysis. Both endocarditis prophylaxis metrics and the RSV/palivizumab metric passed the RAND analysis but fell out during the open comment period. Three metrics passed all analyses, including those for antibiotic prophylaxis in patients with heterotaxy/asplenia, for influenza vaccination compliance in healthcare personnel, and for adherence to recommended regimens of secondary prevention of rheumatic fever. The lack of convincing data to guide quality improvement initiatives in pediatric cardiology is widespread, particularly in infection prevention. Despite this, three metrics were

  9. 10 CFR 600.306 - Metric system of measurement.

    Science.gov (United States)

    2010-01-01

    ... cause significant inefficiencies or loss of markets to United States firms. (b) Recipients are... Requirements for Grants and Cooperative Agreements With For-Profit Organizations General § 600.306 Metric... Competitiveness Act of 1988 (15 U.S.C. 205) and implemented by Executive Order 12770, states that: (1) The metric...

  10. On the topology defined by Thurston's asymmetric metric

    DEFF Research Database (Denmark)

    Papadopoulos, Athanase; Theret, Guillaume

    2007-01-01

    that the topology that the asymmetric metric L induces on Teichmüller space is the same as the usual topology. Furthermore, we show that L satisfies the axioms of a (not necessarily symmetric) metric in the sense of Busemann and conclude that L is complete in the sense of Busemann....

  11. Path integral measure for first-order and metric gravities

    International Nuclear Information System (INIS)

    Aros, Rodrigo; Contreras, Mauricio; Zanelli, Jorge

    2003-01-01

    The equivalence between the path integrals for first-order gravity and the standard torsion-free, metric gravity in 3 + 1 dimensions is analysed. Starting with the path integral for first-order gravity, the correct measure for the path integral of the metric theory is obtained

  12. Converging from Branching to Linear Metrics on Markov Chains

    DEFF Research Database (Denmark)

    Bacci, Giorgio; Bacci, Giovanni; Larsen, Kim Guldstrand

    2015-01-01

    time in the size of the MC. The upper-approximants are Kantorovich-like pseudometrics, i.e. branching-time distances, that converge point-wise to the linear-time metrics. This convergence is interesting in itself, since it reveals a nontrivial relation between branching and linear-time metric...

  13. Effects of Metric Change on Workers’ Tools and Training.

    Science.gov (United States)

    1981-07-01

    understanding of the metric system, and particularly a lack of fluency in converting customary measurements to metric measuremerts, may increase the...assembly, installing, and repairing occupations 84 Painting, plastering, waterproofing, cementing , and related occupations 85 Excavating, grading... cementing , and related occupations 85 Excavating, grading, paving, and related occupations 86 Construction occupations, n.e.c. 89 Structural work

  14. 48 CFR 611.002-70 - Metric system implementation.

    Science.gov (United States)

    2010-10-01

    ... with security, operations, economic, technical, logistical, training and safety requirements. (3) The... total cost of the retrofit, including redesign costs, exceeds $50,000; (ii) Metric is not the accepted... office with an explanation for the disapproval. (7) The in-house operating metric costs shall be...

  15. Empirical analysis of change metrics for software fault prediction

    NARCIS (Netherlands)

    Choudhary, Garvit Rajesh; Kumar, Sandeep; Kumar, Kuldeep; Mishra, Alok; Catal, Cagatay

    2018-01-01

    A quality assurance activity, known as software fault prediction, can reduce development costs and improve software quality. The objective of this study is to investigate change metrics in conjunction with code metrics to improve the performance of fault prediction models. Experimental studies are

  16. Predicting class testability using object-oriented metrics

    NARCIS (Netherlands)

    M. Bruntink (Magiel); A. van Deursen (Arie)

    2004-01-01

    textabstractIn this paper we investigate factors of the testability of object-oriented software systems. The starting point is given by a study of the literature to obtain both an initial model of testability and existing OO metrics related to testability. Subsequently, these metrics are evaluated

  17. Comparative Study of Trace Metrics between Bibliometrics and Patentometrics

    Directory of Open Access Journals (Sweden)

    Fred Y. Ye

    2016-06-01

    Full Text Available Purpose: To comprehensively evaluate the overall performance of a group or an individual in both bibliometrics and patentometrics. Design/methodology/approach: Trace metrics were applied to the top 30 universities in the 2014 Academic Ranking of World Universities (ARWU — computer sciences, the top 30 ESI highly cited papers in the computer sciences field in 2014, as well as the top 30 assignees and the top 30 most cited patents in the National Bureau of Economic Research (NBER computer hardware and software category. Findings: We found that, by applying trace metrics, the research or marketing impact efficiency, at both group and individual levels, was clearly observed. Furthermore, trace metrics were more sensitive to the different publication-citation distributions than the average citation and h-index were. Research limitations: Trace metrics considered publications with zero citations as negative contributions. One should clarify how he/she evaluates a zero-citation paper or patent before applying trace metrics. Practical implications: Decision makers could regularly examinine the performance of their university/company by applying trace metrics and adjust their policies accordingly. Originality/value: Trace metrics could be applied both in bibliometrics and patentometrics and provide a comprehensive view. Moreover, the high sensitivity and unique impact efficiency view provided by trace metrics can facilitate decision makers in examining and adjusting their policies.

  18. Self-dual metrics with self-dual Killing vectors

    International Nuclear Information System (INIS)

    Tod, K.P.; Ward, R.S.

    1979-01-01

    Twistor methods are used to derive a class of solutions to Einstein's vacuum equations, with anti-self dual Weyl tensor. In particular, all metrics with a Killing vector whose derivative is anti-self-dual and which admit a real positive-definite section are exhibited and shown to coincide with the metrics of Hawking. (author)

  19. Scalar metric fluctuations in space-time matter inflation

    International Nuclear Information System (INIS)

    Anabitarte, Mariano; Bellini, Mauricio

    2006-01-01

    Using the Ponce de Leon background metric, which describes a 5D universe in an apparent vacuum: G-bar AB =0, we study the effective 4D evolution of both, the inflaton and gauge-invariant scalar metric fluctuations, in the recently introduced model of space-time matter inflation

  20. 22 CFR 226.15 - Metric system of measurement.

    Science.gov (United States)

    2010-04-01

    ... 22 Foreign Relations 1 2010-04-01 2010-04-01 false Metric system of measurement. 226.15 Section 226.15 Foreign Relations AGENCY FOR INTERNATIONAL DEVELOPMENT ADMINISTRATION OF ASSISTANCE AWARDS TO U.S. NON-GOVERNMENTAL ORGANIZATIONS Pre-award Requirements § 226.15 Metric system of measurement. (a...

  1. Presic-Boyd-Wong Type Results in Ordered Metric Spaces

    Directory of Open Access Journals (Sweden)

    Satish Shukla

    2014-04-01

    Full Text Available The purpose of this paper is to prove some Presic-Boyd-Wong type fixed point theorems in ordered metric spaces. The results of this paper generalize the famous results of Presic and Boyd-Wong in ordered metric spaces. We also initiate the homotopy result in product spaces. Some examples are provided which illustrate the results proved herein.

  2. A heuristic way of obtaining the Kerr metric

    International Nuclear Information System (INIS)

    Enderlein, J.

    1997-01-01

    An intuitive, straightforward way of finding the metric of a rotating black hole is presented, based on the algebra of differential forms. The representation obtained for the metric displays a simplicity which is not obvious in the usual Boyer Lindquist coordinates. copyright 1997 American Association of Physics Teachers

  3. On the L2-metric of vortex moduli spaces

    NARCIS (Netherlands)

    Baptista, J.M.

    2011-01-01

    We derive general expressions for the Kähler form of the L2-metric in terms of standard 2-forms on vortex moduli spaces. In the case of abelian vortices in gauged linear sigma-models, this allows us to compute explicitly the Kähler class of the L2-metric. As an application we compute the total

  4. Probabilistic G-Metric space and some fixed point results

    Directory of Open Access Journals (Sweden)

    A. R. Janfada

    2013-01-01

    Full Text Available In this note we introduce the notions of generalized probabilistic metric spaces and generalized Menger probabilistic metric spaces. After making our elementary observations and proving some basic properties of these spaces, we are going to prove some fixed point result in these spaces.

  5. Socio-Technical Security Metrics (Dagstuhl Seminar 14491)

    NARCIS (Netherlands)

    Gollmann, Dieter; Herley, Cormac; Koenig, Vincent; Pieters, Wolter; Sasse, Martina Angela

    2015-01-01

    This report documents the program and the outcomes of Dagstuhl Seminar 14491 "Socio-Technical Security Metrics". In the domain of safety, metrics inform many decisions, from the height of new dikes to the design of nuclear plants. We can state, for example, that the dikes should be high enough to

  6. Radiating c metric: an example of a proper Ricci Collineation

    International Nuclear Information System (INIS)

    Aulestia, L.; Nunez, L.; Patino, A.; Rago, H.; Herrera, L.

    1984-01-01

    A generalization of the charged c metric to the nonstationary case is given. The possibility of associating the energy-momentum tensor with the electromagnetic or neutrino field is discussed. It is shown that, for a specific choice of the time-dependent parameters, the metric admits at least a two-parameter group of proper Ricci collineations

  7. On the Metric-based Approximate Minimization of Markov Chains

    DEFF Research Database (Denmark)

    Bacci, Giovanni; Bacci, Giorgio; Larsen, Kim Guldstrand

    2018-01-01

    In this paper we address the approximate minimization problem of Markov Chains (MCs) from a behavioral metric-based perspective. Specifically, given a finite MC and a positive integer k, we are looking for an MC with at most k states having minimal distance to the original. The metric considered...

  8. On the Metric-Based Approximate Minimization of Markov Chains

    DEFF Research Database (Denmark)

    Bacci, Giovanni; Bacci, Giorgio; Larsen, Kim Guldstrand

    2017-01-01

    We address the behavioral metric-based approximate minimization problem of Markov Chains (MCs), i.e., given a finite MC and a positive integer k, we are interested in finding a k-state MC of minimal distance to the original. By considering as metric the bisimilarity distance of Desharnais at al...

  9. Implementing Metrics at a District Level. Administrative Guide. Revised Edition.

    Science.gov (United States)

    Borelli, Michael L.; Morelli, Sandra Z.

    Administrative concerns in implementing metrics at a district level are discussed and specific recommendations are made regarding them. The paper considers the extent and manner of staff training necessary, the curricular changes associated with metrics, and the distinctions between elementary and secondary programs. Appropriate instructional…

  10. 20 CFR 435.15 - Metric system of measurement.

    Science.gov (United States)

    2010-04-01

    ... 20 Employees' Benefits 2 2010-04-01 2010-04-01 false Metric system of measurement. 435.15 Section 435.15 Employees' Benefits SOCIAL SECURITY ADMINISTRATION UNIFORM ADMINISTRATIVE REQUIREMENTS FOR... metric system is the preferred measurement system for U.S. trade and commerce. The Act requires each...

  11. Choosing the Greenest Synthesis: A Multivariate Metric Green Chemistry Exercise

    Science.gov (United States)

    Mercer, Sean M.; Andraos, John; Jessop, Philip G.

    2012-01-01

    The ability to correctly identify the greenest of several syntheses is a particularly useful asset for young chemists in the growing green economy. The famous univariate metrics atom economy and environmental factor provide insufficient information to allow for a proper selection of a green process. Multivariate metrics, such as those used in…

  12. 76 FR 53885 - Patent and Trademark Resource Centers Metrics

    Science.gov (United States)

    2011-08-30

    ... DEPARTMENT OF COMMERCE United States Patent and Trademark Office Patent and Trademark Resource Centers Metrics ACTION: Proposed collection; comment request. SUMMARY: The United States Patent and... ``Patent and Trademark Resource Centers Metrics comment'' in the subject line of the message. Mail: Susan K...

  13. Author Impact Metrics in Communication Sciences and Disorder Research

    Science.gov (United States)

    Stuart, Andrew; Faucette, Sarah P.; Thomas, William Joseph

    2017-01-01

    Purpose: The purpose was to examine author-level impact metrics for faculty in the communication sciences and disorder research field across a variety of databases. Method: Author-level impact metrics were collected for faculty from 257 accredited universities in the United States and Canada. Three databases (i.e., Google Scholar, ResearchGate,…

  14. Evaluating hydrological model performance using information theory-based metrics

    Science.gov (United States)

    The accuracy-based model performance metrics not necessarily reflect the qualitative correspondence between simulated and measured streamflow time series. The objective of this work was to use the information theory-based metrics to see whether they can be used as complementary tool for hydrologic m...

  15. Using metrics in stability of stochastic programming problems

    Czech Academy of Sciences Publication Activity Database

    Houda, Michal

    2005-01-01

    Roč. 13, č. 1 (2005), s. 128-134 ISSN 0572-3043 R&D Projects: GA ČR(CZ) GA402/04/1294 Institutional research plan: CEZ:AV0Z10750506 Keywords : stochastic programming * quantitative stability * Wasserstein metrics * Kolmogorov metrics * simulation study Subject RIV: BB - Applied Statistics, Operational Research

  16. A Practical Method for Collecting Social Media Campaign Metrics

    Science.gov (United States)

    Gharis, Laurie W.; Hightower, Mary F.

    2017-01-01

    Today's Extension professionals are tasked with more work and fewer resources. Integrating social media campaigns into outreach efforts can be an efficient way to meet work demands. If resources go toward social media, a practical method for collecting metrics is needed. Collecting metrics adds one more task to the workloads of Extension…

  17. Discriminatory Data Mapping by Matrix-Based Supervised Learning Metrics

    NARCIS (Netherlands)

    Strickert, M.; Schneider, P.; Keilwagen, J.; Villmann, T.; Biehl, M.; Hammer, B.

    2008-01-01

    Supervised attribute relevance detection using cross-comparisons (SARDUX), a recently proposed method for data-driven metric learning, is extended from dimension-weighted Minkowski distances to metrics induced by a data transformation matrix Ω for modeling mutual attribute dependence. Given class

  18. Performance evaluation of routing metrics for wireless mesh networks

    CSIR Research Space (South Africa)

    Nxumalo, SL

    2009-08-01

    Full Text Available for WMN. The routing metrics have not been compared with QoS parameters. This paper is a work in progress of the project in which researchers want to compare the performance of different routing metrics in WMN using a wireless test bed. Researchers...

  19. 27 CFR 4.72 - Metric standards of fill.

    Science.gov (United States)

    2010-04-01

    ... 27 Alcohol, Tobacco Products and Firearms 1 2010-04-01 2010-04-01 false Metric standards of fill. 4.72 Section 4.72 Alcohol, Tobacco Products and Firearms ALCOHOL AND TOBACCO TAX AND TRADE BUREAU, DEPARTMENT OF THE TREASURY LIQUORS LABELING AND ADVERTISING OF WINE Standards of Fill for Wine § 4.72 Metric...

  20. ISS Logistics Hardware Disposition and Metrics Validation

    Science.gov (United States)

    Rogers, Toneka R.

    2010-01-01

    I was assigned to the Logistics Division of the International Space Station (ISS)/Spacecraft Processing Directorate. The Division consists of eight NASA engineers and specialists that oversee the logistics portion of the Checkout, Assembly, and Payload Processing Services (CAPPS) contract. Boeing, their sub-contractors and the Boeing Prime contract out of Johnson Space Center, provide the Integrated Logistics Support for the ISS activities at Kennedy Space Center. Essentially they ensure that spares are available to support flight hardware processing and the associated ground support equipment (GSE). Boeing maintains a Depot for electrical, mechanical and structural modifications and/or repair capability as required. My assigned task was to learn project management techniques utilized by NASA and its' contractors to provide an efficient and effective logistics support infrastructure to the ISS program. Within the Space Station Processing Facility (SSPF) I was exposed to Logistics support components, such as, the NASA Spacecraft Services Depot (NSSD) capabilities, Mission Processing tools, techniques and Warehouse support issues, required for integrating Space Station elements at the Kennedy Space Center. I also supported the identification of near-term ISS Hardware and Ground Support Equipment (GSE) candidates for excessing/disposition prior to October 2010; and the validation of several Logistics Metrics used by the contractor to measure logistics support effectiveness.