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Sample records for roecklein scott norris

  1. Early vitrectomy effective for Norrie disease.

    Science.gov (United States)

    Walsh, Mark K; Drenser, Kimberly A; Capone, Antonio; Trese, Michael T

    2010-04-01

    To review our experience with Norrie disease to determine if early vitrectomy abrogates the natural history of this rare disease; namely, bilateral no light perception visual acuity and phthisis bulbi. We retrospectively reviewed the medical records of all patients seen in our tertiary care pediatric retinal clinical practice from 1988 through 2008 with a potential diagnosis of Norrie disease. Inclusion required not only clinical findings consistent with Norrie disease but also genetics and/or a family history consistent with Norrie disease. Medical record review revealed 14 boys with clinically diagnosed Norrie disease and either Norrie disease gene (NDP) mutations noted on genetic testing (13 patients) and/or a clear family history consistent with Norrie disease (4 patients). All 14 boys with definite Norrie disease had vitrectomy with or without lensectomy in at least 1 eye prior to 12 months of age. Of the 14 boys with definite Norrie disease, 7 maintained at least light perception visual acuity in 1 eye and 3 had no light perception visual acuity bilaterally; visual acuity data were not available for 4 patients. Only 2 of 24 (8%) eyes became phthisical. Historically, no treatment has been offered to mitigate the dismal natural history of Norrie disease. We recommend consideration of early vitrectomy in Norrie disease.

  2. Genetics Home Reference: Norrie disease

    Science.gov (United States)

    ... Email Facebook Twitter Home Health Conditions Norrie disease Norrie disease Printable PDF Open All Close All Enable Javascript to view the expand/collapse boxes. Description Norrie disease is an inherited eye disorder that leads to ...

  3. Audiologic features of Norrie disease.

    Science.gov (United States)

    Halpin, Chris; Owen, Grace; Gutiérrez-Espeleta, Gustavo A; Sims, Katherine; Rehm, Heidi L

    2005-07-01

    Norrie disease is an X-linked recessive disorder in which patients are born blind and develop sensory hearing loss in adolescence. The hearing loss associated with Norrie disease has been shown in a genetically altered knockout mouse to involve dysfunction of the stria vascularis; most other structures are preserved until the later stages of the disease. The objective of this study was to characterize the audiologic phenotype of Norrie disease for comparison with the pathophysiologic mechanism. The design combined two series of clinical audiologic evaluations, with special attention to speech intelligibility. The audiologic results for 12 affected individuals and 10 carriers show that patients with Norrie disease retain high speech intelligibility scores even when the threshold loss is severe. The cochlear mechanism-- failure of the stria vascularis-- accounts for some of the higher values in the wide distribution of speech scores in cases with similar pure tone audiograms.

  4. Epilepsy phenotypes in siblings with Norrie disease.

    Science.gov (United States)

    Okumura, Akihisa; Arai, Eisuke; Kitamura, Yuri; Abe, Shinpei; Ikeno, Mitsuru; Fujimaki, Takuro; Yamamoto, Toshiyuki; Shimizu, Toshiaki

    2015-11-01

    Norrie disease is an X-linked recessive disorder that is characterized by congenital blindness. Although epileptic seizures are observed in some patients with Norrie disease, little is known about this phenomenon. Here, we report the manifestation of epilepsy in siblings with Norrie disease to increase our knowledge of epilepsy in this condition. Three brothers with congenital blindness were diagnosed with Norrie disease after genetic analyses indicated the deletion of exon 2 of the NDP gene. The eldest brother had suffered from epileptic seizures since the age of 11years, and his seizures were resistant to antiepileptic drugs. Although the second brother had no epileptic seizures, the youngest sibling had experiences epileptic seizures since the age of 8years. His seizures were controlled using lamotrigine and levetiracetam. An electroencephalography (EEG) revealed epileptiform discharges in the occipital areas in all three brothers. A study of these patients will increase our knowledge of epilepsy in patients with Norrie disease. Copyright © 2015 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  5. Prenatal exclusion of Norrie's disease.

    OpenAIRE

    Redmond, R M; Graham, C A; Kelly, E D; Coleman, M; Nevin, N C

    1992-01-01

    We report on the use of DNA marker probes and linkage analysis to exclude Norrie's disease in the male fetus of a high risk carrier. There are no clinical markers in females carrying the Norrie's disease gene; thus DNA linkage analysis is an essential technique in the management of families 'at-risk' for this severe ophthalmic disease. The principles of DNA linkage are discussed.

  6. Norrie disease gene is distinct from the monoamine oxidase genes.

    Science.gov (United States)

    Sims, K B; Ozelius, L; Corey, T; Rinehart, W B; Liberfarb, R; Haines, J; Chen, W J; Norio, R; Sankila, E; de la Chapelle, A

    1989-09-01

    The genes for MAO-A and MAO-B appear to be very close to the Norrie disease gene, on the basis of loss and/or disruption of the MAO genes and activities in atypical Norrie disease patients deleted for the DXS7 locus; linkage among the MAO genes, the Norrie disease gene, and the DXS7 locus; and mapping of all these loci to the chromosomal region Xp11. The present study provides evidence that the MAO genes are not disrupted in "classic" Norrie disease patients. Genomic DNA from these "nondeletion" Norrie disease patients did not show rearrangements at the MAOA or DXS7 loci. Normal levels of MAO-A activities, as well as normal amounts and size of the MAO-A mRNA, were observed in cultured skin fibroblasts from these patients, and MAO-B activity in their platelets was normal. Catecholamine metabolites evaluated in plasma and urine were in the control range. Thus, although some atypical Norrie disease patients lack both MAO-A and MAO-B activities, MAO does not appear to be an etiologic factor in classic Norrie disease.

  7. A novel mutation in the Norrie disease gene.

    Science.gov (United States)

    Ott, S; Patel, R J; Appukuttan, B; Wang, X; Stout, J T

    2000-04-01

    Norrie disease is an X-linked recessive disorder characterized by congenital blindness and in some cases mental retardation and deafness.(1) The variability of signs among patients often complicates diagnosis. Signs such as an ocular pseudoglioma, progressive deafness, and mental disturbance are considered classic features.(2) Only one third of patients with Norrie disease have sensorineural deafness, and approximately one half of the affected individuals exhibit mental retardation, often with psychotic features.(3) Histologic analysis has suggested that retinal dysgenesis occurs early in eye development and involves cells in the inner wall of the optic cup.(4) The gene associated with Norrie disease was identified in 1992. (5,6) We report a novel mutation identified in a patient in whom Norrie disease was diagnosed.

  8. Norrie's disease in an Asian family.

    OpenAIRE

    Harendra de Silva, D G; de Silva, D B

    1988-01-01

    Norrie's disease, a congenital progressive oculo-acoustico-cerebral degenerative condition, is a sex linked recessive disorder. Previously described as atrophia oculi congeneti, it is associated with bilateral pseudotumour of the retina, lens, and corneal opacities, and phthisis bulbi. Some patients develop progressive deterioration of mental function and hearing. We report a Sri Lankan family with typical features of Norrie's disease.

  9. [A literature review of Norrie disease].

    Science.gov (United States)

    Ohba, N; Isashiki, Y

    1996-02-01

    Norrie disease is a rare genetic disorder characterized by bilateral congenital blindness. The salient clinical feature early in life is a dense, white, vascularized mass behind each lens due to maldeveloped retina. Cataracts and corneal opacities are developed in young childhood, followed by bulbar atrophies. Histopathologic examination suggests primary vitreoretinal dysplasia because of developmental arrest of the retina in the middle embryonic stage. Occasional patients show psychomotor retardation or progressive hearing loss as part of a multisystem disorder. The disease is transmitted by an X-linked recessive form of inheritance, with sons of female carriers having a 50% risk for expressing the disease. In recent years, a candidate gene for Norrie disease has been isolated and characterized, which encompasses 27 kilobases and consists of three exons interspersed by two introns. Microdeletions and a variety of point mutations in the disease gene were identified in Norrie patients, although the genotype-phenotype correlation remains to be defined, and molecular diagnosis is now available for Norrie disease. The encoded protein has homology to a protein domain involving mucins and TGF beta, which may play an essential role in targeting of retinal/neural connections.

  10. Bilateral Norrie's disease in identical twins.

    OpenAIRE

    Sukumaran, K

    1991-01-01

    A case of Norrie's disease in an identical twins is reported. No positive family history was obtained. The couple had no other children. The older of the twins died at the age of 9 months of uncertain cause. To the best of my knowledge this is the first case of Norrie's disease reported in Malaysia. And its occurrence in an identical twins is very rare.

  11. Norrie disease gene is distinct from the monoamine oxidase genes

    OpenAIRE

    Sims, Katherine B.; Ozelius, Laurie; Corey, Timothy; Rinehart, William B.; Liberfarb, Ruth; Haines, Jonathan; Chen, Wei Jane; Norio, Reijo; Sankila, Eeva; de la Chapelle, Albert; Murphy, Dennis L.; Gusella, James; Breakefield, Xandra O.

    1989-01-01

    The genes for MAO-A and MAO-B appear to be very close to the Norrie disease gene, on the basis of loss and /or disruption of the MAO genes and activities in atypical Norrie disease patients deleted for the DXS7 locus; linkage among the MAO genes, the Norrie disease gene, and the DXS7 locus; and mapping of all these loci to the chromosomal region Xp11. The present study provides evidence that the MAO genes are not disrupted in “classic” Norrie disease patients. Genomic DNA from these “nondelet...

  12. In-utero diagnosis of Norrie disease by ultrasonography.

    Science.gov (United States)

    Redmond, R M; Vaughan, J I; Jay, M; Jay, B

    1993-03-01

    Obstetric ultrasonography of an obligate Norrie disease carrier revealed bilateral retinal detachments in a third trimester male fetus. Postnatal examination confirmed the diagnosis of Norrie disease. DNA linkage analysis with the markers L1.28 and MAO had been uninformative for this family. This report suggests that retinal detachment occurs late in the gestation of the affected fetus.

  13. Real-time Remote Data Online For Norris Geyser Basin in Yellowstone National Park

    Science.gov (United States)

    Perry, J. E.; Lowenstern, J. B.; Clor, L.; Cervelli, P. F.; Allen, S. T.; Heasler, H.; Moloney, T.

    2010-12-01

    Perry, John, Lowenstern, Jacob, Cervelli, Peter, Clor, Laura, Heasler, Henry, Allen, Scott, Moloney, Tim During June 2010, ten 900MHz wireless temperature data loggers (nodes) were installed around the Norris Geyser Basin to monitor geothermal features, streams and soil temperatures. The loggers can provide near real-time updates on temperature variations within 0.2 deg C due to hydrothermal discharges or subsurface fluid migration. Each sensor node is programmed to measure the temperature every two minutes and automatically upload data to the base station computer daily. The hardware consists of a waterproof case containing an M5 logger (made by Marathon Products, Inc.®) with internal memory, lithium D-cell batteries and a 900 MHz, 1-W-transceiver and 5 meter long Teflon-coated probe with a thermistor sensor. Tethered stub or panel antennas are oriented to optimize signal strength to the base station near the Norris Museum. A 0.61 meter-long base-station antenna located 10m high provides signal to the furthest node over 850 meters away with most being “line-of-sight”. A 20-meter coaxial cable and lightning grounding wire connects the base-station antenna to an Ethernet-radio connected to the YNP local-area network. A server located 26-km north at Mammoth Hot Springs requests data at regular intervals (normally daily), archives the information, and then sends it to the USGS for further archiving and internet distribution. During periods of unusual hydrothermal behavior, data can be requested as needed, and it is possible to set user-programmable alarm limits for notification. The RF network is designed to monitor changes from three different sub-basins at Norris (Gray Lakes, Steamboat-Echinus and Porcelain Basin), the main Tantalus Creek drainage, and five individual thermal features (Constant, Porkchop, Steamboat and Echinus Geysers, and Opalescent Spring). The logger installed in Nuphar Lake provides ambient temperatures controlled solely by local

  14. A characteristic phenotypic retinal appearance in Norrie disease.

    Science.gov (United States)

    Drenser, Kimberly A; Fecko, Alice; Dailey, Wendy; Trese, Michael T

    2007-02-01

    To describe a striking retinal finding that the authors have only seen in Norrie disease eyes and to determine if a particular genotype corresponds to this dramatic presentation. This is a retrospective, interventional case report of four patients seen in the clinic over a 1-year period. All patients had analysis of the Norrie gene by direct sequencing. All patients presented with a similar retinal appearance of dense stalk tissue, globular dystrophic retina, and peripheral avascular retina with pigmentary changes. Each patient was found to have a mutation in the Norrie gene affecting a cystine residue in the cystine knot domain. The mutations are predicted to disrupt the structure of the protein product, norrin, which is required for activation of the Wnt receptor:beta-catenin pathway. No other vitreoretinopathy that the authors have seen demonstrates this characteristic retinal presentation of severe retinal dysplasia. All four patients were found to have mutations in the Norrie gene which alter the cystine knot motif. Mutations affecting this domain appear to have devastating effects on retinal development and indicate phenotype correlates with mutations affecting the cystine knot domain.

  15. A novel Norrie disease pseudoglioma gene mutation, c.-1_2delAAT, responsible for Norrie disease in a Chinese family

    OpenAIRE

    Zhang, Xin-Yu; Jiang, Wei-Ying; Chen, Lu-Ming; Chen, Su-Qin

    2013-01-01

    AIM:To investigate the genetic findings and phenotypic characteristics of a Chinese family with Norrie disease (ND).METHODS:Molecular genetic analysis and clinical examinations were performed on a Chinese family with ND. Mutations in the Norrie disease pseudoglioma (NDP) gene were detected by direct sequencing. Haplotypes were constructed and compared with the phenotypes in the family. Evolutionary comparisons and mutant open reading frame (ORF) prediction were also undertaken.RESULTS:Two fam...

  16. Lack of association of the Norrie disease gene with retinoschisis phenotype.

    Science.gov (United States)

    Shastry, B S; Hiraoka, M; Trese, M T

    2000-01-01

    It has been reported recently that mice carrying a disrupted Norrie disease gene produced alterations in the murine eye that are similar to congenital retinoschisis. Therefore, it was of interest to determine whether mutations in the Norrie disease gene can account for the disease in families with retinoschisis that do not carry mutations in the retinoschisis gene. The patient set comprised 5 cases of retinoschisis (1 familial and 4 sporadic), all unrelated to each other. Fundus examination of affected individuals showed foveal and peripheral schisis, and the visual acuity range was 20/40-20/60. Peripheral blood specimens were collected from affected and unaffected family members. DNA was extracted and amplified by polymerase chain reaction amplification of exons of the Norrie disease gene. The amplified products were sequenced by the dideoxy chain termination method. The data revealed no disease-specific sequence alterations in the Norrie disease gene. Although we cannot completely exclude the possibility of the Norrie disease gene as a candidate gene, the above results suggest that the structural and functional changes in the Norrie disease gene are not associated with clinically typical retinoschisis families that do not contain mutations in the coding regions and splice sites of the retinoschisis gene.

  17. Mutations in the NDP gene: contribution to Norrie disease, familial exudative vitreoretinopathy and retinopathy of prematurity.

    Science.gov (United States)

    Dickinson, Joanne L; Sale, Michèle M; Passmore, Abraham; FitzGerald, Liesel M; Wheatley, Catherine M; Burdon, Kathryn P; Craig, Jamie E; Tengtrisorn, Supaporn; Carden, Susan M; Maclean, Hector; Mackey, David A

    2006-01-01

    To examine the contribution of mutations within the Norrie disease (NDP) gene to the clinically similar retinal diseases Norrie disease, X-linked familial exudative vitreoretinopathy (FEVR), Coat's disease and retinopathy of prematurity (ROP). A dataset comprising 13 Norrie-FEVR, one Coat's disease, 31 ROP patients and 90 ex-premature babies of Norrie disease patients. Furthermore, a previously described 14-bp deletion located in the 5' unstranslated region of the NDP gene was detected in three cases of regressed ROP. A second heterozygotic 14-bp deletion was detected in an unaffected ex-premature girl. Only two of the 13 Norrie-FEVR index cases had the full features of Norrie disease with deafness and mental retardation. Two novel mutations within the coding region of the NDP gene were found, one associated with a severe disease phenotypes of Norrie disease and the other with FEVR. A deletion within the non-coding region was associated with only mild-regressed ROP, despite the presence of low birthweight, prematurity and exposure to oxygen. In full-term children with retinal detachment only 15% appear to have the full features of Norrie disease and this is important for counselling parents on the possible long-term outcome.

  18. Prenatal exclusion of Norrie disease with flanking DNA markers.

    Science.gov (United States)

    Gal, A; Uhlhaas, S; Glaser, D; Grimm, T

    1988-10-01

    Three polymorphic DNA markers linked to the locus of Norrie disease were used for indirect genotype analysis in a ten-wk-old fetus at risk for the disease. When haplotypes of the family members and the estimated recombination frequency between Norrie gene and each of the DNA marker loci DXS7, DXS84, and DXS146 were taken into account, the risk that the fetus had inherited the mutation was about 1%.

  19. Scott S Snyder

    Indian Academy of Sciences (India)

    Home; Journals; Pramana – Journal of Physics. Scott S Snyder. Articles written in Pramana – Journal of Physics. Volume 62 Issue 3 March 2004 pp 565-568 Experimental Particle Physics. Prospects for Higgs search at DØ · Scott S Snyder DØ Collaboration · More Details Abstract Fulltext PDF. The status of the Higgs search ...

  20. [Norrie syndrome: identification of carriers by segregation analysis with flanking DNA markers].

    Science.gov (United States)

    Körner, J; Uhlhaas, S; Neugebauer, M; Gal, A

    1989-01-01

    Norrie disease is an X-linked recessive disorder. Affected males present with congenital blindness. Additionally, hearing loss and psychotic behavior may occur at any time. Since carriers are clinically healthy, they can only be identified by genetic means. Daughters of carriers or sisters of affected males have an à priori 50% risk of being carriers themselves. Close linkage has been found between the Norrie disease locus (NDP) and the DNA locus DXS7 mapped to Xp11.3. For genetic counselling, this linkage relationship allows carriers of the disease to be identified in informative families. We describe a large pedigree with Norrie disease. Segregation analysis was carried out with DXS7 and a second flanking marker, DXS255, both linked to NDP. In this way, three females at risk were identified who had a high probability of being carriers for Norrie disease.

  1. Norrie disease in a family with a manifesting female carrier.

    Science.gov (United States)

    Sims, K B; Irvine, A R; Good, W V

    1997-04-01

    To show that Norrie disease can occur in a girl and to describe her ophthalmologic and genetic features. Amplification of DNA polymerase chain reaction and sequencing of asymmetric polymerase chain reaction for exon 3 were performed on the blood specimen obtained from a girl born with bilateral retinal detachments. A female child with bilateral retinal detachment who had 2 uncles in whom Norrie disease had already been diagnosed. The child had a mutation in the third exon (T776-->A; Ile 123-->Asn) identical to the mutation found in her uncles. Norrie disease can occur in girls. The most likely explanation is nonrandom or unfavorable X inactivation, although timing of development of the peripheral retina and its blood supply could render it vulnerable to effects of the mutant allele at a critical developmental phase.

  2. A novel missense Norrie disease mutation associated with a severe ocular phenotype.

    Science.gov (United States)

    Khan, Arif O; Shamsi, Farrukh A; Al-Saif, Amr; Kambouris, Marios

    2004-01-01

    Clinical findings and pedigree analysis led to the diagnosis of severe Norrie disease in two brothers. DNA sequencing demonstrated a novel missense mutation (703G>T) that significantly alters predicted protein structure. Less severe retinal developmental disease may be associated with milder mutations in the Norrie disease gene.

  3. Laser photocoagulation at birth prevents blindness in Norrie's disease diagnosed using amniocentesis.

    Science.gov (United States)

    Chow, Clement C; Kiernan, Daniel F; Chau, Felix Y; Blair, Michael P; Ticho, Benjamin H; Galasso, John M; Shapiro, Michael J

    2010-12-01

    To report the first case of prophylactic laser treatment to prevent blindness in a patient who was diagnosed with Norrie's disease by genetic testing with amniocentesis. Case report. A 2-year-old white boy with Norrie's disease. A 37-week gestational age male with a family history of Norrie's disease was born via Cesarean section after the mother had undergone prenatal amniocentesis fetal-genetic testing at 23 weeks of gestation. A C520T (nonsense) mutation was found in the Norrie's disease gene. After examination under anesthesia confirmed the diagnosis on the first day of life, laser photocoagulation was applied to the avascular retina bilaterally. The patient was followed closely by ophthalmology, pediatrics, and occupational therapy departments. Functional outcome, as documented by Teller visual acuity and formal occupational therapy testing, and anatomic outcome, as documented by Retcam photography and fluorescein angiography. Complete regression of extraretinal fibrovascular proliferation was observed 1 month after laser treatment. No retinal detachment had occurred to date at 24 months. Teller visual acuity at 23 months of life was 20/100 in both eyes. The patient's vision and developmental milestones were age appropriate. Pre-term genetic diagnosis with immediate laser treatment after birth may preserve vision in individuals affected with Norrie's disease. Copyright © 2010 American Academy of Ophthalmology. Published by Elsevier Inc. All rights reserved.

  4. [Norrie-Wardburg syndrome].

    Science.gov (United States)

    Skevas, A; Kastanioudakis, I; Daniilidis, B; Exarchakos, G

    1992-10-01

    We describe a case of a 25-year old patient with typical Norrie-Warburg Syndrome. From the first year of his life he was found to be blind, with bilateral sensorineural loss of hearing. Audiological examination showed symmetrical moderate bilateral sensorineural hearing loss. His hearing loss was refractory to treatment for the last eight years. Because of timely diagnosis of hearing loss and timely fitting of a hearing aid, the patient could study at school and graduate from university education. Disease carriers who are clinically healthy can be identified only via chromosome analysis.

  5. Citation for Scott Doney

    Science.gov (United States)

    Glover, David M.; Doney, Scott

    “A man of genius makes no mistakes. His errors are volitional and are the portals of discovery. James Joyce, Ulysses (1922). ”After collaborating with Scott Doney for the past 14 years I know what Joyce meant. When working with someone as bright as Scott it inevitably happens that you just don't understand. And because we're trained skeptics the question immediately arises, ”has our friend and colleague made a mistake?“ But we're wrong; we just didn't see the portal through which people like Scott had already proceeded. Certainly this is what we reserve these awards of ‘outstandingness’ for; those whose insight lead through the portals of discovery”.

  6. Ocular findings associated with a Cys39Arg mutation in the Norrie disease gene.

    Science.gov (United States)

    Joos, K M; Kimura, A E; Vandenburgh, K; Bartley, J A; Stone, E M

    1994-12-01

    To diagnose the carriers and noncarriers in a family affected with Norrie disease based on molecular analysis. Family members from three generations, including one affected patient, two obligate carriers, one carrier identified with linkage analysis, one noncarrier identified with linkage analysis, and one female family member with indeterminate carrier status, were examined clinically and electrophysiologically. Linkage analysis had previously failed to determine the carrier status of one female family member in the third generation. Blood samples were screened for mutations in the Norrie disease gene with single-strand conformation polymorphism analysis. The mutation was characterized by dideoxy-termination sequencing. Ophthalmoscopy and electroretinographic examination failed to detect the carrier state. The affected individuals and carriers in this family were found to have a transition from thymidine to cytosine in the first nucleotide of codon 39 of the Norrie disease gene, causing a cysteine-to-arginine mutation. Single-strand conformation polymorphism analysis identified a patient of indeterminate status (by linkage) to be a noncarrier of Norrie disease. Ophthalmoscopy and electroretinography could not identify carriers of this Norrie disease mutation. Single-strand conformation polymorphism analysis was more sensitive and specific than linkage analysis in identifying carriers in this family.

  7. Familial cases of Norrie disease detected by copy number analysis.

    Science.gov (United States)

    Arai, Eisuke; Fujimaki, Takuro; Yanagawa, Ai; Fujiki, Keiko; Yokoyama, Toshiyuki; Okumura, Akihisa; Shimizu, Toshiaki; Murakami, Akira

    2014-09-01

    Norrie disease (ND, MIM#310600) is an X-linked disorder characterized by severe vitreoretinal dysplasia at birth. We report the results of causative NDP gene analysis in three male siblings with Norrie disease and describe the associated phenotypes. Three brothers with suspected Norrie disease and their mother presented for clinical examination. After obtaining informed consent, DNA was extracted from the peripheral blood of the proband, one of his brothers and his unaffected mother. Exons 1-3 of the NDP gene were amplified by polymerase chain reaction (PCR), and direct sequencing was performed. Multiplex ligation-dependent probe amplification (MLPA) was also performed to search for copy number variants in the NDP gene. The clinical findings of the three brothers included no light perception, corneal opacity, shallow anterior chamber, leukocoria, total retinal detachment and mental retardation. Exon 2 of the NDP gene was not amplified in the proband and one brother, even when the PCR primers for exon 2 were changed, whereas the other two exons showed no mutations by direct sequencing. MLPA analysis showed deletion of exon 2 of the NDP gene in the proband and one brother, while there was only one copy of exon 2 in the mother. Norrie disease was diagnosed in three patients from a Japanese family by clinical examination and was confirmed by genetic analysis. To localize the defect, confirmation of copy number variation by the MLPA method was useful in the present study.

  8. Presentación de un paciente con enfermedad de Norrie

    OpenAIRE

    Santana Hernández, Elayne Esther; Tamayo Chang, Víctor Jesús; Marrero Infante, Juan Miguel; Márquez Ibáñez, Nilson; Artigas Cuenca, Arianne

    2013-01-01

    La enfermedad de Norrie es un raro trastorno genético ligado al cromosoma X que afecta principalmente a los ojos y casi siempre conduce a la ceguera. Además de los síntomas oculares congénitos, algunos pacientes sufren una pérdida progresiva de la audición sobre todo a partir de su segunda década de vida, mientras que otros afectados pueden tener problemas mentales. Los pacientes con la enfermedad de Norrie pueden desarrollar cataratas y leucocoria, junto con otras afecciones de desarrollo en...

  9. Recombinational event between Norrie disease and DXS7 loci.

    Science.gov (United States)

    Ngo, J T; Spence, M A; Cortessis, V; Sparkes, R S; Bateman, J B

    1988-07-01

    We have identified a family affected with X-linked recessive Norrie disease, in which a recombinational event occurred between the disease locus and the DXS7 locus identified by the probe L1.28. The addition of our family brings the total of published informative families to seven, with a maximum lod score of 7.58 at a recombination frequency of 0.038 +/- 0.036. This finding indicates that the L1.28 probe is useful but may not be completely reliable for prenatal diagnosis and that the gene for Norrie disease is not within the DNA sequence identified by the L1.28 probe.

  10. Further linkage data on Norrie disease.

    Science.gov (United States)

    Kivlin, J D; Sanborn, G E; Wright, E; Cannon, L; Carey, J

    1987-03-01

    We obtained a LOD score of +1.61 using DNA marker L1.28 in 5 generations of a family with Norrie disease, raising the total LOD score to +5.42. There have been no recombinations between the 2 loci in any family to date, making the marker useful for genetic counseling.

  11. Norrie disease resulting from a gene deletion: clinical features and DNA studies.

    OpenAIRE

    Donnai, D; Mountford, R C; Read, A P

    1988-01-01

    We describe a family in which two boys with Norrie disease have a deletion of the DXS7 locus. The deletion does not extend as far distally as the OTC or DXS84 loci. A full clinical description of the patients is given to help establish the range of manifestations of Norrie disease. There is no evidence of any other X linked disease in our patients.

  12. Norrie disease resulting from a gene deletion: clinical features and DNA studies.

    Science.gov (United States)

    Donnai, D; Mountford, R C; Read, A P

    1988-02-01

    We describe a family in which two boys with Norrie disease have a deletion of the DXS7 locus. The deletion does not extend as far distally as the OTC or DXS84 loci. A full clinical description of the patients is given to help establish the range of manifestations of Norrie disease. There is no evidence of any other X linked disease in our patients.

  13. Cochlear Implants and Psychiatric Assessments: a Norrie Disease Case Report.

    Science.gov (United States)

    Jacques, Denis; Dubois, Thomas; Zdanowicz, Nicolas; Gilain, Chantal; Garin, Pierre

    2017-09-01

    It is important to perform psychiatric assessments of adult patients who are candidates for cochlear implants both to screen them for psychiatric disorders and to assess their understanding and compliance with the procedure. Deafness is a factor of difficulty for conducting in-depth psychiatric interviews, but concomitant blindness may make it impossible. After a description of Norrie disease, a rare disease in which blindness and deafness may occur together, we propose a case report of a patient suffering from the disease and who consulted in view of a cochlear implant. Early information on cochlear implants appears to be necessary before total deafness occurs in patients suffering from Norrie disease. An inventory of digital communication tools that can be used by the patient is also highly valuable. Research should be supported for a more systematic use of psychiatric assessments prior to cochlear implants. In the special case of Norrie disease, we recommend early screening for mental retardation and related psychotic disorders and, depending on the patient's level of understanding, preventive information on the benefits and limits of cochlear implants before total deafness occurs.

  14. Localization of the Norrie disease gene mRNA by in situ hybridization.

    Science.gov (United States)

    Hartzer, M K; Cheng, M; Liu, X; Shastry, B S

    1999-07-15

    Norrie disease is a rare X-linked recessive neurodevelopmental disorder. The affected males manifest congenital blindness, which is often associated with hearing loss, mental retardation and psychiatric problems. Genetic linkage studies have localized the gene to the short arm of the X-chromosome and the gene has been isolated recently. The encoded protein is a member of the superfamily of growth factors containing a cystine knot motif and may be involved in cell adhesion and neurodevelopment. Molecular genetic analysis revealed a large number of missense, nonsense, deletion, and splice-site mutations among Norrie patients. In order to further determine the role of the Norrie disease gene, we studied the distribution pattern of its mRNA in the retina and in brain by in situ hybridization. The results show abundant hybridization signals in outer nuclear, inner nuclear, and ganglion cell layers of the retina in all three species (mice, rabbit, and human) examined. There was no significant expression in the vitreous body, lens, and rod outer segment. High expression levels were also observed in the cerebellar granular layer, hippocampus, olfactory bulb, cortex, and epithelium of the rabbit brain. These data suggest that the Norrie disease gene could play a critical role in the differentiation or maintenance of the differentiated state of the retina.

  15. Isolated Norrie disease in a female caused by a balanced translocation t(X,6).

    Science.gov (United States)

    Meire, F M; Lafaut, B A; Speleman, F; Hanssens, M

    1998-12-01

    This is the second report of Norrie disease in a female patient with a de-novo balanced translocation t(X,6) with breakpoint at the location of the Norrie gene. At the age of 3 months, a girl was referred for suspected congenital glaucoma. The right eye was microphthalmic and ultrasonography was compatible with persistent hyperplasia of the primary vitreous. The left eye was also microphthalmic. The left cornea was larger than the right. The anterior chamber was virtual and leukocoria was evident. The eye felt hard digitally. Ultrasonography indicated an organized retinal detachment. The pathologic findings are reported and are compatible with Norrie disease.

  16. [Norrie syndrome (author's transl)].

    Science.gov (United States)

    Schmitz-Valckenberg, P; Scholz, W

    1977-10-01

    The Norrie syndrome, an x-chromosomal linked, recessive genetic disease, is described using ophthalmologic and genetic examinations of a family in three generations. The main symptom of this syndrome is retinal detachment with hemorrhages, which generally leads to blindness in early childhood. In addition to this, in 25--35% of the cases mental retardation and hearing problems are found. Special significance is to be attached to the differential diagnosis of this syndrome because the vascular proliferation on the retina is a non-specific, secondary reaction in children, which also occurs symptomatically in several other diseases.

  17. Inversion (X)(p11.4q22) associated with Norrie disease in a four generation family.

    Science.gov (United States)

    Pettenati, M J; Rao, P N; Weaver, R G; Thomas, I T; McMahan, M R

    1993-03-01

    We report on a 4-generation family in which Norrie disease occurs together with a pericentric inversion of the X chromosome in all affected males and carrier females. The breakpoint in the short arm of the X chromosome appears to be at the purported location of the Norrie disease gene. This is the second report of an association between Norrie disease and a chromosome aberration involving Xp11, and the first report of a specific gene disruption, thus physical gene location, due to a pericentric chromosome inversion.

  18. Norrie disease and exudative vitreoretinopathy in families with affected female carriers.

    Science.gov (United States)

    Shastry, B S; Hiraoka, M; Trese, D C; Trese, M T

    1999-01-01

    Norrie disease (ND) is a rare X-linked recessive disorder characterized by congenital blindness, which is often associated with sensorineural hearing loss and mental retardation. X-linked familial exudative vitreoretinopathy (FEVR) is a hereditary disorder characterized by an abnormality of the peripheral retina and is not associated with systemic diseases. X-linked recessive disorders generally do not affect females. Here we show that female carriers can be associated with manifestation of an X-linked disorder. A four-generation family with an affected female, and a history of congenital blindness and hearing loss, was identified through the pro-band. A second family, with a full-term female infant, was evaluated through ophthalmic examinations and found to exhibit ocular features, such as retinal folds, retinal detachment and peripheral exudates. Peripheral blood specimens were collected from several affected and unaffected family members. DNA was extracted and analyzed by single-strand conformation polymorphism (SSCP) following polymerase chain reaction (PCR) amplification of the exons of the Norrie disease gene. The amplified products were sequenced by the dideoxy chain termination method. In an X-linked four-generation family, a novel missense (A118D) mutation in the third exon of the Norrie disease gene, was identified. The mutation was transmitted through three generations and cosegregated with the disease. The affected maternal grandmother and the unaffected mother carried the same mutation in one of their alleles. In an unrelated sporadic family, a heterozygous missense mutation (C96Y) was identified in the third exon of the Norrie disease gene in an affected individual. Analysis of exon-1 and 2 of the Norrie disease gene did not reveal any additional sequence alterations in these families. The mutations were not detected in the unaffected family members and the 116 normal unrelated controls, suggesting that they are likely to be the pathogenic mutations

  19. Retinoschisislike alterations in the mouse eye caused by gene targeting of the Norrie disease gene.

    Science.gov (United States)

    Ruether, K; van de Pol, D; Jaissle, G; Berger, W; Tornow, R P; Zrenner, E

    1997-03-01

    To investigate the retinal function and morphology of mice carrying a replacement mutation in exon 2 of the Norrie disease gene. Recently, Norrie disease mutant mice have been generated using gene targeting technology. The mutation removes the 56 N-terminal amino acids of the Norrie gene product. Ganzfeld electroretinograms (ERGs) were obtained in five animals hemizygous or homozygous for the mutant gene and in three female animals heterozygous for the mutant gene. As controls, three males carrying the wild-type gene were examined. Electroretinogram testing included rod a- and b-wave V-log I functions, oscillatory potentials, and cone responses. The fundus morphology has been visualized by scanning laser ophthalmoscopy. Rod and cone ERG responses and fundus morphology were not significantly different among female heterozygotes and wild-type mice. In contrast, the hemizygous mice displayed a severe loss of ERG b-wave, leading to a negatively shaped scotopic ERG and a marked reduction of oscillatory potentials. The a-wave was normal at low intensities, and only with brighter flashes was there a moderate amplitude loss. Cone amplitudes were barely recordable in the gene-targeted males. Ophthalmoscopy revealed snowflakelike vitreal changes, retinoschisis, and pigment epithelium irregularities in hemizygotes and homozygotes, but no changes in female heterozygotes. The negatively shaped scotopic ERG in male mice with a Norrie disease gene mutation probably was caused by retinoschisis. Pigment epithelial changes and degenerations of the outer retina are relatively mild. These findings may be a clue to the embryonal retinoschisislike pathogenesis of Norrie disease in humans or it may indicate a different expression of the Norrie disease gene defect in mice compared to that in humans.

  20. Norrie disease: first mutation report and prenatal diagnosis in an Indian family.

    Science.gov (United States)

    Ghosh, Manju; Sharma, Shipra; Shastri, Shivaram; Arora, Sadhna; Shukla, Rashmi; Gupta, Neerja; Deka, Deepika; Kabra, Madhulika

    2012-11-01

    Norrie Disease (ND) is a rare X-linked recessive disorder characterised by congenital blindness due to severe retinal dysgenesis. Hearing loss and intellectual disability is present in 30-50 % cases. ND is caused by mutations in the NDP gene, located at Xp11.3. The authors describe mutation analysis of a proband with ND and subsequently prenatal diagnosis. Sequence analysis of the NDP gene revealed a hemizygous missense mutation arginine to serine in codon 41 (p.Arg41Ser) in the affected child. Mother was carrier for the mutation. In a subsequent di-chorionic di-amniotic pregnancy, the authors performed prenatal diagnosis by mutation analysis on chorionic villi sample at 11 wk of gestation. The fetuses were unaffected. This is a first mutation report and prenatal diagnosis of a familial case of Norrie disease from India. The importance of genetic testing of Norrie disease for confirmation, carrier testing, prenatal diagnosis and genetic counseling is emphasized.

  1. Abnormal protein in the cerebrospinal fluid of patients with a submicroscopic X-chromosomal deletion associated with Norrie disease: preliminary report.

    Science.gov (United States)

    Joy, J E; Poglod, R; Murphy, D L; Sims, K B; de la Chapelle, A; Sankila, E M; Norio, R; Merril, C R

    1991-01-01

    Norrie disease is an X-linked recessive disorder characterized by congenital blindness and, in many cases, mental retardation. Some Norrie disease cases have been shown to be associated with a submicroscopic deletion in chromosomal region Xp11.3. Cerebrospinal fluid (CSF) was collected from four male patients with an X-chromosomal deletion associated with Norrie disease. CSF proteins were resolved using two-dimensional gel electrophoresis and then analyzed by computer using the Elsie V program. Our analysis revealed a protein that appears to be altered in patients with Norrie disease deletion.

  2. Blanco White and Walter Scott Blanco white y Walter Scott

    Directory of Open Access Journals (Sweden)

    Fernando DURÁN LÓPEZ

    2011-01-01

    Full Text Available The first edition of Ivanhoe; a romance. By the author of Waverley was published in Edinburgh in 1820. From the beginning of year 1823, José María Blanco White translated several excerpts from Ivanhoe in the numbers 1-3 of the magazine Variedades, owned by the publisher Rudolph Ackermann. in these articles and other later writings, the translator praised Scott as a model for a new way of painting history in a narrative. This paper studies his ideas on Scott’s historical novel, as well as his translation technique, compared with that of José Joaquín de Mora. En 1820 se publicó en Edimburgo la primera edición de Ivanhoe; a romance. By the author of Waverley. Desde comienzos de 1823, en los tres primeros números de su revista Variedades, promovida por el editor Rudolph Ackermann, José María Blanco White tradujo varios fragmentos de Ivanhoe entre grandes elogios. Asimismo, Blanco White tomó a Scott como modelo de referencia de una nueva manera de pintar la historia por medio de la novela en otros varios escritos críticos de años posteriores. El artículo estudia las ideas de Blanco White acerca de la novela histórica de Scott y su técnica como traductor, comparada con la de José Joaquín Mora.

  3. [Analysis of gene mutation in a Chinese family with Norrie disease].

    Science.gov (United States)

    Zhang, Tian-xiao; Zhao, Xiu-li; Hua, Rui; Zhang, Jin-song; Zhang, Xue

    2012-09-01

    To detect the pathogenic mutation in a Chinese family with Norrie disease. Clinical diagnosis was based on familial history, clinical sign and B ultrasonic examination. Peripheral blood samples were obtained from all available members in a Chinese family with Norrie disease. Genomic DNA was extracted from lymphocytes by the standard SDS-proteinase K-phenol/chloroform method. Two coding exons and all intron-exon boundaries of the NDP gene were PCR amplified using three pairs of primers and subjected to automatic DNA sequence. The causative mutation was confirmed by restriction enzyme analysis and genotyping analysis in all members. Sequence analysis of NDP gene revealed a missense mutation c.220C > T (p.Arg74Cys) in the proband and his mother. Further mutation identification by restriction enzyme analysis and genotyping analysis showed that the proband was homozygote of this mutation. His mother and other four unaffected members (III3, IV4, III5 and II2) were carriers of this mutation. The mutant amino acid located in the C-terminal cystine knot-like domain, which was critical motif for the structure and function of NDP. A NDP missense mutation was identified in a Chinese family with Norrie disease.

  4. Prenatal Diagnosis of a Case of Norrie Disease with Late Development of Bilateral Ocular Malformation.

    Science.gov (United States)

    Wu, Li Hong; Chen, Li-Hong; Xie, Hongning; Xie, Ying-Jun

    2017-06-01

    We report a case of Norrie disease, diagnosed by prenatal ultrasound, confirmed by Sanger sequencing of the DNP gene from the aborted fetal cord blood and histologically. Prenatal ultrasound revealed no abnormality in either eye at 22 +1 and 31 +4 gestational weeks, but at 36 +5 gestational weeks both eyes had massive vitreous cavity opacities with complete retinal detachment. Norrie disease was initially suspected because of an older male sibling with the disease. To our knowledge, prenatal ultrasound diagnosis of Norrie disease has been previously described only one case in 1993 in a 34-week-old fetus. The normal eye development until after 31 + 4 gestational weeks provides insight into the first manifestation and then the rapid progression of the eye disease.

  5. Bilateral persistent fetal vasculature due to a mutation in the Norrie disease protein gene.

    Science.gov (United States)

    Payabvash, Seyedmehdi; Anderson, Jill S; Nascene, David R

    2015-12-01

    We report a case of a 7-week-old boy with bilateral leukocoria and asymmetric microphthalmia who was found to have Norrie disease. Symmetrically hyperdense globes with no evidence of calcification were seen on CT scan. The MRI showed bilateral retinal hemorrhages resulting in conical vitreous chambers-narrow at the optic disc and widened toward the lens-characteristic of persistent fetal vasculature. Genetic evaluation revealed a previously undescribed mutation in the Norrie disease protein gene. © The Author(s) 2015.

  6. ASK Talks with W. Scott Cameron

    Science.gov (United States)

    Cameron, W. Scott

    2002-01-01

    This paper presents an interview with Scott Cameron who is the Capital Systems Manager for the Food and Beverage Global Business Unit of Procter and Gamble. He has been managing capital projects and mentoring other project managers for the past 20 years at Procter and Gamble within its Beauty Care, Health Care, Food and Beverage, and Fabric and Home Care Businesses. Scott also has been an Academy Sharing Knowledge (ASK) feature writer since Volume One.

  7. A novel nonsense mutation in the NDP gene in a Chinese family with Norrie disease

    OpenAIRE

    Liu, Deyuan; Hu, Zhengmao; Peng, Yu; Yu, Changhong; Liu, Yalan; Mo, Xiaoyun; Li, Xiaoping; Lu, Lina; Xu, Xiaojuan; Su, Wei; Pan, Qian; Xia, Kun

    2010-01-01

    Purpose Norrie disease (ND), a rare X-linked recessive disorder, is characterized by congenital blindness and, occasionally, mental retardation and hearing loss. ND is caused by the Norrie Disease Protein gene (NDP), which codes for norrin, a cysteine-rich protein involved in ocular vascular development. Here, we report a novel mutation of NDP that was identified in a Chinese family in which three members displayed typical ND symptoms and other complex phenotypes, such as cerebellar atrophy, ...

  8. Knudsen effects in a Scott effect experiment.

    Science.gov (United States)

    Wells, C. W.; Wood, L. T.; Hildebrandt, A. F.

    1973-01-01

    A thermal torque sometimes observed in Scott effect measurements has been studied experimentally and an explanation for the thermal torque proposed. The magnitude of the thermal torque can be comparable to the Scott torque depending on geometrical and thermal anisotropies. The thermal torque is predicted to decrease with application of an axial magnetic field.

  9. [Analysis of the NDP gene in a Chinese family with X-linked recessive Norrie disease].

    Science.gov (United States)

    Mei, Libin; Huang, Yanru; Pan, Qian; Liang, Desheng; Wu, Lingqian

    2015-05-01

    The purpose of the current research was to investigate the NDP (Norrie disease protein) gene in one Chinese family with Norrie disease (ND) and to characterize the related clinical features. Clinical data of the proband and his family members were collected. Complete ophthalmic examinations were carried out on the proband. Genomic DNA was extracted from peripheral blood leukocytes of 35 family members. Molecular analysis of the NDP gene was performed by polymerase chain reaction and direct sequencing of all exons and flanking regions. A hemizygous NDP missense mutation c.362G > A (p.Arg121Gln) in exon 3 was identified in the affected members, but not in any of the unaffected family individuals. The missense mutation c.362G > A in NDP is responsible for the Norrie disease in this family. This discovery will help provide the family members with accurate and reliable genetic counseling and prenatal diagnosis.

  10. Metric Scott analysis

    Czech Academy of Sciences Publication Activity Database

    Ben Yaacov, I.; Doucha, Michal; Nies, A.; Tsankov, T.

    2017-01-01

    Roč. 318, October (2017), s. 46-87 ISSN 0001-8708 Institutional support: RVO:67985840 Keywords : continuous logic * infinitary logic * Scott sentence Subject RIV: BA - General Mathematics OBOR OECD: Pure mathematics Impact factor: 1.373, year: 2016 http://www.sciencedirect.com/science/article/pii/S0001870816309896?via%3Dihub

  11. Relativistic Scott correction in self-generated magnetic fields

    DEFF Research Database (Denmark)

    Erdos, Laszlo; Fournais, Søren; Solovej, Jan Philip

    2012-01-01

    /3}$ and it is unchanged by including the self-generated magnetic field. We prove the first correction term to this energy, the so-called Scott correction of the form $S(\\alpha Z) Z^2$. The current paper extends the result of \\cite{SSS} on the Scott correction for relativistic molecules to include a self......-generated magnetic field. Furthermore, we show that the corresponding Scott correction function $S$, first identified in \\cite{SSS}, is unchanged by including a magnetic field. We also prove new Lieb-Thirring inequalities for the relativistic kinetic energy with magnetic fields....

  12. Norrie disease: extraocular clinical manifestations in 56 patients.

    Science.gov (United States)

    Smith, Sharon E; Mullen, Thomas E; Graham, Dionne; Sims, Katherine B; Rehm, Heidi L

    2012-08-01

    Norrie disease (ND) is an X-linked recessive disorder characterized by congenital blindness, progressive sensorineural hearing loss and cognitive impairment. The ocular phenotype has been well described, while the extraocular manifestations of the disorder are not well understood. We present the data from the Norrie Disease Registry, which consists of 56 patients with detailed clinical histories and genotype data. This study represents the largest, detailed investigation into the phenotypic spectrum of ND to date and more importantly expands knowledge of the extraocular clinical manifestations. We identify several novel aspects of the syndrome that will improve the management of these patients. In particular, we expand our understanding of the neurologic manifestations in ND and identify a chronic seizure disorder in approximately 10% of all patients. In addition, details of the hearing phenotype are described including the median age of onset (12 years of age) and how genotype affects onset. Moreover, we find vascular disease to be a significant component of ND; and vascular health should be, in the future, a component of patient clinical care. In summary, the results expand our understanding of the phenotypic variability and genotypic heterogeneity in ND patients. Copyright © 2012 Wiley Periodicals, Inc.

  13. Voltage-dependent ion channels in the mouse RPE: comparison with Norrie disease mice.

    Science.gov (United States)

    Wollmann, Guido; Lenzner, Steffen; Berger, Wolfgang; Rosenthal, Rita; Karl, Mike O; Strauss, Olaf

    2006-03-01

    We studied electrophysiological properties of cultured retinal pigment epithelial (RPE) cells from mouse and a mouse model for Norrie disease. Wild-type RPE cells revealed the expression of ion channels known from other species: delayed-rectifier K(+) channels composed of Kv1.3 subunits, inward rectifier K(+) channels, Ca(V)1.3 L-type Ca(2+) channels and outwardly rectifying Cl(-) channels. Expression pattern and the ion channel characteristics current density, blocker sensitivity, kinetics and voltage-dependence were compared in cells from wild-type and Norrie mice. Although no significant differences were observed, our study provides a base for future studies on ion channel function and dysfunction in transgenic mouse models.

  14. Fetal loss in homozygous mutant Norrie disease mice: a new role of Norrin in reproduction.

    Science.gov (United States)

    Luhmann, Ulrich F O; Meunier, Dominique; Shi, Wei; Lüttges, Angela; Pfarrer, Christiane; Fundele, Reinald; Berger, Wolfgang

    2005-08-01

    Mutations in the Norrie disease pseudoglioma gene (NDP) are known to cause X-linked recessive Norrie disease. In addition, NDP mutations have been found in other vasoproliferative retinopathies such as familial exudative vitreoretinopathy, retinopathy of prematurity, and Coats disease, suggesting a role for Norrin in vascular development. Here we report that female mice homozygous for the Norrie disease pseudoglioma homolog (Ndph) knockout allele exhibit almost complete infertility, while heterozygous females and hemizygous males are fertile. Histological examinations and RNA in situ hybridization analyses revealed defects in vascular development and decidualization in pregnant Ndph-/- females from embryonic day 7 (E7) onwards, resulting in embryonic loss. Using RT-PCR analyses we also demonstrate, for the first time, the expression of Ndph in mouse uteri and deciduae as well as the expression of NDP in human placenta. Taken together, these data provide strong evidence for Norrin playing an important role in female reproductive tissues. Copyright 2005 Wiley-Liss, Inc

  15. A novel missense NDP mutation [p.(Cys93Arg)] with a manifesting carrier in an austrian family with Norrie disease.

    Science.gov (United States)

    Parzefall, Thomas; Lucas, Trevor; Ritter, Markus; Ludwig, Martin; Ramsebner, Reinhard; Frohne, Alexandra; Schöfer, Christian; Hengstschläger, Markus; Frei, Klemens

    2014-01-01

    Norrie disease is a rare, X-linked genetic syndrome characterized by combined congenital blindness and progressive hearing impairment. Norrie disease is caused by alterations in the NDP gene encoding the growth factor norrin that plays a key role in vascular development and stabilization of the eye, inner ear and brain. We identified a family with 3 affected deafblind males and a single female carrier presenting with a serous retinal detachment but normal hearing. Genetic analysis revealed a novel c.277T>C missense mutation causing the substitution of a hydrophobic cysteine to a hydrophilic arginine [p.(Cys93Arg)] within the highly conserved cysteine knot domain of the norrin protein. These results should expand the scope for amniocentesis and genetic testing for Norrie disease which is gaining in importance due to novel postnatal therapeutic concepts to alleviate the devastating retinal symptoms of Norrie disease. © 2014 S. Karger AG, Basel.

  16. Elucidation of Molecular Pathogenic Mechanisms of Norrie Disease

    OpenAIRE

    Luhmann, Ulrich F.O.

    2010-01-01

    Summary Norrie disease (ND) is a rare X-linked recessive congenital blindness, sometimes associated with deafness and mental retardation. In this thesis the molecular pathogenic mechanisms of this syndrome should be elucidated using the Ndph knockout mouse model. Gene expression studies but also histology and protein biochemistry were used to characterize the affected organs, eye and brain. Gene expression analyses of eyes at p21 using cDNA subtrac...

  17. Novel mutations in Norrie disease gene in Japanese patients with Norrie disease and familial exudative vitreoretinopathy.

    Science.gov (United States)

    Kondo, Hiroyuki; Qin, Minghui; Kusaka, Shunji; Tahira, Tomoko; Hasebe, Haruyuki; Hayashi, Hideyuki; Uchio, Eiichi; Hayashi, Kenshi

    2007-03-01

    To search for mutations in the Norrie disease gene (NDP) in Japanese patients with familial exudative vitreoretinopathy (FEVR) and Norrie disease (ND) and to delineate the mutation-associated clinical features. Direct sequencing after polymerase chain reaction of all exons of the NDP gene was performed on blood collected from 62 probands (31 familial and 31 simplex) with FEVR, from 3 probands with ND, and from some of their family members. The clinical symptoms and signs in the patients with mutations were assessed. X-inactivation in the female carriers was examined in three FEVR families by using leukocyte DNA. Four novel mutations-I18K, K54N, R115L, and IVS2-1G-->A-and one reported mutation, R97P, in the NDP gene were identified in six families. The severity of vitreoretinopathy varied among these patients. Three probands with either K54N or R115L had typical features of FEVR, whereas the proband with R97P had those of ND. Families with IVS2-1G-->A exhibited either ND or FEVR characteristics. A proband with I18K presented with significant phenotypic heterogeneity between the two eyes. In addition, affected female carriers in a family harboring the K54N mutation presented with different degrees of vascular abnormalities in the periphery of the retina. X-inactivation profiles indicated that the skewing was not significantly different between affected and unaffected women. These observations indicate that mutations of the NDP gene can cause ND and 6% of FEVR cases in the Japanese population. The X-inactivation assay with leukocytes may not be predictive of the presence of a mutation in affected female carriers.

  18. Philip Glass, Scott Walker ja Sigur Ros! / Immo Mihkelson

    Index Scriptorium Estoniae

    Mihkelson, Immo, 1959-

    2007-01-01

    Pimedate Ööde 11. filmifestivali muusikafilme - Austraalia "Glass: Philipi portree 12 osas" (rež. Scott Hicks), Islandi "Sigur Ros kodus" (rež. Dean DeBois), Suurbritannia "Scott Walker: 30 Century Man" (rež. Stephen Kijak)

  19. Norrie disease as part of a complex syndrome explained by a submicroscopic deletion of the X chromosome.

    Science.gov (United States)

    Bleeker-Wagemakers, E M; Zweije-Hofman, I; Gal, A

    1988-11-01

    A 15-year-old male patient with the typical ocular symptoms of Norrie disease is described. Additionally, he presents severe mental retardation, growth disturbances, hypogonadism, and increased susceptibility to infections. This complex syndrome is apparently segregating through three generations: four other male relatives of the patient were blind from birth and died from recurrent infections between the ages of three to 15 months. The DNA sequence of the DXS7 locus (L1.28 probe), known to be closely linked to the Norrie gene, was not found in the patient's DNA. This result suggests that the more complex clinical picture seen is the result of a deletion of the X chromosome spanning DXS7, the Norrie gene, and several neighbouring loci. A detailed clinical description of the patient is given and compared to that of similar cases.

  20. Norrie disease. Diagnosis of a simplex case by DNA analysis.

    Science.gov (United States)

    Chynn, E W; Walton, D S; Hahn, L B; Dryja, T P

    1996-09-01

    Norrie disease is a rare, X-linked recessive disorder characterized by congenital blindness due to malformed retinas. We describe a simplex patient who had leukokoria and whose clinical diagnosis was confirmed only after molecular genetics analysis. DNA analysis was also used to determine the carrier status of relatives of the proband.

  1. Generative complexity of Gray-Scott model

    Science.gov (United States)

    Adamatzky, Andrew

    2018-03-01

    In the Gray-Scott reaction-diffusion system one reactant is constantly fed in the system, another reactant is reproduced by consuming the supplied reactant and also converted to an inert product. The rate of feeding one reactant in the system and the rate of removing another reactant from the system determine configurations of concentration profiles: stripes, spots, waves. We calculate the generative complexity-a morphological complexity of concentration profiles grown from a point-wise perturbation of the medium-of the Gray-Scott system for a range of the feeding and removal rates. The morphological complexity is evaluated using Shannon entropy, Simpson diversity, approximation of Lempel-Ziv complexity, and expressivity (Shannon entropy divided by space-filling). We analyse behaviour of the systems with highest values of the generative morphological complexity and show that the Gray-Scott systems expressing highest levels of the complexity are composed of the wave-fragments (similar to wave-fragments in sub-excitable media) and travelling localisations (similar to quasi-dissipative solitons and gliders in Conway's Game of Life).

  2. Identification of novel missense mutations in the Norrie disease gene associated with one X-linked and four sporadic cases of familial exudative vitreoretinopathy.

    Science.gov (United States)

    Shastry, B S; Hejtmancik, J F; Trese, M T

    1997-01-01

    X-linked Familial Exudative Vitreoretinopathy (XLFEVR) is a hereditary eye disorder that affects both the retina and the vitreous body. It is characterized by an abnormal vascularization of the peripheral retina. It has been previously shown by linkage and candidate gene analysis that XLFEVR and Norrie disease are allelic. In this report we describe four novel mutations (R41K, H42R, K58N, and Y120C) in the Norrie disease gene associated with one X-linked and four sporadic cases of FEVR. One mutation (H42R) was found to be segregating with the disease in three generations (X-linked family), and the others are sporadic. These sequence alterations changed the encoded amino acids in the Norrie disease protein and were not found in 17 unaffected family members or in 36 randomly selected normal individuals. This study provides additional evidence that mutations in the same gene can result in FEVR and Norrie disease. It also demonstrates that it may be beneficial for clinical diagnosis to screen for mutations in the Norrie disease gene in sporadic FEVR cases.

  3. Characterization and mapping of the mouse NDP (Norrie disease) locus (Ndp).

    Science.gov (United States)

    Battinelli, E M; Boyd, Y; Craig, I W; Breakefield, X O; Chen, Z Y

    1996-02-01

    Norrie disease is a severe X-linked recessive neurological disorder characterized by congenital blindness with progressive loss of hearing. Over half of Norrie patients also manifest different degrees of mental retardation. The gene for Norrie disease (NDP) has recently been cloned and characterized. With the human NDP cDNA, mouse genomic phage libraries were screened for the homolog of the gene. Comparison between mouse and human genomic DNA blots hybridized with the NDP cDNA, as well as analysis of phage clones, shows that the mouse NDP gene is 29 kb in size (28 kb for the human gene). The organization in the two species is very similar. Both have three exons with similar-sized introns and identical exon-intron boundaries between exon 2 and 3. The mouse open reading frame is 393 bp and, like the human coding sequence, is encoded in exons 2 and 3. The absence of six nucleotides in the second mouse exon results in the encoded protein being two amino acids smaller than its human counterpart. The overall homology between the human and mouse NDP protein is 95% and is particularly high (99%) in exon 3, consistent with the apparent functional importance of this region. Analysis of transcription initiation sites suggests the presence of multiple start sites associated with expression of the mouse NDP gene. Pedigree analysis of an interspecific mouse backcross localizes the mouse NDP gene close to Maoa in the conserved segment, which runs from CYBB to PFC in both human and mouse.

  4. Listening in the Silences for Fred Newton Scott

    Science.gov (United States)

    Mastrangelo, Lisa

    2009-01-01

    As part of her recent sabbatical, the author proposed going to the University of Michigan Bentley Archives to do research on Fred Newton Scott, founder and chair of the Department of Rhetoric and teacher from 1889 to 1926 at the University of Michigan. Scott ran the only graduate program in rhetoric and composition in the country between those…

  5. 77 FR 7182 - Scott W. Houghton, M.D.; Decision and Order

    Science.gov (United States)

    2012-02-10

    ... DEPARTMENT OF JUSTICE Drug Enforcement Administration [Docket No. 12-09] Scott W. Houghton, M.D... CFR 0.100(b), I order that DEA Certificate of Registration BH8796077, issued to Scott W. Houghton, M.D., be, and it hereby is, revoked. I further order that any pending application of Scott W. Houghton, M.D...

  6. Norris Bradbury Tribute by J. Robert Oppenheimer

    Energy Technology Data Exchange (ETDEWEB)

    Meade, Roger Allen [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2017-04-27

    Stored on a reel of microfilm are three faint and barely readable letters written on the occasion of Norris Bradbury’s fifteen-year anniversary as Director of the (then) Los Alamos Science Laboratory. The first letter, written by Los Alamos Public Relations Officer John V. Young, asks Oppenheimer to send a “congratulatory message to be read at a ceremony honoring Bradbury’s tenure as Laboratory Director. The second letter is Oppenheimer’s response to Young, and the third is Oppenheimer’s congratulatory message to Bradbury. The three letters are transcribed below.

  7. NDP gene mutations in 14 French families with Norrie disease.

    Science.gov (United States)

    Royer, Ghislaine; Hanein, Sylvain; Raclin, Valérie; Gigarel, Nadine; Rozet, Jean-Michel; Munnich, Arnold; Steffann, Julie; Dufier, Jean-Louis; Kaplan, Josseline; Bonnefont, Jean-Paul

    2003-12-01

    Norrie disease is a rare X-inked recessive condition characterized by congenital blindness and occasionally deafness and mental retardation in males. This disease has been ascribed to mutations in the NDP gene on chromosome Xp11.1. Previous investigations of the NDP gene have identified largely sixty disease-causing sequence variants. Here, we report on ten different NDP gene allelic variants in fourteen of a series of 21 families fulfilling inclusion criteria. Two alterations were intragenic deletions and eight were nucleotide substitutions or splicing variants, six of them being hitherto unreported, namely c.112C>T (p.Arg38Cys), c.129C>G (p.His43Gln), c.133G>A (p.Val45Met), c.268C>T (p.Arg90Cys), c.382T>C (p.Cys128Arg), c.23479-1G>C (unknown). No NDP gene sequence variant was found in seven of the 21 families. This observation raises the issue of misdiagnosis, phenocopies, or existence of other X-linked or autosomal genes, the mutations of which would mimic the Norrie disease phenotype. Copyright 2003 Wiley-Liss, Inc.

  8. STS-87 Mission Specialist Winston E. Scott suits up

    Science.gov (United States)

    1997-01-01

    STS-87 Mission Specialist Winston Scott dons his launch and entry suit with the assistance of a suit technician in the Operations and Checkout Building. This is Scotts second space flight. He and the five other crew members will depart shortly for Launch Pad 39B, where the Space Shuttle Columbia awaits liftoff on a 16-day mission to perform microgravity and solar research. Scott is scheduled to perform an extravehicular activity spacewalk with Mission Specialist Takao Doi, Ph.D., of the National Space Development Agency of Japan, during STS-87. He also performed a spacewalk on STS-72.

  9. Ectopic norrin induces growth of ocular capillaries and restores normal retinal angiogenesis in Norrie disease mutant mice.

    Science.gov (United States)

    Ohlmann, Andreas; Scholz, Michael; Goldwich, Andreas; Chauhan, Bharesh K; Hudl, Kristiane; Ohlmann, Anne V; Zrenner, Eberhart; Berger, Wolfgang; Cvekl, Ales; Seeliger, Mathias W; Tamm, Ernst R

    2005-02-16

    Norrie disease is an X-linked retinal dysplasia that presents with congenital blindness, sensorineural deafness, and mental retardation. Norrin, the protein product of the Norrie disease gene (NDP), is a secreted protein of unknown biochemical function. Norrie disease (Ndp(y/-)) mutant mice that are deficient in norrin develop blindness, show a distinct failure in retinal angiogenesis, and completely lack the deep capillary layers of the retina. We show here that the transgenic expression of ectopic norrin under control of a lens-specific promoter restores the formation of a normal retinal vascular network in Ndp(y/-) mutant mice. The improvement in structure correlates with restoration of neuronal function in the retina. In addition, lenses of transgenic mice with ectopic expression of norrin show significantly more capillaries in the hyaloid vasculature that surrounds the lens during development. In vitro, lenses of transgenic mice in coculture with microvascular endothelial cells induce proliferation of the cells. Transgenic mice with ectopic expression of norrin show more bromodeoxyuridine-labeled retinal progenitor cells at embryonic day 14.5 and thicker retinas at postnatal life than wild-type littermates, indicating a putative direct neurotrophic effect of norrin. These data provide direct evidence that norrin induces growth of ocular capillaries and that pharmacologic modulation of norrin might be used for treatment of the vascular abnormalities associated with Norrie disease or other vascular disorders of the retina.

  10. Evaluation of the norrie disease gene in a family with incontinentia pigmenti.

    Science.gov (United States)

    Shastry, B S; Trese, M T

    2000-01-01

    Incontinentia pigmenti (IP) is an ectodermal multisystem disorder which can affect dental, ocular, cardiac and neurologic structures. The ocular changes of IP can have a very similar appearance to the retinal detachment of X-linked familial exudative vitreoretinopathy, which has been shown to be caused by the mutations in the Norrie disease gene. Therefore, it is of interest to determine whether similar mutations in the gene can account for the retinal pathology in patients with IP. To test our hypothesis, we have analyzed the entire Norrie disease gene for a family with IP, by single strand conformational polymorphism followed by DNA sequencing. The sequencing data revealed no disease-specific sequence alterations. These data suggest that ocular findings of IP are perhaps associated with different genes and there is no direct relationship between the genotype and phenotype. Copyright 2000 S. Karger AG, Basel

  11. Hedgehog regulates Norrie disease protein to drive neural progenitor self-renewal.

    Science.gov (United States)

    McNeill, Brian; Mazerolle, Chantal; Bassett, Erin A; Mears, Alan J; Ringuette, Randy; Lagali, Pamela; Picketts, David J; Paes, Kim; Rice, Dennis; Wallace, Valerie A

    2013-03-01

    Norrie disease (ND) is a congenital disorder characterized by retinal hypovascularization and cognitive delay. ND has been linked to mutations in 'Norrie Disease Protein' (Ndp), which encodes the secreted protein Norrin. Norrin functions as a secreted angiogenic factor, although its role in neural development has not been assessed. Here, we show that Ndp expression is initiated in retinal progenitors in response to Hedgehog (Hh) signaling, which induces Gli2 binding to the Ndp promoter. Using a combination of genetic epistasis and acute RNAi-knockdown approaches, we show that Ndp is required downstream of Hh activation to induce retinal progenitor proliferation in the retina. Strikingly, Ndp regulates the rate of cell-cycle re-entry and not cell-cycle kinetics, thereby uncoupling the self-renewal and cell-cycle progression functions of Hh. Taken together, we have uncovered a cell autonomous function for Ndp in retinal progenitor proliferation that is independent of its function in the retinal vasculature, which could explain the neural defects associated with ND.

  12. Collaborations Between Scott and Skidmore

    Directory of Open Access Journals (Sweden)

    Alicia Robinson

    2017-04-01

    Full Text Available This essay examines the collaboration between architect and designer George Gilbert Scott and metalworker Francis Skidmore. It compares their metalwork screens at the cathedrals of Hereford, Lichfield, and Salisbury—projects which sometimes overlapped and were all completed in the relatively short time span between 1861 and 1870—within the wider context of Skidmore’s career. While Scott was lauded in his lifetime and has been much studied since, Skidmore has not often been written about, despite having achieved an impressive scale and pace of work in British cathedrals, parish churches, and town halls. This essay therefore shines particular light on Skidmore’s work as designer and maker, and particularly the high profile commissions for these great cathedrals, restored and enhanced with the aesthetics and ambition of the Victorian era.

  13. Screening for NDP mutations in 44 unrelated patients with familial exudative vitreoretinopathy or Norrie disease.

    Science.gov (United States)

    Yang, Huiqin; Li, Shiqiang; Xiao, Xueshan; Guo, Xiangming; Zhang, Qingjiong

    2012-08-01

    To screen mutations in the norrin (NDP) gene in 44 unrelated Chinese patients with familial exudative vitreoretinopathy (FEVR, 38 cases) or Norrie disease (6 cases) and to describe the associated phenotypes. Of the 44 patients, mutation in FZD4, LRP5, and TSPAN12 was excluded in 38 patients with FEVR in previous study. Sanger sequencing was used to analyze the 2 coding exons and their adjacent regions of NDP in the 44 patients. Clinical data were presented for patients with mutation. NDP variants in 5 of the 6 patients with Norrie disease were identified, including a novel missense mutation (c.164G>A, p.Cys55Phe) in one patient, two known missense mutations (c.122G>A, p.Arg41Lys; c.220C>T, p.Arg74Cys) in two patients, and a gross deletion encompassing the two coding exons in two patients. Of the 5 patients, 3 had a family history and 2 were singleton cases. No mutation in NDP was detected in the 38 patients with FEVR. NDP mutations are common cause of Norrie disease but might be rare cause for FEVR in Chinese.

  14. Isolation of a candidate gene for Norrie disease by positional cloning

    NARCIS (Netherlands)

    Berger, W.; Meindl, A.; van de Pol, T. J.; Cremers, F. P.; Ropers, H. H.; Döerner, C.; Monaco, A.; Bergen, A. A.; Lebo, R.; Warburg, M.

    1992-01-01

    The gene for Norrie disease, an X-linked disorder characterized by progressive atrophy of the eyes, mental disturbances and deafness, has been mapped to chromosome Xp11.4 close to DXS7 and the monoamine oxidase (MAO) genes. By subcloning a YAC with a 640 kilobases (kb) insert which spans the

  15. Role of the Norrie disease pseudoglioma gene in sprouting angiogenesis during development of the retinal vasculature.

    Science.gov (United States)

    Luhmann, Ulrich F O; Lin, Jihong; Acar, Niyazi; Lammel, Stefanie; Feil, Silke; Grimm, Christian; Seeliger, Mathias W; Hammes, Hans-Peter; Berger, Wolfgang

    2005-09-01

    To characterize developmental defects and the time course of Norrie disease in retinal and hyaloid vasculature during retinal development and to identify underlying molecular angiogenic pathways that may be affected in Norrie disease, exudative vitreoretinopathy, retinopathy of prematurity, and Coats' disease. Norrie disease pseudoglioma homologue (Ndph)-knockout mice were studied during retinal development at early postnatal (p) stages (p5, p10, p15, and p21). Histologic techniques, quantitative RT-PCR, ELISA, and Western blot analyses provided molecular data, and scanning laser ophthalmoscopy (SLO) angiography and electroretinography (ERG) were used to obtain in vivo data. The data showed that regression of the hyaloid vasculature of Ndph-knockout mice occurred but was drastically delayed. The development of the superficial retinal vasculature was strongly delayed, whereas the deep retinal vasculature did not form because of the blockage of vessel outgrowth into the deep retinal layers. Subsequently, microaneurysm-like lesions formed. Several angiogenic factors were differentially transcribed during retinal development. Increased levels of hypoxia inducible factor-1alpha (HIF1alpha) and VEGFA, as well as a characteristic ERG pattern, confirmed hypoxic conditions in the inner retina of the Ndph-knockout mouse. These data provide evidence for a crucial role of Norrin in hyaloid vessel regression and in sprouting angiogenesis during retinal vascular development, especially in the development of the deep retinal capillary networks. They also suggest an early and a late phase of Norrie disease and may provide an explanation for similar phenotypic features of allelic retinal diseases in mice and patients as secondary consequences of pathologic hypoxia.

  16. Molecular analysis of the NDP gene in two families with Norrie disease.

    Science.gov (United States)

    Rivera-Vega, M Refugio; Chiñas-Lopez, Silvet; Vaca, Ana Luisa Jimenez; Arenas-Sordo, M Luz; Kofman-Alfaro, Susana; Messina-Baas, Olga; Cuevas-Covarrubias, Sergio Alberto

    2005-04-01

    To describe the molecular defects in the Norrie disease protein (NDP) gene in two families with Norrie disease (ND). We analysed two families with ND at molecular level through polymerase chain reaction, DNA sequence analysis and GeneScan. Two molecular defects found in the NDP gene were: a missense mutation (265C > G) within codon 97 that resulted in the interchange of arginine by proline, and a partial deletion in the untranslated 3' region of exon 3 of the NDP gene. Clinical findings were more severe in the family that presented the partial deletion. We also diagnosed the carrier status of one daughter through GeneScan; this method proved to be a useful tool for establishing female carriers of ND. Here we report two novel mutations in the NDP gene in Mexican patients and propose that GeneScan is a viable mean of establishing ND carrier status.

  17. A novel Norrie disease pseudoglioma gene mutation, c.-1_2delAAT, responsible for Norrie disease in a Chinese family.

    Science.gov (United States)

    Zhang, Xin-Yu; Jiang, Wei-Ying; Chen, Lu-Ming; Chen, Su-Qin

    2013-01-01

    To investigate the genetic findings and phenotypic characteristics of a Chinese family with Norrie disease (ND). Molecular genetic analysis and clinical examinations were performed on a Chinese family with ND. Mutations in the Norrie disease pseudoglioma (NDP) gene were detected by direct sequencing. Haplotypes were constructed and compared with the phenotypes in the family. Evolutionary comparisons and mutant open reading frame (ORF) prediction were also undertaken. Two family members with ocular manifestations were diagnosed with ND. No signs of sensorineural hearing loss were observed in either patient, while one of them showed signs of mild mental retardation. A novel heterozygous mutation in the NDP gene, c.-1_2delAAT, was detected in both patients. The mutation and the mutation bearing haplotype co-segregated with the ND phenotype in males and was transmitted from their mothers and/or grandmothers (II:2). The male without ND did not harbor the mutation. The mutation occurred at the highly conserved nucleotides. ORF finder predicted that the mutation would lead to the production of a truncated protein that lacks the first 11 N-terminal amino acids. A novel mutation, c.-1_2delAAT in the NDP gene, was identified in a Chinese family with ND. This mutation caused ND without obvious sensorineural hearing loss. Mental disorder was found in one but not the other patients. The clinical heterogeneity in the family indicated that other genetic variants and epigenetic factors may also play a role in the disease presentation.

  18. Heroes for the past and present: a century of remembering Amundsen and Scott.

    Science.gov (United States)

    Roberts, Peder

    2011-12-01

    In 1911-1912 Roald Amundsen and Robert Falcon Scott led rival parties in a race to the geographic South Pole. While both parties reached the Pole--Amundsen first--Scott's men died on the return journey. Amundsen became a Norwegian icon through his record-setting travels; Scott became a symbol of courage and devotion to science. The memory of each was invoked at various points during the twentieth century in the context of contemporary Antarctic events. Scott's status as a scientific figure was central to the Scott Polar Research Institute, while Amundsen's lack of scientific legacy became a way for British polar explorers to differentiate themselves from Norwegian contemporaries during the interwar years. After 1945 Scott and Amundsen were again invoked as exemplars of national polar achievement, even as the rise of large-scale science on the continent overshadowed past British and Norwegian achievements. In the present Amundsen and Scott remain wedded to particular values, focused respectively on national achievement and sacrifice in the name of science, while their race has become secondary. Copyright © 2011 Elsevier Ltd. All rights reserved.

  19. On Scott-Phillips' General Account of Communication.

    Science.gov (United States)

    Planer, Ronald J

    2017-12-01

    The purpose of this paper is to critically engage with a recent attempt by Thom Scott-Phillips to offer a general account of communication. As a general account, it is intended to apply equally well to both non-human and human interactions which are prima facie communicative in character. However, so far, Scott-Phillips has provided little detail regarding how his account is supposed to apply to the latter set of cases. After presenting what I take to be the most plausible way of filling in those details, I argue that his account would appear to be too narrow: it (minimally) fails to capture a range of human interactions which strike us as instances of communication. To wit, these are cases in which some but not all of the information an act is designed to convey to a reactor actually reaches that reactor. An alternative account incorporating Scott-Phillips' main insights is then sketched, and it is suggested that this account, or something like it, would accommodate the full range of non-human and human interactions that are intuitively communicative.

  20. Ornithine aminotransferase (OAT): recombination between an X-linked OAT sequence (7.5 kb) and the Norrie disease locus.

    Science.gov (United States)

    Ngo, J T; Bateman, J B; Spence, M A; Cortessis, V; Sparkes, R S; Kivlin, J D; Mohandas, T; Inana, G

    1990-01-01

    A human ornithine aminotransferase (OAT) locus has been mapped to the Xp11.2, as has the Norrie disease locus. We used a cDNA probe to investigate a 3-generation UCLA family with Norrie disease; a 4.2-kb RFLP was detected and a maximum lod score of 0.602 at zero recombination fraction was calculated. We used the same probe to study a second multigeneration family with Norrie disease from Utah. A different RFLP of 7.5 kb in size was identified and a recombinational event between the OAT locus represented by this RFLP and the disease loci was observed. Linkage analysis of these two loci in this family revealed a maximum load score of 1.88 at a recombination fraction of 0.10. Although both families have affected members with the same disease, the lod scores are reported separately because the 4.2- and 7.5-kb RFLPs may represent two different loci for the X-linked OAT.

  1. Rakesh K. Kapania named Norris and Laura Mitchell Professor of Aerospace Engineering

    OpenAIRE

    Crumbley, Liz

    2008-01-01

    Rakesh K. Kapania, a professor in the Department of Aerospace and Ocean Engineering in the College of Engineering at Virginia Tech, was appointed the Norris and Laura Mitchell Professor of Aerospace Engineering by the Virginia Tech Board of Visitors during the board's quarterly meeting March 31.

  2. Retinal phenotype-genotype correlation of pediatric patients expressing mutations in the Norrie disease gene.

    Science.gov (United States)

    Wu, Wei-Chi; Drenser, Kimberly; Trese, Michael; Capone, Antonio; Dailey, Wendy

    2007-02-01

    To correlate the ophthalmic findings of patients with pediatric vitreoretinopathies with mutations occurring in the Norrie disease gene (NDP). One hundred nine subjects with diverse pediatric vitreoretinopathies and 54 control subjects were enrolled in the study. Diagnoses were based on retinal findings at each patient's first examination. Samples of DNA from each patient underwent polymerase chain reaction amplification and direct sequencing of the NDP gene. Eleven male patients expressing mutations in the NDP gene were identified in the test group, whereas the controls demonstrated wild-type NDP. All patients diagnosed as having Norrie disease had mutations in the NDP gene. Four of the patients with Norrie disease had mutations involving a cysteine residue in the cysteine-knot motif. Four patients diagnosed as having familial exudative vitreoretinopathy were found to have noncysteine mutations. One patient with retinopathy of prematurity had a 14-base deletion in the 5' untranslated region (exon 1), and 1 patient with bilateral persistent fetal vasculature syndrome expressed a noncysteine mutation in the second exon. Mutations disrupting the cysteine-knot motif corresponded to severe retinal dysgenesis, whereas patients with noncysteine mutations had varying degrees of avascular peripheral retina, extraretinal vasculature, and subretinal exudate. Patients exhibiting severe retinal dysgenesis should be suspected of carrying a mutation that disrupts the cysteine-knot motif in the NDP gene.

  3. Genotype-phenotype variations in five Spanish families with Norrie disease or X-linked FEVR.

    Science.gov (United States)

    Riveiro-Alvarez, Rosa; Trujillo-Tiebas, Maria José; Gimenez-Pardo, Ascension; Garcia-Hoyos, Maria; Cantalapiedra, Diego; Lorda-Sanchez, Isabel; Rodriguez de Alba, Marta; Ramos, Carmen; Ayuso, Carmen

    2005-09-02

    Norrie disease (OMIM 310600) is a rare X-linked disorder characterized by congenital blindness in males. Approximately 40 to 50% of the cases develop deafness and mental retardation. X-linked familial exudative vitreoretinopathy (XL-FEVR) is a hereditary ocular disorder characterized by a failure of peripheral retinal vascularization. Both X-linked disorders are due to mutations in the NDP gene, which encodes a 133 amino acid protein called Norrin, but autosomal recessive (AR) and autosomal dominant (AD) forms of FEVR have also been described. In this study, we report the molecular findings and the related phenotype in five Spanish families affected with Norrie disease or XL-FEVR due to mutations of the NDP gene. The study was conducted in 45 subjects from five Spanish families. These families were clinically diagnosed with Norrie disease or similar conditions. The three exons of the NDP gene were analyzed by automatic DNA sequencing. Haplotype analyses were also performed. Two new nonsense mutations, apart from other mutations previously described in the NDP gene, were found in those patients affected with ND or X-linked FEVR. An important genotype-phenotype variation was found in relation to the different mutations of the NDP gene. In fact, the same mutation may be responsible for different phenotypes. We speculate that there might be other molecular factors that interact in the retina with Norrin, which contribute to the resultant phenotypes.

  4. A novel Norrie disease pseudoglioma gene mutation, c.-1_2delAAT, responsible for Norrie disease in a Chinese family

    Directory of Open Access Journals (Sweden)

    Xin-Yu Zhang

    2013-12-01

    Full Text Available AIM:To investigate the genetic findings and phenotypic characteristics of a Chinese family with Norrie disease (ND.METHODS:Molecular genetic analysis and clinical examinations were performed on a Chinese family with ND. Mutations in the Norrie disease pseudoglioma (NDP gene were detected by direct sequencing. Haplotypes were constructed and compared with the phenotypes in the family. Evolutionary comparisons and mutant open reading frame (ORF prediction were also undertaken.RESULTS:Two family members with ocular manifestations were diagnosed with ND. No signs of sensorineural hearing loss were observed in either patient, while one of them showed signs of mild mental retardation. A novel heterozygous mutation in the NDP gene, c.-1_2delAAT, was detected in both patients. The mutation and the mutation bearing haplotype co-segregated with the ND phenotype in males and was transmitted from their mothers and/or grandmothers (II:2. The male without ND did not harbor the mutation. The mutation occurred at the highly conserved nucleotides. ORF finder predicted that the mutation would lead to the production of a truncated protein that lacks the first 11 N-terminal amino acids.CONCLUSION:A novel mutation, c.-1_2delAAT in the NDP gene, was identified in a Chinese family with ND. This mutation caused ND without obvious sensorineural hearing loss. Mental disorder was found in one but not the other patients. The clinical heterogeneity in the family indicated that other genetic variants and epigenetic factors may also play a role in the disease presentation.

  5. Molecular modelling of the Norrie disease protein predicts a cystine knot growth factor tertiary structure.

    Science.gov (United States)

    Meitinger, T; Meindl, A; Bork, P; Rost, B; Sander, C; Haasemann, M; Murken, J

    1993-12-01

    The X-lined gene for Norrie disease, which is characterized by blindness, deafness and mental retardation has been cloned recently. This gene has been thought to code for a putative extracellular factor; its predicted amino acid sequence is homologous to the C-terminal domain of diverse extracellular proteins. Sequence pattern searches and three-dimensional modelling now suggest that the Norrie disease protein (NDP) has a tertiary structure similar to that of transforming growth factor beta (TGF beta). Our model identifies NDP as a member of an emerging family of growth factors containing a cystine knot motif, with direct implications for the physiological role of NDP. The model also sheds light on sequence related domains such as the C-terminal domain of mucins and of von Willebrand factor.

  6. Wave-splitting in the bistable Gray-Scott model

    DEFF Research Database (Denmark)

    Rasmussen, K.E.; Mazin, W.; Mosekilde, Erik

    1996-01-01

    The Gray-Scott model describes a chemical reaction in which an activator species grows autocatalytically on a continuously fed substrate. For certain feed rates and activator life times the model shows the coexistence of two homogeneous steady states. The blue state, where the activator concentra......The Gray-Scott model describes a chemical reaction in which an activator species grows autocatalytically on a continuously fed substrate. For certain feed rates and activator life times the model shows the coexistence of two homogeneous steady states. The blue state, where the activator...

  7. Genetics Home Reference: Aarskog-Scott syndrome

    Science.gov (United States)

    ... A characteristic of X-linked inheritance is that fathers cannot pass X-linked traits to their sons. Evidence suggests that Aarskog-Scott syndrome is inherited in an autosomal dominant or autosomal recessive pattern in some families, although ...

  8. Repair of Total Tractional Retinal Detachment in Norrie Disease: Report of Technique and Successful Surgical Outcome.

    Science.gov (United States)

    Todorich, Bozho; Thanos, Aristomenis; Yonekawa, Yoshihiro; Capone, Antonio

    2017-03-01

    Norrie disease is a rare, but devastating cause of pediatric retinal detachment, universally portending a poor visual prognosis. This paper describes successful surgical management of an infant with total retinal detachment associated with Norrie disease mutation. The infant was a full-term white male who presented with bilateral total funnel retinal detachments (RDs). He underwent genetic testing, which demonstrated single-point mutation 133 G>A transition in exon 2 of the NDP gene. The retinal detachment was managed with translimbal iridectomy, lensectomy, capsulectomy, and vitrectomy. Careful dissection of the retrolental membranes resulted in opening of the funnel. Single-stage surgery in this child's eye achieved re-attachment of the posterior pole with progressive reabsorption of subretinal fluid and cholesterol without the need for external drainage. Fluorescein angiography, performed at 2 months postoperatively, demonstrated perfusion of major vascular arcades, but with significant abnormalities and aneurysmal changes of higher-order vessels, suggestive of retinal and vascular dysplasia. The child has maintained brisk light perception vision. Early surgical intervention with careful dissection of tractional tissues can potentially result in good anatomic outcomes in some patients with Norrie disease-associated retinal detachment. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:260-262.]. Copyright 2017, SLACK Incorporated.

  9. An estimating function approach to inference for inhomogeneous Neyman-Scott processes

    DEFF Research Database (Denmark)

    Waagepetersen, Rasmus Plenge

    “This paper is concerned with inference for a certain class of inhomogeneous Neyman-Scott point processes depending on spatial covariates. Regression parameter estimates obtained from a simple estimating function are shown to be asymptotically normal when the “mother” intensity for the Neyman-Scott...

  10. A mutation in the Norrie disease gene (NDP) associated with X-linked familial exudative vitreoretinopathy.

    Science.gov (United States)

    Chen, Z Y; Battinelli, E M; Fielder, A; Bundey, S; Sims, K; Breakefield, X O; Craig, I W

    1993-10-01

    Familial exudative vitreoretinopathy (FEVR) is a hereditary disorder characterized by an abnormality of the peripheral retina. Both autosomal dominant (adFEVR) and X-linked (XLFEVR) forms have been described, but the biochemical defect(s) underlying the symptoms are unknown. Molecular analysis of the Norrie gene locus (NDP) in a four generation FEVR family (shown previously to exhibit linkage to the X-chromosome markers DXS228 and MAOA (Xp11.4-p11.3)) reveals a missense mutation in the highly conserved region of the NDP gene, which caused a neutral amino acid substitution (Leu124Phe), was detected in all of the affected males, but not in the unaffected family members, nor in normal controls. The observations suggest that phenotypes of both XLFEVR and Norrie disease can result from mutations in the same gene.

  11. Scott Brothers Windows and Doors Information Sheet

    Science.gov (United States)

    Scott Brothers Windows and Doors (the Company) is located in Bridgeville, Pennsylvania. The settlement involves renovation activities conducted at property constructed prior to 1978, located in Pittsburgh, Pennsylvania.

  12. 78 FR 5854 - Application of Scott Air, LLC for Certificate Authority

    Science.gov (United States)

    2013-01-28

    ... DEPARTMENT OF TRANSPORTATION Office of the Secretary Application of Scott Air, LLC for Certificate Authority AGENCY: Department of Transportation. ACTION: Notice of order to show cause (Order 2013-1-12... to show cause why it should not issue an order finding Scott Air, LLC fit, willing, and able, and...

  13. Comments on ''The non-linear saturation of a magnetic island'' by J. Norris

    International Nuclear Information System (INIS)

    Thyagaraja, A.

    1990-01-01

    Recent results derived on non-linear saturation of tearing modes (Norris, J., Plasma Phys. Contr. Fusion, 1989, Vol. 31, 699) are examined in relation to previously published literature. It is explicitly demonstrated that they are at variance in several important respects with those due to previous workers in this area. (author)

  14. An Overview of Justice in Sir Walter Scott Waverley Novels: The Heart of Mid-Lothian

    Directory of Open Access Journals (Sweden)

    Enrique García Díaz

    2014-12-01

    Full Text Available Although Sir Walter Scott is a well-known writer most of his readers know that he became an advocate in 1792, when he was admitted to the bar. Since then Scott and other advocates walked the floor at Parliament House (home of the Faculty of Advocates and the Court of Session waiting to be hired. Scott’s own experiences as a fledgling advocate are echoed in those of Alain Fairford in his novel Redgauntlet (Scott 1824, which provides a vivid picture of Parliament House in the eighteenth century. During his life, Scott combined extensive writing and editing issues with his daily work as Clerk of Session and Sheriff-Depute of Selkirkshire. Walter Scott was not unaware of Justice and Law and The Heart of Mid-Lothian is the novel in which he introduces to the reader the Scottish Legal System during the eighteenth century. However, there are few more examples that I will explain. Aunque Sir Walter Scott es un conocido escritor, la mayoría de sus lectores saben que en 1792 se hizo abogado, cuando fue admitido en el colegio de abogados. Desde entonces Scott y otros abogados rondaron el Parlamento con la esperanza de ser contratados. Las propias experiencias de Scott como un abogado novel se reflejan en las de Alain Fairford en su novela Redgauntlet (Scott 1824, lo que ofrece una vívida imagen del Parlamento (sede de la facultad de Derecho y Tribunal Supremo en el siglo XVIII. Durante su vida, Scott compaginó una profusa actividad como escritor y editor con su trabajo diario como juez en Selkirk. Walter Scott conocía la justicia y el derecho y El corazón de Mid-Lothian es la novela en la presenta al lector el régimen jurídico de Escocia durante el siglo XVIII. Sin embargo, se explicarán algunos otros ejemplos. DOWNLOAD THIS PAPER FROM SSRN: http://ssrn.com/abstract=2543538

  15. Executive dysfunctions as part of the behavioural phenotype of Aarskog-Scott syndrome

    NARCIS (Netherlands)

    Egger, J.I.M.; Verhoeven, W.M.A.; Janssen, G.T.L.; Aken, L. van; Hoogeboom, A.J.M.

    2012-01-01

    Introduction Aarskog syndrome (AAS) also called Aarskog-Scott syndrome faciodigitogenital syndrome or faciogenital dysplasia is a genetically heterogeneous developmental disorder, first described in 1970 by the Norwegian pediatrician Dagfin Aarskog and further delineated by Scott in 1971. It is a

  16. X-linked recessive primary retinal dysplasia is linked to the Norrie disease locus.

    Science.gov (United States)

    Ravia, Y; Braier-Goldstein, O; Bat-Miriam, K M; Erlich, S; Barkai, G; Goldman, B

    1993-08-01

    X-linked primary retinal dysplasia (PRD) refers to an abnormal proliferation of retinal tissue causing either its neural elements or its glial tissue to form folds, giving rise to gliosis. A Jewish family of oriental origin was previously reported by Godel and Goodman, in which a total of five males suffer from different degrees of blindness. The authors postulated that the described findings are distinguished from Norrie disease, since in this case no clinical findings, other than those associated with the eyes, were noticed in the affected males. In addition, two of the carrier females exhibit minimal eye changes. We have performed linkage analysis of the family using the L1.28, p58-1 and m27 beta probes, and DXS426 and MAOB associated microsatellites. Our results map the gene responsible for the disorder between the MAOB and DXS426, m27 beta and p58-1 loci, on the short arm of the X chromosome at Xp11.3, which suggest the possibility that the same gene is responsible for both primary retinal dysplasia and Norrie disease.

  17. Interview met professor Joan Wallach Scott

    NARCIS (Netherlands)

    Bijl, Greetje; Tijhoff, Esmeralda

    2012-01-01

    Joan Scott, professor at the School of Social Science in the Institute for Avanced Study in Princeton, New Jersey (USA), was the keynote speaker at the conference 'Uitsluitend emancipatie' in de Beurs van Berlage in Amsterdam in October 2012. An interview on gender, history, feminism and her book

  18. Generalization of the Moszkovski-Scott method

    International Nuclear Information System (INIS)

    Balbutsev, E.B.

    1976-01-01

    A constant separation parameter is proposed to be used in the Moszkovski-Scott method for solving the Bethe-Goldstone equation. After such a modification one can apply the method to odd states of relative motion, not only to even ones. Some essential inaccuracies of the original method are eliminated, as well

  19. Norrie disease: linkage analysis using a 4.2-kb RFLP detected by a human ornithine aminotransferase cDNA probe.

    Science.gov (United States)

    Ngo, J T; Bateman, J B; Cortessis, V; Sparkes, R S; Mohandas, T; Inana, G; Spence, M A

    1989-05-01

    Previous study has shown that the usual DNA marker for Norrie disease, the L1.28 probe which identifies the DXS7 locus, can recombine with the disease locus. In this study, we used a human ornithine aminotransferase (OAT) cDNA which detects OAT-related DNA sequences mapped to the same region on the X chromosome as that of the L1.28 probe to investigate the family with Norrie disease who exhibited the recombinational event. When genomic DNA from this family was digested with the PvuII restriction endonuclease, we found a restriction fragment length polymorphism (RFLP) of 4.2 kb in size. This fragment was absent in the affected males and cosegregated with the disease locus; we calculated a lod score of 0.602, at theta = 0.00. No deletion could be detected by chromosomal analysis or on Southern blots with other enzymes. These results suggest that one of the OAT-related sequences on the X chromosome may be in close proximity to the Norrie disease locus and represent the first report which indicates that the OAT cDNA may be useful for the identification of carrier status and/or prenatal diagnosis.

  20. The Norrie disease gene maps to a 150 kb region on chromosome Xp11.3.

    Science.gov (United States)

    Sims, K B; Lebo, R V; Benson, G; Shalish, C; Schuback, D; Chen, Z Y; Bruns, G; Craig, I W; Golbus, M S; Breakefield, X O

    1992-05-01

    Norrie disease is a human X-linked recessive disorder of unknown etiology characterized by congenital blindness, sensory neural deafness and mental retardation. This disease gene was previously linked to the DXS7 (L1.28) locus and the MAO genes in band Xp11.3. We report here fine physical mapping of the obligate region containing the Norrie disease gene (NDP) defined by a recombination and by the smallest submicroscopic chromosomal deletion associated with Norrie disease identified to date. Analysis, using in addition two overlapping YAC clones from this region, allowed orientation of the MAOA and MAOB genes in a 5'-3'-3'-5' configuration. A recombination event between a (GT)n polymorphism in intron 2 of the MAOB gene and the NDP locus, in a family previously reported to have a recombination between DXS7 and NDP, delineates a flanking marker telomeric to this disease gene. An anonymous DNA probe, dc12, present in one of the YACs and in a patient with a submicroscopic deletion which includes MAOA and MAOB but not L1.28, serves as a flanking marker centromeric to the disease gene. An Alu-PCR fragment from the right arm of the MAO YAC (YMAO.AluR) is not deleted in this patient and also delineates the centromeric extent of the obligate disease region. The apparent order of these loci is telomere ... DXS7-MAOA-MAOB-NDP-dc12-YMAO.AluR ... centromere. Together these data define the obligate region containing the NDP gene to a chromosomal segment less than 150 kb.

  1. Ocular phenotypes associated with two mutations (R121W, C126X) in the Norrie disease gene.

    Science.gov (United States)

    Kellner, U; Fuchs, S; Bornfeld, N; Foerster, M H; Gal, A

    1996-06-01

    To describe the ocular phenotypes associated with 2 mutations in the Norrie disease gene including a manifesting carrier. Ophthalmological examinations were performed in 2 affected males and one manifesting carrier. Genomic DNA was analyzed by direct sequencing of the Norrie disease gene. Family I: A 29-year-old male had the right eye enucleated at the age of 3 years. His left eye showed severe temporal dragging of the retina and central scars. Visual acuity was 20/300. DNA analysis revealed a C-to-T transition of the first nucleotide in codon 121 predicting the replacement of arginine-121 by tryptophan (R121W). Both the mother and maternal grandmother carry the same mutation in heterozygous form. Family 2: A 3-month-old boy presented with severe temporal dragging of the retina on both eyes and subsequently developed retinal detachment. Visual acuity was limited to light perception. His mother's left eye was amaurotic and phthitic. Her right eye showed severe retinal dragging, visual acuity was reduced to 20/60. DNA analysis revealed a T-to-A transversion of the third nucleotide in codon 126 creating a stop codon (C126X). The mother and maternal grandmother were carriers. Mutations in the Norrie disease gene can lead to retinal malformations of variable severity both in hemizygous males and manifesting carriers.

  2. W. Richard Scott, Institutions and Organizations: Ideas, Interests, and Identities

    DEFF Research Database (Denmark)

    Jakobsen, Michael

    2014-01-01

    Book review of: W. Richard Scott: Institutions and Organizations: Ideas, Interests, and Identities. 4th edition. Thousand Oaks, CA: SAGE Publications, 2014. xiii, 345 pp.......Book review of: W. Richard Scott: Institutions and Organizations: Ideas, Interests, and Identities. 4th edition. Thousand Oaks, CA: SAGE Publications, 2014. xiii, 345 pp....

  3. 78 FR 60929 - Notice of Public Meeting of the Fort Scott Council

    Science.gov (United States)

    2013-10-02

    .... Such requests must be stated prominently at the beginning of the comments. The Trust will make... PRESIDIO TRUST Notice of Public Meeting of the Fort Scott Council AGENCY: The Presidio Trust... Scott Council (Council) will be held from 10 a.m. to 12:30 p.m. on Thursday, October 17, 2013. The...

  4. Under the Radar: The First Woman in Radio Astronomy, Ruby Payne-Scott

    Science.gov (United States)

    Miller Goss, W.

    2012-05-01

    Under the Radar, the First Woman in Radio Astronomy, Ruby Payne-Scott W. Miller Goss, NRAO Socorro NM Ruby Payne-Scott (1912-1981) was an eminent Australian scientist who made major contributions to the WWII radar effort (CSIR) from 1941 to 1945. In late 1945, she pioneered radio astronomy efforts at Dover Heights in Sydney, Australia at a beautiful cliff top overlooking the Tasman Sea. Again at Dover Heights, Payne-Scott carried out the first interferometry in radio astronomy using an Australian Army radar antenna as a radio telescope at sun-rise, 26 January 1946. She continued these ground breaking activities until 1951. Ruby Payne-Scott played a major role in discovering and elucidating the properties of Type III bursts from the sun, the most common of the five classes of transient phenomena from the solar corona. These bursts are one of the most intensively studied forms of radio emission in all of astronomy. She is also one of the inventors of aperture synthesis in radio astronomy. I examine her career at the University of Sydney and her conflicts with the CSIR hierarchy concerning the rights of women in the work place, specifically equal wages and the lack of permanent status for married women. I also explore her membership in the Communist Party of Australia as well as her partially released Australian Scientific Intelligence Organization file. Payne-Scott’s role as a major participant in the flourishing radio astronomy research of the post war era remains a remarkable story. She had a number of strong collaborations with the pioneers of early radio astronomy in Australia: Pawsey, Mills, Christiansen, Bolton and Little. I am currently working on a popular version of the Payne-Scott story; “Making Waves, The Story of Ruby Payne-Scott: Australian Pioneer Radio Astronomer” will be published in 2013 by Springer in the Astronomers’ Universe Series.

  5. Sir Charles Scott Sherrington (1857–1952)

    Indian Academy of Sciences (India)

    Twentieth century bore witness to remarkable scientists whohave advanced our understanding of the brain. Among them,Sir Charles Scott Sherrington's ideas about the way in whichthe central nervous system operates has continuing relevanceeven today. He received honorary doctorates from twentytwouniversities and ...

  6. Sir Charles Scott Sherrington (1857–1952)

    Indian Academy of Sciences (India)

    2016-08-26

    Aug 26, 2016 ... Twentieth century bore witness to remarkable scientists whohave advanced our understanding of the brain. Among them,Sir Charles Scott Sherrington's ideas about the way in whichthe central nervous system operates has continuing relevanceeven today. He received honorary doctorates from ...

  7. Clinical reinvestigation and linkage analysis in the family with Episkopi blindness (Norrie disease).

    Science.gov (United States)

    Wolff, G; Mayerová, A; Wienker, T F; Atalianis, P; Ioannou, P; Warburg, M

    1992-11-01

    We present the results of a clinical and genetic reinvestigation of the Cypriot family affected by an X chromosomally inherited eye disease originally published by Taylor et al, who coined the term Episkopi blindness. The pedigree was extended to 160 members, including 16 affected males out of 48 males at risk for the disease, most of whom were seen by one of us (PA). Affected males are blind with no associated symptoms and apparently are not mentally retarded. Thirty-nine family members agreed to blood sampling for genetic investigations. RFLP analysis was performed using probes from the region known to be deleted in some Norrie patients and polymorphic markers (DXS77, DXS7, MAOA, DXS255) from the proximal short arm of the X chromosome. There was no deletion for any of the probes in the affected males. Linkage analysis yielded positive lod scores for all informative markers (Z (DXS255, theta = 0) = 6.54, Z (MAOA, theta = 0) = 2.23, Z (DXS7, theta = 0) = 2.13). Thus, the conclusion that Episkopi blindness and Norrie disease (NDP, MIM *310600) are the same entity based on clinical evidence is now reinforced by gene mapping.

  8. Plasma amine oxidase activities in Norrie disease patients with an X-chromosomal deletion affecting monoamine oxidase.

    Science.gov (United States)

    Murphy, D L; Sims, K B; Karoum, F; Garrick, N A; de la Chapelle, A; Sankila, E M; Norio, R; Breakefield, X O

    1991-01-01

    Two individuals with an X-chromosomal deletion were recently found to lack the genes encoding monoamine oxidase type A (MAO-A) and MAO-B. This abnormality was associated with almost total (90%) reductions in the oxidatively deaminated urinary metabolites of the MAO-A substrate, norepinephrine, and with marked (100-fold) increases in an MAO-B substrate, phenylethylamine, confirming systemic functional consequences of the genetic enzyme deficiency. However, urinary concentrations of the deaminated metabolites of dopamine and serotonin (5-HT) were essentially normal. To investigate other deaminating systems besides MAO-A and MAO-B that might produce these metabolites of dopamine and 5-HT, we examined plasma amine oxidase (AO) activity in these two patients and two additional patients with the same X-chromosomal deletion. Normal plasma AO activity was found in all four Norrie disease-deletion patients, in four patients with classic Norrie disease without a chromosomal deletion, and in family members of patients from both groups. Marked plasma amine metabolite abnormalities and essentially absent platelet MAO-B activity were found in all four Norrie disease-deletion patients, but in none of the other subjects in the two comparison groups. These results indicate that plasma AO is encoded by gene(s) independent of those for MAO-A and MAO-B, and raise the possibility that plasma AO, and perhaps the closely related tissue AO, benzylamine oxidase, as well as other atypical AOs or MAOs encoded independently from MAO-A and MAO-B may contribute to the oxidative deamination of dopamine and 5-HT in humans.

  9. Peripheral retinopathy in offspring of carriers of Norrie disease gene mutations. Possible transplacental effect of abnormal Norrin.

    Science.gov (United States)

    Mintz-Hittner, H A; Ferrell, R E; Sims, K B; Fernandez, K M; Gemmell, B S; Satriano, D R; Caster, J; Kretzer, F L

    1996-12-01

    The Norrie disease (ND) gene (Xp11.3) (McKusick 310600) consists of one untranslated exon and two exons partially translated as the Norrie disease protein (Norrin). Norrin has sequence homology and computer-predicted tertiary structure of a growth factor containing a cystine knot motif, which affects endothelial cell migration and proliferation. Norrie disease (congenital retinal detachment), X-linked primary retinal dysplasia (congenital retinal fold), and X-linked exudative vitreoretinopathy (congenital macular ectopia) are allelic disorders. Blood was drawn for genetic studies from members of two families to test for ND gene mutations. Sixteen unaffected family members were examined ophthalmologically. If any retinal abnormality were identified, fundus photography and fluorescein angiography was performed. Family A had ND (R109stp), and family B had X-linked exudative vitreoretinopathy (R121L). The retinas of 11 offspring of carrier females were examined: three of seven carrier females, three of three otherwise healthy females, and one of one otherwise healthy male had peripheral inner retinal vascular abnormalities. The retinas of five offspring of affected males were examined: none of three carrier females and none of two otherwise healthy males had this peripheral retinal finding. Peripheral inner retinal vascular abnormalities similar to regressed retinopathy of prematurity were identified in seven offspring of carriers of ND gene mutations in two families. These ophthalmologic findings, especially in four genetically healthy offspring, strongly support the hypothesis that abnormal Norrin may have an adverse transplacental (environmental) effect on normal inner retinal vasculogenesis.

  10. La Jolie Fille de Perth de Bizet  ou comment trahir et honorer Walter Scott Bizet’s Jolie Fille de Perth or How to Betray and Honour Walter Scott

    Directory of Open Access Journals (Sweden)

    Gilles Couderc

    2011-11-01

    Full Text Available What remains of Walter Scott’s Fair Maid of Perth in Bizet’s 1867 Jolie Fille de Perth, an opera in 4acts on a libretto by Jules Adenis and Vernoy de Saint-Georges? Not much when compared to other Scott-inspired operas. Little historical context or local colour, even in Bizet’s music. Some characters remotely linked to Scott in a libretto that mostly abides by the rules of French opera or opéra-comique of the time and recycles the dramatic ingredients favoured by Saint-Georges, a purveyor of libretti for opera or the ballet second only to Scribe, who engendered such international successes as Flotow’s Martha and Balfe’s Bohemian Girl, whose gipsy, long before his Carmen, haunts Bizet’s “Scottish” opera. Yet the work pays indirect homage to Scott, whose historical novels contributed to the birth of the French “grand opera”, by rewriting scenes or situations drawn from Scott. In spite of borrowing freely from French grand opera and opéra-comique, Bizet here attempts to find his own musical expression and his opera reflects aspects of Second Empire French society and the roles it assigned to women, before the appearance of his revolutionary Carmen on the stage.Que reste t’il du roman de Walter Scott The Fair Maid of Perth dans la Jolie Fille de Perth de Bizet, opéra en 4 actes de 1867 sur un livret de Vernoy de Saint-Georges, vieux routier du théâtre lyrique, et Jules Adenis ? Pas grand-chose par rapport aux opéras inspirés par Scott. Peu d’Ecosse, une absence remarquable de couleur locale ou historique, des personnages vaguement inspirés de Scott pour un livret qui se plie surtout aux règles de l’opéra français et de l’opéra-comique à la manière de Scribe et recycle les ingrédients habituels des livrets de Saint-Georges, père de succès internationaux comme la Martha de Flotow et de la Bohemian Girl de Balfe, dont la figure exotique de la bohémienne, longtemps avant Carmen, hante l’opéra

  11. A novel c.240_241insGG mutation in NDP gene in a family with Norrie disease.

    Science.gov (United States)

    Andarva, Monavvar; Jamshidi, Javad; Ghaedi, Hamid; Daftarian, Narsis; Emamalizadeh, Babak; Alehabib, Elham; Taghavi, Shaghyegh; Pouriran, Ramin; Darvish, Hossein

    2018-03-01

    Norrie disease (ND) is a rare, X-linked recessive disorder with the main characteristic of early childhood blindness. The aim of the present study was to identify the genetic cause of the disease and the phenotypic characteristics of the patients in an Iranian family with four affected males with ND. Norrie disease pseudoglioma (NDP) gene was sequenced and clinical examination was performed on patients. A GG dinucleotide insertion in exon 3 (c.240_241insGG) of NDP was detected in all patients. The mutation caused a frameshift and an early stop codon (p.Phe81Glyfs*23). A novel mutation was found in the NDP gene in the affected males of the family. As the mutation was absent in the normal male members of the family, it should be the genetic cause of the disease. © 2017 Optometry Australia.

  12. Astronaut Scott Parazynski during egress training

    Science.gov (United States)

    1994-01-01

    Astronaut Scott E. Parazynski looks at fellow STS-66 mission specialist Joseph R. Tanner, (foreground) during a rehearsal of procedures to be followed during the launch and entry phases of their scheduled November 1994 flight. This rehearsal, held in the crew compartment trainer (CCT) of JSC's Shuttle mockup and integration laboratory, was followed by a training session on emergency egress procedures.

  13. Thinking Visually: An Interview with Scott Bennett.

    Science.gov (United States)

    Gamble, Harriet

    2002-01-01

    Presents an interview with Scott Bennett, an artist of abstract art and traditional craft. Focuses on issues such as the role of art in his life, how his art has developed over time, and his process of creating his works of art. Includes directions for a glazing project. (CMK)

  14. Coats' disease, Turner syndrome, and von Willebrand disease in a patient with Wildtype Norrie disease pseudoglioma.

    Science.gov (United States)

    Desai, Rajen U; Saffra, Norman A; Krishna, Rati P; Rosenberg, Steven E

    2011-01-01

    The authors describe a girl diagnosed as having Coats' disease, Turner syndrome (45X karyotype), and type 1 von Willebrand disease. She tested negative for the Norrie disease pseudoglioma (NDP) gene located on the X-chromosome, which has been suspected of contributing to Coats' disease. Copyright 2010, SLACK Incorporated.

  15. 78 FR 77791 - Dakota, Minnesota & Eastern Railroad Corporation-Abandonment Exemption-in Scott County, Iowa

    Science.gov (United States)

    2013-12-24

    ... DEPARTMENT OF TRANSPORTATION Surface Transportation Board [Docket No. AB 337 (Sub-No. 7X)] Dakota, Minnesota & Eastern Railroad Corporation--Abandonment Exemption--in Scott County, Iowa Dakota, Minnesota... as Blackhawk Spur, between milepost 0.33+/- and milepost 0.99 +/- in Scott County, Iowa (the Line...

  16. Reframing Michael Scott: Exploring Inappropriate Workplace Communication

    Science.gov (United States)

    Schaefer, Zachary A.

    2010-01-01

    Individuals who work in professional settings interact with others who may exhibit a variety of cultural beliefs and decision-making approaches. Page (2007) argues that cognitive diversity (i.e., how people approach and attempt to solve problems) is a vital asset in effective organizations. Michael Scott, who portrays the inept main character on…

  17. Norrie-Warburg syndrome: two novel mutations in patients with classical clinical phenotype.

    Science.gov (United States)

    Gal, A; Veske, A; Jojart, G; Grammatico, B; Huber, B; Gu, S; del Porto, G; Senyi, K

    1996-01-01

    Norrie-Warburg syndrome (NWS) is a rare X-linked disorder characterized by blindness, which is invariable, deafness and mental disturbances, which are present occasionally. We describe here two novel mutations, a missense mutation (C126S) and a 1-base pair insertion (insT466/T467), together with a recurrent mutation (M1V), found in patients presenting with the classical clinical phenotype of NWS. All three mutations are likely to result in prominent structural changes of the norrin protein.

  18. Utilization of gene mapping and candidate gene mutation screening for diagnosing clinically equivocal conditions: a Norrie disease case study.

    Science.gov (United States)

    Chini, Vasiliki; Stambouli, Danai; Nedelea, Florina Mihaela; Filipescu, George Alexandru; Mina, Diana; Kambouris, Marios; El-Shantil, Hatem

    2014-06-01

    Prenatal diagnosis was requested for an undiagnosed eye disease showing X-linked inheritance in a family. No medical records existed for the affected family members. Mapping of the X chromosome and candidate gene mutation screening identified a c.C267A[p.F89L] mutation in NPD previously described as possibly causing Norrie disease. The detection of the c.C267A[p.F89L] variant in another unrelated family confirms the pathogenic nature of the mutation for the Norrie disease phenotype. Gene mapping, haplotype analysis, and candidate gene screening have been previously utilized in research applications but were applied here in a diagnostic setting due to the scarcity of available clinical information. The clinical diagnosis and mutation identification were critical for providing proper genetic counseling and prenatal diagnosis for this family.

  19. Co-segregation of Norrie disease and idiopathic pulmonary hypertension in a family with a microdeletion of the NDP region at Xp11.3-p11.4.

    Science.gov (United States)

    Staropoli, John F; Xin, Winnie; Sims, Katherine B

    2010-11-01

    Norrie disease is a rare X-linked congenital retinal vasculopathy that may be accompanied by sensorineural deafness, mental retardation, and other neurological deficits. Here we present a family in which Norrie disease co-segregated with either early-onset idiopathic pulmonary hypertension or sudden death preceded by a period of progressive dyspnea. Neither Norrie disease, nor its atypical variants described to date, have been associated with this extended clinical phenotype. Molecular analysis of the Norrie disease gene (NDP) and adjacent loci was performed by multiplex ligation-dependent probe amplification and comparative genomic hybridisation. Affected males in this family showed an inherited hemizygous deletion restricted to NDP and two immediately telomeric genes, monoamine oxidase-B (MAO-B) and monoamine oxidase-A (MAO-A), which encode closely related enzymes that metabolize biogenic amines including serotonin, dopamine, and norepinephrine. Sequencing of the deletion junction showed an unusual pattern in which a region of microhomology flanked intervening genomic sequence. Because abnormalities of biogenic amines, particularly serotonin, have been implicated in the pathophysiology of pulmonary hypertension, we propose that presumed MAO deficiency in these patients may represent a novel risk factor for pulmonary hypertension, particularly forms with very early onset. Fine-mapping of other microdeletions at this locus may provide insights into additional mechanisms for nonrecurrent genomic rearrangements at this and other chromosomal loci.

  20. Probably Norrie's disease due to mutation. Two sporadic sibships of two males each, a necropsy of one case, and, given Norrie's disease, a calculation of the gene mutation frequency.

    OpenAIRE

    Phillips, C I; Newton, M; Duvall, J; Holloway, S; Levy, A M

    1986-01-01

    Two sibships, each with two affected males but no other affected family members, are described. All four patients at birth had small eyes with white masses visible behind clear lenses. Support for a diagnosis of Norrie's disease lies in the probable mental retardation and sudden death of one child and mental retardation in the other in one of the families, and strong support in the sensorineural deafness in one child in the other family. A necropsy was performed on the dead child. Both eyes s...

  1. Astronauts Scott and Armstrong undergoe water egress training

    Science.gov (United States)

    1966-01-01

    Astronauts Neil A. Armstrong (on left), command pilot, and David R. Scott, pilot of the Gemini 8 prime crew, use a boilerplate model of a Gemini spacecraft during water egress training in the Gulf of Mexico. Three Manned Spacecraft Center swimmers assist in the training exercise.

  2. Long-term consequences of developmental vascular defects on retinal vessel homeostasis and function in a mouse model of Norrie disease.

    Science.gov (United States)

    Beck, Susanne C; Feng, Yuxi; Sothilingam, Vithiyanjali; Garcia Garrido, Marina; Tanimoto, Naoyuki; Acar, Niyazi; Shan, Shenliang; Seebauer, Britta; Berger, Wolfgang; Hammes, Hans-Peter; Seeliger, Mathias W

    2017-01-01

    Loss of Norrin signalling due to mutations in the Norrie disease pseudoglioma gene causes severe vascular defects in the retina, leading to visual impairment and ultimately blindness. While the emphasis of experimental work so far was on the developmental period, we focus here on disease mechanisms that induce progression into severe adult disease. The goal of this study was the comprehensive analysis of the long-term effects of the absence of Norrin on vascular homeostasis and retinal function. In a mouse model of Norrie disease retinal vascular morphology and integrity were studied by means of in vivo angiography; the vascular constituents were assessed in detailed histological analyses using quantitative retinal morphometry. Finally, electroretinographic analyses were performed to assess the retinal function in adult Norrin deficient animals. We could show that the primary developmental defects not only persisted but developed into further vascular abnormalities and microangiopathies. In particular, the overall vessel homeostasis, the vascular integrity, and also the cellular constituents of the vascular wall were affected in the adult Norrin deficient retina. Moreover, functional analyses indicated to persistent hypoxia in the neural retina which was suggested as one of the major driving forces of disease progression. In summary, our data provide evidence that the key to adult Norrie disease are ongoing vascular modifications, driven by the persistent hypoxic conditions, which are ineffective to compensate for the primary Norrin-dependent defects.

  3. Astronauts Armstrong and Scott arrive at Hickam Field, Hawaii

    Science.gov (United States)

    1966-01-01

    Astronauts Neil A. Armstrong (center), command pilot, and David R. Scott, pilot, arrive at Hickam Field, Hawaii on their way from Naha, Okinawa, to Cape Kennedy, Florida. Astronaut Walter M. Schirra Jr. is at extreme left.

  4. STS-100 Crew Interview: Scott Parazynski

    Science.gov (United States)

    2001-01-01

    STS-100 Mission Specialist Scott Parazynski is seen being interviewed. He answers questions about his inspiration to become an astronaut and his career path. He gives details on the mission's goals and significance, the rendezvous and docking of Endeavour with the International Space Station (ISS), the mission's spacewalks, and installation and capabilities of the Space Station robotic arm, UHF antenna, and Rafaello Logistics Module. Parazynski then discusses his views about space exploration as it becomes an international collaboration.

  5. Combined Quantification of the Global Proteome, Phosphoproteome, and Proteolytic Cleavage to Characterize Altered Platelet Functions in the Human Scott Syndrome.

    Science.gov (United States)

    Solari, Fiorella A; Mattheij, Nadine J A; Burkhart, Julia M; Swieringa, Frauke; Collins, Peter W; Cosemans, Judith M E M; Sickmann, Albert; Heemskerk, Johan W M; Zahedi, René P

    2016-10-01

    The Scott syndrome is a very rare and likely underdiagnosed bleeding disorder associated with mutations in the gene encoding anoctamin-6. Platelets from Scott patients are impaired in various Ca 2+ -dependent responses, including phosphatidylserine exposure, integrin closure, intracellular protein cleavage, and cytoskeleton-dependent morphological changes. Given the central role of anoctamin-6 in the platelet procoagulant response, we used quantitative proteomics to understand the underlying molecular mechanisms and the complex phenotypic changes in Scott platelets compared with control platelets. Therefore, we applied an iTRAQ-based multi-pronged strategy to quantify changes in (1) the global proteome, (2) the phosphoproteome, and (3) proteolytic events between resting and stimulated Scott and control platelets. Our data indicate a limited number of proteins with decreased (70) or increased (64) expression in Scott platelets, among those we confirmed the absence of anoctamin-6 and the strong up-regulation of aquaporin-1 by parallel reaction monitoring. The quantification of 1566 phosphopeptides revealed major differences between Scott and control platelets after stimulation with thrombin/convulxin or ionomycin. In Scott platelets, phosphorylation levels of proteins regulating cytoskeletal or signaling events were increased. Finally, we quantified 1596 N-terminal peptides in activated Scott and control platelets, 180 of which we identified as calpain-regulated, whereas a distinct set of 23 neo-N termini was caspase-regulated. In Scott platelets, calpain-induced cleavage of cytoskeleton-linked and signaling proteins was downregulated, in accordance with an increased phosphorylation state. Thus, multipronged proteomic profiling of Scott platelets provides detailed insight into their protection against detrimental Ca 2+ -dependent changes that are normally associated with phosphatidylserine exposure. © 2016 by The American Society for Biochemistry and Molecular

  6. Walter Dill Scott and the Student Personnel Movement

    Science.gov (United States)

    Biddix, J. Patrick; Schwartz, Robert A.

    2012-01-01

    Walter Dill Scott (1869-1955), tenth president of Northwestern University and pioneer of industrial psychology, is an essential architect of student personnel work. This study of his accomplishments, drawing on records from the Northwestern University archives, tells a story about the people he influenced and his involvement in codifying what was…

  7. Scott Fitzgerald: famous writer, alcoholism and probable epilepsy

    Directory of Open Access Journals (Sweden)

    Mariana M. Wolski

    Full Text Available ABSTRACT Scott Fitzgerald, a world-renowned American writer, suffered from various health problems, particularly alcohol dependence, and died suddenly at the age of 44. According to descriptions in A Moveable Feast, by Ernest Hemingway, Fitzgerald had episodes resembling complex partial seizures, raising the possibility of temporal lobe epilepsy.

  8. Astronauts Armstrong and Scott during photo session outside KSC

    Science.gov (United States)

    1966-01-01

    Astronauts Neil A. Armstrong (left), command pilot, and David R. Scott, pilot, the Gemini 8 prime crew, during a photo session outside the Kennedy Space Center (KSC) Mission Control Center. They are standing in front of a radar dish.

  9. Cerebrovascular disease associated with Aarskog-Scott syndrome

    International Nuclear Information System (INIS)

    DiLuna, Michael L.; Amankulor, Nduka M.; Gunel, Murat; Johnson, Michele H.

    2007-01-01

    Faciogenital dysplasia, also known as Aarskog-Scott syndrome (AAS), is an X-linked dominant congenital disorder characterized by multiple facial, musculoskeletal, dental, neurological and urogenital abnormalities, ocular manifestations, congenital heart defects, low IQ and behavioral problems. Here we describe an unusual presentation of dysplastic carotid artery, basilar artery malformation or occlusion and posterior circulation aneurysm in a 13-year-old male with AAS. (orig.)

  10. Cerebrovascular disease associated with Aarskog-Scott syndrome

    Energy Technology Data Exchange (ETDEWEB)

    DiLuna, Michael L.; Amankulor, Nduka M.; Gunel, Murat [Yale University School of Medicine, Department of Neurosurgery, New Haven, CT (United States); Johnson, Michele H. [Yale University School of Medicine, Department of Diagnostic Radiology, New Haven, CT (United States)

    2007-05-15

    Faciogenital dysplasia, also known as Aarskog-Scott syndrome (AAS), is an X-linked dominant congenital disorder characterized by multiple facial, musculoskeletal, dental, neurological and urogenital abnormalities, ocular manifestations, congenital heart defects, low IQ and behavioral problems. Here we describe an unusual presentation of dysplastic carotid artery, basilar artery malformation or occlusion and posterior circulation aneurysm in a 13-year-old male with AAS. (orig.)

  11. The influence of the Scott effect on the determination of q0

    International Nuclear Information System (INIS)

    Kruszewski, A.; Semeniuk, I.

    1975-01-01

    The statistical model for taking into account the Scott effect was constructed. The suggestion that clusters with exceptionally bright first-ranked cluster member possess fainter than average second and third-ranked galaxies is not substantiated by raw observational data. The first-ranked galaxies are brighter and less cluster richness dependent than expected from the statistical model. The bias due to the Scott effect may increase q 0 by up to 0.5 but with proper care it should be possible to take it into account even without employing complicated statistical models. (author)

  12. The Wnt signaling pathway in familial exudative vitreoretinopathy and Norrie disease.

    Science.gov (United States)

    Warden, Scott M; Andreoli, Christopher M; Mukai, Shizuo

    2007-01-01

    The Wnt signaling pathway is highly conserved among species and has an important role in many cell biological processes throughout the body. This signaling cascade is involved in regulating ocular growth and development, and recent findings indicate that this is particularly true in the retina. Mutations involving different aspects of the Wnt signaling pathway are being linked to several diseases of retinal development. The aim of this article is to first review the Wnt signaling pathway. We will then describe two conditions, familial exudative vitreoretinopathy (FEVR) and Norrie disease (ND), which have been shown to be caused in part by defects in the Wnt signaling cascade.

  13. Long-term consequences of developmental vascular defects on retinal vessel homeostasis and function in a mouse model of Norrie disease.

    Directory of Open Access Journals (Sweden)

    Susanne C Beck

    Full Text Available Loss of Norrin signalling due to mutations in the Norrie disease pseudoglioma gene causes severe vascular defects in the retina, leading to visual impairment and ultimately blindness. While the emphasis of experimental work so far was on the developmental period, we focus here on disease mechanisms that induce progression into severe adult disease. The goal of this study was the comprehensive analysis of the long-term effects of the absence of Norrin on vascular homeostasis and retinal function. In a mouse model of Norrie disease retinal vascular morphology and integrity were studied by means of in vivo angiography; the vascular constituents were assessed in detailed histological analyses using quantitative retinal morphometry. Finally, electroretinographic analyses were performed to assess the retinal function in adult Norrin deficient animals. We could show that the primary developmental defects not only persisted but developed into further vascular abnormalities and microangiopathies. In particular, the overall vessel homeostasis, the vascular integrity, and also the cellular constituents of the vascular wall were affected in the adult Norrin deficient retina. Moreover, functional analyses indicated to persistent hypoxia in the neural retina which was suggested as one of the major driving forces of disease progression. In summary, our data provide evidence that the key to adult Norrie disease are ongoing vascular modifications, driven by the persistent hypoxic conditions, which are ineffective to compensate for the primary Norrin-dependent defects.

  14. Ron Scott d/b/a White Dog Painting Information Sheet

    Science.gov (United States)

    Ron Scott d/b/a White Dog Painting (the Company) is located in Kansas City, Missouri. The settlement involves renovation activities conducted at property constructed prior to 1978, located in Kansas City, Missouri.

  15. A Máquina Teórica: O Discurso de Christopher Norris sobre o Pós-Modernismo na Década de Noventa

    Directory of Open Access Journals (Sweden)

    Adam Sharman

    1997-10-01

    Full Text Available opresente ensaio oferece uma exegese crítica dos sete livros que, até o final de 1997, o teórico e crítico britânico Christopher Norman dedicou ao pós-modernismo, considerando-se que a temática de Norris é a teoria pósmodernista, não os artefatos culturais ou processos pósmodernistas pelos quais ele demonstra pouco interesse. Nessa trajetória, são entrelaçados os fios de diversas questões: o entendimento de Norris do pós-modernismo; as objeções que ele faz à teoria pós-modernista; o teor de sua discordância de pensadores como Baudrillard com relação à Guerra do Golfo; a nota de desalento que pauta sua visão do pós-modernismo; o impacto do contexto político de direita de Margaret Thatcher sobre sua visão pessimista dos intelectuais pós-modernistas no início da década. Sobretudo, o ensaio rastreia a crítica reiterada de Norris à excessiva extensão conferida à lingüística saussureana, na sua opinião, a raiz de um mal-elaborado "relativismo cultural", bem como seu amplo comentário sobre o afastamento de Michel Foucault do pensamento de Kant. Norris crê que esse encontro ilustra, de forma exemplar, o afastamento pós-modernista dos valores iluministas de verdade, crítica e razão universal, cujo corolário, para ele, é a eliminação de qualquer vinculação, baseada em princípios, entre a prática intelectual e o domínio ético-político. O ensaio oferece, paralelamente, um número de críticas a Norris (inclusive seu hábito de processar diversas posições numa máquina teórica abstrata e imperdoável, aiJ ressaltar as muitas alternativas à teoria pós-modernista que ele explora (filosofias pós-analíticas do significado, filosofia da ciência, realismo causal, a desconstrução de Derrida e de Man. O ensaio conclui estabelecendo uma distinção entre relatividade e relativismo e enfatizando que também a epistemologia necessita de uma ética e de uma responsabilidade -com relação aos textos

  16. Theorizing Steampunk in Scott Westerfeld's YA Series Leviathan

    Science.gov (United States)

    Mielke, Tammy L.; LaHaie, Jeanne M.

    2015-01-01

    In this article, we offer an explanation of steampunk and theorize the genre and its functions within Scott Westerfeld's YA series Leviathan. In order to do so, we examine the "cogs" of the genre machine and its use of nostalgic longing for a revised past/future to rebel against present day cultural norms. Critics note that steampunk…

  17. Mutations of the Norrie gene in Korean ROP infants.

    Science.gov (United States)

    Kim, Jeong Hun; Yu, Young Suk; Kim, Jiyeon; Park, Seong Sup

    2002-12-01

    The present study was conducted to evaluate if there is a Norrie disease gene (ND gene) mutation involved in the retinopathy of prematurity (ROP), and to identify the possibility of a genetic abnormality that may be linked to the presence of ROP. Nineteen premature Korean infants, with a low birth weight (1500 g or less) or low gestational age (32 weeks or less), were included in the study. Eighteen infants had ROP, and the other did not. Genomic DNA was isolated from the peripheral blood leukocytes of these patients, and all three exons and their flanking areas, all known ND gene mutations regions, were evaluated following amplification by a polymerase chain reaction, but no ND gene mutations were detected. Any disagreement between the relationship of ROP to the ND gene mutation will need to be clarified by further investigation.

  18. A novel nonsense mutation in the NDP gene in a Chinese family with Norrie disease.

    Science.gov (United States)

    Liu, Deyuan; Hu, Zhengmao; Peng, Yu; Yu, Changhong; Liu, Yalan; Mo, Xiaoyun; Li, Xiaoping; Lu, Lina; Xu, Xiaojuan; Su, Wei; Pan, Qian; Xia, Kun

    2010-12-08

    Norrie disease (ND), a rare X-linked recessive disorder, is characterized by congenital blindness and, occasionally, mental retardation and hearing loss. ND is caused by the Norrie Disease Protein gene (NDP), which codes for norrin, a cysteine-rich protein involved in ocular vascular development. Here, we report a novel mutation of NDP that was identified in a Chinese family in which three members displayed typical ND symptoms and other complex phenotypes, such as cerebellar atrophy, motor disorders, and mental disorders. We conducted an extensive clinical examination of the proband and performed a computed tomography (CT) scan of his brain. Additionally, we performed ophthalmic examinations, haplotype analyses, and NDP DNA sequencing for 26 individuals from the proband's extended family. The proband's computed tomography scan, in which the fifth ventricle could be observed, indicated cerebellar atrophy. Genome scans and haplotype analyses traced the disease to chromosome Xp21.1-p11.22. Mutation screening of the NDP gene identified a novel nonsense mutation, c.343C>T, in this region. Although recent research has shown that multiple different mutations can be responsible for the ND phenotype, additional research is needed to understand the mechanism responsible for the diverse phenotypes caused by mutations in the NDP gene.

  19. The importance of biochemical and genetic findings in the diagnosis of atypical Norrie disease.

    Science.gov (United States)

    Rodríguez-Muñoz, Ana; García-García, Gema; Menor, Francisco; Millán, José M; Tomás-Vila, Miguel; Jaijo, Teresa

    2018-01-26

    Norrie disease (ND) is a rare X-linked disorder characterized by bilateral congenital blindness. ND is caused by a mutation in the Norrie disease pseudoglioma (NDP) gene, which encodes a 133-amino acid protein called norrin. Intragenic deletions including NDP and adjacent genes have been identified in ND patients with a more severe neurologic phenotype. We report the biochemical, molecular, clinical and radiological features of two unrelated affected males with a deletion including NDP and MAO genes. Biochemical and genetic analyses were performed to understand the atypical phenotype and radiological findings. Biogenic amines in cerebrospinal fluid (CSF) were measured by high-performance liquid chromatography. The coding exons of NDP gene were amplified by polymerase chain reaction. Multiplex ligation-dependent probe amplification and chromosomal microarray were carried out on both affected males. Computed tomography and magnetic resonance imaging were performed on the two patients. In one patient, the serotonin and catecholamine metabolite levels in CSF were virtually undetectable. In both patients, genetic studies revealed microdeletions in the Xp11.3 region, involving the NDP, MAOA and MAOB genes. Radiological examination demonstrated brain and cerebellar atrophy. We suggest that alterations caused by MAO deficit may remain during the first years of life. Clinical phenotype, biochemical findings and neuroimaging can guide the genetic study in patients with atypical ND and help us to a better understanding of this disease.

  20. STS-105 Crew Interview: Scott Horowitz

    Science.gov (United States)

    2001-01-01

    STS-105 Commander Scott Horowitz is seen during a prelaunch interview. He answers questions about his inspiration to become an astronaut, his career path, training for the mission, and his role in the mission's activities. He gives details on the mission's goals, which include the transfer of supplies from the Discovery Orbiter to the International Space Station (ISS) and the change-over of the Expedition 2 and Expedition 3 crews (the resident crews of ISS). Horowitz discusses the importance of the ISS in the future of human spaceflight.

  1. Nursery Pest Management of Phytolyma lata Walker (Scott) Attack ...

    African Journals Online (AJOL)

    The establishment of plantations of Milicia excelsa has been constrained by the gall-forming psyllid Phytolyma lata Walker (Scott) that causes extensive damage to young plants. We present findings of an experiment aimed at preventing Phytolyma attack on Milicia seedlings in the nursery using chemical control and ...

  2. Reliability and validity of the Salford-Scott Nursing Values Questionnaire in Turkish.

    Science.gov (United States)

    Ulusoy, Hatice; Güler, Güngör; Yıldırım, Gülay; Demir, Ecem

    2018-02-01

    Developing professional values among nursing students is important because values are a significant predictor of the quality care that will be provided, the clients' recognition, and consequently the nurses' job satisfaction. The literature analysis showed that there is only one validated tool available in Turkish that examines both the personal and the professional values of nursing students. The aim of this study was to assess the reliability and validity of the Salford-Scott Nursing Values Questionnaire in Turkish. This study was a Turkish linguistic and cultural adaptation of a research tool. Participants and research context: The sample of this study consisted of 627 undergraduate nursing students from different geographical areas of Turkey. Two questionnaires were used for data collection: a socio-demographic form and the Salford-Scott Nursing Values Questionnaire. For the Salford-Scott Nursing Values Questionnaire, construct validity was examined using factor analyses. Ethical considerations: The study was approved by the Cumhuriyet University Faculty of Medicine Research Ethics Board. Students were informed that participation in the study was entirely voluntary and anonymous. Item content validity index ranged from 0.66 to 1.0, and the total content validity index was 0.94. The Kaiser-Meyer-Olkin measure of sampling was 0.870, and Bartlett's test of sphericity was statistically significant (x 2 = 3108.714, p < 0.001). Construct validity was examined using factor analyses and the six factors were identified. Cronbach's alpha was used to assess the internal consistency reliability and the value of 0.834 was obtained. Our analyses showed that the Turkish version of Salford-Scott Nursing Values Questionnaire has high validity and reliability.

  3. SCOTT: A time and amplitude digitizer ASIC for PMT signal processing

    Science.gov (United States)

    Ferry, S.; Guilloux, F.; Anvar, S.; Chateau, F.; Delagnes, E.; Gautard, V.; Louis, F.; Monmarthe, E.; Le Provost, H.; Russo, S.; Schuller, J.-P.; Stolarczyk, Th.; Vallage, B.; Zonca, E.; KM3NeT Consortium

    2013-10-01

    SCOTT is an ASIC designed for the readout electronics of photomultiplier tubes developed for KM3NeT, the cubic-kilometer scale neutrino telescope in Mediterranean Sea. To digitize the PMT signals, the multi-time-over-threshold technique is used with up to 16 adjustable thresholds. Digital outputs of discriminators feed a circular sampling memory and a “first in first out” digital memory. A specific study has shown that five specifically chosen thresholds are suited to reach the required timing accuracy. A dedicated method based on the duration of the signal over a given threshold allows an equivalent timing precision at any charge. To verify that the KM3NeT requirements are fulfilled, this method is applied on PMT signals digitized by SCOTT.

  4. Microdeletion in the X-chromosome and prenatal diagnosis in a family with Norrie disease.

    Science.gov (United States)

    Zhu, D P; Antonarakis, S E; Schmeckpeper, B J; Diergaarde, P J; Greb, A E; Maumenee, I H

    1989-08-01

    We have studied a three-generation family in which Norrie disease is segregating and have performed prenatal diagnosis on the fetus of an obligatory carrier. Deletions at loci DXS7 and DXS77 defined by probes L1.28, L1.28-p59, and pX59 were detected in the affected male. DNA studies of chorionic villus biopsy material indicated that the male fetus had inherited the normal allele from the carrier mother. This prediction was confirmed on eye examination at age 5 months.

  5. 2015-2016 Travel and Hospitality Expense Reports for Scott Gilmore ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Ruxandra Staicu

    Purpose: Board meetings. Date(s):. 2015-07-13 to 2015-07-14. Destination(s):. Ottawa. Airfare: Other. Transportation: $31.46. Accommodation: Meals and. Incidentals: Other: Total: $31.46. Comments: 2015-2016 Travel and Hospitality Expense. Reports for Scott Gilmore, Governor.

  6. Vascular defects and sensorineural deafness in a mouse model of Norrie disease.

    Science.gov (United States)

    Rehm, Heidi L; Zhang, Duan-Sun; Brown, M Christian; Burgess, Barbara; Halpin, Chris; Berger, Wolfgang; Morton, Cynthia C; Corey, David P; Chen, Zheng-Yi

    2002-06-01

    Norrie disease is an X-linked recessive syndrome of blindness, deafness, and mental retardation. A knock-out mouse model with an Ndp gene disruption was studied. We examined the hearing phenotype, including audiological, histological, and vascular evaluations. As is seen in humans, the mice had progressive hearing loss leading to profound deafness. The primary lesion was localized to the stria vascularis, which houses the main vasculature of the cochlea. Fluorescent dyes showed an abnormal vasculature in this region and eventual loss of two-thirds of the vessels. We propose that one of the principal functions of norrin in the ear is to regulate the interaction of the cochlea with its vasculature.

  7. Marion duPont Scott Equine Medical Center uses innovative lameness treatment

    OpenAIRE

    Lee, Kate

    2009-01-01

    Virginia Tech's Marion duPont Scott Equine Medical Center is now offering an equine lameness therapy that prevents further degeneration of the affected joint and offers a longer-lasting benefit than traditional steroid treatment.

  8. Two novel mutations in the Norrie disease gene associated with the classical ocular phenotype.

    Science.gov (United States)

    Caballero, M; Veske, A; Rodriguez, J J; Lugo, N; Schroeder, B; Hesse, L; Gal, A

    1996-12-01

    Norrie disease (ND) is a rare X-linked recessive disorder characterized by congenital blindness due to a degenerative and proliferative dysplasia of the neuroretina and, occasionally, by deafness and mental handicap. Here, we report two novel mutations detected in patients with the classical eye features of ND. Both the one-base pair insertion in exon II (544/545 insA) and the two-base pair deletion in the start codon (418delTG) of the ND gene predict a functional 'null allele', i.e. the complete absence of the corresponding gene product.

  9. Cataplexy and monoamine oxidase deficiency in Norrie disease.

    Science.gov (United States)

    Vossler, D G; Wyler, A R; Wilkus, R J; Gardner-Walker, G; Vlcek, B W

    1996-05-01

    Norrie disease (ND) is an X-linked recessive disorder causing ocular atrophy, mental retardation, deafness, and dysmorphic features. Virtually absent monoamine oxidase (MAO) type-A and -B activity has been found in some boys with chromosome deletions. We report the coexistence of cataplexy and abnormal REM sleep organization with ND. Three related boys, referred for treatment of medically refractory atonic spells and apneas, underwent extended EEG-video-polysomnographic monitoring. They demonstrated attacks of cataplexy and inappropriate periods of REM sleep during which they were unarousable. One boy also had generalized tonic-clonic seizures. Previous testing revealed that all three have complete ND gene deletions. In all subjects, platelet MAO-B activity was absent, serum serotonin levels were markedly increased, and plasma catecholamine levels were normal. Data from the canine narcolepsy syndrome model implicate abnormal catecholaminergic and cholinergic activities in the pathogenesis of cataplexy. Our findings suggest that abnormal MAO activity or an imbalance between serotonin and other neurotransmitter levels may be involved in the pathogenesis of human cataplexy.

  10. Evaluating safety of concrete gravity dam on weak rock: Scott Dam

    International Nuclear Information System (INIS)

    Goodman, R.E.; Ahlgren, C.S.

    2000-01-01

    Scott Dam is owned and operated by Pacific Gas and Electric Co. (PG and E) as part of the Potter Valley Project. Although it is an unimpressive concrete gravity dam [233 m (765 ft) long with maximum water surface 33.4 m (110 ft) above tail water], the dam has unusually complex and weak foundation rocks; thick condition caused design changes during construction, numerous subsequent special investigations, and several corrections and additions. A main stumbling block to clarification of the dam safety issue for Scott Dam has always been difficulty in characterizing the foundation material. This paper discusses an approach to this problem as well s how the safety of the dam was subsequently confirmed. Following a comprehensive program of research, investigations, and analysis from 1991 to 1997

  11. Distinguishing different types of inhomogeneity in Neyman-Scott point processes

    Czech Academy of Sciences Publication Activity Database

    Mrkvička, Tomáš

    2014-01-01

    Roč. 16, č. 2 (2014), s. 385-395 ISSN 1387-5841 Institutional support: RVO:60077344 Keywords : clustering * growing clusters * inhomogeneous cluster centers * inhomogeneous point process * location dependent scaling * Neyman-Scott point process Subject RIV: BA - General Mathematics Impact factor: 0.913, year: 2014

  12. 2015-2016 Expense report for Scott Gilmore | IDRC - International ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    2015-07-13

    2015-2016 Expense report for Scott Gilmore. Total travel expenses: CA$31.46. Download expense report. July 13, 2015 to July 14, 2015. CA$31.46. What we do · Funding · Resources · About IDRC. Knowledge. Innovation. Solutions. Careers · Contact Us · Site map. Sign up now for IDRC news and views sent directly to ...

  13. Eesti tervishoid on tõesti hea. Aitäh, USA! / Scott Abel

    Index Scriptorium Estoniae

    Abel, Scott

    2010-01-01

    Ameeriklane Scott Abel kirjutab, et president Barack Obama tervishoiureform mõjutab arstiabi ka Eestis. Vastukaja artiklile: Turay, Abdul. Kindla individualismi traditsioon // Postimees (2010) 30. märts, lk. 12

  14. STS-103 Crew Interviews: Scott Kelly

    Science.gov (United States)

    1999-01-01

    Live footage of a preflight interview with Pilot Scott J. Kelly is seen. The interview addresses many different questions including why Kelly became an astronaut, the events that led to his interest, any role models that he had, and his inspiration. Other interesting information that this one-on-one interview discusses is an explanation of the why this required mission to service the Hubble Space Telescope must take place at such an early date, replacement of the gyroscopes, transistors, and computers. Also discussed are the Chandra X Ray Astrophysics Facility, and a brief touch on Kelly's responsibility during any of the given four space walks scheduled for this mission.

  15. STS-82 Pilot Scott Horowitz arrives for TCDT

    Science.gov (United States)

    1997-01-01

    STS-82 Pilot Scott J. 'Doc' Horowitz arrives at KSCs Shuttle Landing Facility in a T-38 jet from Houston, TX. Horowitz and the other six crew members are at KSC to participate in the Terminal Countdown Demonstration Test (TCDT), a dress rehearsal for launch. The crew aboard the Space Shuttle Discovery on STS-82 will conduct the second Hubble Space Telescope servicing mission. The 10-day flight is targeted for a Feb. 11 liftoff.

  16. John Scott Haldane: The father of oxygen therapy

    Directory of Open Access Journals (Sweden)

    K C Sekhar

    2014-01-01

    Full Text Available John Scott Haldane was a versatile genius who solved several problems of great practical significance. His ability to look beyond the laboratory and investigate theory added crucial findings in the field of respiratory physiology. His work on high altitude physiology, diving physiology, oxygen therapy, and carbon monoxide poisoning led to a sea change in clinical medicine and improved safety and reduced mortality and morbidity in many high risk situations.

  17. Astronaut Scott Parazynski in hatch of CCT during training

    Science.gov (United States)

    1994-01-01

    Astronaut Scott E. Parazynski, STS-66 mission specialist, poses near the hatchway of the crew compartment trainer (CCT) (out of frame) in JSC's Shuttle mockup and integration laboratory. Crew members were about to begin a rehearsal of procedures to be followed during the launch and entry phases of their flight. That rehearsal was followed by a training session on emergency egress procedures.

  18. Exploring cell apoptosis and senescence to understand and treat cancer: an interview with Scott Lowe

    Directory of Open Access Journals (Sweden)

    2015-11-01

    Full Text Available Scott W. Lowe is currently principal investigator at the Memorial Sloan-Kettering Cancer Center. After beginning his studies in chemical engineering, he decided to take another path and became fascinated by biochemistry, genetics and molecular biology, which ultimately led to an interest in human disease, particularly cancer. During his PhD at the Massachusetts Institute of Technology (MIT, Scott had the opportunity to benefit from the exceptional mentorship of Earl Ruley, David Housman and Tyler Jacks, and contributed to elucidating how the p53 (TP53 tumor suppressor gene limits oncogenic transformation and modulates the cytotoxic response to conventional chemotherapy. This important work earned him a fellowship from the Cold Spring Harbor Laboratory, which helped to launch his independent career. Scott is now a leading scientist in the cancer field and his work has helped to shed light on mechanisms of cell apoptosis and senescence to better understand and treat cancer. In this interview, he talks about this incredible scientific journey.

  19. Next-generation sequencing reveals a novel NDP gene mutation in a Chinese family with Norrie disease

    OpenAIRE

    Huang, Xiaoyan; Tian, Mao; Li, Jiankang; Cui, Ling; Li, Min; Zhang, Jianguo

    2017-01-01

    Purpose: Norrie disease (ND) is a rare X-linked genetic disorder, the main symptoms of which are congenital blindness and white pupils. It has been reported that ND is caused by mutations in the NDP gene. Although many mutations in NDP have been reported, the genetic cause for many patients remains unknown. In this study, the aim is to investigate the genetic defect in a five-generation family with typical symptoms of ND. Methods: To identify the causative gene, next-generation sequencing bas...

  20. An estimating function approach to inference for inhomogeneous Neyman-Scott processes

    DEFF Research Database (Denmark)

    Waagepetersen, Rasmus

    2007-01-01

    This article is concerned with inference for a certain class of inhomogeneous Neyman-Scott point processes depending on spatial covariates. Regression parameter estimates obtained from a simple estimating function are shown to be asymptotically normal when the "mother" intensity for the Neyman-Sc...

  1. Scott Morgan Johnson Middle School: Personalization Leads to Unlimited Success

    Science.gov (United States)

    Principal Leadership, 2013

    2013-01-01

    The well-known lyrics may be "The Eyes of Texas Are Upon You," but at Scott Morgan Johnson Middle School in McKinney, TX, it's definitely the "eye of the tiger" that sets the bar for Tiger PRIDE (perseverance, respect, integrity, determination, and excellence). This article describes how those ideals have been infused…

  2. La Emulsión de Scott en la Cultura Hispanoamericana.

    Directory of Open Access Journals (Sweden)

    Alfredo Jácome Roca

    2005-06-01

    Varios de los empresarios que fueron pioneros en la industria farmacéutica contaron con algún aceite de hígado de bacalao entre sus primeros productos. En 1876, dos químicos que incursionaron en la industria, llamados Alfred B. Scott y Samuel W. Bowne, empezaron a comerciar en Nueva York la nueva Emulsión de Scott. La fórmula original incluía el aceite de hígado de bacalao –traído de Noruega en grandes cantidades– y los hipofosfitos de lima y soda. No obstante la buena fama que rodeaba sus ingredientes, la comercialización incluyó la propaganda masiva con afirmaciones ciertamente exageradas, que se aprovechaban de la credulidad del público y de la ausencia de mecanismos regulatorios. Se utilizaba tanto el humor como el temor de los parroquianos en postales, almanaques, avisos, que mostraban niños rosados y cachetones. Estos dibujos –y las botellas mismas– hacen actualmente las delicias de los coleccionistas y el negocio de los anticuarios. Una litografía aparecida en 1895 afirma que «la Emulsión de Scott genera vitalidad, carnes, fuerza y la promesa de salud para las personas de todas las edades». Otra estrategia –que aún en tiempos modernos se usa para productos populares– era la de los testimonios de personas que atestiguaban la bondad de la emulsión en su caso concreto. Un aviso que apareció en 1900 en el Greensburg Morning Tribune daba información detallada sobre la escrófula o enfermedad de las linfadenopatías y sobre la consunción, como a la sazón se llamaba a la tuberculosis. «La gente afectada con escrófula a menudo desarrolla consunción; los síntomas más prominentes de la escrófula son la anemia, la secreción de los oídos, las erupciones descamativas, el crecimiento y drenaje de las glándulas del cuello, que pronostican la pronta aparición de la consunción. Todo esto se puede interrumpir, prevenir la consunción y recuperar la salud con el uso precoz de… la Emulsión de Scott». Las niñas que declinaban

  3. Detection of a new submicroscopic Norrie disease deletion interval with a novel DNA probe isolated by differential Alu PCR fingerprint cloning

    NARCIS (Netherlands)

    Bergen, A. A.; Wapenaar, M. C.; Schuurman, E. J.; Diergaarde, P. J.; Lerach, H.; Monaco, A. P.; Bakker, E.; Bleeker-Wagemakers, E. M.; van Ommen, G. J.

    1993-01-01

    Differential Alu PCR fingerprint cloning was used to isolate a DNA probe from the Xp11.4-->p11.21 region of the human X chromosome. This novel sequence, cpXr318 (DXS742), detects a new submicroscopic deletion interval at the Norrie disease locus (NDP). Combining our data with the consensus genetic

  4. Aldred scott warthin: Pathologist and teacher par excellence

    Directory of Open Access Journals (Sweden)

    Vineeth G Nair

    2017-01-01

    Full Text Available Born in 1866, Aldred Scott Warthin was a pathologist and teacher of great repute. Even though many know him from his eponyms, the true value of his achievements, and how far he was ahead of his peers, is known to but a few modern day medical students. It was in fact, based on his work, that Henry Lynch came up with his theories on the genetic nature of cancer. He died in 1931 leaving a lot of work unfinished.

  5. STS-106 Crew Interviews: Scott D. Altman

    Science.gov (United States)

    2000-01-01

    Live footage of a preflight interview with Pilot Scott D. Altman is seen. The interview addresses many different questions including why Altman became a pilot, the events that led to his interest, his career path through the Navy, and then finally, his selection by NASA as an astronaut. Other interesting information discussed in this one-on-one interview was his work on the movie set of "Top Gun," the highlights of his Navy career, and possible shorter time frame turnarounds for missions. Altman also mentions the scheduled docking with the new International Space Station (ISS) after the arrival of the Zvezda Service Module.

  6. STS-82 Pilot Scott Horowitz at SLF

    Science.gov (United States)

    1997-01-01

    STS-82 Pilot Scott J. 'Doc' Horowitz flashes a wide grin for photographers after he lands his T-38 jet at KSCs Shuttle Landing Facility. Horowitz and the other six members of the STS-82 crew came from their home base at Johnson Space Center in Houston, TX, to spend the last few days before launch at KSC. STS-82 is scheduled for liftoff on Feb. 11 during a 65-minute launch window which opens at 3:56 a.m. EST. The 10-day flight aboard the Space Shuttle Discovery will be the second Hubble Space Telescope servicing mission.

  7. Exploring cell apoptosis and senescence to understand and treat cancer: an interview with Scott Lowe.

    Science.gov (United States)

    Lowe, Scott; Cifra, Alessandra

    2015-11-01

    Scott W. Lowe is currently principal investigator at the Memorial Sloan-Kettering Cancer Center. After beginning his studies in chemical engineering, he decided to take another path and became fascinated by biochemistry, genetics and molecular biology, which ultimately led to an interest in human disease, particularly cancer. During his PhD at the Massachusetts Institute of Technology (MIT), Scott had the opportunity to benefit from the exceptional mentorship of Earl Ruley, David Housman and Tyler Jacks, and contributed to elucidating how the p53 (TP53) tumor suppressor gene limits oncogenic transformation and modulates the cytotoxic response to conventional chemotherapy. This important work earned him a fellowship from the Cold Spring Harbor Laboratory, which helped to launch his independent career. Scott is now a leading scientist in the cancer field and his work has helped to shed light on mechanisms of cell apoptosis and senescence to better understand and treat cancer. In this interview, he talks about this incredible scientific journey. © 2015. Published by The Company of Biologists Ltd.

  8. Universos ficcionais: o romanesco em Walter Scott e José de Alencar

    Directory of Open Access Journals (Sweden)

    Marcos Roberto Flamínio Peres

    2016-10-01

    Full Text Available Tamanha é a força do romanesco em Walter Scott que ele foi capaz de dar origem a duas linhas de força críticas antagônicas: uma tendendo a situá-lo dentro do conjunto da literatura ocidental, reatualizando arquétipos ancestrais (Frye; outra considerando-o a quintessência do romance histórico por representar momentos cruciais por que passava a sociedade capitalista entre os séculos XVIII e XIX (Lukács. À luz desse pano de fundo teórico contrastivo, este artigo busca analisar Waverley (1814, obra mais influente de Scott, em comparação com As minas de prata (1865-1866, romance mais ambicioso de José de Alencar e que lança mão de estratégias narrativas similares.

  9. 78 FR 3479 - Notice of Public Meeting of Fort Scott Council

    Science.gov (United States)

    2013-01-16

    ... submitted on cards that will be provided at the meeting, via mail to Laurie Fox, Presidio Trust, 103... stated prominently at the beginning of the comments. The Trust will make available for public inspection... PRESIDIO TRUST Notice of Public Meeting of Fort Scott Council AGENCY: The Presidio Trust. ACTION...

  10. PEOPLE IN PHYSICS: Interview with Scott Durow, Software Engineer, Oxford

    Science.gov (United States)

    Burton, Conducted by Paul

    1998-05-01

    Scott Durow was educated at Bootham School, York. He studied Physics, Mathematics and Chemistry to A-level and went on to Nottingham University to read Medical Physics. After graduating from Nottingham he embarked on his present career as a Software Engineer based in Oxford. He is a musician in his spare time, as a member of a band and playing the French horn.

  11. Finality regained: A co-algebraic study of Scott-sets and Multisets

    NARCIS (Netherlands)

    D'Agostino, G.; Visser, A.

    1999-01-01

    In this paper we study iterated circular multisets in a coalgebraic frame- work. We will produce two essentially different universes of such sets. The unisets of the first universe will be shown to be precisely the sets of the Scott universe. The unisets of the second universe will be precisely

  12. Scott Foresman-Addison Wesley Elementary Mathematics. What Works Clearinghouse Intervention Report

    Science.gov (United States)

    What Works Clearinghouse, 2010

    2010-01-01

    "Scott Foresman-Addison Wesley Elementary Mathematics" is a core curriculum for students at all ability levels in prekindergarten through grade 6. The program supports students' understanding of key math concepts and skills and covers a range of mathematical content across grades. The What Works Clearinghouse (WWC) reviewed 12 studies on…

  13. Marion duPont Scott Equine Medical Center offers new treatment for lameness

    OpenAIRE

    Musick, Marjorie

    2006-01-01

    The Virginia-Maryland Regional College of Veterinary Medicine's Marion duPont Scott Equine Medical Center has begun offering a new therapy for treating lameness associated with osteoarthritis and cartilage damage in horses, a problem that affects all segments of the equine industry.

  14. 100 years since Scott reached the pole: a century of learning about the physiological demands of Antarctica.

    Science.gov (United States)

    Halsey, Lewis G; Stroud, Mike A

    2012-04-01

    The 1910-1913 Terra Nova Expedition to the Antarctic, led by Captain Robert Falcon Scott, was a venture of science and discovery. It is also a well-known story of heroism and tragedy since his quest to reach the South Pole and conduct research en route, while successful was also fateful. Although Scott and his four companions hauled their sledges to the Pole, they died on their return journey either directly or indirectly from the extreme physiological stresses they experienced. One hundred years on, our understanding of such stresses caused by Antarctic extremes and how the body reacts to severe exercise, malnutrition, hypothermia, high altitude, and sleep deprivation has greatly advanced. On the centenary of Scott's expedition to the bottom of the Earth, there is still controversy surrounding whether the deaths of those five men could have, or should have, been avoided. This paper reviews present-day knowledge related to the physiology of sustained man-hauling in Antarctica and contrasts this with the comparative ignorance about these issues around the turn of the 20th century. It closes by considering whether, with modern understanding about the effects of such a scenario on the human condition, Scott could have prepared and managed his team differently and so survived the epic 1,600-mile journey. The conclusion is that by carrying rations with a different composition of macromolecules, enabling greater calorific intake at similar overall weight, Scott might have secured the lives of some of the party, and it is also possible that enhanced levels of vitamin C in his rations, albeit difficult to achieve in 1911, could have significantly improved their survival chances. Nevertheless, even with today's knowledge, a repeat attempt at his expedition would by no means be bound to succeed.

  15. Scott Foresman-Addison Wesley Elementary Mathematics. What Works Clearinghouse Intervention Report. Updated

    Science.gov (United States)

    What Works Clearinghouse, 2013

    2013-01-01

    "Scott Foresman-Addison Wesley Elementary Mathematics" is a core mathematics curriculum for students in prekindergarten through grade 6. The program aims to improve students' understanding of key math concepts through problem-solving instruction, hands-on activities, and math problems that involve reading and writing. The curriculum…

  16. Making waves the story of Ruby Payne-Scott : Australian pioneer radio astronomer

    CERN Document Server

    Goss, M

    2013-01-01

    This book is an abbreviated, partly re-written version of "Under the Radar - The First Woman in Radio Astronomy: Ruby Payne-Scott." It addresses a general readership interested in historical and sociological aspects of astronomy and presents the biography of Ruby Payne-Scott (1912 – 1981). As the first female radio astronomer (and one of the first people in the world to consider radio astronomy), she made classic contributions to solar radio physics. She also played a major role in the design of the Australian government's Council for Scientific and Industrial Research radars, which were in turn of vital importance in the Southwest Pacific Theatre in World War II. These radars were used by military personnel from Australia, the United States and New Zealand. From a sociological perspective, her career offers many examples of the perils of being a female academic in the first half of the 20th century. Written in an engaging style and complemented by many historical photographs, this book offers fascinating...

  17. Clinical, biochemical, and neuropsychiatric evaluation of a patient with a contiguous gene syndrome due to a microdeletion Xp11.3 including the Norrie disease locus and monoamine oxidase (MAOA and MAOB) genes.

    Science.gov (United States)

    Collins, F A; Murphy, D L; Reiss, A L; Sims, K B; Lewis, J G; Freund, L; Karoum, F; Zhu, D; Maumenee, I H; Antonarakis, S E

    1992-01-01

    Norrie disease is a rare X-linked recessive disorder characterized by blindness from infancy. The gene for Norrie disease has been localized to Xp11.3. More recently, the genes for monoamine oxidase (MAOA, MAOB) have been mapped to the same region. This study evaluates the clinical, biochemical, and neuropsychiatric data in an affected male and 2 obligate heterozygote females from a single family with a submicroscopic deletion involving Norrie disease and MAO genes. The propositus was a profoundly retarded, blind male; he also had neurologic abnormalities including myoclonus and stereotopy-habit disorder. Both obligate carrier females had a normal IQ. The propositus' mother met diagnostic criteria for "chronic hypomania and schizotypal features." The propositus' MAO activity was undetectable and the female heterozygotes had reduced levels comparable to patients receiving MAO inhibiting antidepressants. MAO substrate and metabolite abnormalities were found in the propositus' plasma and CSF. This study indicates that subtle biochemical and possibly neuropsychiatric abnormalities may be detected in some heterozygotes with the microdeletion in Xp11.3 due to loss of the gene product for the MAO genes; this deletion can also explain some of the complex phenotype of this contiguous gene syndrome in the propositus.

  18. Mutations in the Norrie disease gene.

    Science.gov (United States)

    Schuback, D E; Chen, Z Y; Craig, I W; Breakefield, X O; Sims, K B

    1995-01-01

    We report our experience to date in mutation identification in the Norrie disease (ND) gene. We carried out mutational analysis in 26 kindreds in an attempt to identify regions presumed critical to protein function and potentially correlated with generation of the disease phenotype. All coding exons, as well as noncoding regions of exons 1 and 2, 636 nucleotides in the noncoding region of exon 3, and 197 nucleotides of 5' flanking sequence, were analyzed for single-strand conformation polymorphisms (SSCP) by polymerase chain reaction (PCR) amplification of genomic DNA. DNA fragments that showed altered SSCP band mobilities were sequenced to locate the specific mutations. In addition to three previously described submicroscopic deletions encompassing the entire ND gene, we have now identified 6 intragenic deletions, 8 missense (seven point mutations, one 9-bp deletion), 6 nonsense (three point mutations, three single bp deletions/frameshift) and one 10-bp insertion, creating an expanded repeat in the 5' noncoding region of exon 1. Thus, mutations have been identified in a total of 24 of 26 (92%) of the kindreds we have studied to date. With the exception of two different mutations, each found in two apparently unrelated kindreds, these mutations are unique and expand the genotype database. Localization of the majority of point mutations at or near cysteine residues, potentially critical in protein tertiary structure, supports a previous protein model for norrin as member of a cystine knot growth factor family (Meitinger et al., 1993). Genotype-phenotype correlations were not evident with the limited clinical data available, except in the cases of larger submicroscopic deletions associated with a more severe neurologic syndrome.(ABSTRACT TRUNCATED AT 250 WORDS)

  19. Comparative genomics on Norrie disease gene.

    Science.gov (United States)

    Katoh, Masuko; Katoh, Masaru

    2005-05-01

    DAND1 (NBL1), DAND2 (CKTSF1B1 or GREM1 or GREMLIN), DAND3 (CKTSF1B2 or GREM2 or PRDC), DAND4 (CER1), DAND5 (CKTSF1B3 or GREM3 or DANTE), MUC2, MUC5AC, MUC5B, MUC6, MUC19, WISP1, WISP2, WISP3, VWF, NOV and Norrie disease (NDP or NORRIN) genes encode proteins with cysteine knot domain. Cysteine-knot superfamily proteins regulate ligand-receptor interactions for a variety of signaling pathways implicated in embryogenesis, homeostasis, and carcinogenesis. Although Ndp is unrelated to Wnt family members, Ndp is claimed to function as a ligand for Fzd4. Here, we identified and characterized rat Ndp, cow Ndp, chicken ndp and zebrafish ndp genes by using bioinformatics. Rat Ndp gene, consisting of three exons, was located within AC105563.4 genome sequence. Cow Ndp and chicken ndp complete CDS were derived from CB467544.1 EST and BX932859.2 cDNA, respectively. Zebrafish ndp gene was located within BX572627.5 genome sequence. Rat Ndp (131 aa) was a secreted protein with C-terminal cysteine knot-like (CTCK) domain. Rat Ndp showed 100, 96.9, 95.4, 87.8 and 66.4 total-amino-acid identity with mouse Ndp, cow Ndp, human NDP, chicken ndp and zebrafish ndp, respectively. Exon-intron structure of mammalian Ndp orthologs was well conserved. FOXA2, CUTL1 (CCAAT displacement protein), LMO2, CEBPA (C/EBPalpha)-binding sites and triple POU2F1 (OCT1)-binding sites were conserved among promoters of mammalian Ndp orthologs.

  20. Twenty years of audiology in a patient with Norrie disease.

    Science.gov (United States)

    Halpin, Chris; Sims, Katherine

    2008-11-01

    To describe disease progression and treatment outcomes over a 20-year period (ages 5-25) in a young man with Norrie disease (occuloacousticocerebral dysplasia), ND; OMIM #310600. Affected individuals are born blind and develop progressive sensory loss with onset in adolescence. This disease is X-linked and has been associated with mutations of the NDP gene (Xp11.4). The patient was followed using repeated audiograms, as well as reports of educational progress and hearing aid use. The specific mutation was found by molecular analysis. The patient demonstrated progressive sensory loss with good preservation of word recognition. The loss was initially high frequency and asymmetric in adolescence and became more severe, more symmetric and affected practically all frequencies by the end of childhood. Educational progress was affected by the cognitive effects of the syndrome, and hearing aid use was very effective. A bilateral progressive sensory loss with good preservation of word recognition was documented in detail. The residual word recognition supported good use of hearing aids in this case.

  1. STS-101 Crew Interview / Scott Horowitz

    Science.gov (United States)

    2000-01-01

    Live footage of a preflight interview with Pilot Scott J. Horowitz is seen. The interview addresses many different questions including why Horowitz became an astronaut, the events that led to his interest, any role models that he had, and his inspiration. Other interesting information that this one-on-one interview discusses is the reaction and reasons for the splitting-up of the objectives for STS-101 with STS-106. Horowitz also mentions the scheduled space-walk, docking with the International Space Station (ISS), the new glass cockpit of Atlantis, the repairs of equipment and change of the batteries. Horowitz also discusses his responsibilities during the space-walk, and docking of the spacecraft. He stresses that he will have an added challenge during the space-walk, his inability to see where he needs to place the Extravehicular Activities (EVA) crew.

  2. X-linked juvenile retinoschisis: mutations at the retinoschisis and Norrie disease gene loci?

    Science.gov (United States)

    Hiraoka, M; Rossi, F; Trese, M T; Shastry, B S

    2001-01-01

    Juvenile retinoschisis (RS) and Norrie disease (ND) are X-linked recessive retinal disorders. Both disorders, in the majority of cases, are monogenic and are caused by mutations in the RS and ND genes, respectively. Here we report the identification of a family in which mutations in both the RS and ND genes are segregating with RS pathology. Although the mutations identified in this report were not functionally characterized with regard to their pathogenicity, it is likely that both of them are involved in RS pathology in the family analyzed. This suggests the complexity and digenic nature of monogenic human disorders in some cases. If this proves to be a widespread problem, it will complicate the strategies used to identify the genes involved in diseases and to develop methods for intervention.

  3. STS-82 Pilot Scott J. 'Doc' Horowitz Suit Up

    Science.gov (United States)

    1997-01-01

    STS-82 Pilot Scott J. 'Doc' Horowitz puts on a glove of his launch and entry suit with assistance from a suit technician in the Operations and Checkout Building. This is Horowitz''';s second space flight. He and the six other crew members will depart shortly for Launch Pad 39A, where the Space Shuttle Discovery awaits liftoff on a 10-day mission to service the orbiting Hubble Space Telescope (HST). This will be the second HST servicing mission. Four back-to-back spacewalks are planned.

  4. The Paradoxical World of The Great Gatsby by F. Scott Fitzgerald

    OpenAIRE

    ŠANDEROVÁ, Milada

    2015-01-01

    In The Great Gatsby F. Scott Fitzgerald created a world of fundamental contradictions. Whether talking about the way the whole society works, the immense differences among social classes, the characters, or the tension between attributes of a particular character. Therefore, the goal of this bachelor thesis is to analyse the world of this novel as the world built on paradoxes.

  5. Molecular dissection of Norrie disease.

    Science.gov (United States)

    Berger, W

    1998-01-01

    Norrie disease (ND) is a severe form of congenital blindness accompanied by mental retardation and/or deafness in at least one third of the patients. This article summarizes advances in the molecular genetic analysis of this disease during the last 13 years, including mapping and cloning of the human gene and the generation and characterization of a mouse model. Genetic linkage studies and physical mapping strategies have assigned the ND locus to the proximal short arm of the human X chromosome. The identification of chromosomal rearrangements in several patients, such as microdeletions, enabled the isolation of the ND gene by a positional cloning approach. Numerous point mutations in this gene have been identified in three distinct clinical entities: (1) ND, (2) familial and sporadic exudative vitreoretinopathy, and (3) retinopathy of prematurity. The gene encodes a relatively small protein, consisting of 133 amino acids. The function of the gene product is yet unknown, although homologies with known proteins and molecular modelling data suggest a role in the regulation of cell interaction or differentiation processes. A mouse model has been generated to shed more light on early pathogenic events involved in ND and allelic disorders. The mouse homologous protein is highly identical (94%) to the human polypeptide. The gene is expressed in the neuronal layers of the mouse retina, the cerebellum and olfactory epithelium. Mutant mice show snowflake-like opacities within the vitreous, dysgenesis of the ganglion cell layer and occasionally degeneration of photoreceptor cells. The mouse phenotype does not include phthisis bulbi and, overall, resembles a mild form of ND. Electrophysiological studies revealed a severely altered electroretinogram b-wave. These results suggest a primary defect in the inner neuronal layers of the retina. Defects in the vitreous and photoreceptor cell layer are most likely secondary effects. Further histological, functional and molecular

  6. 76 FR 71611 - Notice of Establishment of the Fort Winfield Scott Advisory Committee

    Science.gov (United States)

    2011-11-18

    ... (``Committee''). The Committee will advise the Executive Director of the Presidio Trust on matters pertaining... of once every three months. Nominations: The Presidio Trust will consider nominations of all... PRESIDIO TRUST Notice of Establishment of the Fort Winfield Scott Advisory Committee AGENCY: The...

  7. John Norris, Collision Course: NATO, Russian and Kosovo (foreword by Strobe Talbott. Westport: Praeger Publishers, 2005. 333 pages.

    Directory of Open Access Journals (Sweden)

    Guillaume Colin

    2006-11-01

    Full Text Available Recension requires thoroughness in reading and examining a book, including its cover. In this connection, the cover of John Norris' book bodes ill for its content. A physical map with mountains, waterways, a few localities - most of which are half hidden by the title. However, enough of them can be made out (Argun, Aksay, Khasavyurt, Gudermes... to understand that this is no map of Kosovo but that of Chechnya! Could anyone fail to suspect an unfortunate mistake, a slip of the pen confirming ...

  8. Rapport de frais de 2015-2016 pour Scott Gilmore | CRDI - Centre ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Accueil · À propos du CRDI · Obligation de rendre compte · Transparence · Déplacements et accueil. Rapport de frais de 2015-2016 pour Scott Gilmore. Total des frais de déplacement : CAD$31.46. Télécharger la version PDF de ce rapport. 13 juillet 2015 au 14 juillet 2015. CAD$31.46. Ce que nous faisons · Financement ...

  9. The Pleasures and Lessons of Academic Mythbusting: An Interview with Scott Lilienfeld

    Science.gov (United States)

    Zinn, Tracy E.

    2010-01-01

    Scott O. Lilienfeld is a professor of psychology at Emory University, in Atlanta, Georgia. Dr. Lilienfeld is founder and editor of the journal, "Scientific Review of Mental Health Practice," and is past president of the Society for a Science of Clinical Psychology. He has been a member of 11 journal editorial boards, including the…

  10. STS-90 Pilot Scott Altman in white room before launch

    Science.gov (United States)

    1998-01-01

    STS-90 Pilot Scott Altman is assisted by NASA and USA closeout crew members immediately preceding launch for the nearly 17-day Neurolab mission. Investigations during the Neurolab mission will focus on the effects of microgravity on the nervous system. Linnehan and six fellow crew members will shortly enter the orbiter at KSC's Launch Pad 39B, where the Space Shuttle Columbia will lift off during a launch window that opens at 2:19 p.m. EDT, April 17.

  11. Random attractors for stochastic lattice reversible Gray-Scott systems with additive noise

    Directory of Open Access Journals (Sweden)

    Hongyan Li

    2015-10-01

    Full Text Available In this article, we prove the existence of a random attractor of the stochastic three-component reversible Gray-Scott system on infinite lattice with additive noise. We use a transformation of addition involved with Ornstein-Uhlenbeck process, for proving the pullback absorbing property and the pullback asymptotic compactness of the reaction diffusion system with cubic nonlinearity.

  12. Coretta Scott King Award Winner Javaka Steptoe Stands Tall "In Daddy's Arms."

    Science.gov (United States)

    Peck, Jackie; Hendershot, Judy

    1999-01-01

    Offers an interview with artist and author Javaka Steptoe, winner of the Coretta Scott King award for his book "In Daddy's Arms I Am Tall: African Americans Celebrating Fathers." Discusses his background in the arts, the variety of media he uses, how he begins thinking about his illustrations, his work with children's art, and aspects of his work.…

  13. Formaldehyde as a carbon and electron shuttle between autotroph and heterotroph populations in acidic hydrothermal vents of Norris Geyser Basin, Yellowstone National Park

    Energy Technology Data Exchange (ETDEWEB)

    Moran, James J.; Whitmore, Laura M.; Isern, Nancy G.; Romine, Margaret F.; Riha, Krystin M.; Inskeep, William P.; Kreuzer, Helen W.

    2016-03-19

    The Norris Geyser Basin in Yellowstone National Park contains a large number of hydrothermal systems, which host microbial populations supported by primary productivity associated with a suite of chemolithotrophic metabolisms. We demonstrate that Metallosphaera yellowstonesis MK1, a facultative autotrophic archaeon isolated from a hyperthermal acidic hydrous ferric oxide (HFO) spring in Norris Geyser Basin, excretes formaldehyde during autotrophic growth. To determine the fate of formaldehyde in this low organic carbon environment, we incubated native microbial mat (containing M. yellowstonensis) from a HFO spring with 13C-formaldehyde. Isotopic analysis of incubation-derived CO2 and biomass showed that formaldehyde was both oxidized and assimilated by members of the community. Autotrophy, formaldehyde oxidation, and formaldehyde assimilation displayed different sensitivities to chemical inhibitors, suggesting that distinct sub-populations in the mat selectively perform these functions. Our results demonstrate that electrons originally resulting from iron oxidation can energetically fuel autotrophic carbon fixation and associated formaldehyde excretion, and that formaldehyde is both oxidized and assimilated by different organisms within the native microbial community. Thus, formaldehyde can effectively act as a carbon and electron shuttle connecting the autotrophic, iron oxidizing members with associated heterotrophic members in the HFO community.

  14. STS-87 Mission Specialists Scott and Doi with EVA coordinator Laws participate in the CEIT for their

    Science.gov (United States)

    1997-01-01

    Participating in the Crew Equipment Integration Test (CEIT) at Kennedy Space Center are STS-87 crew members, assisted by Glenda Laws, extravehicular activity (EVA) coordinator, Johnson Space Center, at left. Next to Laws is Mission Specialist Takao Doi, Ph.D., of the National Space Development Agency of Japan, who is looking on as Mission Specialist Winston Scott gets a hands-on look at some of the equipment. The STS-87 mission will be the fourth United States Microgravity Payload and flight of the Spartan-201 deployable satellite. During the mission, scheduled for a Nov. 19 liftoff from KSC, Dr. Doi and Scott will both perform spacewalks.

  15. Modernity in Two Great American Writers' Vision: Ernest Miller Hemingway and Scott Fitzgerald

    Science.gov (United States)

    Keshmiri, Fahimeh; Darzikola, Shahla Sorkhabi

    2016-01-01

    Scott Fitzgerald and Ernest Hemingway, American memorable novelists have had philosophic ideas about modernity. In fact their idea about existential interests of American, and the effects of American system on society, is mirrored in their creative works. All through his early works, Fitzgerald echoes the existential center of his era. Obviously,…

  16. Norrie disease and MAO genes: nearest neighbors.

    Science.gov (United States)

    Chen, Z Y; Denney, R M; Breakefield, X O

    1995-01-01

    The Norrie disease and MAO genes are tandemly arranged in the p11.4-p11.3 region of the human X chromosome in the order tel-MAOA-MAOB-NDP-cent. This relationship is conserved in the mouse in the order tel-MAOB-MAOA-NDP-cent. The MAO genes appear to have arisen by tandem duplication of an ancestral MAO gene, but their positional relationship to NDP appears to be random. Distinctive X-linked syndromes have been described for mutations in the MAOA and NDP genes, and in addition, individuals have been identified with contiguous gene syndromes due to chromosomal deletions which encompass two or three of these genes. Loss of function of the NDP gene causes a syndrome of congenital blindness and progressive hearing loss, sometimes accompanied by signs of CNS dysfunction, including variable mental retardation and psychiatric symptoms. Other mutations in the NDP gene have been found to underlie another X-linked eye disease, exudative vitreo-retinopathy. An MAOA deficiency state has been described in one family to date, with features of altered amine and amine metabolite levels, low normal intelligence, apparent difficulty in impulse control and cardiovascular difficulty in affected males. A contiguous gene syndrome in which all three genes are lacking, as well as other as yet unidentified flanking genes, results in severe mental retardation, small stature, seizures and congenital blindness, as well as altered amine and amine metabolites. Issues that remain to be resolved are the function of the NDP gene product, the frequency and phenotype of the MAOA deficiency state, and the possible occurrence and phenotype of an MAOB deficiency state.

  17. VizieR Online Data Catalog: AIMSS Project. I. Compact Stellar Systems (Norris+, 2014)

    Science.gov (United States)

    Norris, M. A.; Kannappan, S. J.; Forbes, D. A.; Romanowsky, A. J.; Brodie, J. P.; Faifer, F. R.; Huxor, A.; Maraston, C.; Moffett, A. J.; Penny, S. J.; Pota, V.; Smith-Castelli, A.; Strader, J.; Bradley, D.; Eckert, K. D.; Fohring, D.; McBride, J.; Stark, D. V.; Vaduvescu, O.

    2015-04-01

    The majority of our southern spectroscopic observations to date have been obtained using the Southern Astrophysical Research (SOAR) Telescope and the Goodman spectrograph in longslit and MOS modes. We used the South African Large Telescope (SALT) to observe fainter targets requiring exposure times impractically long to be used as filler targets for SOAR observing and which cannot be observed with Keck. As part of a study examining the GCs and UCDs of the shell elliptical NGC 3923 we obtained deep Gemini/GMOS spectroscopy of three UCDs (see Norris et al., 2012MNRAS.421.1485N, Cat. J/MNRAS/421/1485 for further details). The observations were made in MOS mode. The majority of our Northern hemisphere candidates were spectroscopically confirmed using the DEIMOS and ESI instruments on the Keck telescope . We also obtained spectra of NGC 4649 UCD1 with the IDS instrument on the Isaac Newton Telescope. (2 data files).

  18. Magic neutrino mass matrix and the Bjorken-Harrison-Scott parameterization

    International Nuclear Information System (INIS)

    Lam, C.S.

    2006-01-01

    Observed neutrino mixing can be described by a tribimaximal MNS matrix. The resulting neutrino mass matrix in the basis of a diagonal charged lepton mass matrix is both 2-3 symmetric and magic. By a magic matrix, I mean one whose row sums and column sums are all identical. I study what happens if 2-3 symmetry is broken but the magic symmetry is kept intact. In that case, the mixing matrix is parameterized by a single complex parameter U e3 , in a form discussed recently by Bjorken, Harrison, and Scott

  19. Identification of A Novel Missense Mutation in The Norrie Disease Gene: The First Molecular Genetic Analysis and Prenatal Diagnosis of Norrie Disease in An Iranian Family.

    Science.gov (United States)

    Talebi, Farah; Ghanbari Mardasi, Farideh; Mohammadi Asl, Javad; Lashgari, Ali; Farhadi, Freidoon

    2018-07-01

    Norrie disease (ND) is a rare X-linked recessive disorder, which is characterized by congenital blindness and, in several cases, accompanied with mental retardation and deafness. ND is caused by mutations in NDP, located on the proximal short arm of the X chromosome (Xp11.3). The disease has been observed in many ethnic groups worldwide, however, no such case has been reported from Iran. In this study, we present the molecular analysis of two patients with ND and the subsequent prenatal diagnosis. Screening of NDP identified a hemizygous missense mutation (p.Ser133Cys) in the affected male siblings of the family. The mother was the carrier for the mutation (p.Ser133Cys). In a subsequent chorionic amniotic pregnancy, we carried out prenatal diagnosis by sequencing NDP in the chorionic villi sample at 11 weeks of gestation. The fetus was carrying the mutation and thus unaffected. This is the first mutation report and prenatal diagnosis of an Iranian family with ND, and highlights the importance of prenatal diagnostic screening of this congenital disorder and relevant genetic counseling. Copyright© by Royan Institute. All rights reserved.

  20. Mission Specialist Scott Parazynski checks his flight suit

    Science.gov (United States)

    1998-01-01

    STS-95 Mission Specialist Scott E. Parazynski gets help with his flight suit in the Operations and Checkout Building from a suit technician George Brittingham. The final fitting takes place prior to the crew walkout and transport to Launch Pad 39B. Targeted for launch at 2 p.m. EST on Oct. 29, the mission is expected to last 8 days, 21 hours and 49 minutes, and return to KSC at 11:49 a.m. EST on Nov. 7. The STS-95 mission includes research payloads such as the Spartan solar-observing deployable spacecraft, the Hubble Space Telescope Orbital Systems Test Platform, the International Extreme Ultraviolet Hitchhiker, as well as the SPACEHAB single module with experiments on space flight and the aging process.

  1. 2015-2016 Rapports sur les frais de voyage et d'accueil pour Scott ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Ruxandra Staicu

    Réunion du Conseil des gouverneurs. Date(s):. 2015-07-13 à 2015-07-14. Destination(s):. Ottawa. Billet d'avion: Frais de transport au sol ou autrement: 31.46 $. Frais de logement: Repas et frais divers: Autre frais: Total: 31.46 $. Commentaires: 2015-2016 Rapports sur les frais de voyage et d'accueil pour Scott Gilmore, ...

  2. Bethe, Oppenheimer, Teller and the Fermi Award: Norris Bradbury Speaks

    Energy Technology Data Exchange (ETDEWEB)

    Meade, Roger Allen [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2017-04-28

    In 1956 the Enrico Fermi Presidential Award was established to recognize scientists, engineers, and science policymakers who gave unstintingly over their careers to advance energy science and technology. The first recipient was John von Neumann. .1 Among those scientists who were thought eligible for the award were Hans Bethe, J. Robert Oppenheimer, and Edward Teller. In 1959 Norris Bradbury was asked to comment on the relative merits of each these three men, whom he knew well from their affiliation with Los Alamos. Below is a reproduction of the letter Bradbury sent to Dr. Warren C. Johnson of the AEC’s General Advisory Committee(GAC) containing his evaluation of each man. The letter might surprise those not accustomed to Bradbury’s modus operandi of providing very detailed and forthright answers to the AEC. The letter, itself, was found in cache of old microfilm. Whether because of the age of the microfilm or the quality of the filming process, portions of the letter are not legible. Where empty brackets appear, the word or words could not be read or deduced. Words appearing in brackets are guesses that appear, from the image, to be what was written. These guesses, of course, are just that – guesses.

  3. Mission Specialist Scott Parazynski arrives at KSC

    Science.gov (United States)

    1998-01-01

    STS-95 Mission Specialist Scott E. Parazynski notes the time on his watch upon his late arrival aboard a T-38 jet at the Shuttle Landing Facility. Parazynski's first plane experienced problems at the stop at Tyndall AFB and he had to wait for another jet and pilot to finish the flight to KSC. He joined other crewmembers Mission Commander Curtis L. Brown Jr., Pilot Steven W. Lindsey, Mission Specialist Stephen K. Robinson, Payload Specialist John H. Glenn Jr., senator from Ohio, Mission Specialist Pedro Duque, with the European Space Agency (ESA), and Payload Specialist Chiaki Mukai, with the National Space Development Agency of Japan (NASDA), for final pre-launch preparations. STS-95 is expected to launch at 2 p.m. EST on Oct. 29, last 8 days, 21 hours and 49 minutes, and land at 11:49 a.m. EST on Nov. 7.

  4. Submicroscopic interstitial deletion of the X chromosome explains a complex genetic syndrome dominated by Norrie disease.

    Science.gov (United States)

    Gal, A; Wieringa, B; Smeets, D F; Bleeker-Wagemakers, L; Ropers, H H

    1986-01-01

    Norrie disease (ND), an X-linked recessive disorder, is characterized by congenital blindness followed by bulbar atrophy. We have examined a three-generation family in which ND is part of a complex X-linked syndrome with severe mental retardation, hypogonadism, growth disturbances, and increased susceptibility to infections as additional features. This syndrome is apparently due to an interstitial deletion, as evidenced by the failure of the L1.28 DNA probe (DXS7 locus, Xp11.3) to detect complementary DNA sequences on the defective X chromosome of an affected male and of several obligatory heterozygotes. Attempts to further define this deletion with other DNA probes from the proximal short arm of the X chromosome or by prometaphase chromosome analysis were unsuccessful.

  5. Studies on the presence and spatial distribution of anthropogenic pollutants in the glacial basin of Scott Glacier in the face of climate change (Fiord Bellsund, Spitsbergen)

    Science.gov (United States)

    Lehmann, Sara; Kociuba, Waldemar; Franczak, Łukasz; Gajek, Grzegorz; Łeczyński, Leszek; Kozak, Katarzyna; Szopińska, Małgorzata; Ruman, Marek; Polkowska, Żaneta

    2014-10-01

    The study area covered the NW part of the Wedel Jarlsberg Land (SW part of the Svalbard Archipelago). The primary study object was the catchment of the Scott Glacier in the vicinity of the Research Station of of Maria Curie-Skłodowska University in Lublin - Calypsobyen. The Scott River catchment (of glacial hydrological regime) has an area of approximately 10 km2, 40% of which is occupied by the valley Scott Glacier in the phase of strong recession. The present study concerns the determination of physical and chemical parameters (pH, conductivity, TOC) and concentrations of pollutants (phenols, aldehydes).

  6. STS-90 Pilot Scott Altman arrives at KSC for TCDT

    Science.gov (United States)

    1998-01-01

    STS-90 Pilot Scott Altman poses in the cockpit of his T-38 jet trainer aircraft after arriving at the KSC Shuttle Landing Facility along with other members of the crew from NASAs Johnson Space Center to begin Terminal Countdown Demonstration Test (TCDT) activities. The TCDT is held at KSC prior to each Space Shuttle flight to provide crews with the opportunity to participate in simulated countdown activities. Columbia is targeted for launch of STS-90 on April 16 at 2:19 p.m. EST and will be the second mission of 1998. The mission is scheduled to last nearly 17 days.

  7. Two Thai families with Norrie disease (ND): association of two novel missense mutations with severe ND phenotype, seizures, and a manifesting carrier.

    Science.gov (United States)

    Yamada, K; Limprasert, P; Ratanasukon, M; Tengtrisorn, S; Yingchareonpukdee, J; Vasiknanonte, P; Kitaoka, T; Ghadami, M; Niikawa, N; Kishino, T

    2001-04-15

    We describe two Thai families with Norrie disease (ND) in three generations, including 10 affected males and one manifesting female. All affected males in each family had severely defective eye development with complete loss of vision. In addition, three male patients (one from family 1 and two from family 2) suffered from epilepsy, and one female carrier from one family manifested blindness with phthisis bulbi in her right eye. Mutation analysis of the ND gene (NDP) revealed two different novel missense mutations (L16P and S75P) that co-segregated with ND in each family, suggesting that the newly appearing proline at codon 16 or codon 75 alters the conformation of the ND protein and contributes to the severe phenotype of ND in each family. Other studies suggest that epileptic seizures or growth retardation that is associated with ND is the consequence of loss of contiguous genes, because most such patients had deletions extending beyond the Norrie locus. Our finding that the three affected males in the two families with the missense mutations had epilepsy does not support a contiguous gene effect, but favors the pleiotropism of NDP, at least as far as the epileptic manifestation is concerned. The unilateral blindness in the female carrier may have been due to non-random X-inactivation. Copyright 2001 Wiley-Liss, Inc.

  8. Conservation assessment for the Siskiyou Mountains salamander and Scott Bar salamander in northern California.

    Energy Technology Data Exchange (ETDEWEB)

    Vinikour, W. S.; LaGory, K. E.; Adduci, J. J.; Environmental Science Division

    2006-10-20

    The purpose of this conservation assessment is to summarize existing knowledge regarding the biology and ecology of the Siskiyou Mountains salamander and Scott Bar salamander, identify threats to the two species, and identify conservation considerations to aid federal management for persistence of the species. The conservation assessment will serve as the basis for a conservation strategy for the species.

  9. Runoff Variability in the Scott River (SW Spitsbergen in Summer Seasons 2012–2013 in Comparison with the Period 1986–2009

    Directory of Open Access Journals (Sweden)

    Franczak Łukasz

    2016-09-01

    Full Text Available River runoff variability in the Scott River catchment in the summer seasons 2012 and 2013 has been presented in comparison to the multiannual river runoff in 1986–2009. Both in particular seasons and in the analysed multiannual, high variability of discharge rate was recorded. In the research periods 2012–2013, a total of 11 952 water stages and 20 flow rates were measured in the analysed cross-section for the determination of 83 daylong discharges. The mean multiannual discharge of the Scott River amounted to 0.96 m3·s−1. The value corresponds to a specific runoff of 94.6 dm3·s−1·km2, and the runoff layer 937 mm. The maximum values of daily discharge amounted to 5.07 m3·s−1, and the minimum values to 0.002 m3·s−1. The highest runoff occurs in the second and third decade of July, and in the first and second decade of August. The regime of the river is determined by a group of factors, and particularly meteorological conditions affecting the intensity of ablation, and consequently river runoff volume. We found a significant correlation (0.60 in 2012 and 0.67 in 2013 between the air temperature and the Scott River discharge related to the Scott Glacier ice melt.

  10. An assessment of the complications of the Brantley Scott artificial sphincter.

    Science.gov (United States)

    Heathcote, P S; Galloway, N T; Lewis, D C; Stephenson, T P

    1987-08-01

    A Brantley Scott artificial sphincter has been inserted into 95 patients since 1981; more than half of the patients had lower urinary tract neuropathy and most of the others post-TUR incontinence. The main problem with the device has been cuff failure (12), which should be resolved by the new "dipped" cuffs. The major surgical complication has been erosion (10), usually associated with infection. Twenty-four patients had variable degrees of incontinence but the artificial sphincter remains the cornerstone of continence control when other methods have failed or are inappropriate.

  11. Overview of the mutation spectrum in familial exudative vitreoretinopathy and Norrie disease with identification of 21 novel variants in FZD4, LRP5, and NDP.

    Science.gov (United States)

    Nikopoulos, Konstantinos; Venselaar, Hanka; Collin, Rob W J; Riveiro-Alvarez, Rosa; Boonstra, F Nienke; Hooymans, Johanna M M; Mukhopadhyay, Arijit; Shears, Deborah; van Bers, Marleen; de Wijs, Ilse J; van Essen, Anthonie J; Sijmons, Rolf H; Tilanus, Mauk A D; van Nouhuys, C Erik; Ayuso, Carmen; Hoefsloot, Lies H; Cremers, Frans P M

    2010-06-01

    Wnt signaling is a crucial component of the cell machinery orchestrating a series of physiological processes such as cell survival, proliferation, and migration. Among the plethora of roles that Wnt signaling plays, its canonical branch regulates eye organogenesis and angiogenesis. Mutations in the genes encoding the low density lipoprotein receptor protein 5 (LRP5) and frizzled 4 (FZD4), acting as coreceptors for Wnt ligands, cause familial exudative vitreoretinopathy (FEVR). Moreover, mutations in the gene encoding NDP, a ligand for these Wnt receptors, cause Norrie disease and FEVR. Both FEVR and Norrie disease share similar phenotypic characteristics, including abnormal vascularization of the peripheral retina and formation of fibrovascular masses in the eye that can lead to blindness. In this mutation update, we report 21 novel variants for FZD4, LRP5, and NDP, and discuss the putative functional consequences of missense mutations. In addition, we provide a comprehensive overview of all previously published variants in the aforementioned genes and summarize the phenotypic characteristics in mouse models carrying mutations in the orthologous genes. The increasing molecular understanding of Wnt signaling, related to ocular development and blood supply, offers more tools for accurate disease diagnosis that may be important in the development of therapeutic interventions.

  12. "I Have a Dream, Too!": The American Dream in Coretta Scott King Award-Winning Books

    Science.gov (United States)

    Parsons, Linda T.; Castleman, Michele

    2011-01-01

    The Coretta Scott King (CSK) Award, instituted in 1969 and recognized as an official award by the American Library Association (ALA) in 1982, is conferred annually to an African American author and an illustrator for their outstanding contributions to literature about the Black experience for children and young adults. A partial impetus for the…

  13. Mission Specialist Scott Parazynski arrives late at KSC

    Science.gov (United States)

    1998-01-01

    The T-38 jet aircraft arrives at the Shuttle Landing Facility carrying STS-95 Mission Specialist Scott E. Parazynski (second seat). The pilot is astronaut Kent Rominger. Parazynski's first plane experienced problems at the stop at Tyndall AFB and he had to wait for another jet and pilot to finish the flight to KSC. He joined other crewmembers Mission Commander Curtis L. Brown Jr., Pilot Steven W. Lindsey, Mission Specialist Stephen K. Robinson, Payload Specialist John H. Glenn Jr., senator from Ohio, Mission Specialist Pedro Duque, with the European Space Agency (ESA), and Payload Specialist Chiaki Mukai, with the National Space Development Agency of Japan (NASDA), for final pre-launch preparations. STS-95 is expected to launch at 2 p.m. EST on Oct. 29, last 8 days, 21 hours and 49 minutes, and land at 11:49 a.m. EST on Nov. 7.

  14. STS-87 Mission Specialist Scott poses in his launch and entry spacesuit at LC 39B during TCDT

    Science.gov (United States)

    1997-01-01

    STS-87 Mission Specialist Winston Scott poses in his orange launch and entry spacesuit with NASA suit technicians at Launch Pad 39B during Terminal Countdown Demonstration Test (TCDT) activities. The crew of the STS-87 mission is scheduled for launch Nov. 19 aboard the Space Shuttle Columbia. Scott will be performing an extravehicular activity (EVA) spacewalk during the mission. The TCDT is held at KSC prior to each Space Shuttle flight providing the crew of each mission opportunities to participate in simulated countdown activities. The TCDT ends with a mock launch countdown culminating in a simulated main engine cut-off. The crew also spends time undergoing emergency egress training exercises at the pad and has an opportunity to view and inspect the payloads in the orbiter's payload bay.

  15. James Edward Scott: The Leadership Journey of a Senior-Level African American Student Affairs Officer

    Science.gov (United States)

    Willis, Salatha T.

    2013-01-01

    The purpose of this study was to examine, understand, and describe the life, leadership, and influence of Dr. James Edward Scott on higher education and more specifically student affairs; as one of the most well-known and respected African American male chief student affairs officers in the late 20th and early 21st centuries. Using a qualitative…

  16. STS-90 Pilot Scott Altman is suited up for launch

    Science.gov (United States)

    1998-01-01

    STS-90 Pilot Scott Altman is assisted during suit-up activities by Lockheed Suit Technician Valerie McNeil from Johnson Space Center in KSC's Operations and Checkout Building. Altman and the rest of the STS-90 crew will shortly depart for Launch Pad 39B, where the Space Shuttle Columbia awaits a second liftoff attempt at 2:19 p.m. EDT. His first trip into space, Altman is participating in a life sciences research flight that will focus on the most complex and least understood part of the human body - - the nervous system. Neurolab will examine the effects of spaceflight on the brain, spinal cord, peripheral nerves and sensory organs in the human body.

  17. First record of the Calanoid Copepod Pseudodiaptomus serricaudatus (Scott, T. 1894), (Copepoda: Calanoida: Pseudodiaptomidae) in the equatorial Indian ocean.

    Digital Repository Service at National Institute of Oceanography (India)

    Rebello, V.; Narvekar, J.; Gadi, P.; Venenkar, A.; Gauns, M.; PrasannaKumar, S.

    , Pondicherry University, Port Blair, Andaman 3Happy Home Apartment, Near Canara Bank, Fatorda, Margao, Goa-403602 Abstract Pseudodiaptomus serricaudatus (Scott, T. 1894), a planktonic copepod belonging to the family Pseudodiaptomidae, though has...

  18. The Great Kanto earthquake and F. Scott Fitzgerald

    Science.gov (United States)

    Kawakatsu, Hitoshi; Bina, Craig R.

    How many recall the following striking sentence from The Great Gatsby by F. Scott Fitzgerald, which appears on the second page of the novel, where Fitzgerald first introduces Gatsby? “If personality is an unbroken series of successful gestures, then there was something gorgeous about him, some heightened sensitivity to the promises of life, as if he were related to one of those intricate machines that register earthquakes ten thousand miles away.”This line may have failed to focus our attention when we first read the book in our younger days. Now, however, as a Japanese seismologist and an American geophysicist (and student of Japanese culture), we would be greatly remiss for failing to take greater note of this statement. Indeed, as The Great Gatsby was published in 1925, it occurred to us that the earthquake Fitzgerald might have been thinking of was the Great Kanto earthquake, which occurred on September 1, 1923 and devastated the Tokyo metropolitan area.

  19. Hedonism And Materialism As Negative Effects Of Social Changes In American Society Potrayed In The Novel This Side Of Paradise Written By F. Scott Fitzgerald

    OpenAIRE

    Elysia, Irene Nyssa

    2015-01-01

    Judul skripsi ini adalah ‘HEDONISM AND MATERIALISM AS NEGATIVE EFFECTS OF SOCIAL CHANGES IN AMERICAN SOCIETY POTRAYED IN THE NOVEL THIS SIDE OF PARADISE WRITTEN BY F. SCOTT FITZGERALD’. Sesuai dengan judulnya, skripsi ini membahas tentang fenomena hedonisme dan materialisme yang terjadi di Amerika pada awal tahun 1920an, sebagai dampak negatif dari Perang Dunia I. Fenomena ini dapat dibuktikan dari gambaran yang dipaparkan oleh Scott melalui novel ini, yaitu tentang kondisi masyarakat terutam...

  20. Mafic Materials in Scott Crater? A Test for Lunar Reconnaissance Orbiter

    Science.gov (United States)

    Cooper, Bonnie L.

    2007-01-01

    Clementine 750 nm and multispectral ratio data, along with Lunar Orbiter and radar data, were used to study the crater Scott in the lunar south polar region. The multispectral data provide evidence for mafic materials, impact melts, anorthositic materials, and a small pyroclastic deposit. High-resolution radar data and Lunar Orbiter photography for this area show differences in color and surface texture that correspond with the locations of the hypothesized mafic and anorthositic areas on the crater floor. This region provides a test case for the upcoming Lunar Reconnaissance Orbiter. Verification of the existence of a mafic deposit at this location is relevant to future lunar resource utilization planning.

  1. A novel contiguous deletion involving NDP, MAOB and EFHC2 gene in a patient with familial Norrie disease: bilateral blindness and leucocoria without other deficits.

    Science.gov (United States)

    Jia, Bei; Huang, Liping; Chen, Yaoyu; Liu, Siping; Chen, Cuihua; Xiong, Ke; Song, Lanlin; Zhou, Yulai; Yang, Xinping; Zhong, Mei

    2017-12-01

    Contiguous microdeletions of the Norrie disease pseudoglioma (NDP) region on chromosome Xp11.3 have been widely confirmed as contributing to the typical clinical features of Norrie disease (ND). However, the precise relation between genotype and phenotype could vary. The contiguous deletion of NDP and its neighbouring genes, MAOA/B and EFHC2, reportedly leads to syndromic clinical features such as microcephaly, intellectual disability, and epilepsy. Herewe report a novel contiguous microdeletion of the NDP region containing the MAOB and EFHC2 genes,which causes eye defects but no cognitive disability.We detected a deletion of 494.6 kb atXp11.3 in both the proband and carrier mother. This deletionwas then used as the molecular marker in prenatal diagnosis for two subsequent pregnancies. The deletion was absent in one of the foetuses, who remain without any abnormalities at 2 years of age. The proband shows the typical ocular clinical features of ND including bilateral retinal detachment, microphthalmia, atrophic irides, corneal opacification, and cataracts, but no symptoms of microcephaly, intellectual disability, and epilepsy. This familial study demonstrates that a deficiency in one of two MAO genes may not lead to psychomotor delay, and deletion of EFHC2 may not cause epilepsy. Our observations provide new information on the genotype-phenotype relations of MAOA/B and EFHC2 genes involved in the contiguous deletions of ND.

  2. An animal model for Norrie disease (ND): gene targeting of the mouse ND gene.

    Science.gov (United States)

    Berger, W; van de Pol, D; Bächner, D; Oerlemans, F; Winkens, H; Hameister, H; Wieringa, B; Hendriks, W; Ropers, H H

    1996-01-01

    In order to elucidate the cellular and molecular processes which are involved in Norrie disease (ND), we have used gene targeting technology to generate ND mutant mice. The murine homologue of the ND gene was cloned and shown to encode a polypeptide that shares 94% of the amino acid sequence with its human counterpart. RNA in situ hybridization revealed expression in retina, brain and the olfactory bulb and epithelium of 2 week old mice. Hemizygous mice carrying a replacement mutation in exon 2 of the ND gene developed retrolental structures in the vitreous body and showed an overall disorganization of the retinal ganglion cell layer. The outer plexiform layer disappears occasionally, resulting in a juxtaposed inner and outer nuclear layer. At the same regions, the outer segments of the photoreceptor cell layer are no longer present. These ocular findings are consistent with observations in ND patients and the generated mouse line provides a faithful model for study of early pathogenic events in this severe X-linked recessive neurological disorder.

  3. Adapting Scott and Bruce's General Decision-Making Style Inventory to Patient Decision Making in Provider Choice.

    Science.gov (United States)

    Fischer, Sophia; Soyez, Katja; Gurtner, Sebastian

    2015-05-01

    Research testing the concept of decision-making styles in specific contexts such as health care-related choices is missing. Therefore, we examine the contextuality of Scott and Bruce's (1995) General Decision-Making Style Inventory with respect to patient choice situations. Scott and Bruce's scale was adapted for use as a patient decision-making style inventory. In total, 388 German patients who underwent elective joint surgery responded to a questionnaire about their provider choice. Confirmatory factor analyses within 2 independent samples assessed factorial structure, reliability, and validity of the scale. The final 4-dimensional, 13-item patient decision-making style inventory showed satisfactory psychometric properties. Data analyses supported reliability and construct validity. Besides the intuitive, dependent, and avoidant style, a new subdimension, called "comparative" decision-making style, emerged that originated from the rational dimension of the general model. This research provides evidence for the contextuality of decision-making style to specific choice situations. Using a limited set of indicators, this report proposes the patient decision-making style inventory as valid and feasible tool to assess patients' decision propensities. © The Author(s) 2015.

  4. Scott Redford: A New Approach to the Permeability of Political Symbolism in Rum Seljuk Turkey

    Directory of Open Access Journals (Sweden)

    Philip Bockholt

    2017-05-01

    Full Text Available As his work transcends what is seen as iconography, from a strictly art history perspective, the choice of Scott Redford for portrayal in this rubric may seem surprising. However, regarding the applicability of iconographical approaches to the wider domain of cultural studies, precisely his adaptation of art history methods, which integrate disparate source material in a quest for meaning, sparked the interest of this issue of META. For most scholars in the field of Islamic history, researching premodern times normally involves reading narrative sources, that is, chronicles. Despite the so-called "documentary turn" taking place in Mamluk and Ottoman Syria, scholars of the Middle East lack the vast array of archival material that is available to their colleagues working on Medieval Europe. Thus, taking into account other types of material generally neglected by historians might be useful (more in the tradition of archaeologists and art historians who do include material culture in general. This article discusses Scott Redford's approach to combining written sources, epigraphy, and archaeological findings of the Seljuks of Rum in 13th century Anatolia in order to gain more insight into the iconography of power in a remote Islamic past.

  5. Richard J. Hill, Picturing Scotland through the Waverley Novels: Walter Scott and the Origins of the Victorian Illustrated Novel.

    Directory of Open Access Journals (Sweden)

    Jacqueline Irene Cannata

    2012-10-01

    Full Text Available Richard J. Hill, Picturing Scotland through the Waverley Novels: Walter Scott and the Origins of the Victorian Illustrated Novel . Farnham, Surrey, and Burlington, VT: Ashgate, 2010. Pp. 236. ISBN 978-0-7546-6806-0. US$99.99.

  6. Professor John Scott, folate and neural tube defects.

    Science.gov (United States)

    Hoffbrand, A Victor

    2014-02-01

    John Scott (1940-2013) was born in Dublin where he was to spend the rest of his career, both as an undergraduate and subsequently Professor of Biochemistry and Nutrition at Trinity College. His research with the talented group of scientists and clinicians that he led has had a substantial impact on our understanding of folate metabolism, mechanisms of its catabolism and deficiency. His research established the leading theory of folate involvement with vitamin B12 in the pathogenesis of vitamin B12 neuropathy. He helped to establish the normal daily intake of folate and the increased requirements needed either in food or as a supplement before and during pregnancy to prevent neural tube defects. He also suggested a dietary supplement of vitamin B12 before and during pregnancy to reduce the risk of neural tube defects. It would be an appropriate epitaph if fortification of food with folic acid became mandatory in the UK and Ireland, as it is in over 70 other countries. © 2013 John Wiley & Sons Ltd.

  7. No Absolutism Here: Harm Predicts Moral Judgment 30× Better Than Disgust-Commentary on Scott, Inbar, & Rozin (2016).

    Science.gov (United States)

    Gray, Kurt; Schein, Chelsea

    2016-05-01

    Moral absolutism is the idea that people's moral judgments are insensitive to considerations of harm. Scott, Inbar, and Rozin (2016, this issue) claim that most moral opponents to genetically modified organisms are absolutely opposed-motivated by disgust and not harm. Yet there is no evidence for moral absolutism in their data. Perceived risk/harm is the most significant predictor of moral judgments for "absolutists," accounting for 30 times more variance than disgust. Reanalyses suggest that disgust is not even a significant predictor of the moral judgments of absolutists once accounting for perceived harm and anger. Instead of revealing actual moral absolutism, Scott et al. find only empty absolutism: hypothetical, forecasted, self-reported moral absolutism. Strikingly, the moral judgments of so-called absolutists are somewhat more sensitive to consequentialist concerns than those of nonabsolutists. Mediation reanalyses reveal that moral judgments are most proximally predicted by harm and not disgust, consistent with dyadic morality. © The Author(s) 2016.

  8. Identification of missense mutations in the Norrie disease gene associated with advanced retinopathy of prematurity.

    Science.gov (United States)

    Shastry, B S; Pendergast, S D; Hartzer, M K; Liu, X; Trese, M T

    1997-05-01

    Retinopathy of prematurity (ROP) is a retinal vascular disease occurring in infants with short gestational age and low birth weight and can lead to retinal detachment (ROP stages 4 and 5). X-linked familial exudative vitreoretinopathy is phenotypically similar to ROP and has been associated with mutations in the Norrie disease (ND) gene in some cases. To determine if similar mutations in the ND gene may play a role in the development of advanced ROP. Clinical examination and molecular genetic analysis were performed on 16 children, including 2 dizygotic and 1 monozygotic twin pairs, and their parents from 13 families. Sequencing of the amplified products revealed missense mutations (R121W and L108P) in the third exon of the ND gene in 4 patients. These mutations were not present in an unaffected premature twin, 2 children with regressed stage 3 ROP, the parents, or in 50 unrelated healthy control subjects. These findings suggest that mutations in the ND gene may play a role in the development of severe ROP in premature infants.

  9. Linkage analysis of Norrie disease with an X-chromosomal ornithine aminotransferase locus.

    Science.gov (United States)

    Bateman, J B; Kojis, T L; Cantor, R M; Heinzmann, C; Ngo, J T; Spence, M A; Inana, G; Kivlin, J D; Curtis, D; Sparkes, R S

    1993-01-01

    Norrie disease is a rare disease of newborn males caused by prenatal or perinatal retinal detachment, which may be associated with mental retardation, psychosis, and/or hearing loss. DXS7 (L1.28) and MAO A and B loci have been linked to the ND locus on the short arm of the X chromosome. Sequences homologous to OAT also have been mapped to the short arm of the X chromosome. We performed linkage analyses between the ND locus and one of the OAT-like clusters of sequences on the X chromosome (OATL1), using a ScaI RFLP in a ND family, and increased the previously calculated lod score (z) to over 3 (3.38; theta = 0.05). Similarly, we calculated a lod score of 4.06 (theta = 0.01) between the OATL1 and DXS7 loci. Alone, the OATL1 ScaI RFLP system is expected to be informative in 48% of females. If this system were used in combination with the DXS7 TaqI polymorphism, 71% of females would be informative for at least one of the markers and 21% would be informative for both. Because the OATL1 ScaI RFLP is a relatively common polymorphism, this system should be useful for the identification of ND carriers and affected male fetuses and newborns.

  10. Controlling Listeria monocytogenes Scott A on Surfaces of Fully Cooked Turkey Deli Product Using Organic Acid-Containing Marinades as Postlethality Dips.

    Science.gov (United States)

    Casco, Gerardo; Johnson, Jennifer L; Taylor, T Matthew; Gaytán, Carlos N; Brashears, Mindy M; Alvarado, Christine Z

    2015-01-01

    This study evaluated the efficacy of organic acids applied singly or in combination as postlethality dips to sliced uncured turkey deli loaves to inhibit the growth of Listeria monocytogenes (Lm) Scott A. Treatments consisted of sodium lactate (SL; 3.6%), potassium lactate (PL; 3.6%), sodium citrate (SC; 0.75%), a combination of SL and sodium diacetate (SDA; 0.25%), and a combination of SL/PL/SDA, alongside appropriate negative and positive controls. Products were inoculated with 10(4)-10(5) CFU/mL streptomycin-resistant (1500 μg/mL) Lm Scott A prior to treatment. Products were then stored at ~4°C and sampled at 0, 7, 14, 21, 28, 42, and 56 d. The SL/SDA combination applied to turkey slices extended the lag phase through 21 days of refrigerated storage. Numbers of Lm Scott A rose by 0.7 log10 CFU/g through the 56 d storage period. The application of the SL/PL/SDA treatment to turkey product surfaces extended the lag phase through 42 d, with pathogen numbers declining after 21 d. Combination organic acid dips prolonged the lag phase for 2 to 6 wk on turkey product surfaces and can be useful as antimicrobial agents for Lm control on postlethality exposed sliced deli products.

  11. Case 3724 - Metochus abbreviatus Scott, 1874 (Insecta, Heteroptera): proposed precedence over Rhyparochromus erosus Walker, 1872 (currently Metochus erosus)

    Science.gov (United States)

    The purpose of this application, under Article 23.9.3 of the Code, is to conserve the widely used specific name Metochus abbreviatus Scott, 1874, for a species of rhyparochromid bugs from East Asia. The name is threatened by the senior subjective synonym Metochus erosus (Walker, 1872), which has bee...

  12. A phyt osociological classification of the vegetation of the Jack Scott Nature Reserve*

    Directory of Open Access Journals (Sweden)

    B. J. Coetzee

    1974-12-01

    Full Text Available The vegetation of the Jack Scott Nature Reserve in the Central Bankenveld Veld Type is classified chiefly by the Braun-Blanquet Table Method. Habitat features, physiognomy, total floristic composition, differentiating species, woody plants and prominent grasses and forbs are presented for each community. Characterizing habitat features, in order of importance for the communities, are: exposure, soil texture, geology, slope, aspect, degree of rockiness and previous ploughing. The classification correlates well with the major physiographic and climatic variation in the Reserve and generally does not cut across main physiognomic types. The communities are potentially homogeneous management units.

  13. A análise de redes na sociologia e nos estudos sobre grupos econômicos: entrevista com John Scott

    Directory of Open Access Journals (Sweden)

    Rodolfo Palazzo Dias

    2017-07-01

    Full Text Available Com o objetivo de tornar mais acessível ao público brasileiro a área de Análise de Redes Sociais (ARS, Em Tese realizou uma entrevista com Jonh Scott, renomado autor dessa área.

  14. Dynamics of the Davydov–Scott soliton with location or velocity mismatch of its high-frequency component

    Energy Technology Data Exchange (ETDEWEB)

    Blyakhman, L.G.; Gromov, E.M.; Onosova, I.V.; Tyutin, V.V., E-mail: vtyutin@hse.ru

    2017-05-03

    The dynamics of a two-component Davydov–Scott (DS) soliton with a small mismatch of the initial location or velocity of the high-frequency (HF) component was investigated within the framework of the Zakharov-type system of two coupled equations for the HF and low-frequency (LF) fields. In this system, the HF field is described by the linear Schrödinger equation with the potential generated by the LF component varying in time and space. The LF component in this system is described by the Korteweg–de Vries equation with a term of quadratic influence of the HF field on the LF field. The frequency of the DS soliton's component oscillation was found analytically using the balance equation. The perturbed DS soliton was shown to be stable. The analytical results were confirmed by numerical simulations. - Highlights: • The dynamics of the Davydov–Scott soliton with initial location or velocity mismatch of the HF component was investigated. • The study was performed within the framework of coupled linear Schrödinger and KdV equations for the HF and LF fields. • Analytical and numerical approaches were used. • The frequency of the DS soliton component oscillation was found. • Stability of the perturbed DS solitons was demonstrated.

  15. Next-generation sequencing reveals a novel NDP gene mutation in a Chinese family with Norrie disease.

    Science.gov (United States)

    Huang, Xiaoyan; Tian, Mao; Li, Jiankang; Cui, Ling; Li, Min; Zhang, Jianguo

    2017-11-01

    Norrie disease (ND) is a rare X-linked genetic disorder, the main symptoms of which are congenital blindness and white pupils. It has been reported that ND is caused by mutations in the NDP gene. Although many mutations in NDP have been reported, the genetic cause for many patients remains unknown. In this study, the aim is to investigate the genetic defect in a five-generation family with typical symptoms of ND. To identify the causative gene, next-generation sequencing based target capture sequencing was performed. Segregation analysis of the candidate variant was performed in additional family members using Sanger sequencing. We identified a novel missense variant (c.314C>A) located within the NDP gene. The mutation cosegregated within all affected individuals in the family and was not found in unaffected members. By happenstance, in this family, we also detected a known pathogenic variant of retinitis pigmentosa in a healthy individual. c.314C>A mutation of NDP gene is a novel mutation and broadens the genetic spectrum of ND.

  16. Next-generation sequencing reveals a novel NDP gene mutation in a Chinese family with Norrie disease

    Directory of Open Access Journals (Sweden)

    Xiaoyan Huang

    2017-01-01

    Full Text Available Purpose: Norrie disease (ND is a rare X-linked genetic disorder, the main symptoms of which are congenital blindness and white pupils. It has been reported that ND is caused by mutations in the NDP gene. Although many mutations in NDP have been reported, the genetic cause for many patients remains unknown. In this study, the aim is to investigate the genetic defect in a five-generation family with typical symptoms of ND. Methods: To identify the causative gene, next-generation sequencing based target capture sequencing was performed. Segregation analysis of the candidate variant was performed in additional family members using Sanger sequencing. Results: We identified a novel missense variant (c.314C>A located within the NDP gene. The mutation cosegregated within all affected individuals in the family and was not found in unaffected members. By happenstance, in this family, we also detected a known pathogenic variant of retinitis pigmentosa in a healthy individual. Conclusion: c.314C>A mutation of NDP gene is a novel mutation and broadens the genetic spectrum of ND.

  17. Genetic factors in human sleep disorders with special reference to Norrie disease, Prader-Willi syndrome and Moebius syndrome.

    Science.gov (United States)

    Parkes, J D

    1999-06-01

    Sleep-wake problems are common in specific inborn errors of metabolism and structure of the central nervous system. Psychological factors, behavioural difficulties, metabolic disturbances, and widespread rather than focal damage to the nervous system are present in many of these diseases and all influence the sleep-wake cycle. However, a number of conditions cause relatively focal damage to the neuroanatomical substrate of sleeping and waking. These include fatal familial insomnia, with involvement of the prion protein gene on chromosome 20, Norrie disease, the Prader-Willi syndrome and the Moebius syndrome. The last three important conditions, although rare, are considered in detail in this review. They result in sensory deprivation, hypothalamic and mid-brain damage, and involve the X-chromosome, chromosome 15, and chromosome 13, respectively. These conditions cause a wide variety of sleep disturbance, including parasomnias, daytime sleepiness, and a condition like cataplexy. The place of the relevant gene products in normal sleep regulation needs further exploration.

  18. STS-103 Pilot Scott Kelly during TCDT activities

    Science.gov (United States)

    1999-01-01

    STS-103 Pilot Scott J. Kelly is ready to take his turn at driving a small armored personnel carrier that is part of emergency egress training during Terminal Countdown Demonstration Test (TCDT) activities. Behind him (left) is Mission Specialist Jean-Frangois Clervoy of France, who is with the European Space Agency. At right is Mission Specialist Steven L. Smith. The tracked vehicle could be used by the crew in the event of an emergency at the pad during which the crew must make a quick exit from the area. The TCDT also provides simulated countdown exercises and opportunities to inspect the mission payloads in the orbiter's payload bay. STS-103 is a 'call-up' mission due to the need to replace and repair portions of the Hubble Space Telescope. Although Hubble is operating normally and conducting its scientific observations, only three of its six gyroscopes are working properly. Four EVA's are planned to make the necessary repairs and replacements on the telescope. The other STS-103 crew members are Commander Curtis L. Brown Jr. and Mission Specialists C. Michael Foale (Ph.D.), John M. Grunsfeld (Ph.D.), and Claude Nicollier of Switzerland, who also is with the European Space Agency. The mission is targeted for launch Dec. 6 at 2:37 a.m. EST.

  19. International recognition for ageing research: John Scott Award-2014 to Leonard Hayflick and Paul Moorhead

    OpenAIRE

    Rattan, Suresh

    2014-01-01

    It is with great pleasure and pride that we share the news of the award of the 2014 “City of Philadelphia John Scott Award”, to Dr. Leonard Hayflick and Dr. Paul Moorhead, for their research on ageing. The press release announcing the award states that: “from the first awarded in 1822, the Award is the oldest scientific award in the United States and, as a legacy to Benjamin Franklin, they are in the historic company of past winners who include Marie Curie, Thomas Edison, Jonas Salk, Irving L...

  20. Correlation of ophthalmic examination with carrier status in females potentially harboring a severe Norrie disease gene mutation.

    Science.gov (United States)

    Khan, Arif O; Aldahmesh, Mohammed A; Meyer, Brian

    2008-04-01

    To correlate ophthalmic findings with carrier status for a severe Norrie disease (ND) gene mutation (C95F). Prospective interventional case series. Six potential carriers and 1 obligate carrier from a family harboring the mutation. An ophthalmologist blind to the pedigree performed a full ophthalmic examination for the 7 asymptomatic family members. A peripheral blood sample was collected from each for ND gene sequencing. Ophthalmic examination findings (with attention to the presence or absence of retinal findings) and results of ND gene sequencing. Three carriers were identified by molecular genetics, and all 3 of them had peripheral retinal abnormality. However, 3 of the 4 genetically identified noncarriers also exhibited peripheral retinal abnormality. Two of these noncarriers with retinal findings were the offspring of a confirmed noncarrier. The genetically identified noncarrier with a normal peripheral retinal examination was the daughter of an obligate carrier. The presence of peripheral retinal changes was not useful for carrier prediction in a family harboring ND. There are likely additional loci responsible for phenotypic expression.

  1. Pipeline corridors through wetlands -- Impacts on plant communities: Norris Brook Crossing Peabody, Massachusetts

    Energy Technology Data Exchange (ETDEWEB)

    Shem, L.M.; Van Dyke, G.D.; Zimmerman, R.E. [Argonne National Lab., IL (United States)

    1994-12-01

    The goal of the Gas Research Institute Wetland Corridors Program is to document impacts of existing pipelines on the wetlands they traverse. To accomplish this goal, 12 existing wetland crossings were surveyed. These sites varied in elapsed time since pipeline construction, wetland type, pipeline installation techniques, and right-of-way (ROW) management practices. This report presents the results of a survey conducted August 17--19, 1992, at the Norris Brook crossing in the town of Peabody, Essex County, Massachusetts. The pipeline at this site was installed during September and October 1990. A backhoe was used to install the pipeline. The pipe was assembled on the adjacent upland and slid into the trench, after which the backhoe was used again to fill the trench and cover the pipeline. Within two years after pipeline construction, a dense vegetative community, composed predominantly of native perennial species, had become established on the ROW. Compared with adjacent natural areas undisturbed by pipeline installation, there was an increase in purple loosestrife and cattail within the ROW, while large woody species were excluded from the ROW. As a result of the ROW`s presence, habitat diversity, edge-type habitat, and species diversity increased within the site. Crooked-stem aster, Aster prenanthoides (a species on the Massasschusetts list of plants of special concern), occurred in low numbers in the adjacent natural areas and had reinvaded the ROW in low numbers.

  2. Scotts Valley Energy Office and Human Capacity Building that will provide energy-efficiency services and develop sustainable renewable energy projects.

    Energy Technology Data Exchange (ETDEWEB)

    Anderson, Temashio [Scotts Valley Band of Pomo Indians

    2013-06-28

    The primary goal of this project is to develop a Scotts Valley Energy Development Office (SVEDO). This office will further support the mission of the Tribe's existing leadership position as the DOE Tribal Multi-County Weatherization Energy Program (TMCWEP) in creating jobs and providing tribal homes and buildings with weatherization assistance to increase energy efficiency, occupant comfort and improved indoor air quality. This office will also spearhead efforts to move the Tribe towards its further strategic energy goals of implementing renewable energy systems through specific training, resource evaluation, feasibility planning, and implementation. Human capacity building and continuing operations are two key elements of the SVEDO objectives. Therefore, the project will 1) train and employ additional Tribal members in energy efficiency, conservation and renewable resource analyses and implementation; 2) purchase materials and equipment required to implement the strategic priorities as developed by the Scotts Valley Tribe which specifically include implementing energy conservation measures and alternative energy strategies to reduce energy costs for the Tribe and its members; and 3) obtain a dedicated office and storage space for ongoing SVEDO operations.

  3. Overproduction and partial purification of the Norrie disease gene product, norrin, from a recombinant baculovirus.

    Science.gov (United States)

    Shastry, Barkur S; Trese, Michael T

    2003-12-05

    Abnormal vascularization of the peripheral retina and retinal detachment are common clinical characteristics of Norrie disease (ND), familial exudative vitreoretinopathy, Coats' disease, and retinopathy of prematurity. Although little is known about the molecular basis of these diseases, studies have shown that all of these diseases are associated with mutations in the ND gene. In spite of this, little is known about norrin, its molecular mechanism of action, and its functional relationship with the development of abnormal retinal vasculature. To obtain a large quantity of norrin for structural and functional studies, we have overproduced it in insect cells. For this purpose, a cDNA fragment (869 bp) was isolated from a human retinal cDNA library by amplification and was cloned into an expression vector. The purified plasmid was co-transfected with wild-type linearized Bac-N-Blue DNA into S. frugiperda Sf21 insect cells. The recombinant virus plaques were purified and clones were selected based on the level of recombinant protein expressed in Sf21 cells infected with a purified recombinant virus. From these, a high-titer stock was generated and subsequently used to prepare a fused protein on a large scale. The protein was partially purified by the process of immobilized metal affinity chromatography and the use of ion exchange chromatography

  4. Aurora 7 the Mercury space flight of M. Scott Carpenter

    CERN Document Server

    Burgess, Colin

    2016-01-01

    TO A NATION enthralled by the heroic exploits of the Mercury astronauts, the launch of Lt. Cmdr. Scott Carpenter on NASA’s second orbital space flight was a renewed cause for pride, jubilation and celebration. Within hours, that excitement had given way to stunned disbelief and anxiety as shaken broadcasters began preparing the American public for the very real possibility that an American astronaut and his spacecraft may have been lost at sea. In fact, it had been a very close call. Completely out of fuel and forced to manually guide Aurora 7 through the frightening inferno of re-entry, Carpenter brought the Mercury spacecraft down to a safe splashdown in the ocean. In doing so, he controversially overshot the intended landing zone. Despite his efforts, Carpenter’s performance on the MA-7 mission was later derided by powerful figures within NASA. He would never fly into space again. Taking temporary leave of NASA, Carpenter participated in the U.S. Navy’s pioneering Sealab program. For a record 30 days...

  5. Scott Correction for Large Atoms and Molecules in a Self-Generated Magnetic Field

    DEFF Research Database (Denmark)

    Erdös, Laszlo; Fournais, Søren; Solovej, Jan Philip

    2012-01-01

    constant. We show that, in the simultaneous limit $Z\\to\\infty$, $\\al\\to 0$ such that $\\kappa =Z\\al^2$ is fixed, the ground state energy of the system is given by a two term expansion $c_1Z^{7/3} + c_2(\\kappa) Z^2 + o(Z^2)$. The leading term is given by the non-magnetic Thomas-Fermi theory. Our result shows......We consider a large neutral molecule with total nuclear charge $Z$ in non-relativistic quantum mechanics with a self-generated classical electromagnetic field. To ensure stability, we assume that $Z\\al^2\\le \\kappa_0$ for a sufficiently small $\\kappa_0$, where $\\al$ denotes the fine structure...... that the magnetic field affects only the second (so-called Scott) term in the expansion....

  6. Further developments of the Neyman-Scott clustered point process for modeling rainfall

    Science.gov (United States)

    Cowpertwait, Paul S. P.

    1991-07-01

    This paper provides some useful results for modeling rainfall. It extends work on the Neyman-Scott cluster model for simulating rainfall time series. Several important properties have previously been found for the model, for example, the expectation and variance of the amount of rain captured in an arbitrary time interval (Rodriguez-Iturbe et al., 1987a), In this paper additional properties are derived, such as the probability of an arbitrary interval of any chosen length being dry. In applications this is a desirable property to have, and is often used for fitting stochastic rainfall models to field data. The model is currently being used in rainfall time series research directed toward improving sewage systems in the United Kingdom. To illustrate the model's performance an example is given, where the model is fitted to 10 years of hourly data taken from Blackpool, England.

  7. Harnessing the Power of Emotion for Social Change: Review of Numbers and Nerves: Information, Emotion, and Meaning in a World of Data by Scott Slovic and Paul Slovic (2015

    Directory of Open Access Journals (Sweden)

    Anne M.W. Kelly

    2017-01-01

    Full Text Available Scott Slovic and Paul Slovic (Eds.. Numbers and Nerves: Information, Emotion, and Meaning in a World of Data (Corvallis, OR: Oregon State University Press, 2015. 272 pp. ISBN 978-0-87071-776-5. Literature and environment professor Scott Slovic, and his father, psychologist Paul Slovic, editors of this collection of essays and interviews, describe and demonstrate the psychological effects which hamper our ability to comprehend and respond appropriately to large numerical data. The collection then offers a brief survey of art works which, by first appealing to viewers’ emotions, can potentially move the viewer to a better understanding of numbers.

  8. Landscapes of The Mind: The Setting as Psyche in Nathaniel Hawthorne's The Scarlet Letter And F. Scott Fitzgerald's The Great Gatsby Landscapes of The Mind: The Setting as Psyche in Nathaniel Hawthorne's The Scarlet Letter And F. Scott Fitzgerald's The Great Gatsby

    Directory of Open Access Journals (Sweden)

    Cruce Stark

    2009-09-01

    Full Text Available In the closing moment of F. Scott Fitzgerald's The Great Gateby, Nick returns for one last look at what once was Gateby's house. But instead of physical buildings, he has a vision of an earlier time, a vision of the "old island" that "flowered once for Dutch sailor's eyes -- a fresh green breast of the new world." "For a transitory enchanted moment," Nick thinks, "man must have held his breath in the presence of this continent, compelled into an aesthetic contemplation he neither understood nor desired, face to face for the last time in history with something commensurate to his capacity for wonder (182." In the closing moment of F. Scott Fitzgerald's The Great Gateby, Nick returns for one last look at what once was Gateby's house. But instead of physical buildings, he has a vision of an earlier time, a vision of the "old island" that "flowered once for Dutch sailor's eyes -- a fresh green breast of the new world." "For a transitory enchanted moment," Nick thinks, "man must have held his breath in the presence of this continent, compelled into an aesthetic contemplation he neither understood nor desired, face to face for the last time in history with something commensurate to his capacity for wonder (182."

  9. A novel missense mutation in the NDP gene in a child with Norrie disease and severe neurological involvement including infantile spasms.

    Science.gov (United States)

    Lev, Dorit; Weigl, Yuval; Hasan, Mariana; Gak, Eva; Davidovich, Michael; Vinkler, Chana; Leshinsky-Silver, Esther; Lerman-Sagie, Tally; Watemberg, Nathan

    2007-05-01

    Norrie disease (ND) is a rare X-linked recessive disorder characterized by congenital blindness and in some cases, mental retardation and deafness. Other neurological complications, particularly epilepsy, are rare. We report on a novel mutation identified in a patient with ND and profound mental retardation. The patient was diagnosed at the age of 6 months due to congenital blindness. At the age of 8 months he developed infantile spasms, which were diagnosed at 11 months as his EEG demonstrated hypsarrhythmia. Mutation analysis of the ND gene (NDP) of the affected child and his mother revealed a novel missense mutation at position c.134T > A resulting in amino acid change at codon V45E. To the best of our knowledge, such severe neurological involvement has not been previously reported in ND patients. The severity of the phenotype may suggest the functional importance of this site of the NDP gene.

  10. Expression of the Norrie disease gene (Ndp) in developing and adult mouse eye, ear, and brain.

    Science.gov (United States)

    Ye, Xin; Smallwood, Philip; Nathans, Jeremy

    2011-01-01

    The Norrie disease gene (Ndp) codes for a secreted protein, Norrin, that activates canonical Wnt signaling by binding to its receptor, Frizzled-4. This signaling system is required for normal vascular development in the retina and for vascular survival in the cochlea. In mammals, the pattern of Ndp expression beyond the retina is poorly defined due to the low abundance of Norrin mRNA and protein. Here, we characterize Ndp expression during mouse development by studying a knock-in mouse that carries the coding sequence of human placental alkaline phosphatase (AP) inserted at the Ndp locus (Ndp(AP)). In the CNS, Ndp(AP) expression is apparent by E10.5 and is dynamic and complex. The anatomically delimited regions of Ndp(AP) expression observed prenatally in the CNS are replaced postnatally by widespread expression in astrocytes in the forebrain and midbrain, Bergman glia in the cerebellum, and Müller glia in the retina. In the developing and adult cochlea, Ndp(AP) expression is closely associated with two densely vascularized regions, the stria vascularis and a capillary plexus between the organ of Corti and the spiral ganglion. These observations suggest the possibility that Norrin may have developmental and/or homeostatic functions beyond the retina and cochlea. Copyright © 2010 Elsevier B.V. All rights reserved.

  11. Environmental Assessment of the Gering-Stegall 115-kV Transmission Line Consolidation Project, Scotts Bluff County, Nebraska

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1995-05-01

    The Department of Energy (DOE), Western Area Power Administration (Western) proposes to consolidate segments of two transmission lines near the Gering Substation in Gering, Nebraska. The transmission lines are both located in Scotts Bluff County, Nebraska. The transmission lines are both located in Scotts Bluff County, Nebraska, within the city of Gering. Presently, there are three parallel 115-kilovolt (kV) transmission lines on separate rights-of-way (ROW) that terminate at the Gering Substation. The project would include dismantling the Archer-Gering wood-pole transmission line and rebuilding the remaining two lines on single-pole steel double circuit structures. The project would consolidate the Gering-Stegall North and Gering-Stegall South 115-kV transmission lines on to one ROW for a 1.33-mile segment between the Gering Substation and a point west of the Gering Landfill. All existing wood-pole H-frame structures would be removed, and the Gering-Stegall North and South ROWs abandoned. Western is responsible for the design, construction, operation, and maintenance of the line. Western prepared an environmental assessment (EA) that analyzed the potential environmental impacts of the proposed construction, operation, and maintenance of the 115-kV transmission line consolidation. Based on the analyses in the EA, the DOE finds that the proposed action is not a major Federal action significantly affecting the quality of the human environment, within the meaning of the National Environmental Policy Act of 1969 (NEPA).

  12. Environmental Assessment of the Gering-Stegall 115-kV Transmission Line Consolidation Project, Scotts Bluff County, Nebraska

    International Nuclear Information System (INIS)

    1995-05-01

    The Department of Energy (DOE), Western Area Power Administration (Western) proposes to consolidate segments of two transmission lines near the Gering Substation in Gering, Nebraska. The transmission lines are both located in Scotts Bluff County, Nebraska. The transmission lines are both located in Scotts Bluff County, Nebraska, within the city of Gering. Presently, there are three parallel 115-kilovolt (kV) transmission lines on separate rights-of-way (ROW) that terminate at the Gering Substation. The project would include dismantling the Archer-Gering wood-pole transmission line and rebuilding the remaining two lines on single-pole steel double circuit structures. The project would consolidate the Gering-Stegall North and Gering-Stegall South 115-kV transmission lines on to one ROW for a 1.33-mile segment between the Gering Substation and a point west of the Gering Landfill. All existing wood-pole H-frame structures would be removed, and the Gering-Stegall North and South ROWs abandoned. Western is responsible for the design, construction, operation, and maintenance of the line. Western prepared an environmental assessment (EA) that analyzed the potential environmental impacts of the proposed construction, operation, and maintenance of the 115-kV transmission line consolidation. Based on the analyses in the EA, the DOE finds that the proposed action is not a major Federal action significantly affecting the quality of the human environment, within the meaning of the National Environmental Policy Act of 1969 (NEPA)

  13. Identification of genes involved in Ca2+ ionophore A23187-mediated apoptosis and demonstration of a high susceptibility for transcriptional repression of cell cycle genes in B lymphoblasts from a patient with Scott syndrome

    Directory of Open Access Journals (Sweden)

    Meyer Dominique

    2005-10-01

    Full Text Available Abstract Background In contrast to other agents able to induce apoptosis of cultured cells, Ca2+ ionophore A23187 was shown to elicit direct activation of intracellular signal(s. The phenotype of the cells derived from patients having the hemorrhagic disease Scott syndrome, is associated with an abnormally high proportion of apoptotic cells, both in basal culture medium and upon addition of low ionophore concentrations in long-term cultures. These features are presumably related to the mutation also responsible for the defective procoagulant plasma membrane remodeling. We analyzed the specific transcriptional re-programming induced by A23187 to get insights into the effect of this agent on gene expression and a defective gene regulation in Scott cells. Results The changes in gene expression upon 48 hours treatment with 200 nM A23187 were measured in Scott B lymphoblasts compared to B lymphoblasts derived from the patient's daughter or unrelated individuals using Affymetrix microarrays. In a similar manner in all of the B cell lines, results showed up-regulation of 55 genes, out of 12,000 represented sequences, involved in various pathways of the cell metabolism. In contrast, a group of 54 down-regulated genes, coding for histones and proteins involved in the cell cycle progression, was more significantly repressed in Scott B lymphoblasts than in the other cell lines. These data correlated with the alterations of the cell cycle phases in treated cells and suggested that the potent effect of A23187 in Scott B lymphoblasts may be the consequence of the underlying molecular defect. Conclusion The data illustrate that the ionophore A23187 exerts its pro-apoptotic effect by promoting a complex pattern of genetic changes. These results also suggest that a subset of genes participating in various steps of the cell cycle progress can be transcriptionally regulated in a coordinated fashion. Furthermore, this research brings a new insight into the defect

  14. Scott y Zelda Fitzgerald y el psicoanálisis: la construcción de "Suave es la noche"

    OpenAIRE

    Esteve Díaz, Nuria

    2016-01-01

    Esta tesis aporta una investigación original que pretende analizar la novela Suave es la noche (Tender is the Night, 1934) del escritor norteamericano Francis Scott Key Fitzgerald desde la perspectiva de las humanidades médicas y de manera particular, de las relaciones entre medicina y literatura. En Suave es la noche existen amplias referencias médicas y conceptos psicoanalíticos relacionados con la psiquiatría europea de finales del siglo XIX y comienzos del XX, por lo que cobra especial in...

  15. Features of ozone intraannual variability in polar regions based on ozone sounding data obtained at the Resolute and Amundsen-Scott stations

    Energy Technology Data Exchange (ETDEWEB)

    Gruzdev, A.N.; Sitnov, S.A. (AN SSSR, Institut Fiziki Atmosfery, Moscow (USSR))

    1991-04-01

    Ozone sounding data obtained at the Resolute and Amundsen-Scott stations are used to analyze ozone intraannual variability in Southern and Northern polar regions. For the Arctic, in particular, features associated with winter stratospheric warmings, stratospheric-tropospheric exchange, and the isolated evolution of surface ozone are noted. Correlative connections between ozone and temperature making it possible to concretize ozone variability mechanisms are analyzed. 31 refs.

  16. Phenotypic heterogeneity associated with a novel mutation (Gly112Glu) in the Norrie disease protein.

    Science.gov (United States)

    Allen, R C; Russell, S R; Streb, L M; Alsheikheh, A; Stone, E M

    2006-02-01

    To determine the molecular pathology and clinical severity of two pedigrees with a history of early retinal detachment and peripheral retinal vascular abnormalities. Longitudinal cohort study. A longitudinal clinical study and DNA analysis was performed on 49 family members of two pedigrees. Nine individuals were found to be hemizygous for a mutation at codon 112 (Gly112Glu) of the Norrie disease protein (NDP) in one pedigree. Significant phenotypic heterogeneity was found. The proband presented with a unilateral subtotal retinal detachment at the age of 3 years, and subsequently developed a slowly progressive tractional retinal detachment involving the macula in the contralateral eye at the age of 4 years. One individual had only mild peripheral retinal pigmentary changes with normal vision at the age of 79 years. The remaining seven individuals had varying degrees of peripheral retinal vascular abnormalities and anterior segment findings. Seven affected members of a second pedigree affected by a previously reported mutation, Arg74Cys, also demonstrated wide ocular phenotypic variation. A novel mutation (Gly112Glu), which represents the most carboxy located, NDP mutation reported, results in significant phenotypic heterogeneity. These data support the contention that the spectrum of ocular disease severity associated with these NDP mutations is broad. Use of terms that characterize this entity by phenotypic appearance, such as familial exudative vitreoretinopathy, do not adequately communicate the potential spectrum of severity of this disorder to affected or carrier family members.

  17. The Third Turn toward the Social: Nancy Welch's "Living Room," Tony Scott's "Dangerous Writing," and Rhetoric and Composition's Turn toward Grassroots Political Activism

    Science.gov (United States)

    Kinney, Kelly; Girshin, Thomas; Bowlin, Barrett

    2013-01-01

    This review essay examines recent texts by Nancy Welch and Tony Scott, both of which use embodied activism as a starting point for their inquiries. Taken together, these works point to a distinct shift in composition studies' turn toward the social, one that calls on workers both within and outside the academy to actively engage in grassroots…

  18. ROBERT VENTURI, DENISE SCOTT BROWN Y STEVEN IZENOUR: LEARNING FROM LAS VEGAS

    Directory of Open Access Journals (Sweden)

    Ignacio Senra Fernández-Miranda

    2013-05-01

    Full Text Available RESUMEN Poco tiene que ver la edición original de 1972 de Learning from las Vegas, the forgotten symbolism of the architectural form, con la edición revisada de 1977, que fue traducida al español y publicada por Gustavo Gili en 1978. El gran formato del libro original (38x28cm se justificaba por la importancia del material gráfico desplegado, un ejercicio de análisis que aspiraba a descubrir nuevas técnicas de representación capaces de reproducir una realidad tan compleja sensorialmente como la de la ciudad de las Vegas. La drástica transformación, impulsada por los propios autores, suponía un abaratamiento y por tanto una mayor difusión del libro, pero sobretodo trataba de acabar con el conflicto entre su crítica al diseño Bauhaus y el diseño Bauhaus tardío del libro original, como señaló la propia Denise Scott Brown en el prologo de la edición revisada de 1977.

  19. Possible Mafic Patches in Scott Crater Highlight the Need for Resource Exploration on the Lunar South Polar Region

    Science.gov (United States)

    Cooper, Bonnie L.

    2007-01-01

    Possible areas of mafic material on the rim and floor of Scott crater (82.1 deg S, 48.5 deg E) are suggested by analysis of shadow-masked Clementine false-color-ratio images. Mafic materials common in mare and pyroclastic materials can produce more oxygen than can highlands materials, and mafic materials close to the south pole may be important for propellant production for a future lunar mission. If the dark patches are confirmed as mafic materials, this finding would suggest that other mafic patches may exist, even closer to the poles, which were originally mapped as purely anorthositic.

  20. Mutation spectrum of the Norrie disease pseudoglioma (NDP) gene in Indian patients with FEVR.

    Science.gov (United States)

    Musada, Ganeswara Rao; Jalali, Subhadra; Hussain, Anjli; Chururu, Anupama Reddy; Gaddam, Pramod Reddy; Chakrabarti, Subhabrata; Kaur, Inderjeet

    2016-01-01

    Mutations in the Norrie disease pseudoglioma (NDP; Xp11.3) gene have been involved in retinal blood vessel formation and neural differentiation and are implicated in familial exudative vitreoretinopathy (FEVR) cases. However, the role of the gene has not been explored in the Indian context. Thus, this study was designed to understand the involvement of NDP among Indian patients with FEVR. The study cohort comprised 225 subjects, including unrelated patients with FEVR (n = 110) and ethnically matched healthy subjects (n = 115) recruited from a tertiary eye care center in India. The entire coding regions, intron-exon boundaries, along with the 5' and 3' untranslated regions of NDP were screened with resequencing following standard protocols. The spectrum of the observed variants was analyzed in conjunction with data available from other populations. Eight potentially pathogenic mutations (p.His4ArgfsX21, p.Asp23GlufsX9, p.Ile48ValfsX55, p.His50Asp, p.Ser57*, p.Gly113Asp, p.Arg121Gln, and p.Cys126Arg, including five novel ones), were observed in the coding region of the NDP gene in ten unrelated FEVR probands (9%). The novel changes were not observed in the control subjects and were unavailable in the dbSNP, ESP5400, NIEHS95, and ExAC databases. All probands with NDP mutations exhibited classical features of the disease as observed among patients with FEVR worldwide. This is perhaps the first study to demonstrate the involvement of NDP among patients with Indian FEVR that further expands its mutation spectrum. The data generated could have broad implications in genetic counseling, disease management, and early intervention for a better prognosis in FEVR.

  1. La diaspora americana in Europa: il caso degli espatriati in Tender is the Night di F. Scott Fitzgerald

    Directory of Open Access Journals (Sweden)

    Elisa A. Pantaleo

    2015-11-01

    Full Text Available From 1921 to 1930, F. Scott Fitzgerald travelled to Europe four times, and he spent almost four years in France and one year in Switzerland. While living abroad in the multicultural environment of Paris and of the French Riviera, his attitude towards Europe underwent a major change. Tender is the Night marks a significant transition from the narrow nationalism of Fitzgerald’s first travel correspondence to an increased sensitivity towards European otherness. The cultural encounter with Europe – that in the novel is rendered through an hybridization of the language and the characters –helped the author to reinterpret his identity in a cosmopolitan perspective.

  2. Robinson Rancheria Strategic Energy Plan; Middletown Rancheria Strategic Energy Plan, Scotts Valley Rancheria Strategic Energy Plan, Elem Indian Colony Strategic Energy Plan, Upperlake Rancheria Strategic Energy Plan, Big Valley Rancheria Strategic Energy Plan

    Energy Technology Data Exchange (ETDEWEB)

    McGinnis and Associates LLC

    2008-08-01

    The Scotts Valley Band of Pomo Indians is located in Lake County in Northern California. Similar to the other five federally recognized Indian Tribes in Lake County participating in this project, Scotts Valley Band of Pomo Indians members are challenged by generally increasing energy costs and undeveloped local energy resources. Currently, Tribal decision makers lack sufficient information to make informed decisions about potential renewable energy resources. To meet this challenge efficiently, the Tribes have committed to the Lake County Tribal Energy Program, a multi Tribal program to be based at the Robinson Rancheria and including The Elem Indian Colony, Big Valley Rancheria, Middletown Rancheria, Habematolel Pomo of Upper Lake and the Scotts Valley Pomo Tribe. The mission of this program is to promote Tribal energy efficiency and create employment opportunities and economic opportunities on Tribal Lands through energy resource and energy efficiency development. This program will establish a comprehensive energy strategic plan for the Tribes based on Tribal specific plans that capture economic and environmental benefits while continuing to respect Tribal cultural practices and traditions. The goal is to understand current and future energy consumption and develop both regional and Tribe specific strategic energy plans, including action plans, to clearly identify the energy options for each Tribe.

  3. Mutation spectrum of the Norrie disease pseudoglioma (NDP) gene in Indian patients with FEVR

    Science.gov (United States)

    Musada, Ganeswara Rao; Jalali, Subhadra; Hussain, Anjli; Chururu, Anupama Reddy; Gaddam, Pramod Reddy; Chakrabarti, Subhabrata

    2016-01-01

    Purpose Mutations in the Norrie disease pseudoglioma (NDP; Xp11.3) gene have been involved in retinal blood vessel formation and neural differentiation and are implicated in familial exudative vitreoretinopathy (FEVR) cases. However, the role of the gene has not been explored in the Indian context. Thus, this study was designed to understand the involvement of NDP among Indian patients with FEVR. Methods The study cohort comprised 225 subjects, including unrelated patients with FEVR (n = 110) and ethnically matched healthy subjects (n = 115) recruited from a tertiary eye care center in India. The entire coding regions, intron–exon boundaries, along with the 5′ and 3′ untranslated regions of NDP were screened with resequencing following standard protocols. The spectrum of the observed variants was analyzed in conjunction with data available from other populations. Results Eight potentially pathogenic mutations (p.His4ArgfsX21, p.Asp23GlufsX9, p.Ile48ValfsX55, p.His50Asp, p.Ser57*, p.Gly113Asp, p.Arg121Gln, and p.Cys126Arg, including five novel ones), were observed in the coding region of the NDP gene in ten unrelated FEVR probands (9%). The novel changes were not observed in the control subjects and were unavailable in the dbSNP, ESP5400, NIEHS95, and ExAC databases. All probands with NDP mutations exhibited classical features of the disease as observed among patients with FEVR worldwide. Conclusions This is perhaps the first study to demonstrate the involvement of NDP among patients with Indian FEVR that further expands its mutation spectrum. The data generated could have broad implications in genetic counseling, disease management, and early intervention for a better prognosis in FEVR. PMID:27217716

  4. Absence of a Scott correction for the total binding energy of noninteracting fermions in a smooth potential well

    International Nuclear Information System (INIS)

    Huxtable, B.D.

    1988-01-01

    It is shown, for V in a particular class of smooth functions, that the total binding energy, E(Z), of Z noninteracting Fermions in the potential well Z 4/3 V(Z 1/3 X) obeys E(Z) = c TF (V)Z 7/3 + O(Z 5/3 ) as Z → ∞. Here c TF (V) is the coefficient predicted by Thomas-Fermi theory. This result is consistent with the conjectured Scott correction, which occurs at order Z 2 , to the total binding energy of an atomic number Z. This correction is thought to arise only because V(x)∼ - |x| -1 near x = 0 in the atomic problem, and so V is not a smooth function

  5. Contiguous deletion of the NDP, MAOA, MAOB, and EFHC2 genes in a patient with Norrie disease, severe psychomotor retardation and myoclonic epilepsy.

    Science.gov (United States)

    Rodriguez-Revenga, L; Madrigal, I; Alkhalidi, L S; Armengol, L; González, E; Badenas, C; Estivill, X; Milà, M

    2007-05-01

    Norrie disease (ND) is an X-linked disorder, inherited as a recessive trait that, therefore, mostly affects males. The gene responsible for ND, called NDP, maps to the short arm of chromosome X (Xp11.4-p11.3). We report here an atypical case of ND, consisting of a patient harboring a large submicroscopic deletion affecting not only the NDP gene but also the MAOA, MAOB, and EFHC2 genes. Microarray comparative genomic hybridization (CGH) analysis showed that 11 consecutive bacterial artificial chromosome (BAC) clones, mapping around the NDP gene, were deleted. These clones span a region of about 1 Mb on Xp11.3. The deletion was ascertained by fluorescent in situ hybridization (FISH) analysis with different BAC clones located within the region. Clinical features of the proband include bilateral retinal detachment, microcephaly, severe psychomotor retardation without verbal language skills acquired, and epilepsy. The identification and molecular characterization of this case reinforces the idea of a new contiguous gene syndrome that would explain the complex phenotype shared by atypical ND patients.

  6. True Grit: In Tracking down the Real Story of a Legendary Hero of the Old West, Vaunda Micheaux Nelson also Nabbed the Coretta Scott King Award

    Science.gov (United States)

    Fleishhacker, Joy

    2010-01-01

    When Vaunda Micheaux Nelson donned a black Stetson to become the biographer of Deputy U.S. Marshal Bass Reeves, she had no idea that her square-shooting book about an unsung African-American hero of the Old West would win over a posse of fans and earn her the prestigious 2010 Coretta Scott King (CSK) Author Award. "Bad News for Outlaws"…

  7. Deviations of the lepton mapping matrix form the harrison-perkins-scott form

    International Nuclear Information System (INIS)

    Friedberg, R.; Lee, T.D.

    2010-01-01

    We propose a simple set of hypotheses governing the deviations of the leptonic mapping matrix from the Harrison-Perkins-Scott (HPS) form. These deviations are supposed to arise entirely from a perturbation of the mass matrix in the charged lepton sector. The perturbing matrix is assumed to be purely imaginary (thus maximally T-violating) and to have a strength in energy scale no greater (but perhaps smaller) than the muon mass. As we shall show,it then follows that the absolute value of the mapping matrix elements pertaining to the tau lepton deviate by no more than O((m μ /m τ ) 2 ) ≅ 3.5 x 10 -3 from their HPS values. Assuming that(m μ /m τ ) 2 can be neglected, we derive two simple constraints on the four parameters θ12, θ23, θ31, and δ of the mapping matrix. These constraints are independent of the details of the imaginary T-violating perturbation of the charged lepton mass matrix. We also show that the e and μ parts of the mapping matrix have a definite form governed by two parameters α and β; any deviation of order m μ /m τ can be accommodated by adjusting these two parameters. (authors)

  8. Global gene expression analysis in a mouse model for Norrie disease: late involvement of photoreceptor cells.

    Science.gov (United States)

    Lenzner, Steffen; Prietz, Sandra; Feil, Silke; Nuber, Ulrike A; Ropers, H-Hilger; Berger, Wolfgang

    2002-09-01

    Mutations in the NDP gene give rise to a variety of eye diseases, including classic Norrie disease (ND), X-linked exudative vitreoretinopathy (EVRX), retinal telangiectasis (Coats disease), and advanced retinopathy of prematurity (ROP). The gene product is a cystine-knot-containing extracellular signaling molecule of unknown function. In the current study, gene expression was determined in a mouse model of ND, to unravel disease-associated mechanisms at the molecular level. Gene transcription in the eyes of 2-year-old Ndp knockout mice was compared with that in the eyes of age-matched wild-type control animals, by means of cDNA subtraction and microarrays. Clones (n = 3072) from the cDNA subtraction libraries were spotted onto glass slides and hybridized with fluorescently labeled RNA-derived targets. More than 230 differentially expressed clones were sequenced, and their expression patterns were verified by virtual Northern blot analysis. Numerous gene transcripts that are absent or downregulated in the eye of Ndp knockout mice are photoreceptor cell specific. In younger Ndp knockout mice (up to 1 year old), however, all these transcripts were found to be expressed at normal levels. The identification of numerous photoreceptor cell-specific transcripts with a reduced expression in 2-year-old, but not in young, Ndp knockout mice indicates that normal gene expression in these light-sensitive cells of mutant mice is established and maintained over a long period and that rods and cones are affected relatively late in the mouse model of ND. Obviously, the absence of the Ndp gene product is not compatible with long-term survival of photoreceptor cells in the mouse.

  9. Novel and recurrent NDP gene mutations in familial cases of Norrie disease and X-linked exudative vitreoretinopathy.

    Science.gov (United States)

    Pelcastre, Erika L; Villanueva-Mendoza, Cristina; Zenteno, Juan C

    2010-05-01

    To present the results of molecular analysis of the NDP gene in Mexican families with Norrie disease (ND) and X-linked familial exudative vitreoretinopathy (XL-FEVR). Two unrelated families with ND and two with XL-FEVR were studied. Clinical diagnosis was suspected on the basis of a complete ophthalmologic examination. Molecular methods included DNA isolation from peripheral blood leucocytes, polymerase chain reaction amplification and direct nucleotide sequencing analysis of the complete coding region and exon-intron junctions of NDP. Haplotype analysis using NDP-linked microsatellites markers was performed in both ND families. A novel Norrin missense mutation, p.Arg41Thr, was identified in two apparently unrelated families with ND. Haplotype analysis demonstrated that affected males in these two families shared the same ND-linked haplotype, suggesting a common origin for this novel mutation. The previously reported p.Arg121Trp and p.Arg121Gln Norrin mutations were identified in the two families with XL-FEVR. Our results expand the mutational spectrum in ND. This is the first report of ND resulting from mutation at arginine position 41 of Norrin. Interestingly, mutations at the same residue but resulting in a different missense change were previously described in subjects with XL-FEVR (p.Arg41Lys) or persistent fetal vasculature syndrome (p.Arg41Ser), indicating that the novel p.Arg41Thr change causes a more severe retinal phenotype. Preliminary data suggest a founder effect for the ND p.Arg41Thr mutation in these two Mexican families.

  10. Multi-time-over-threshold technique for photomultiplier signal processing: Description and characterization of the SCOTT ASIC

    International Nuclear Information System (INIS)

    Ferry, S.; Guilloux, F.; Anvar, S.; Chateau, F.; Delagnes, E.; Gautard, V.; Louis, F.; Monmarthe, E.; Le Provost, H.; Russo, S.; Schuller, J.-P.; Stolarczyk, Th.; Vallage, B.; Zonca, E.

    2012-01-01

    KM3NeT aims to build a cubic-kilometer scale neutrino telescope in the Mediterranean Sea based on a 3D array of photomultiplier tubes. A dedicated ASIC, named SCOTT, has been developed for the readout electronics of the PMTs: it uses up to 16 adjustable thresholds to digitize the signals with the multi-time-over-threshold technique. Digital outputs of discriminators feed a circular sampling memory and a “first in first out” digital memory for derandomization. At the end of the data processing, the ASIC produces a digital waveform sampled at 800 MHz. A specific study was carried out to process PMT data and has showed that five specifically chosen thresholds are suited to reach the required timing precision. A dedicated method based on the duration of the signal over a given threshold allows an equivalent timing precision at any charge. A charge estimator using the information from the thresholds allows a charge determination within less than 20% up to 60 pe.

  11. Multi-time-over-threshold technique for photomultiplier signal processing: Description and characterization of the SCOTT ASIC

    Science.gov (United States)

    Ferry, S.; Guilloux, F.; Anvar, S.; Chateau, F.; Delagnes, E.; Gautard, V.; Louis, F.; Monmarthe, E.; Le Provost, H.; Russo, S.; Schuller, J.-P.; Stolarczyk, Th.; Vallage, B.; Zonca, E.; Representing the KM3NeT Consortium

    2012-12-01

    KM3NeT aims to build a cubic-kilometer scale neutrino telescope in the Mediterranean Sea based on a 3D array of photomultiplier tubes. A dedicated ASIC, named SCOTT, has been developed for the readout electronics of the PMTs: it uses up to 16 adjustable thresholds to digitize the signals with the multi-time-over-threshold technique. Digital outputs of discriminators feed a circular sampling memory and a “first in first out” digital memory for derandomization. At the end of the data processing, the ASIC produces a digital waveform sampled at 800 MHz. A specific study was carried out to process PMT data and has showed that five specifically chosen thresholds are suited to reach the required timing precision. A dedicated method based on the duration of the signal over a given threshold allows an equivalent timing precision at any charge. A charge estimator using the information from the thresholds allows a charge determination within less than 20% up to 60 pe.

  12. Retinopathy of prematurity: mutations in the Norrie disease gene and the risk of progression to advanced stages.

    Science.gov (United States)

    Haider, M Z; Devarajan, L V; Al-Essa, M; Srivastva, B S; Kumar, H; Azad, R; Rashwan, N

    2001-04-01

    Retinopathy of prematurity (ROP) is a retinal vascular disease that occurs in infants with short gestational age and low birth weight and may lead to retinal detachment and blindness. Missense mutations in the Norrie disease (ND) gene have been associated with the risk of progression to advanced stages in cases of ROP from the US and also in clinically similar ND and familial exudative vitreoretinopathy. We have screened two ND gene mutations, namely A105T and Val60Glu, by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) and allele-specific PCR methods, respectively, in 210 Kuwaiti premature newborns to replicate these findings in a different ethnic group. In the Kuwaiti premature newborn cohort, 115 of 210 babies had no eye problems and served as controls, while 95 were cases of ROP. In 71 of 95 ROP cases, the disease regressed spontaneously on or before stage 3, while in 24 of 95 ROP cases the disease progressed to advanced stages 4 and 5. In case of missense mutation (A105T), the AA genotype was detected in 96% of controls compared with 87% of ROP cases (NS); similarly no significant difference was found between spontaneously regressed ROP cases and those who progressed to advanced stages. For the Val60Glu mutation, no significant association was detected between the genotype and progression of ROP to advanced stages. Unlike data from the US, our findings from a Kuwaiti cohort of ROP cases and controls suggest a lack of association between the two ND gene mutations (A105T and Val60Glu) and ROP and the risk of progression of the disease to advanced stages.

  13. The Italian validation of the Salford-Scott Nursing Values Questionnaire.

    Science.gov (United States)

    Mecugni, Daniela; Albinelli, Patrizia; Pellegrin, Joellemarie; Finotto, Stefano

    2015-03-01

    To properly direct nursing training and to improve the professional practice to become more effective, it is important to understand students' values. Literature review has shown that there have been changes in students' values in the last 20 years. In contemporary students, a general decrease in altruism has been observed, but also a larger appreciation for honesty toward patients has been declared. The analyzed literature did not find validated tools available in Italian that explore personal and professional values of nursing students. This study was an Italian linguistic and cultural adaptation of a research tool. The authors aimed to validate, for the Italian context, the Salford-Scott Nursing Values Questionnaire, enhanced by Johnson to explore the nursing profession's values. The Beaton Model was used as well as Valmi's. These models require five phases, with the goal of producing a pre-final version of the instrument for it to then be administered to a sample of the target and expert population. The study was approved by the Council of the Nursing Degree University course of the Modena and Reggio Emilia University, Reggio Emilia site, and the identity of the subjects was protected at every moment of the testing. Face validation was achieved since the clarity percentile for each item was 100%. Content validity was also reached, measured from the content validity index and the scale validity index. The study has confirmed the reliability of the instrument's internal consistence with a value of Cronbach's alpha on 0.95 of total of items. The reliability of the test-retest confirms the stability of the instrument in time (r = 0.908; p = 0.01). The study concludes that the instrument is ready to be administered to the target population, a sample group of nursing students. © The Author(s) 2014.

  14. Intrafamilial variability of the ocular phenotype in a Polish family with a missense mutation (A63D) in the Norrie disease gene.

    Science.gov (United States)

    Zaremba, J; Feil, S; Juszko, J; Myga, W; van Duijnhoven, G; Berger, W

    1998-09-01

    To describe the phenotypic variability in a Polish Norrie disease (ND) family associated with the missense mutation A63D. A patient with spared vision from a Polish ND family underwent detailed ophthalmological examinations including slit-lamp biomicroscopy, ultrasound (USG), angiography, Goldmann kinetic visual field, and electroretinography (ERG). Mutation screening was carried out using the single-strand conformation polymorphism (SSCP) technique and subsequent DNA sequencing of the coding part of the ND gene. A mutation was detected (exon 3, A63D) in a large Polish family with 12 affected males, all but one presenting with classical ND symptoms. In one male, partially preserved vision was observed up to 40 years of age (distance acuity of the right eye 1/50 and left eye 2/50). Slit-lamp examination revealed remnants of a persistent primary vitreous and hyaloid artery. Upon angiography, the retina was vascularized within the posterior pole but not in the periphery. The ERG revealed pathological changes characteristic for chorioretinal degenerations. Within one family, individuals with identical sequence alterations in the ND gene can show remarkable phenotypic variability of the ocular symptoms. These findings indicate the involvement of additional factors (epigenetic or genetic) in ocular pathogenesis of ND.

  15. Road Bridges and Culverts, MDTA Culverts, Culverts on John F. Kennedy Highway (I95), Baltimore Harbor Tunnel Throughway, Francis Scott Key Bridge, Bay bridge, Nice Bridge, Published in 2010, 1:1200 (1in=100ft) scale, Maryland Transportation Authority.

    Data.gov (United States)

    NSGIC State | GIS Inventory — Road Bridges and Culverts dataset current as of 2010. MDTA Culverts, Culverts on John F. Kennedy Highway (I95), Baltimore Harbor Tunnel Throughway, Francis Scott Key...

  16. Dendrobium nobile Lindley and its bibenzyl component moscatilin are able to protect retinal cells from ischemia/hypoxia by dowregulating placental growth factor and upregulating Norrie disease protein.

    Science.gov (United States)

    Chao, Wen-Haur; Lai, Ming-Yi; Pan, Hwai-Tzong; Shiu, Huei-Wen; Chen, Mi-Mi; Chao, Hsiao-Ming

    2018-06-22

    Presumably, progression of developmental retinal vascular disorders is mainly driven by persistent ischemia/hypoxia. An investigation into vision-threatening retinal ischemia remains important. Our aim was to evaluate, in relation to retinal ischemia, protective effects and mechanisms of Dendrobium nobile Lindley (DNL) and its bibenzyl component moscatilin. The therapeutic mechanisms included evaluations of levels of placental growth factor (PLGF) and Norrie disease protein (NDP). An oxygen glucose deprivation (OGD) model involved cells cultured in DMEM containing 1% O 2 , 94% N 2 and 0 g/L glucose. High intraocular pressure (HIOP)-induced retinal ischemia was created by increasing IOP to 120 mmHg for 60 min in Wistar rats. The methods included electroretinogram (ERG), histopathology, MTT assay and biochemistry. When compared with cells cultured in DMEM containing DMSO (DMSO+DMEM), cells subjected to OGD and pre-administrated with DMSO (DMSO+OGD) showed a significant reduction in the cell viability and NDP expression. Moreover, cells that received OGD and 1 h pre-administration of 0.1 μM moscatilin (Pre-OGD Mos 0.1 μM) showed a significant counteraction of the OGD-induced decreased cell viability. Furthermore, compared with the DMSO+OGD group (44.54 ± 3.15%), there was significant elevated NDP levels in the Pre-OGD Mos 0.1 μM group (108.38 ± 29.33%). Additionally, there were significant ischemic alterations, namely reduced ERG b-wave, less numerous retinal ganglion cells, decreased inner retinal thickness, and reduced/enhanced amacrine's ChAT/Müller's GFAP or vimentin immunolabelings. Moreover, there were significantly increased protein levels of HIF-1α, VEGF, PKM2, RBP2 and, particularly, PLGF (pg/ml; Sham vs. Vehicle: 15.11 ± 1.58 vs. 39.53 ± 5.25). These ischemic effects were significantly altered when 1.0 g/Kg/day DNL (DNL1.0 + I/R or I/R+ DNL1.0) was applied before and/or after ischemia, but not vehicle (Vehicle+I/R). Of

  17. STS-103 Pilot Scott Kelly and MS John Grunsfeld try on oxygen masks

    Science.gov (United States)

    1999-01-01

    In the bunker at Launch Pad 39B, STS-103 Pilot Scott J. Kelly (left) and Mission Specialist John M. Grunsfeld (Ph.D.) (right) try on oxygen masks during Terminal Countdown Demonstration Test (TCDT) activities. The TCDT provides the crew with emergency egress training, opportunities to inspect their mission payloads in the orbiter's payload bay, and simulated countdown exercises. Other crew members taking part are Commander Curtis L. Brown Jr. and Mission Specialists Steven L. Smith, C. Michael Foale (Ph.D.), and Jean-Frangois Clervoy of France and Claude Nicollier of Switzerland, who are with the European Space Agency. STS-103 is a 'call-up' mission due to the need to replace and repair portions of the Hubble Space Telescope, including the gyroscopes that allow the telescope to point at stars, galaxies and planets. The STS-103 crew will be replacing a Fine Guidance Sensor, an older computer with a new enhanced model, an older data tape recorder with a solid-state digital recorder, a failed spare transmitter with a new one, and degraded insulation on the telescope with new thermal insulation. The crew will also install a Battery Voltage/Temperature Improvement Kit to protect the spacecraft batteries from overcharging and overheating when the telescope goes into a safe mode. Four EVA's are planned to make the necessary repairs and replacements on the telescope. The mission is targeted for launch Dec. 6 at 2:37 a.m. EST.

  18. Retinal vascular abnormalities and dragged maculae in a carrier with a new NDP mutation (c.268delC) that caused severe Norrie disease in the proband.

    Science.gov (United States)

    Lin, Phoebe; Shankar, Suma P; Duncan, Jacque; Slavotinek, Anne; Stone, Edwin M; Rutar, Tina

    2010-02-01

    Norrie disease (ND) is caused by mutations in the ND pseudoglioma (NDP) gene (MIM 300658) located at chromosome Xp11.4-p11.3. ND is characterized by abnormal retinal vascular development and vitreoretinal disorganization presenting at birth. Systemic manifestations include sensorineural deafness, progressive mental disorder, behavioral and psychological problems, growth failure, and seizures. Other vitreoretinopathies that are associated with NDP gene mutations include X-linked familial exudative vitreoretinopathy, Coats disease, persistent fetal vasculature, and retinopathy of prematurity. Phenotypic variability associated with NDP gene mutations has been well documented in affected male patients. However, there are limited data on signs in female carriers, with mild peripheral retinal abnormalities reported in both carrier and noncarrier females of families with NDP gene mutations. Here, we report a family harboring a single base-pair deletion, c.268delC, in the NDP gene causing a severe ND phenotype in the male proband and peripheral retinal vascular abnormalities with dragged maculae similar to those observed in familial exudative vitreoretinopathy in his carrier mother. Copyright (c) 2010 American Association for Pediatric Ophthalmology and Strabismus. Published by Mosby, Inc. All rights reserved.

  19. PORTER S FIVE FORCES MODEL SCOTT MORTON S FIVE FORCES MODEL BAKOS TREACY MODEL ANALYZES STRATEGIC INFORMATION SYSTEMS MANAGEMENT

    Directory of Open Access Journals (Sweden)

    Indra Gamayanto

    2004-01-01

    Full Text Available Wollongong City Council (WCC is one of the most progressive and innovative local government organizations in Australia. Wollongong City Council use Information Technology to gain the competitive advantage and to face a global economy in the future. Porter's Five Force model is one of the models that can be using at Wollongong City Council because porter's five Forces model has strength in relationship between buyer and suppliers (Bargaining power of suppliers and bargaining power of buyers. Other model such as Scott Morton's Five Forces model has strength to analyze the social impact factor, so to gain competitive advantage in the future and have a good IT/IS strategic planning; this model can be use also. Bakos & Treacy model almost the same as Porter's model but Bakos & Treacy model can also be applying into Wollongong City Council to improve the capability in Transforming organization, efficiency, and effectiveness.

  20. Review of behavioral health integration in primary care at Baylor Scott and White Healthcare, Central Region.

    Science.gov (United States)

    Jolly, John B; Fluet, Norman R; Reis, Michael D; Stern, Charles H; Thompson, Alexander W; Jolly, Gillian A

    2016-04-01

    The integration of behavioral health services in primary care has been referred to in many ways, but ultimately refers to common structures and processes. Behavioral health is integrated into primary care because it increases the effectiveness and efficiency of providing care and reduces costs in the care of primary care patients. Reimbursement is one factor, if not the main factor, that determines the level of integration that can be achieved. The federal health reform agenda supports changes that will eventually permit behavioral health to be fully integrated and will allow the health of the population to be the primary target of intervention. In an effort to develop more integrated services at Baylor Scott and White Healthcare, models of integration are reviewed and the advantages and disadvantages of each model are discussed. Recommendations to increase integration include adopting a disease management model with care management, planned guideline-based stepped care, follow-up, and treatment monitoring. Population-based interventions can be completed at the pace of the development of alternative reimbursement methods. The program should be based upon patient-centered medical home standards, and research is needed throughout the program development process.

  1. Disease: H00589 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available H00589 Familial exudative vitreoretinopathy (FEVR), including: Exudative vitreoretinopathy... 1 (EVR1); Exudative vitreoretinopathy2, X-linked ... (EVR2); Exudative vitreoretinopathy 4 (EVR4); Exudative vitreoretinopathy... 5 (EVR5); Norrie disease (ND) Familial exudative vitreoretinopathy (FEVR) and Norri...RP5 [HSA:4041] [KO:K03068] (EVR5) TSPAN12 [HSA:23554] [KO:K17355] ... Exudative vitreoretinopathy 3 (EVR3) ...ion spectrum in familial exudative vitreoretinopathy and Norrie disease with iden

  2. NATO’s Future Role in the Arctic

    Science.gov (United States)

    2016-05-01

    www.arctic-council.org/index.php/en/about-us 4 Kuross, “An Ambiguous Role: NATO in the Arctic.” 5 Derek Reveron & Kathleen Mahoney- Norris , Human...The RAND Corporation, April 1989, http://www.rand.org/pubs/papers/P7558.html, 12-3. 22 Reveron & Mahoney- Norris , Human Security in a Borderless...trying-to-start-a- war-in-the-arctic-its-just-keeping-out-the-riffraff Reveron, Derek & Mahoney- Norris , Kathleen. Human Security in a Borderless

  3. 1995 Emerging Leaders in Healthcare. The new leaders: Gita Budd, Colene Daniel, Elizabeth Gallup, Scott Wordelman.

    Science.gov (United States)

    Southwick, K

    1995-01-01

    Fierce pressures for cost containment. Demands for quality improvements. The drive toward patient-centered care. The push for community involvement. Insistent voices of payers, patients, consumers, physicians. Accumulated tensions amid the chaos of change. Balancing all of these demands while inspiring and encouraging the professionals and other workers within the healthcare organization requires a high level of leadership ability. One that insists on the best from everyone involved in a healthcare system--from physicians to staff, nurses to social workers. And then strives for more. The four young executives who are this year's Emerging Leaders in Healthcare have all pushed their systems beyond traditional boundaries into new territory, helping their patients, their employees, their physicians, and their communities rise to new levels of achievement. At the same time, these leaders emphasize teamwork and consensus-style management, so that their co-workers feel like they're participating in the changes, not being victimized by them. Gita Budd, Colene Daniel, Elizabeth Gallup, and Scott Wordelman are winners of the 1995 award from The Healthcare Forum and Korn/Ferry International that honors ¿dynamic, decisive young leaders (under 40) with the proven ability to nurture the growth of the industry.¿ Korn/Ferry International and The Healthcare Forum are proud to present 1995's Emerging Leaders.

  4. The application of business models to medical research: interviews with two founders of directed-philanthropy foundations. Interview with Scott Johnson and Don Listwin by Kathryn A. Phillips.

    Science.gov (United States)

    Scott, Johnson; Listwin, Don

    2007-01-01

    A new trend in research funding has emerged: directed philanthropy, in which the donor plays an active, hands-on role in managing the research by applying a "business model." Although such efforts now represent only a small portion of foundation funding, they have potentially far-reaching implications because (1) the approach of using a business model is being applied more broadly and (2) the success or failure of these efforts may portend the fate of larger translational efforts. The author conducted interviews with Scott Johnson of the Myelin Repair Foundation and Don Listwin of the Canary Foundation in the fall of 2006.

  5. Norrie disease gene sequence variants in an ethnically diverse population with retinopathy of prematurity.

    Science.gov (United States)

    Hutcheson, Kelly A; Paluru, Prasuna C; Bernstein, Steven L; Koh, Jamie; Rappaport, Eric F; Leach, Richard A; Young, Terri L

    2005-07-14

    Retinopathy of prematurity (ROP) is a leading cause of visual loss in the pediatric population. Mutations in the Norrie disease gene (NDP) are associated with heritable retinal vascular disorders, and have been found in a small subset of patients with severe retinopathy of prematurity. Varying rates of progression to threshold disease in different races may have a genetic basis, as recent studies suggest that the incidence of NDP mutations may vary in different groups. African Americans, for example, are less likely to develop severe degrees of ROP. We screened a large cohort of ethnically diverse patients for mutations in the entire NDP. A total of 143 subjects of different ethnic backgrounds were enrolled in the study. Fifty-four patients had severe ROP (Stage 3 or worse). Of these, 38 were threshold in at least one eye (with a mean gestational age of 26.1 weeks and mean birth weight of 788.4 g). There were 36 patients with mild or no ROP, 31 parents with no history of retinal disease or prematurity, and 22 wild type (normal) controls. There were 70 African American subjects, 55 Caucasians, and 18 of other races. Severe ROP was noted in 29 African American subjects, 17 Caucasians, and 8 of other races. Seven polymerase chain reaction primer pairs spanning the NDP were optimized for denaturing high performance liquid chromatography and direct sequencing. Three primer pairs covered the coding region, and the remaining four spanned the 3' and 5' untranslated regions (UTR). Six of 54 (11%) infants with severe ROP had polymorphisms in the NDP. Five of the infants were African American, and one was Caucasian. Two parents were heterozygous for the same polymorphism as their child. One parent-child pair had a single base pair (bp) insertion in the 3' UTR region. Another parent-child pair had two mutations: a 14 bp deletion in the 5' UTR region of exon 1 and a single nucleotide polymorphism in the 5' UTR region of exon 2. No coding region sequence changes were found. No

  6. Spermiogenesis and sperm ultrastructure in Calicotyle affinis Scott, 1911 (Platyhelminthes, Monogenea, Monopisthocotylea, Monocotylidae

    Directory of Open Access Journals (Sweden)

    Bruňanská M.

    2017-12-01

    Full Text Available Spermatological characteristics of Calicotyle affinis Scott, 1911, an endoparasitic monocotylid monogenean from the cloaca of a holocephalan fish Chimaera monstrosa L, have been investigated by means of transmission electron microscopy for the first time. Spermiogenesis exhibits features basically similar to those of the congeneric Calicotyle kroyeri and Calicotyle australiensis, but there are some new findings with respect to the formation and fine structure of the spermatozoon including the remarkable complex end-piece (EP. Morphogenesis of the EP, which is located at the anterior (proximal region of the late spermatid, includes two stages: (1 the centriolar region is continuous with a cytoplasmic mass of the zone of differentiation, the electron-dense surface of the spermatid undergoes significant changes in the sculpturing and the inner core of developing spermatid is electron-lucent; (2 after central fusion of the arching membranes a definitive structure of the EP is subsequently evolved, finally comprising 3 – 4 electron-dense discs attached to a central common electron-lucent column. The EP is considered as a synapomorphy of the genera Calicotyle + Dictyocotyle. The mature spermatozoon of C. affinis comprises the EP, two parallel axonemes of almost equal lengths with the 9 + “1” trepaxonematan pattern, mitochondrion, nucleus, and a reduced number of parallel cortical microtubules (1 – 3. The posterior (distal extremity of the mature spematozoon contains a single tapering axoneme. Ultrastructural characteristics of the mature spermatozoon of C. affinis coincide mostly with those of congeneric C. australiensis. Variations of the spermatological characters within the genus Calicotyle, between Calicotyle and enigmatic Dictyocotyle as well as other monocotylid monogeneans are discussed.

  7. Unedited Version

    Indian Academy of Sciences (India)

    5

    Patient with Familial Norrie Disease: Bilateral Blindness and Leucocoria ..... Lin RC, Shih JC (2013) Monoamine oxidase A and A/B knockout mice display autistic-like ... spectrum in familial exudative vitreoretinopathy and Norrie disease with ...

  8. Sequence analysis and transcript identification within 1.5 MB of DNA deleted together with the NDP and MAO genes in atypical Norrie disease patients presenting with a profound phenotype.

    Science.gov (United States)

    Suárez-Merino, B; Bye, J; McDowall, J; Ross, M; Craig, I W

    2001-06-01

    Mutations at the Norrie disease gene locus, NDP, manifest in a broad range of defects. These range from a relatively mild, late-onset, exudative vitreoretinopathy to congenital blindness and sensorineural deafness combined, in some cases, with mental retardation. In addition, extensive deletions involving the NDP locus, located at Xp11.3, the adjacent monoamine oxidadase genes MAOA and MAOB, and additional material, result in a more severe pattern of symptoms. The phenotypes include all or some of the following; mental retardation, involuntary movements, hypertensive crises and hypogonadism. We extended an existing YAC contig to embrace the boundaries of three of the largest deletions and converted this into four PAC contigs. Computer analysis and experimental data have resulted in the identification of several putative loci, including a phosphatase inhibitor 2-like gene (dJ154.1) and a 250-bp sequence which resembles a homeobox domain (dA113.3), 1.2 Mb and 400 kb respectively from the MAO/NDP cluster. The pattern of expression of dJ154.1 suggests that it may represent an important factor contributing to the complex phenotypes of these deletion patients. Hum Mutat 17:523, 2001. Copyright 2001 Wiley-Liss, Inc.

  9. Insertion and deletion mutations in the dinucleotide repeat region of the Norrie disease gene in patients with advanced retinopathy of prematurity.

    Science.gov (United States)

    Hiraoka, M; Berinstein, D M; Trese, M T; Shastry, B S

    2001-01-01

    Retinopathy of prematurity (ROP) is a leading cause of blindness in premature children. It is a multifactorial disorder which causes fibrovascular tissue changes that affect the retina in low birth-weight and short gestational age infants. To determine the prevalence of Norrie disease (ND) gene mutations, clinical examination and molecular genetic analyses were performed in 100 pre-term babies of different ethnic backgrounds who developed advanced ROP. The leukocyte DNA was extracted, amplified by the polymerase chain reaction (PCR), and analyzed by single-strand conformation polymorphism (SSCP), G/T and C/A scanning, and by DNA sequencing. All three exons, including splice sites and the 3'-untranslated region, were screened. Of the 100 patients analyzed, 2 patients with advanced ROP showed a mobility shift in the DNA. In 1 patient, this mobility shift was caused by the insertion of an additional 12-bp CT repeat in exon 1, and in the second patient, there was a 14-bp deletion in the same exon of the ND gene, as evidenced by direct sequencing of the amplified products. Similar analyses of exons 2 and 3 and the 3'-untranslated region failed to detect additional mutations in the gene. None of the 130 normal, unrelated controls revealed similar changes. Taking into account the above results, as well as those of other studies, it appears that the ND gene mutations can account for 3% of cases of advanced ROP. Although the ND gene is not frequently involved in advanced ROP, the present large-scale study further supports the hypothesis that genetic influences may play an important role in the development of severe ROP in some premature infants.

  10. The Quest for Success and Power in F. Scott Fitzgerald's Novel The Beautiful and Damned

    Directory of Open Access Journals (Sweden)

    Ala Eddin Sadeq

    2017-02-01

    Full Text Available This study aims at investigating the concepts of success and power, as depicted by F. Scott Fitzgerald in The Beautiful and Damned (2009. Cultural change motivates individuals to work harder to achieve success, which in turn makes them influential. The study reveals that the concepts of success and power are controversial, as their means vary from one theorist to another.  Waldo Emerson, for example, believes that success is connected to happiness.  He, therefore, lists down features that characterize successful people. To succeed, one must learn to follow their desires, an argument that is expounded by the ideology of the American Dream.  Friedrich Nietzsche, however, explains that individuals are motivated to lead due to the fact that power brings about the superman. To achieve the status of the superman, Nietzsche believes that individuals develop the will to power and are able to influence others (Nietzsche, 1968. Fitzgerald, on the other hand, makes it clear that power leads to liberty. The novel provides a deep analysis of the quest for power and success. The main characters are Gloria, Joseph, and Anthony who helps to demonstrate the quest for success and power. Richard Caramel is also a character whose role explains the pursuit of true happiness. He is depicted as powerful because he influences the society through his writings. He has a strong determination to be a writer, which motivates him to work hard and to seek further success.

  11. Information geometric analysis of phase transitions in complex patterns: the case of the Gray-Scott reaction–diffusion model

    International Nuclear Information System (INIS)

    Har-Shemesh, Omri; Quax, Rick; Hoekstra, Alfons G; Sloot, Peter M A

    2016-01-01

    The Fisher–Rao metric from information geometry is related to phase transition phenomena in classical statistical mechanics. Several studies propose to extend the use of information geometry to study more general phase transitions in complex systems. However, it is unclear whether the Fisher–Rao metric does indeed detect these more general transitions, especially in the absence of a statistical model. In this paper we study the transitions between patterns in the Gray-Scott reaction–diffusion model using Fisher information. We describe the system by a probability density function that represents the size distribution of blobs in the patterns and compute its Fisher information with respect to changing the two rate parameters of the underlying model. We estimate the distribution non-parametrically so that we do not assume any statistical model. The resulting Fisher map can be interpreted as a phase-map of the different patterns. Lines with high Fisher information can be considered as boundaries between regions of parameter space where patterns with similar characteristics appear. These lines of high Fisher information can be interpreted as phase transitions between complex patterns. (paper: disordered systems, classical and quantum)

  12. Aarskog-Scott syndrome: clinical update and report of nine novel mutations of the FGD1 gene.

    Science.gov (United States)

    Orrico, A; Galli, L; Faivre, L; Clayton-Smith, J; Azzarello-Burri, S M; Hertz, J M; Jacquemont, S; Taurisano, R; Arroyo Carrera, I; Tarantino, E; Devriendt, K; Melis, D; Thelle, T; Meinhardt, U; Sorrentino, V

    2010-02-01

    Mutations in the FGD1 gene have been shown to cause Aarskog-Scott syndrome (AAS), or facio-digito-genital dysplasia (OMIM#305400), an X-linked disorder characterized by distinctive genital and skeletal developmental abnormalities with a broad spectrum of clinical phenotypes. To date, 20 distinct mutations have been reported, but little phenotypic data are available on patients with molecularly confirmed AAS. In the present study, we report on our experience of screening for mutations in the FGD1 gene in a cohort of 60 European patients with a clinically suspected diagnosis of AAS. We identified nine novel mutations in 11 patients (detection rate of 18.33%), including three missense mutations (p.R402Q; p.S558W; p.K748E), four truncating mutations (p.Y530X; p.R656X; c.806delC; c.1620delC), one in-frame deletion (c.2020_2022delGAG) and the first reported splice site mutation (c.1935+3A>C). A recurrent mutation (p.R656X) was detected in three independent families. We did not find any evidence for phenotype-genotype correlations between type and position of mutations and clinical features. In addition to the well-established phenotypic features of AAS, other clinical features are also reported and discussed. Copyright 2010 Wiley-Liss, Inc.

  13. Causative get-constructions in the dialogued passages in F. Scott Fitzgerald’s novels The Beautiful and Damned and Tender Is the Night as gender-conditioned structures

    Directory of Open Access Journals (Sweden)

    Gołąbek Rafał

    2015-05-01

    Full Text Available It goes without saying that in modern sociolinguistics there is a consensus with regard to the fact that the language of males and females differs. The initial sections of the article briefly address the peculiarities of gendered speech as to provide a theoretical background for checking whether the causative get is used similarly or differently by men and women in the two of F. Scott Fitzgerald’s novels: The Beautiful and Damned and Tender Is the Night. The basic expectation formed is that the motifs for triggering the use of causative get are of social rather than structural nature. Before the analysis is carried out, the group of the English periphrastic causatives are sketchily characterized. Generally, what has been found is that there is a clear, socially-motivated pattern of how F. Scott Fitzgerald uses the causative get in the dialogued occurrences in his two novels. Get is a characteristic of men’s talk, but it is also the expected form while female characters address male ones - hence the verb is labelled as “masculine” get. Moreover, it has been discovered that there does not seem to be any particular pattern in either the speaker’s mood or the speaker’s attitude expressed that would trigger the use of the causative verb in question. Yet, what seems to be a well-defined tendency, when it comes to the speaker-hearer power relation, is that the speaker usually assumes a more superior position than the hearer when he or she uses the causative verb. The superiority in most cases is strongly associated with masculinity. Hence, what is postulated is that the causative get is labelled not only as “masculine" but also as “superior”.

  14. Connecting Numbers with Emotion: Review of Numbers and Nerves: Information, Emotion, and Meaning in a World of Data by Scott Slovic and Paul Slovic (2015

    Directory of Open Access Journals (Sweden)

    Samuel L. Tunstall

    2017-01-01

    Full Text Available Scott Slovic and Paul Slovic (Eds.. Numbers and Nerves: Information, Emotion, and Meaning in a World of Data (Corvallis, OR: Oregon State University Press, 2015. 272 pp. ISBN 978-0-87071-776-5. It is common to view quantitative literacy as reasoning with respect to numbers. In Numbers and Nerves, the contributors to the volume make clear that we should attend not only to how students consciously reason with numbers, but also how our innate biases influence our actions when faced with numbers. Beginning with the concepts of psychic numbing, and then psuedoinefficacy, the contributors to the volume examine how our behaviors when faced with large numbers are often not mathematically rational. I consider the implications of these phenomena for the Numeracy community.

  15. A C597-->A polymorphism in the Norrie disease gene is associated with advanced retinopathy of prematurity in premature Kuwaiti infants.

    Science.gov (United States)

    Haider, M Z; Devarajan, L V; Al-Essa, M; Kumar, H

    2002-01-01

    Retinopathy of prematurity (ROP) is a retinal vascular disease which occurs in infants with a short gestational age and low birth weight and may lead to retinal detachment and blindness. In some premature infants, ROP progresses to advanced stages despite rigorous intervention, but in the majority, it spontaneously regresses before the threshold stage. Genetic factors, e.g. mutations in the Norrie disease (ND) gene, have been implicated in determining the progression of ROP to advanced stages. We have identified a novel C597A polymorphism of the ND gene; we screened this and another mutation in the ND gene, C110G, in 210 premature Kuwaiti infants using PCR-RFLP, DNA sequence analysis and DNA enzyme immunoassay hybridization to investigate their association with advanced-stage ROP. In this cohort of premature Kuwaiti newborns, 115 of 210 babies had no eye problems and served as controls, while 95 were found to have ROP. In 71 of the 95 ROP cases, the disease spontaneously regressed at or before stage 3, while in 24 of 95 ROP cases, the disease progressed to advanced stages 4 or 5. The incidence of the AA genotype of the C597A polymorphism was considerably higher in advanced-stage ROP cases (83.3%) compared to spontaneously regressing ROP cases (0%) and the normal controls (10.4%) (p < 0.0001). For the other genotypes, no significant difference was detected between the controls and ROP cases. In the case of the C110G mutation in the ND gene, no significant differences were detected between the controls and ROP cases, and the majority of subjects had a CC genotype in all three groups. Copyright 2002 National Science Council, ROC and S. Karger AG, Basel

  16. In Search of Activist Pedagogies in SMTE

    Science.gov (United States)

    Alsop, Steve; Bencze, Larry

    2012-01-01

    David Burns and Stephen Norris's (2012) article entitled "Activist Environmental Education and Moral Philosophy" offers a thought-provoking response to the CJSMTE special edition. The authors would like to thank these authors for their supportive and philosophically adroit arguments. Burns and Norris provide an opportunity to continue…

  17. Play the game in the opening scene A multidisciplinary lens for understanding (videoludic movies, including Super Mario Bros., Resident Evil and Scott Pilgrim vs. the World

    Directory of Open Access Journals (Sweden)

    Enrico Gandolfi

    2015-09-01

    Full Text Available The aim of this article is to create a multidisciplinary tool concerning the passage from the medium of videogames to cinema. According to concepts taken from Media Studies, Cultural Studies, Semiotics and Game Studies, we will explore the multiple dimensions and the related connections that occur in the film linearization of digital interaction: production issues, narrative and aesthetic elements, heuristics and mechanics in-game and on the big screen, and so on. The framework will be tested through three paradigmatic case studies: Super Mario, Resident Evil, and Scott Pilgrim vs. the World. The overall intent is to give scholars, and also practitioners, a holistic perspective on this peculiar type of crossmedia process, pointing out virtuous productive strategies as ruinous ones.

  18. New record and redescription of Calanopia thompsoni A. Scott, 1909 (Copepoda, Calanoida, Pontellidae) from the Red Sea, with notes on the taxonomic status of C. parathompsoni Gaudy, 1969 and a key to species.

    Science.gov (United States)

    Al-Aidaroos, Ali M; Salama, Adnan J; El-Sherbiny, Mohsen M

    2016-01-01

    During a plankton sampling programme around Al-Wajh area, Saudi Arabian coast of the northern Red Sea, a copepod Calanopia thompsoni A. Scott, 1909 (Calanoida: Pontellidae) was reported for the first time in the Red Sea. Both sexes are fully redescribed and compared to previous descriptions as well as the closely related species, Calanopia parathompsoni. The zoogeographical distribution of the species confirms that it is of Indo-Pacific origin. A dichotomous key for the identification of males and females of the species of Calanopia is included.

  19. Norrie-Syndrom

    OpenAIRE

    Flügel, T; Pflug, C; Niessen, A

    2016-01-01

    Hintergrund: Vorgestellt wurde in unserer Klinik ein 17-jähriger Junge mit angeborener Blindheit. Er leidet zusätzlich seit dem 14. Lebensjahr unter einer schubweise progredienten Schwerhörigkeit. Aktuell zeigte sich eine mittelgradige Schwerhörigkeit beidseits. Eine humangenetische Abklärung der Augenveränderungen hatte in der frühen Kindheit keine Auffälligkeiten ergeben. Die Familienanamnese ist bezogen auf Hörstörungen oder Blindheit leer. Wegen der neu aufgetretenen progredienten Schwer...

  20. Norries disease

    Directory of Open Access Journals (Sweden)

    Saini J

    1992-01-01

    Full Text Available A 2-month-old male infant was found to have Norrie′s disease. The clinical presentation and detailed histological features diagnostic of the disease are discussed. This is the first authentic, histologically proven case of Norrie′s disease from India. The absence of hearing loss and mental retardation at the time of presentation at the early stage of infancy and the fact that the case was sporadic do not detract from the diagnosis. However the child at the age of one year developed hearing loss.

  1. On Consumerism, Collective Action, and Whether Art Teaches Anything

    Science.gov (United States)

    Ruitenberg, Claudia W.

    2014-01-01

    In this review essay, Claudia Ruitenberg discusses Trevor Norris's "Consuming Schools," René Arcilla's "Mediumism," and Martha Nussbaum's "Not for Profit." While the primary focus of each book is different--with Norris concentrating on the pressures of consumerism and commercialism on K-12 schooling,…

  2. The case of Scott Ortiz: a clash between criminal justice and public health

    Directory of Open Access Journals (Sweden)

    Tobia Maria S

    2006-07-01

    Full Text Available Abstract The criminal justice system creates particular challenges for persons with HIV and Hepatitis C, many of whom have a history of injection drug use. The case of Scott Ortiz, taken from public trial and sentencing transcripts, reveals the manner in which incarceration may delay learning of important health problems such as Hepatitis C infection. In addition, the case of Mr. Ortiz suggests the bias in sentencing that a former injection drug user may face. Collaboration between the Montefiore Medical Center residency in Social Medicine and a Bronx legal services agency, Bronx Defenders, yielded the discovery that a decade after diagnosis with HIV and after long term incarceration, Mr. Ortiz was infected with Hepatitis C. Mr. Ortiz only became aware of his advanced Hepatitis C and liver damage during his trial. The second important aspect of this case centers on the justification for lengthy sentence for a burglary conviction. The presiding Judge in Mr. Ortiz's case acknowledged that because of his advanced illness, Mr. Ortiz posed no threat to society as a burglar (the crime for which he was convicted. But the Judge elected to use his discretion to sentence Mr. Ortiz to a term of 15 years to life (as opposed to a minimum of two to four years based on the idea that the public health would be served by preventing Mr. Ortiz from returning to the life of a street addict, sharing dirty needles with others. Mr. Ortiz reports distant injection drug use, no evidence of current or recent drug use was presented during Mr. Ortiz's trial and he reports no injection drug use for over a decade. In this case, bias against a former injection drug user, masquerading as concern for public health, is used to justify a lengthier sentence. Mr. Ortiz's lack of awareness of his Hepatitis C infection despite long term incarceration, combined with the justification for his dramatically increased sentence, provide examples of how persons within the criminal justice

  3. Establish Best Practices for Supervision of Instructors

    Science.gov (United States)

    2012-09-01

    active participants in the feedback process, allowing them to voice their opinions and collaboratively set goals ( Norris , 1991). In the foreign...language instructional context, instructors may desire to participate in the feedback process ( Norris , 1991). Accordingly, effective instructional...13%) and Russian (11%) were the most commonly reported language offerings, followed by Chinese (9%), French (9%), Portuguese (9%), and Spanish (9

  4. 2012 Aerospace Medical Certification Statistical Handbook

    Science.gov (United States)

    2013-12-01

    2012 Aerospace Medical Certification Statistical Handbook Valerie J. Skaggs Ann I. Norris Civil Aerospace Medical Institute Federal Aviation...Certification Statistical Handbook December 2013 6. Performing Organization Code 7. Author(s) 8. Performing Organization Report No. Skaggs VJ, Norris AI 9...2.57 Hayfever 14,477 2.49 Asthma 12,558 2.16 Other general heart pathology (abnormal ECG, open heart surgery, etc.). Wolff-Parkinson-White syndrome

  5. Effect of Vertical Shoot-Positioned, Scott-Henry, Geneva Double-Curtain, Arch-Cane, and Parral Training Systems on the Volatile Composition of Albariño Wines

    Directory of Open Access Journals (Sweden)

    Mar Vilanova

    2017-09-01

    Full Text Available Viticultural practices influence both grape and wine quality. The influence of training systems on volatile composition was investigated for Albariño wine from Rías Baixas AOC in Northwest Spain. The odoriferous contribution of the compounds to the wine aroma was also studied. Volatile compounds belonging to ten groups (alcohols, C6-compounds, ethyl esters, acetates, terpenols, C13-norisoprenoids, volatile phenols, volatile fatty acids, lactones and carbonyl compounds were determined in Albariño wines from different training systems, Vertical Shoot-Positioned (VSP, Scott-Henry (SH, Geneva Double-Curtain (GDC, Arch-Cane (AC, and Parral (P during 2010 and 2011 vintages. Wines from GDC showed the highest total volatile composition with the highest concentrations of alcohols, ethyl esters, fatty acids, and lactones families. However, the highest levels of terpenes and C13-norisoprenoids were quantified in the SH system. A fruitier aroma was observed in Albariño wines from GDC when odor activity values were calculated.

  6. Obituary: John Norris Bahcall, 1934-2005

    Science.gov (United States)

    Striker, Jeremiah P.; Bahcall, Neta A.

    2007-12-01

    John Norris Bahcall, one of the most creative and influential astrophysicists of his generation — a scientist who helped prove what makes the Sun shine and helped make the Hubble Space Telescope a reality — passed away in Pasadena, California, on 17 August 2005. Bahcall died peacefully in his sleep from a rare blood disorder. For the past 35 years, Bahcall was the Richard Black Professor of Natural Sciences at the Institute for Advanced Study in Princeton, New Jersey, where he created one of the leading astrophysics programs in the world. Active and working to the end, Bahcall said that he was always grateful for a full and happy life that exceeded his wildest expectations. Bahcall died as he lived, surrounded by the family he loved, embracing life to its fullest, happy, working and joking to the end. Bahcall's stellar career encompassed seminal contributions in numerous fields of astrophysics as well as extraordinary leadership on behalf of the scientific community, including the American Astronomical Society, the American Physical Society, the National Academy of Sciences, NASA, and Congress. Bahcall's contributions made him one of the scientific leaders of his time. He had been recognized by numerous awards including the 1998 National Medal of Science from President Clinton, the Gold Medal of the Royal Astronomical Society, the Medal of the Swedish Royal Academy, the Dan David Award, the Fermi Award, the first Hans Bethe Prize, the Franklin Medal, the Comstock Prize in physics, NASA's Exceptional Scientific Achievement Medal, NASA's Distinguished Public Service Medal, and the top awards of the American Astronomical Society — including the Russell Award, the Heineman Prize, and the Warner Prize. Bahcall was elected to the National Academy of Sciences in 1976 and to the American Philosophical Society in 2001. He was the recipient of Honorary Degrees from numerous universities around the world. Bahcall's scientific interests and expertise ranged from neutrino

  7. When Democracy Is Not Enough

    DEFF Research Database (Denmark)

    Charron, Nicholas

    2014-01-01

    Book review of: Making Democratic Governance Work: How Regimes Shape Prosperity, Welfare, and Peace. By Pippa Norris. New York: Cambridge University Press, 2012. 279 pp., $30.99 paperback (ISBN-13: 978-1-107-60269-4).......Book review of: Making Democratic Governance Work: How Regimes Shape Prosperity, Welfare, and Peace. By Pippa Norris. New York: Cambridge University Press, 2012. 279 pp., $30.99 paperback (ISBN-13: 978-1-107-60269-4)....

  8. Self-field ac losses in biaxially aligned Y endash Ba endash Cu endash O tape conductors

    International Nuclear Information System (INIS)

    Iijima, Y.; Hosaka, M.; Sadakata, N.; Saitoh, T.; Kohno, O.; Takeda, K.

    1997-01-01

    Self-field ac losses were measured by the conventional ac four-probe method in biaxially aligned Y endash Ba endash Cu endash O tapes using polycrystalline Hastelloy tapes with textured yttria-stabilized-zirconia buffer layers. The ac losses increased in proportion to the fourth power of transport current in the high J c sample, and agreed well with Norris close-quote equation for thin strip conductors. However, the low J c sample had rather higher losses than Norris close-quote prediction, suggesting excessive magnetic flux penetration caused by percolated current paths. The results confirmed Norris close-quote prediction of the low ac losses for thin strip conductors, and indicated the importance of removing percolated structures of current paths to avoid higher ac losses than the theoretical predictions based on uniform conductors. copyright 1997 American Institute of Physics

  9. 4- Norris.pmd

    African Journals Online (AJOL)

    chifaou.amzat

    2011-08-20

    Aug 20, 2011 ... current problems/opportunities and the possible solutions. .... are wheat, lucerne, tomatoes, spinach, watermelons, cabbages, groundnuts, ..... following activities: hydroponics, greenhouses, high-value crops, specialised.

  10. Beyond Line of Sight (BLOS) Command and Control (C2) Capability to Improve Disaster Response and Recovery

    Science.gov (United States)

    2013-09-01

    accessed Sept. 19, 2012). 8 Ibid. 9 Guy Norris , “Real-Time Intelligence, Surveillance & Reconnaissance (ISR) Data Sharing Technology for the “Af/Pak...in 2012.19 Spanish for the word “straight,” a derecho is a term used to describe a widespread, long- lived, straight-line windstorm that is...Network (NPSBN).” Illinois First Net. http://www.illinois.gov/firstnet/NPSBN/Pages/default.aspx. Norris , Guy. “ Real-Time Intelligence, Surveillance

  11. Targeting Prostate Cancer with Bifunctional Modulators of the Androgen Receptor

    Science.gov (United States)

    2015-06-01

    Wittmann, B.; Dwyer, M.; Cui, H.; Dye, D.; McDonnell, D.; Norris , J. Inhibition of prostate cancer cell growth by second-site androgen receptor antagonists...Wittmann, B.; Dwyer, M.; Cui, H.; Dye, D.; McDonnell, D.; Norris , J. Inhibition of prostate cancer cell growth by second-site androgen receptor...important clin- ical problem in diseases such as asthma (51, 52), ne- phrotic syndrome (53), and malignancies such as acute lymphoblastic leukemia (54

  12. Targeting Histone Abnormality in Triple Negative Breast Cancer

    Science.gov (United States)

    2016-08-01

    M.D., Ph.D. 3 1. Huang Y, Johnson KR, Norris JS, Fan W. NF-κB/IκB signaling pathway may contribute to the mediation of paclitaxel-induced...of paclitaxel-induced apoptosis in human tumor cell lines. Mol. Pharmacol., 61: 105-113, 2002. PMID: 11752211 3. Huang Y, Fang Y, Dziadyk JM, Norris ...has been shown to have important roles in many diseases including cancer.17,18 In this study, we addressed the following clinically relevant issues

  13. Michael Scott

    Directory of Open Access Journals (Sweden)

    Ersin Hussein

    2015-04-01

    While Michael has contributed significantly to the field of classics and ancient history by publishing extensively, he has also enjoyed great success in engaging wider audiences with the ancient world. He regularly talks in schools around the country, writes books intended for the popular market as well as articles for national and international newspapers and magazines. Michael's experience in writing and presenting a range of programmes intended for TV and radio audiences has made him a household name. He has written and presented programmes for the National Geographic, History Channel, Nova, and the BBC including Delphi: bellybutton of the ancient world (BBC4; Guilty Pleasures: luxury in the ancient and medieval words (BBC4; Jesus: rise to power (Natural Geographic; Ancient Discoveries (History Channel; Who were the Greeks? (BBC2; The Mystery of the X Tombs (BBC2/Nova; The Greatest Show on Earth (BBC4, in conjunction with the Open University. He has also presented a radio series for BBC Radio 4, Spin the Globe. Michael's most recent programme, Roman Britain from the Air, was aired on ITV in December 2014. In this interview, I talk to him about his engagement with other disciplines within the humanities, his forthcoming book project, and his experiences writing and presenting TV and radio documentaries.

  14. Fisheries Handbook of Engineering Requirements and Biological Criteria,

    Science.gov (United States)

    1986-01-01

    species whose desease organisms. Deep wells require pumping and fre- specific function is to supply game fish. There is a large quently carry an excess...1966. 126(3266):212-213. August 2, 1957. 22. Fry, F.E.J. and K.S. Norris , "The transportation of live 30. Eisler, Ronald and Tadeus Backiel...8217" 301. June, 1967. chloretone and M.S. 222 on eastern brook trout." The Progressive Fish-Culturist, 20(3):104-110, July, 1958. 24. Norris , Kenneth S

  15. Gamma-ray Burst X-ray Flares Light Curve Fitting

    Science.gov (United States)

    Aubain, Jonisha

    2018-01-01

    Gamma Ray Bursts (GRBs) are the most luminous explosions in the Universe. These electromagnetic explosions produce jets demonstrated by a short burst of prompt gamma-ray emission followed by a broadband afterglow. There are sharp increases of flux in the X-ray light curves known as flares that occurs in about 50% of the afterglows. In this study, we characterized all of the X-ray afterglows that were detected by the Swift X-ray Telescope (XRT), whether with flares or without. We fit flares to the Norris function (Norris et al. 2005) and power laws with breaks where necessary (Racusin et al. 2009). After fitting the Norris function and power laws, we search for the residual pattern detected in prompt GRB pulses (Hakkila et al. 2014, 2015, 2017), that may indicate a common signature of shock physics. If we find the same signature in flares and prompt pulses, it provides insight into what causes them, as well as, how these flares are produced.

  16. Quantum theory and the flight from realism philosophical responses to quantum mechanics

    CERN Document Server

    Norris, Christopher

    2002-01-01

    This book is a critical introduction to the long-standing debate concerning the conceptual foundations of quantum mechanics and the problems it has posed for physicists and philosophers from Einstein to the present. Quantum theory has been a major infulence on postmodernism, and presents significant problems for realists. Keeping his own realist position in check, Christopher Norris subjects a wide range of key opponents and supporters of realism to a high and equal level of scrutiny. With a characteristic combination of rigour and intellectual generosity, he draws out the merits and weaknesses from opposing arguments. In a sequence of closely argued chapters, Norris examines the premises of orthodox quantum theory, as developed most influentially by Bohr and Heisenberg, and its impact on varous philosophical developments. These include the ideas developed by W.V Quine, Thomas Kuhn, Michael Dummett, Bas van Fraassen, and Hilary Puttnam. In each case, Norris argues, these thinkers have been influenced by the...

  17. 75 FR 35629 - Standard Instrument Approach Procedures, and Takeoff Minimums and Obstacle Departure Procedures...

    Science.gov (United States)

    2010-06-23

    ... West Union, IA, George L Scott Muni, GPS RWY 17, Orig, CANCELLED West Union, IA, George L Scott Muni, GPS RWY 35, Orig, CANCELLED West Union, IA, George L Scott Muni, RNAV (GPS) RWY 17, Orig West Union, IA, George L Scott Muni, RNAV (GPS) RWY 35, Orig West Union, IA, George L Scott Muni, Takeoff...

  18. Discussion of “Geology and diamond distribution of the 140/141 kimberlite, Fort à la Corne, central Saskatchewan, Canada”, by A. Berryman, B.H. Scott-Smith and B.C. Jellicoe (Lithos v. 76, p. 99 114)

    Science.gov (United States)

    Kjarsgaard, Bruce A.; Leckie, Dale A.; Zonneveld, John-Paul

    2007-09-01

    A wide variety of geological data and geological observations by numerous geoscientists do not support a two-stage crater excavation and in-fill model, or a champagne glass-shaped geometry for the 169 or 140/141 kimberlite bodies in the Fort à la Corne kimberlite field, Saskatchewan as described by Berryman, A., Scott Smith, B.H., Jellicoe, B., (2004). Rather, these kimberlite bodies are best described as polygenetic kimberlite tephra cones and tuff rings with associated feeder vents of variable geometry as shown by previous workers for the 169 kimberlite, the 140/141 kimberlite and the Star kimberlite. The domal tephra cone geometry is preserved due to burial by conformable Cretaceous marine mudstones and siltstones and is not an artifact of Quaternary glacial processes.

  19. Cerebroretinal microangiopathy with calcifications and cysts associated with CTC1 and NDP mutations.

    Science.gov (United States)

    Romaniello, Romina; Arrigoni, Filippo; Citterio, Andrea; Tonelli, Alessandra; Sforzini, Cinzia; Rizzari, Carmelo; Pessina, Marco; Triulzi, Fabio; Bassi, Maria Teresa; Borgatti, Renato

    2013-12-01

    Mutations in the conserved telomere maintenance component 1 (CTC1) gene were recently described in Coats plus syndrome and in cerebroretinal microangiopathy with calcifications and cysts. Norrie disease protein (NDP) gene was found mutated in Norrie disease, in Familial Exudative Vitreoretinopathy, and in Coats syndrome. Here we describe a boy affected by Norrie disease who developed typical features of cerebroretinal microangiopathy with calcifications and cysts. Direct sequencing of the CTC1 and NDP genes in this patient shows the presence of compound heterozygosity for 2 mutations in CTC1 (c.775G>A, pV259M and a novel microdeletion c.1213delG) and a missense mutation in the NDP gene (c.182T>C, p.L61P). Based on these genetic findings and on the expression of both genes in endothelial cells, we postulate that microangiopathy might be a primary underlying pathologic abnormality in cerebroretinal microangiopathy with calcifications and cysts. This hypothesis is further supported by magnetic resonance imaging (MRI) data showing multiple minute calcifications in the deep gray nuclei and in terminal arteriolar zones.

  20. Demography of Honors: The Census of U.S. Honors Programs and Colleges

    Science.gov (United States)

    Scott, Richard I.; Smith, Patricia J.; Cognard-Black, Andrew J.

    2017-01-01

    Beginning in 2013 and spanning four research articles, we have implemented an empirical analysis protocol for honors education that is rooted in demography (Scott; Scott and Smith; Smith and Scott "Growth"; Smith and Scott, "Demography"). The goal of this protocol is to describe the structure and distribution of the honors…

  1. A new anchialine Stephos Scott from the Yucatan Peninsula with notes on the biogeography and diversity of the genus (Copepoda, Calanoida, Stephidae

    Directory of Open Access Journals (Sweden)

    Eduardo Suárez-Morales

    2017-04-01

    Full Text Available Surveys of the anchialine crustacean fauna of the Yucatan Peninsula (YP, Mexico, have revealed the occurrence of calanoid copepods. The genus Stephos Scott, 1892, belonging to the family Stephidae is among the most frequent and widely distributed groups in anchialine caves but has not been hitherto recorded from the YP. Recent collections from an anchialine cave in an island off the northern coast of the YP yielded many specimens of a new species of Stephos. The new taxon, S. fernandoi sp. n., is described here based on male and female specimens. The new species is clearly distinguished from its congeners by the following characters: male left fifth leg with three terminal lamellae plus subdistal process, right leg with distal row of peg-like elements; female fifth leg with single long, acute apical process; genital double-somite with two rows each of 4 long spinules adjacent to operculum; legs 2-4 with articulated setae. The diversity of the genus shows regional differences; the Australia-Western Pacific region is the most diverse (eleven species, followed by the Mediterranean (seven species and the Northeastern Atlantic (six species; only four species are known from the Northwestern Tropical Atlantic (NWTA. The morphology of the female fifth leg was examined to explore possible biogeographic trends in the genus; patterns suggest multiple colonization events in the highly diverse regions and a relatively recent radiation in the NWTA, characterized by anchialine forms. The introduction of stephid copepods in the region may be a relatively recent event derived from colonization of benthopelagic ancestral forms and subsequent invasion onto cave habitats. The new species appears to be linked to the strictly anchialine Miostephos.

  2. Nuga selga : endine lähikondne teeb maha president Bushi valitsusaja / Kaivo Kopli

    Index Scriptorium Estoniae

    Kopli, Kaivo

    2008-01-01

    Endine Valge Maja pressiesindaja Scott McClellan kirjutas kritiseeriva raamatu "What Happened" president George W. Bushist ja tema kaastöötajatest. Vt. samas: "Me oleme segaduses. See pole Scott, keda me tundsime"; Väljavõtteid Scott McClellani raamatust

  3. 75 FR 41896 - Colville Indian Plywood and Veneer Colville Tribal Enterprise Corporation Wood Products Division...

    Science.gov (United States)

    2010-07-19

    ... Logging, Lone Rock Contracting, Mawdsley Logging, Mccuen Jones, San Poil Logging, Scott Thorndike, Silver... McCuen Jones, San Poil Logging, Scott Thorndike, Silver Nichol Trucking and Stensgar Logging were... Logging, Scott Thorndike, Silver Nichol Trucking and Stensgar Logging, Omak, Washington, who became...

  4. Time scale of hydrothermal water-rock reactions in Yellowstone National Park based on radium isotopes and radon

    International Nuclear Information System (INIS)

    Clark, J.F.; Turekian, K.K.

    1990-01-01

    We have measured 224 Ra (3.4 d), 228 Ra (5.7 yr), and 226 Ra (1620 yr) and chloride in hot spring waters from the Norris-Mammoth Corridor, Yellowstone National Park. Two characteristic cold-water components mix with the primary hydrothermal water: one for the travertine-depositing water related to the Mammoth Hot Springs and the other for the sinter-depositing Norris Geyser Basin springs. The Mammoth Hot Springs water is a mixture of the primary hydrothermal fluid with meteoric waters flowing through the Madison Limestone, as shown by the systematic decrease of the ( 228 Ra/ 226 Ra) activity ratio proceeding northward. The Norris Geyser Basin springs are mixtures of primary hydrothermal water with different amounts of cold meteoric water with no modification of the primary hydrothermal ( 228 Ra/ 226 Ra) activity ratio. Using a solution and recoil model for radium isotope supply to the primary hydrothermal water, a mean water-rock reaction time prior to expansion at 350degC and supply to the surface is 540 years assuming that 250 g of water are involved in the release of the radium from one gram of rock. The maximum reaction time allowed by our model is 1150 years. (orig.)

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

    International Nuclear Information System (INIS)

    Tsukamoto, Osami

    2005-01-01

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

  6. Transport losses in single and assembled coated conductors with textured-metal substrate with reduced magnetism

    International Nuclear Information System (INIS)

    Amemiya, N.; Jiang, Z.; Li, Z.; Nakahata, M.; Kato, T.; Ueyama, M.; Kashima, N.; Nagaya, S.; Shiohara, S.

    2008-01-01

    Transport losses in a coated conductor with a textured-metal substrate with reduced magnetism were studied experimentally. The substrate is with a clad structure, and HoBCO superconductor layer is deposited on the substrate with buffer layers. The measured transport loss of a sample whose critical current is 126.0 A falls between Norris's strip value and Norris's ellipse value. The increase in the measured transport loss from Norris's strip value can be attributed to its non-uniform lateral J c distribution. The same buffered clad tape was placed under an IBAD-MOCVD coated conductor with a non-magnetic substrate, and its transport loss was measured. The comparison between the measured transport loss of this sample and that of the identical IBAD-MOCVD coated conductor without the buffered clad tape indicates that the increase in the transport loss due to this buffered clad tape is small. The transport losses of hexagonal assemblies of IBAD-MOCVD coated conductors, whose structure simulates that of superconducting power transmission cables, were also measured where the buffered clad tapes were under-lied or over-lied on the coated conductors. The increase in the transport loss of hexagonal assemblies of coated conductors due to the buffered clad tapes is at an allowable level

  7. 75 FR 41896 - Colville Indian Precision Pine, Colville Tribal Enterprise Corporation, Wood Products Division...

    Science.gov (United States)

    2010-07-19

    ... Logging, Lone Rock Contracting, Mawdsley Logging, McCuen Jones, San Poil Logging, Scott Thorndike, Silver..., Laramie Logging, Lone Rock Contracting, Mawdsley Logging, McCuen Jones, San Poil Logging, Scott Thorndike... Contracting, Mawdsley Logging, McCuen Jones, San Poil Logging, Scott Thorndike, Silver Nichol Trucking and...

  8. Is Comparability of 14C Dates an Issue?: A Status Report on the Fourth International Radiocarbon Intercomparison

    NARCIS (Netherlands)

    Bryant, C.; Carmi, I.; Cook, G.T.; Gulliksen, S.; Harkness, D.D.; Heinemeier, J.; McGee, E.; Naysmith, P.; Possnert, G.; Scott, E.M.; Plicht, J. van der; Strydonck, M. van

    2001-01-01

    For more than 15 years, the radiocarbon community has participated in a series of laboratory intercomparisons in response to the issue of comparability of measurements as perceived within the wider user communities (Scott et al. 1990; Rozanski et al. 1992; Guiliksen and Scott 1995; Scott et al.

  9. Anne Scott Sørensen, Ole Martin Høystad, Erling Bjurström and Halvard Vike Nye kulturstudier - En innføring, Oslo: Spartacus Forlag AS/Scandinavian Academic Press, 2008

    Directory of Open Access Journals (Sweden)

    Gösta Arvastson

    2009-10-01

    Full Text Available Nye kulturstudier [New Cultural Studies] is the first introduction to cultural studies in Scandinavia and an impressive presentation of the subject. The book aims to explain how cultural studies emerged as an interdisciplinary field in humanities and social sciences. Other introductions to cultural research in eth-nology and anthropology have been produced - but this one is different, since it is more comprehensive and am-bitious. Nye kulturstudier is the result of in-terdisciplinary collaboration between four colleagues from Norway, Sweden and Denmark. Senior lecturer Anne Scott Sørensen and Professor Ole Martin Høystad are affiliated to the Institute for Literature, Media and Cultural Studies at the University of Southern Denmark in Odense. Professor Erling Bjurström belongs to Tema Q at Linköping Uni-versity, and Professor Halvard Vike works at the Institute for Social Anthro-pology at Oslo University. The authors comment that they are oriented towards different subjects and educational pro-grammes at their respective universities. The book begins with a background to the theories and scientific traditions. This is followed by Cultural Analysis and Methodology, a chapter on Identity, Globalisation and Multiculturalism, one on Taste, Lifestyle and Consumption and, finally, by Nature, Body and Ex-perience Landscapes.

  10. Incidence of Old World screw-worm fly, Chrysomya bezziana in Iraq

    International Nuclear Information System (INIS)

    Al-Taweel, Ayad A.; Al-Izzi, Mohammed A.J.; Jassim, Fadhil A.

    2000-01-01

    The Old World screw-worm fly (OWSWF), Chrysomya bezziana Villenuve, is a member of the insect family Calliphoridae and is an obligate parasite of warm-blooded animals in the tropics and sub-tropics (Norris and Murray 1964). Flies lay their eggs on the edge of wounds or body orifices; the resulting larvae invade the host tissues and produce lesions and infertility if the genitals become infested (Humphrey et al. 1980). Recorded hosts include cattle (Bos indicus), sheep (Ovis aries), goats (Caprus hircus), dogs (Canis familiaris), cats (Felis domesticus) and man (Homo sapiens) (Patton 1920, 1922, Stoddar and Peck 1962, Norris and Murray 1964). This investigation describes the incidence of myiasis caused by C. bezziana in Iraq from September 1996 to March 1998

  11. Virginia Tech Student Support

    National Research Council Canada - National Science Library

    Shende, Aproova; Nair, Arun; Jiang, Wen; Das, Kaushik; Ramesh, Kaliat; Hemker, Kevin; Igusa, Takeru; Marzouk, Osama; Young, Amanda; Lear, Matthew

    2008-01-01

    .... higher education has faced. In an attempt to help its community cope with this heartbreaking event, the university administration decided to close Norris Hall, the site of many of the shootings...

  12. Editorial | Scott | Ergonomics SA

    African Journals Online (AJOL)

    Ergonomics SA. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 23, No 1 (2011) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register. DOWNLOAD FULL TEXT Open Access DOWNLOAD FULL TEXT ...

  13. FLOODPLAIN, SCOTT COUNTY, IOWA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Floodplain Mapping/Redelineation study deliverables depict and quantify the flood risks for the study area. The primary risk classifications used are the...

  14. Persistent hyperplastic primary vitreous: congenital malformation of the eye.

    Science.gov (United States)

    Shastry, Barkur S

    2009-12-01

    Persistent hyperplastic primary vitreous (PHPV), also known as persistent fetal vasculature, is a rare congenital developmental malformation of the eye, caused by the failure of regression of the primary vitreous. It is divided into anterior and posterior types and is characterized by the presence of a vascular membrane located behind the lens. The condition can be of an isolated type or can occur with other ocular disorders. Most cases of PHPV are sporadic, but it can be inherited as an autosomal dominant or recessive trait. Inherited PHPV also occurs in several breeds of dogs and cats. In a limited number of cases, Norrie disease and FZD4 genes are found to be mutated in unilateral and bilateral PHPV. These genes when mutated also cause Norrie disease pseudoglioma and familial exudative vitreoretinopathy that share some of the clinical features with PHPV. Mice lacking arf and p53 tumour suppressor genes as well as Norrie disease pseudoglioma and LRP5 genes suggest that these genes are needed for hyaloid vascular regression. These experiments also indicate that abnormalities in normal apoptosis and defects in Wnt signalling pathway may be responsible for the pathogenesis of PHPV. Identification of other candidate genes in the future may provide a better understanding of the pathogenesis of the condition that may lead to a better therapeutic approach and better management.

  15. Factors related to respiration influencing survival and respiratory function in patients with amyotrophic lateral sclerosis: a retrospective study.

    Science.gov (United States)

    Leonardis, L; Dolenc Grošelj, L; Vidmar, G

    2012-12-01

    Various breathing abnormalities (Neurology 2009; 73: 1218) have been proposed as indicators for the introduction of non-invasive positive-pressure ventilation (NIV) in patients with amyotrophic lateral sclerosis (ALS). We were interested in the usefulness of symptoms of respiratory insufficiency and abnormal results of daytime arterial gas analyses (AGA) as predictors of survival and the effect of NIV on respiratory volumes and pressures. Reported symptoms, respiratory subscore of the ALS Functional Rating Scale (ALSFRS-r), Norris scale (Norris-r), and AGA were retrospectively analyzed in 189 ALS patients. Longitudinal follow-up of forced vital capacity (FVC), maximal inspiratory and expiratory pressure (MIP, MEP), and sniff nasal pressure (SNP) were analyzed with regard to the introduction of NIV. Respiratory symptoms were a bad prognostic sign (P = 0.007). Abnormalities in Norris-r, ALSFRS-r, pO(2), pCO(2), and oxygen saturation tended to be associated with a shorter survival, although they were not statistically significant. NIV prolonged survival and reduced the decline in FVC (P = 0.007), MIP, MEP, and SNP (the last three were not statistically significant). Symptoms, abnormal FVC, and AGA do not always coincide, and they can appear in a different sequence. Any respiratory abnormality should prompt the clinician to start discussing NIV with the patient. NIV prolongs survival and improves respiratory function. © 2012 The Author(s) European Journal of Neurology © 2012 EFNS.

  16. Human-Centered Command and Control of Future Autonomous Systems

    Science.gov (United States)

    2013-06-01

    introduce challenges with situation awareness, automation reliance, and accountability (Bainbridge, 1983). If not carefully designed and integrated...into users’ tasks, automation’s costs can quickly outweigh its benefits. A tempting solution to compensate for inherent human cognitive limitations is... Drury & Scott, 2008; Nehme, Scott, Cummings, & Furusho, 2006; Scott & Cummings, 2006). However, there have not been detailed prescriptive task

  17. Towards a more adaptive co - management of natural resources ...

    African Journals Online (AJOL)

    2014-06-01

    Jun 1, 2014 ... being channelled into Madagascar's environmental sector since the mid-1980s, the ...... firmly embedded in Malagasy environmental legislation, there is little potential to ..... UBC Press, British Colombia. Norris, S. 2006.

  18. The Clinical Observation on 10 cases of patients with Hemifacial Spasm Treated by Soyeom Pharmacupuncture at G20(Pungji

    Directory of Open Access Journals (Sweden)

    Jin Heo

    2010-06-01

    Full Text Available Objectives : The main purpose of this research is to evaluate the effect of treatment with Soyeom Pharmacupuncture at G20(Punji for ten patients with hemifacial spasm. Methods : We have treated them with acupuncture treatment and Soyeom Pharmacupuncture at G20(Pungji, and evaluated the effect by Scott`s scale. Results : After treatment, the grades of spasm intensity classified by Scott`s description were improved in 9 cases. Conclusion : This data suggested that Soyeom Pharmacupuncture at G20(Pungji for hemifacial spasm was effective and will be attempted to the patients with it.

  19. Even-aged intensive management: two case histories

    Science.gov (United States)

    Harold M. Klaiber

    1977-01-01

    In 1967 Scott Paper Company merged with the S. D. Warren Company and S. D. Warren became a division of Scott. In 1969 the S. D. Warren timberlands in the Bingham, Maine area were transferred from the Warren Division to the Northeast Operations of Scott. Included in this transfer were approximately 700 acres of tree plantations which had been established in the 1920...

  20. Dicty_cDB: SHK686 [Dicty_cDB

    Lifescience Database Archive (English)

    Full Text Available 27420.1 LscoSEQ7816 Leucosporidium scottii pBluescript (EcoRI-XhoI) Leucosporidium scott...ii cDNA clone LscoSEQ7816, mRNA sequence. 54 3e-07 2 EB021565 |EB021565.1 LscoSEQ13808 Leucosporidium scott...ii pBluescript (EcoRI-XhoI) Leucosporidium scottii cDNA clone LscoSEQ13808, mRNA sequence. 54 3e-07 2 ...EB020998 |EB020998.1 LscoSEQ13129 Leucosporidium scottii pBluescript (EcoRI-XhoI) Leucosporidium scott...ii cDNA clone LscoSEQ13129, mRNA sequence. 54 3e-07 2 EB018390 |EB018390.1 LscoSEQ10064 Leucosporidium scott

  1. Congenital hearing loss. Is CT enough?

    African Journals Online (AJOL)

    Mahmoud Agha

    2014-01-24

    Jan 24, 2014 ... Congenital hearing loss is one of the developmental disorders that may be not clearly .... Alport syndrome, Klippel-Feil, Norrie disease and Waarden- .... Bismuth eye shield was routinely used for all patients (AttenuRad;.

  2. Journal of Genetics | Indian Academy of Sciences

    Indian Academy of Sciences (India)

    Genetic analysis of 55 northern Vietnamese patients with Wilson disease: ..... with familial Norrie disease: bilateral blindness and leucocoria without other deficits ... A novel missense mutation of ADAR1gene in a Chinese family leading to ...

  3. 75 FR 60126 - Performance Review Board Members

    Science.gov (United States)

    2010-09-29

    .... Childress Kenneth Y. Choe Richard M. Church Frank D. Cipolloni H. Westley Clark Henry Claypool Norris W... L. Hurst Jeanne C. Ireland Karen E. Jackson Holli B. Jaffe John S. Jarman George E. Jenkins Alfred C...

  4. Various scoring systems for predicting mortality in Intensive Care Unit

    African Journals Online (AJOL)

    2015-12-07

    Dec 7, 2015 ... Mortality rate was higher in patients admitted from wards other than surgery ... evaluate the predictability of various severity of illness scores, and ..... Livingston BM, MacKirdy FN, Howie JC, Jones R, Norrie JD. Assessment of.

  5. Estimation of the additive and dominance variances in SA Landrace ...

    African Journals Online (AJOL)

    NORRIS

    South African Journal of Animal Science 2006, 36 (4) ... Fuerst (1996) simulated a genetic model with different levels of additive, dominance and additive by additive genetic effects to .... However, a simulation study by Norris et al. (2002) ...

  6. Real-Time Teleguidance of a Non-Surgeon Crew Medical Officer Performing Orthopedic Surgery at the Amundsen-Scott South Pole Station During Winter-Over

    Science.gov (United States)

    Otto, Christian

    2010-01-01

    The Amundsen-Scott South Pole Research station located at the geographic South Pole, is the most isolated, permanently inhabited human outpost on Earth. Medical care is provided to station personnel by a non-surgeon crew medical officer (CMO). During the winter-over period from February to October, the station is isolated, with no incoming or outgoing flights due to severe weather conditions. In late June, four months after the station had closed for the austral winter, a 31 year old meteorologist suffered a complete rupture of his patellar tendon while sliding done an embankment. An evacuation was deemed to be too risky to aircrews due to the extreme cold and darkness. A panel of physicians from Massachusetts General Hospital, Johns Hopkins University and the University of Texas Medical Branch were able to assess the patient remotely via telemedicine and agreed that surgery was the only means to restore mobility and prevent long term disability. The lack of a surgical facility and a trained surgical team were overcome by conversion of the clinic treatment area, and intensive preparation of medical laypersons as surgical assistants. The non-surgeon CMO and CMO assistant at South Pole, were guided through the administration of spinal anesthetic, and the two-hour operative repair by medical consultants at Massachusetts General Hospital. Real-time video of the operative field, directions from the remote consultants and audio communication were provided by videoconferencing equipment, operative cameras, and high bandwidth satellite communications. In real-time, opening incision/exposure, tendon relocation, hemostatsis, and operative closure by the CMO was closely monitored and guided and by the remote consultants. The patient s subsequent physical rehabilitation over the ensuing months of isolation was also monitored remotely via telemedicine. This was the first time in South Pole s history that remote teleguidance had been used for surgery and represents a model for

  7. Tropospheric ozone annual variation and possible troposphere-stratosphere coupling in the Arctic and Antarctic as derived from ozone soundings at Resolute and Amundsen-Scott stations

    Energy Technology Data Exchange (ETDEWEB)

    Gruzdev, A.N.; Sitnov, S.A. (Russian Academy of Sciences, Moscow (Russian Federation). Inst. of Atmospheric Physics)

    1993-01-01

    The tropospheric ozone annual variation in the northern and southern polar regions is analyzed from ozone sounding data obtained at Resolute during a 15-year period and Amundsen-Scott during a 7-year period. The phase of ozone annual variation above Resolute changes (increases) gradually from the stratosphere across the tropopause to the middle troposphere. Unlike this, the phase of the Antarctic ozone annual harmonic has a discontinuity in the layer of the changing tropopause level, so that the annual harmonic in the upper troposphere, lower stratosphere is 4-to-5 months out of phase (earlier) to that above and beneath. Above both the Arctic and Antarctic stations, the ozone mixing ratio and its vertical gradient evolve in a similar manner in the wide layer from the lower stratosphere to the middle troposphere. This likely points out that ozone in this layer is controlled from above. An indication of the stratospheric-tropospheric ozone exchange above Resolute is noted from mid-winter to spring. The analysis of columnar tropospheric ozone changes gives a lower estimate of the cross-tropopause ozone flux up to 5x10[sup 10] mol cm[sup -2] s[sup -1]. Above the South Pole, the cross-tropopause ozone flux is not usually large. There is also some evidence that early in the spring, when the stratospheric ozone 'hole' is developed, the stratospheric-tropospheric exchange conducts the influence of the 'hole' into the upper troposphere, where the integrated ozone destruction is estimated to be 8x10[sup 10] mol cm[sup -2] s[sup -1]. Correlation analysis gives no ozone-tropopause correlation in the Antarctic in winter, while in other seasons as well as during all seasons in the Arctic, there are negative correlation peaks just above the tropopause. (19 refs., 6 figs.).

  8. Calculations of void swelling in Type 316 stainless steel after a temperature change using the VS8 code

    International Nuclear Information System (INIS)

    Windsor, M.E.; Matthews, J.R.

    1985-06-01

    The report compares measurements made by Norris and Buswell of void swelling in irradiated Type 316 steel after a temperature change from 475 to 575 C, and vice versa, with calculated swelling using the VS8 FACSIMILE code. (author)

  9. 76 FR 5405 - NASA Advisory Council; Science Committee; Astrophysics Subcommittee; Meeting

    Science.gov (United States)

    2011-01-31

    ... Committee; Astrophysics Subcommittee; Meeting AGENCY: National Aeronautics and Space Administration. ACTION... amended, the National Aeronautics and Space Administration (NASA) announces a meeting of the Astrophysics... contacting Marian Norris. The agenda for the meeting includes the following topics: --Astrophysics Division...

  10. 76 FR 35474 - Colville Indian Plywood and Veneer, Colville Tribal Enterprise Corporation Wood Products Division...

    Science.gov (United States)

    2011-06-17

    ... Logging, Mccuen Jones, San Poil Logging, Scott Thorndike, Silver Nichol Trucking and Stensgar Logging..., Mawdsley Logging, McCuen Jones, San Poil Logging, Scott Thorndike, Silver Nichol Trucking and Stensgar...

  11. Mina ka olen Ameerika / Michele Norris

    Index Scriptorium Estoniae

    Norris, Michele

    2016-01-01

    Washingtoni uus riiklik afroameerika ajaloo ja kultuuri muuseum ning selle mitmekülgne kollektsioon näitavad afroameeriklaste kannatuste, visaduse ja triumfide isiklikku poolt. Muuseumi peakuraatoriks on Rex Ellis

  12. Possible words and fixed stress in the segmentation of Slovak speech

    NARCIS (Netherlands)

    Hanuliková, A.; McQueen, J.M.; Mitterer, H.A.

    2010-01-01

    The possible-word constraint (PWC; Norris, McQueen, Cutler, & Butterfield, 1997) has been proposed as a language-universal segmentation principle: Lexical candidates are disfavoured if the resulting segmentation of continuous speech leads to vowelless residues in the input—for example, single

  13. Agyepong et al., Afr J Tradit Complement Altern Med. (2014) 11(3 ...

    African Journals Online (AJOL)

    cadewumi

    As a result of indiscriminate use of antibiotics in the treatment of these ... been found in the stem bark (Chakraborty et al., 1995), and essential oils including β- ... then powdered using laboratory mill machine (Type 8, Christy and Norris Limited, ...

  14. Jogging forward, thinking back

    OpenAIRE

    Raboteau, Albert Jordy, III

    2009-01-01

    Kristina Anderson was an avid jogger before she was wounded in Norris Hall on April 16, 2007. She needed rehabilitation to walk again, much less to run. But she is jogging regularly again now, moving forward with her life without forgetting.

  15. Language-universal constraints on speech segmentation

    NARCIS (Netherlands)

    Norris, D.; McQueen, J.M.; Cutler, A.; Butterfield, S.; Kearns, R.K.

    2001-01-01

    Two word-spotting experiments are reported that examine whether the Possible-Word Constraint (PWC; Norris, McQueen, Cutler & Butterfield, 1997) is a language-specific or language-universal strategy for the segmentation of continuous speech. The PWC disfavors parses which leave an impossible residue

  16. Is APOE ε3 a favourable factor for the longevity: an association study ...

    Indian Academy of Sciences (India)

    2011-08-19

    Aug 19, 2011 ... ents lived for more than 90 years of age were excluded from this study. .... frequent in elderly individuals and their offspring than in controls, and that this was .... Norris C. et al. 2007 A comprehensive view of sex-specific issues.

  17. Engineer. The Professional Bulletin of Army Engineers. Volume 42. May-August 2012

    Science.gov (United States)

    2012-08-01

    Olsen The Extraordinary Career of Lieutenant General Frank A. Camm— Three Tenets for Building Great Engineers Norris Bradbury stands next to a...successful. Three departments were organized to teach mathematics, English grammar, French, Spanish , his- tory, law, geography, and penmanship.9 Although

  18. Manganese Health Research Program (MHRP)

    Science.gov (United States)

    2008-01-01

    to Prion 21 Klein Bradley Dept. of Biomedical Sciences & Pathobiology College of Veterinary Medicine Virginia Tech, Blacksburg, VA...PhD Struve, Melanie , BS Norris, Amy, PhD Higgins, Alan, PhD 5f. WORK UNIT NUMBER 8. PERFORMING ORGANIZATION REPORT NUMBER 7

  19. 78 FR 20358 - NASA Advisory Council; Science Committee; Heliophysics Subcommittee; Meeting

    Science.gov (United States)

    2013-04-04

    ... 10 working days prior to the meeting: full name; gender; date/place of birth; citizenship; visa... --Heliophysics Strategic Objectives and Performance Goals Science Mission Directorate Science Plan --Heliophysics... working days prior to the meeting to Marian Norris. Patricia D. Rausch, Advisory Committee Management...

  20. Most Recent Sampling Results for Annex III Building

    Science.gov (United States)

    Contains email from Scott Miller, US EPA to Scott Kramer. Subject: Most Recent Sampling Results for Annex III Building. (2:52 PM) and Gore(TM) Surveys Analytical Results U.S. Geological Survey, Montgomery, AL.

  1. Väärt klassikast tehti neegrite räpparilugu / Andres Laasik

    Index Scriptorium Estoniae

    Laasik, Andres, 1960-2016

    2005-01-01

    Francis Scott Fitzgeraldi romaani "Suur Gatsby" ekraniseering "G" : stsenarist ja režissöör Christopher Scott Sherot : operaator Horaciop Marquines : Ameerika Ühendriigid 2002. Film "tõlgib" teose ameerika populaarse neegrikultuuri keelde

  2. ''The ambipolar diffusion time scale and the location of star formation in magnetic interstellar clouds'': Setting the record straight

    International Nuclear Information System (INIS)

    Mouschovias, T.C.

    1984-01-01

    The point of a recent (1983) paper by Scott is that a previous paper (1979) by Mouschovias has concluded ''erroneously'' that star formation takes place off center in a cloud because of the use of an ''improver'' definition of a time scale for ambipolar diffusion. No such conclusion, Scott claims, follows from a ''proper'' definition, such as the ''traditional'' one by Spitzer. (i) Scott misrepresents the reasoning that led to the conclusion in the paper which he criticized. (ii) He is also wrong: both the ''traditional'' and the ''improper'' definitions vary similarly with radius, and both can have an off-center minimum; the spatial variation of the degree of ionization is the determining factor: not the specific value of the time scale at the origin, as Scott claims

  3. AC power losses in Bi-2223/Ag HTS tapes

    International Nuclear Information System (INIS)

    Savvides, N.; Reilly, D.; Mueller, K.-H.; Herrmann, J.

    1998-01-01

    Full text: We report measurements at 77 K of the transport ac losses of Bi-2223/Ag composite tapes. The investigated tapes vary from single filament to multifilament construction and include both conventional tapes and other conductor shapes with twisted filaments. The self-field ac losses were determined at 77 K and 60 Hz as a function of ac current amplitude (0 - 100 A). We observe different behaviour among tapes depending on their quality and strain history. For 'good' virgin tapes the experimental data are well described by the Norris equations for the dependence of power loss P on the amplitude I m of the transport current. The data of good monofilament tapes are fitted to the Norris equation P ∼ I m n for an elliptical cross section (ie. n = 3) and the data of good multifilament tapes are fitted to the Norris equation for a rectangular strip (ie. n = 4). Many specimens, however, show a range of behaviour with lower values of n. Based on our work on the effect of strain on the dc transport properties of tapes, we carried out detailed investigations of the effect of controlled applied bend strain on the ac loss. Our results show that irreversible damage to superconducting filaments (ie. cracks) cause the ac loss to rise and n to decrease with increasing strain. In addition, applied strains much greater than the irreversible strain limit cause the ac loss to increase by several orders of magnitude and become ohmic in character with n = 2. Theoretical work is in progress to model the observed behaviour

  4. Supra-subduction zone extensional magmatism in Vermont and adjacent Quebec: Implications for early Paleozoic Appalachian tectonics

    Science.gov (United States)

    Kim, J.; Coish, R.; Evans, M.; Dick, G.

    2003-01-01

    Metadiabasic intrusions of the Mount Norris Intrusive Suite occur in fault-bounded lithotectonic packages containing Stowe, Moretown, and Cram Hill Formation lithologies in the northern Vermont Rowe-Hawley belt, a proposed Ordovician arc-trench gap above an east-dipping subduction zone. Rocks of the Mount Norris Intrusive Suite are characteristically massive and weakly foliated, have chilled margins, contain xenoliths, and have sharp contacts that both crosscut and are parallel to early structural fabrics in the host metasedimentary rocks. Although the mineral assemblage of the Mount Norris Intrusive Suite is albite + actinolite + epidote + chlorite + calcite + quartz, intergrowths of albite + actinolite are probably pseudomorphs after plagioclase + clinopyroxene. The metadiabases are subalkaline, tholeiitic, hypabyssal basalts with preserved ophitic texture. A backarc-basin tectonic setting for the intrusive suite is suggested by its LREE (light rare earth element) enrichment, negative Nb-Ta anomalies, and Ta/Yb vs. Th/Yb trends. Although no direct isotopic age data are available, the intrusions are broadly Ordovician because their contacts are clearly folded by the earliest Acadian (Silurian-Devonian) folds. Field evidence and geochemical data suggest compelling along-strike correlations with the Coburn Hill Volcanics of northern Vermont and the Bolton Igneous Group of southern Quebec. Isotopic and stratigraphic age constraints for the Bolton Igneous Group bracket these backarc magmas to the 477-458 Ma interval. A tectonic model that begins with east-dipping subduction and progresses to outboard west-dipping subduction after a syncollisional polarity reversal best explains the intrusion of deformed metamorphosed metasedimentary rocks by backarc magmas.

  5. HYDRAULICS, SCOTT COUNTY, KENTUCKY, USA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Recent developments in digital terrain and geospatial database management technology make it possible to protect this investment for existing and future projects to...

  6. FLOODPLAIN, SCOTT COUNTY, KENTUCKY USA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Floodplain Mapping/Redelineation study deliverables depict and quantify the flood risks for the study area. The primary risk classifications used are the...

  7. FLOODPLAIN, SCOTT COUNTY, MISSOURI, USA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  8. TERRAIN, SCOTT COUNTY, KENTUCKY USA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Terrain data, as defined in FEMA Guidelines and Specifications, Appendix N: Data Capture Standards, describes the digital topographic data that was used to create...

  9. Search Results | Page 853 | IDRC - International Development ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Scott Gilmore. Scott is a social entrepreneur and writer. He is a columnist for Maclean's magazine and the President of Anchor Chain, which helps small businesses in frontier markets connect to investment and international supply chains. Profile.

  10. Search Results | Page 854 | IDRC - International Development ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Scott Gilmore. Scott is a social entrepreneur and writer. He is a columnist for Maclean's magazine and the President of Anchor Chain, which helps small businesses in frontier markets connect to investment and international supply chains. Profile.

  11. Monitoring gas and heat emissions at Norris Geyser Basin, Yellowstone National Park, USA based on a combined eddy covariance and Multi-GAS approach

    Science.gov (United States)

    Lewicki, J. L.; Kelly, P. J.; Bergfeld, D.; Vaughan, R. G.; Lowenstern, J. B.

    2017-11-01

    We quantified gas and heat emissions in an acid-sulfate, vapor-dominated area (0.04-km2) of Norris Geyser Basin, located just north of the 0.63 Ma Yellowstone Caldera and near an area of anomalous uplift. From 14 May to 3 October 2016, an eddy covariance system measured half-hourly CO2, H2O and sensible (H) and latent (LE) heat fluxes and a Multi-GAS instrument measured (1 Hz frequency) atmospheric H2O, CO2 and H2S volumetric mixing ratios. We also measured soil CO2 fluxes using the accumulation chamber method and temperature profiles on a grid and collected fumarole gas samples for geochemical analysis. Eddy covariance CO2 fluxes ranged from - 56 to 885 g m- 2 d- 1. Using wavelet analysis, average daily eddy covariance CO2 fluxes were locally correlated with average daily environmental parameters on several-day to monthly time scales. Estimates of CO2 emission rate from the study area ranged from 8.6 t d- 1 based on eddy covariance measurements to 9.8 t d- 1 based on accumulation chamber measurements. Eddy covariance water vapor fluxes ranged from 1178 to 24,600 g m- 2 d- 1. Nighttime H and LE were considered representative of hydrothermal heat fluxes and ranged from 4 to 183 and 38 to 504 W m- 2, respectively. The total hydrothermal heat emission rate (H + LE + radiant) estimated for the study area was 11.6 MW and LE contributed 69% of the output. The mean ± standard deviation of H2O, CO2 and H2S mixing ratios measured by the Multi-GAS system were 9.3 ± 3.1 parts per thousand, 467 ± 61 ppmv, and 0.5 ± 0.6 ppmv, respectively, and variations in the gas compositions were strongly correlated with diurnal variations in environmental parameters (wind speed and direction, atmospheric temperature). After removing ambient H2O and CO2, the observed variations in the Multi-GAS data could be explained by the mixing of relatively H2O-CO2-H2S-rich fumarole gases with CO2-rich and H2O-H2S-poor soil gases. The fumarole H2O/CO2 and CO2/H2S end member ratios (101.7 and 27

  12. Monitoring gas and heat emissions at Norris Geyser Basin, Yellowstone National Park, USA based on a combined eddy covariance and Multi-GAS approach

    Science.gov (United States)

    Lewicki, Jennifer L.; Kelly, Peter; Bergfeld, Deborah; Vaughan, R. Greg; Lowenstern, Jacob B.

    2017-01-01

    We quantified gas and heat emissions in an acid-sulfate, vapor-dominated area (0.04-km2) of Norris Geyser Basin, located just north of the 0.63 Ma Yellowstone Caldera and near an area of anomalous uplift. From 14 May to 3 October 2016, an eddy covariance system measured half-hourly CO2, H2O and sensible (H) and latent (LE) heat fluxes and a Multi-GAS instrument measured (1 Hz frequency) atmospheric H2O, CO2 and H2S volumetric mixing ratios. We also measured soil CO2 fluxes using the accumulation chamber method and temperature profiles on a grid and collected fumarole gas samples for geochemical analysis. Eddy covariance CO2 fluxes ranged from − 56 to 885 g m− 2 d− 1. Using wavelet analysis, average daily eddy covariance CO2 fluxes were locally correlated with average daily environmental parameters on several-day to monthly time scales. Estimates of CO2emission rate from the study area ranged from 8.6 t d− 1 based on eddy covariance measurements to 9.8 t d− 1 based on accumulation chamber measurements. Eddy covariance water vapor fluxes ranged from 1178 to 24,600 g m− 2 d− 1. Nighttime H and LEwere considered representative of hydrothermal heat fluxes and ranged from 4 to 183 and 38 to 504 W m− 2, respectively. The total hydrothermal heat emission rate (H + LE + radiant) estimated for the study area was 11.6 MW and LE contributed 69% of the output. The mean ± standard deviation of H2O, CO2 and H2S mixing ratios measured by the Multi-GAS system were 9.3 ± 3.1 parts per thousand, 467 ± 61 ppmv, and 0.5 ± 0.6 ppmv, respectively, and variations in the gas compositions were strongly correlated with diurnal variations in environmental parameters (wind speed and direction, atmospheric temperature). After removing ambient H2O and CO2, the observed variations in the Multi-GAS data could be explained by the mixing of relatively H2O-CO2-H2S-rich fumarole gases with CO2-rich and H2O-H2S-poor soil gases. The

  13. Search Results | Page 850 | IDRC - International Development ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Scott Gilmore. Scott is a social entrepreneur and writer. He is a columnist for Maclean's magazine and the President of Anchor Chain, which helps small businesses in frontier markets connect to investment and international supply chains. Profile ...

  14. Bob Scott: Tallinn 2011 saab oma preemiaraha kätte / Bob Scott ; intervjueerinud Teele Tammeorg

    Index Scriptorium Estoniae

    Scott, Bob

    2010-01-01

    Euroopa kultuuripealinnade hindamiskomisjoni esimees räägib oma kohtumisest Tallinna linnapea Edgar Savisaare ja kultuuriministri Laine Jänesega ning sellest, et Tallinnal ja Eestil tuleb lisatoetuse saamiseks saata Euroopa Komisjonile vaid kiri, milles nad kinnitavad, et kultuuripealinna programmi jaoks on raha olemas

  15. Multivariate density estimation theory, practice, and visualization

    CERN Document Server

    Scott, David W

    2015-01-01

    David W. Scott, PhD, is Noah Harding Professor in the Department of Statistics at Rice University. The author of over 100 published articles, papers, and book chapters, Dr. Scott is also Fellow of the American Statistical Association (ASA) and the Institute of Mathematical Statistics. He is recipient of the ASA Founder's Award and the Army Wilks Award. His research interests include computational statistics, data visualization, and density estimation. Dr. Scott is also Coeditor of Wiley Interdisciplinary Reviews: Computational Statistics and previous Editor of the Journal of Computational and

  16. Thermomagnetic torques in polyatomic gases

    Science.gov (United States)

    Hildebrandt, A. F.; Wood, C. T.

    1972-01-01

    The application of the Scott effect to the dynamics of galactic and stellar rotation is investigated. Efforts were also made to improve the sensitivity and stability of torque measurements and understand the microscopic mechanism that causes the Scott effect.

  17. Why not model spoken word recognition instead of phoneme monitoring?

    NARCIS (Netherlands)

    Vroomen, J.; de Gelder, B.

    2000-01-01

    Norris, McQueen & Cutler present a detailed account of the decision stage of the phoneme monitoring task. However, we question whether this contributes to our understanding of the speech recognition process itself, and we fail to see why phonotactic knowledge is playing a role in phoneme

  18. Effects of reaction temperature on size and optical properties of ...

    Indian Academy of Sciences (India)

    Administrator

    influential factors in shape control of CdSe nanocrystals by changing the ratio of .... four different temperatures (200, 220, 240 and 280°C). During the whole .... J, Wu A M, Gambhir S S and Weiss S 2005 Science 307 538. Murray C B, Norris ...

  19. The Case of the Missing Cyanogen-rich AGB Stars in Galactic Globular Clusters

    DEFF Research Database (Denmark)

    Campbell, S. W.; Yong, D.; Wylie-de Boer, E. C.

    2012-01-01

    The handful of available observations of AGB stars in Galactic Globular Clusters suggest that the GC AGB populations are dominated by cyanogen-weak stars (eg. Norris et al. 1981; Sneden et al. 2000). This contrasts strongly with the distributions on the RGB (and other) populations, which generall...

  20. The Florida Prostate Cancer Research Training Opportunities for Outstanding Leaders (ReTOOL (registered trademark)) Program: Creating Opportunities for Minority HBCU Students

    Science.gov (United States)

    2013-03-01

    underserved. (MA Haynes & BD Smedley (Eds). National Academy Press, Washington DC. 2. Norris KC, Agodoa LY (2005). The need for health professionals... Robert Vessella, PhD University of Washington Medical Center 44 10:30 – 10:45 am S-10 A Targeted Approach to Prostate Radiotherapy Alan

  1. The Role of the Capase-8 Inhibitor FLIP in Androgen-Withdrawal Induced Death of Prostate Epithelium

    Science.gov (United States)

    2006-01-01

    DL, Norris JS. Resistance of prostate cancer cells to soluble TNF- related apoptosis- inducing ligand (TRAIL/Apo2L) can be overcome by doxorubicin or...Bueso-Ramos C, Chatterjee D, Pantazis P, Aggarwal BB. Curcumin downregulates cell survival mechanisms in human prostate cancer cell lines. Oncogene

  2. Wnt signaling: Ig-norrin the dogma.

    Science.gov (United States)

    Clevers, Hans

    2004-06-08

    Secreted Wnt proteins trigger the intracellular Wnt signaling cascade upon engagement of dedicated Frizzled-Lrp receptor complexes. Unexpectedly, a non-Wnt ligand for this receptor complex has now been discovered. This novel ligand, Norrin, is mutated in the hereditary ocular Norrie syndrome. Copyright 2004 Elsevier Ltd.

  3. Sperm whale clicks

    DEFF Research Database (Denmark)

    Møhl, Bertel; Wahlberg, Magnus; Madsen, Peter T.

    2000-01-01

    . A sound generator weighing upward of 10 tons and with a cross-section of 1 m is expected to generate high-intensity, directional sounds. This prediction from the Norris and Harvey theory is not supported by published data for sperm whale clicks ~source levels of 180 dB re 1 mPa and little, if any......In sperm whales ~Physeter catodon L. 1758! the nose is vastly hypertrophied, accounting for about one-third of the length or weight of an adult male. Norris and Harvey @in Animal Orientation and Navigation, NASA SP-262 ~1972!, pp. 397–417# ascribed a sound-generating function to this organ complex......, directionality!. Either the theory is not borne out or the data is not representative for the capabilities of the sound-generating mechanism. To increase the amount of relevant data, a five-hydrophone array, suspended from three platforms separated by 1 km and linked by radio, was deployed at the slope...

  4. [Study on Vis/NIR spectra detecting system for watermelons and quality predicting in motion].

    Science.gov (United States)

    Tian, Hai-Qing; Ying, Yi-Bin; Xu, Hui-Rong; Lu, Hui-Shan; Xie, Li-Juan

    2009-06-01

    To make Vis/NIR diffuse transmittance technique applied to quality prediction for watermelon in motion, the dynamic spectra detecting system was rebuilt. Spectra detecting experiments were conducted and the effects of noises caused by motion on spectra were analyzed. Then the least--square filtering method and Norris differential filtering method were adopted to eliminate the effects of noise on spectra smoothing, and statistical models between the spectra and soluble solids content were developed using partial least square method. The performance of different models was assessed in terms of correlation coefficients (r) of validation set of samples, root mean square errors of calibration (RMSEC) and root mean square errors of prediction (RMSEP). Calibration and prediction results indicated that Norris differential method was an effective method to smooth spectra and improve calibration and prediction results, especially, with r of 0.895, RMSEC of 0.549, and RMSEP of 0.760 for the calibration and prediction result of the first derivative spectra.

  5. Search Results | Page 853 | IDRC - International Development ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Results 8521 - 8530 of 8531 ... Scott Gilmore. Scott is a social entrepreneur and writer. He is a columnist for Maclean's magazine and the President of Anchor Chain, which helps small businesses in frontier markets connect to investment and international supply chains.

  6. ‘Shattered glass’: Assessing the influence of mass media on legitimacy and entrepreneurs’ adoption of new organizational practices

    NARCIS (Netherlands)

    Kuijpers, Johannes Cornelis; Ehrenhard, Michel Léon; Groen, Aard

    2017-01-01

    Legitimacy defined as a generalized assumption of desirability or appropriateness of an action or idea (Ashford & Gibbs, 1990; Suchman, 1995) is argued to play an important role in the maintenance and change of organizations and institutions (Scott, 2008; Scott, Ruef, Mendel, & Caronna, 2000).

  7. Africa Development - Vol 36, No 1 (2011)

    African Journals Online (AJOL)

    Can It be Achieved? Partnering Towards Improving Livelihoods in the Ganspan Settlement, Norther n Cape Province, South Africa · EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT. D Norris, EC Van Zyl, WT Hashe, MS Mafuma, MP Senyolo, SP Mngomezulu, ...

  8. A Game of Simon Says: Latin America’s Left Turn and Its Effects on US Security

    Science.gov (United States)

    2008-05-01

    Fred P. Stone, Lt Col, PhD, Director of Research John T. Ackerman, PhD, Series Editor Kathleen Mahoney-Norris, PhD, Essay Advisor Air University...lopsided than even the lyrics from the 966 Beatles’ song “Taxman” envisioned. Alvaro Hurtado of the Interna- tional Labour Organization states that

  9. Chronic kidney disease in sub-Saharan Africa: Hypothesis for ...

    African Journals Online (AJOL)

    The world's disease profile is changing and chronic ... morbidity and mortality in the world, accounts for ... contribute substantially to the disparate burden ... the year 2030, more than 70% of patients with ... 2002. New York: National Kidney Foundation; 2002. 7. Pugsley D, Norris KC, ... and projections to the year 2010.

  10. A novel contiguous deletion involving NDP, MAOB and EFHC2 gene ...

    Indian Academy of Sciences (India)

    Home; Journals; Journal of Genetics; Volume 96; Issue 6. A novel contiguous deletion involving NDP, MAOBand EFHC2gene in a patient with familial Norrie disease: bilateral blindness and leucocoria without other deficits. BEI JIA LIPING HUANG YAOYU CHEN SIPING LIU CUIHUA CHEN KE XIONG LANLIN SONG YULAI ...

  11. CONSTANTINE AND CHRISTIANITY: THE FORMATION OF ...

    African Journals Online (AJOL)

    STM

    As a result of Constantine‟s policies, the Christian Church and the Roman .... public organization, thus paving the way for the Catholic .... time, however, church teachings were being integrated into the state .... partner with him in controlling the empire. .... Charles Norris Cochrane, Christianity and Classical Culture, 186. 23.

  12. Author Details

    African Journals Online (AJOL)

    Feeley, A B. Vol 8, No 3 (2014) - Articles Added sugar and dietary sodium intake from purchased fast food, confectionery, sweetened beverages and snacks among Sowetan adolescents A B Feeley, PhD; S A Norris, PhD Medical Research Council/Wits Developmental Pathways for Health Research Unit, Departm

  13. China and Japan’s Strategic Nuclear Relationship

    Science.gov (United States)

    2009-09-01

    Nuclear Numerology Chinese Style,” Arms Control Today (March, 2005); Robert S. Norris, “Chinese Nuclear Forces 2008,” Bulletin of the Atomic...Bulletin of the Atomic Scientists, May/June. ———. 2005. “Letters to the Editor: Nuclear Numerology Chinese Style,” Arms Control Today, March

  14. A novel contiguous deletion involving NDP, MAOB and EFHC2 gene ...

    Indian Academy of Sciences (India)

    Home; Journals; Journal of Genetics; Volume 96; Issue 6. A novel contiguous deletion involving NDP, MAOBitalic> and EFHC2italic> gene in a patient with familial Norrie disease: bilateral blindness and leucocoria without other deficits. BEI JIA LIPING HUANG YAOYU CHEN SIPING LIU CUIHUA CHEN KE XIONG LANLIN ...

  15. 75 FR 33837 - NASA Advisory Council; Science Committee; Astrophysics Subcommittee; Meeting

    Science.gov (United States)

    2010-06-15

    ... than 10 working days prior to the meeting: Full name; gender; date/ place of birth; citizenship; visa... following topics: --Astrophysics Division Update --Ethics Briefing --Government Performance and Results Act... information 3 working days in advance by contacting Marian Norris via e-mail at [email protected] or by...

  16. Evaluation of the evolving stress field of the Yellowstone volcanic plateau, 1988 to 2010, from earthquake first-motion inversions

    Science.gov (United States)

    Russo, E.; Waite, G. P.; Tibaldi, A.

    2017-03-01

    Although the last rhyolite eruption occurred around 70 ka ago, the silicic Yellowstone volcanic field is still considered active due to high hydrothermal and seismic activity and possible recent magma intrusions. Geodetic measurements document complex deformation patterns in crustal strain and seismic activity likewise reveal spatial and temporal variations in the stress field. We use earthquake data recorded between 1988 and 2010 to investigate these variations and their possible causes in more detail. Earthquake relocations and a set of 369 well-constrained, double-couple, focal mechanism solutions were computed. Events were grouped according to location and time to investigate trends in faulting. The majority of the events have normal-faulting solutions, subordinate strike-slip kinematics, and very rarely, reverse motions. The dominant direction of extension throughout the 0.64 Ma Yellowstone caldera is nearly ENE, consistent with the perpendicular direction of alignments of volcanic vents within the caldera, but our study also reveals spatial and temporal variations. Stress-field solutions for different areas and time periods were calculated from earthquake focal mechanism inversion. A well-resolved rotation of σ3 was found, from NNE-SSW near the Hebgen Lake fault zone, to ENE-WSW near Norris Junction. In particular, the σ3 direction changed throughout the years around Norris Geyser Basin, from being ENE-WSW, as calculated in the study by Waite and Smith (2004), to NNE-SSW, while the other σ3 directions are mostly unchanged over time. The presence of ;chocolate tablet; structures, with two sets of nearly perpendicular normal faults, was identified in many stages of the deformation history both in the Norris Geyser Basin area and inside the caldera.

  17. Development and Evaluation of New Algorithms for the Retrieval of Wind and Internal Wave Parameters from Shipborne Marine Radar Data

    Science.gov (United States)

    2012-12-01

    marine radar to survey ocean waves close to the spanish coast. In Proc. of the WMO/IOC Workshop on Operational Ocean Monitoring using Surface Based...Linear feature detection and enhancement in noisy images via the Radon transform. Pattern Recognit. Lett., 4(4):279–284, 1986. [79] A. Norris . The

  18. Overcoming Degraded Communications under A2AD: A Doctrinal Solution

    Science.gov (United States)

    2014-05-15

    LCDR Travis K. Suggs, USN 5e. TASK NUMBER Paper Advisor: Prof. Patrick Sweeney, Ph.D.& CAPT Andrew Norris , USCG 5f. WORK UNIT NUMBER...Newport, RI, 2010),.9. 6 war syndrome .” 16 The Joint Force is slowly recovering from nearly a decade of combat operations in Iraq and Afghanistan

  19. Põhjamaade arhitektuuri noorem põlvkond

    Index Scriptorium Estoniae

    1997-01-01

    17. oktoobrist 1997. a. Rotermanni soolalaos Soome ja Eesti Arhitektuurimuuseumi koostööna avatud Põhjamaade väljapanek "Northern Factor : The New Generation of the North" 1996. a. Veneetsia biennaalilt. Kuraator - M.-R. Norri. Tema loengust 23. oktoobril. Toimus Oulu arhitektide Ulla ja Lasse Vahtera tööde esitlus

  20. Proceedings of the Second International Congress on Recent Developments in Air- and Structure-Borne Sound and Vibration (2nd) Held in Auburn University, Alabama on 4-6 March 1992. Volume 2

    Science.gov (United States)

    1992-03-06

    METHOD OF SOUND FIELDS IN INDUSTRIAL HALLS .................. 1669 V.L Ledenyov and A.L Antonov, Tambov Institute of Chemical Machine Building, Russia...1163 Njunin, Boris N., 667 Lebedeva, I.V., 1327 Norris, Andrew, 753 Ledenyov , V.I., 1669 Norton, M.P., 621 Lee, Gilbert F., 763 Novikov, 1.1., 725

  1. Teatro-cine Eileen Norris - California (EE.UU.

    Directory of Open Access Journals (Sweden)

    Martin, Albert C.

    1978-10-01

    Full Text Available This building forms a part of the substructure of the Department of the Cinema of the University of Southern California. Among its various functions, it includes the practical teaching of cinematography. It consists of a ground floor for accesses and auditorium, with a surface measuring 1,950 m2, and a basement of the same extension, designed for a film library and film deposit. The auditorium has a capacity for 350 spectators and is equipped with a screen adaptable to the projection of 16, 35 and 70 mm films, as well as equipment for the projection of slides and video tapes. It can also be conditioned as a lecture hall, for which purpose, complete sets of equipment fort simultaneous translation have been installed. On the outside, the architecture presents a neoclassical design, based on a portico of four large white cement columns, which combines with Marble exteriors on the walls.

    Este edificio forma parte de la infraestructura del Departamento de Cine de la Universidad de California del Sur. Entre sus diversas funciones comprende la enseñanza práctica de la cinematografía. Consta de una planta baja destinada a accesos y auditorio, de 1.950 m2 de superficie, y un sótano, de la misma extensión, destinado a biblioteca de cine y a depósito de películas. El auditorio tiene capacidad para 350 espectadores y cuenta con una pantalla adaptable a la proyección de films de 16, 35 y 70 mm, así como con equipos para la proyección de diapositivas y cintas de video-tapes. Puede acondicionarse, igualmente, como sala de conferencias, para lo cual se han instalado unos completos equipos de traducción simultánea. Exteriormente, la arquitectura presenta un diseño neoclásico, basado en un pórtico de cuatro grandes columnas de cemento blanco que combina con revestimientos de mármol y en los paramentos verticales.

  2. Sosiaal-wetenskaplike kritiese eksegese van Nuwe-Testamentiese ...

    African Journals Online (AJOL)

    UPuser

    Nicht viele Mächtige: Annäherungen an eine Soziologie des. Urchristentums, in Befreiungserfahrungen: Studien zur Sozialgeschichte des. Neuen Testaments, 247-256. München: Kaiser. Scott, B B [1989] 1990. Hear then the parable: A commentary on the parables of. Jesus. Minneapolis, MN: Fortress. Scott, J C 1990.

  3. Champagne og naiv dekadence

    DEFF Research Database (Denmark)

    Bjerre, Thomas Ærvold

    2007-01-01

    Portrætartikel om den amerikanske forfatter F. Scott Fitzgerald i anledningen af den danske udgivelse af De smukke og fortabte. Udgivelsesdato: 24. august......Portrætartikel om den amerikanske forfatter F. Scott Fitzgerald i anledningen af den danske udgivelse af De smukke og fortabte. Udgivelsesdato: 24. august...

  4. Journal of Astrophysics and Astronomy | Indian Academy of Sciences

    Indian Academy of Sciences (India)

    2016-01-27

    Jan 27, 2016 ... We have carried out a Principal Component Analysis (PCA) of the temporal and spectral variables of 24 long-lag, wide-pulse gamma-ray bursts (GRBs) presented by Norris et al. (2005). Taking all eight temporal and spectral parameters into account, our analysis shows that four principal components are ...

  5. Biodiversity and Peace: Where Technology and Montessori Come Together in the Children's Eternal Rainforest, Costa Rica

    Science.gov (United States)

    Norris, Jeff

    2016-01-01

    Jeff Norris, initially shocked by the Montessorians who are calling technology into question, states that technology can offer a means of development for the child who is concurrently supporting and learning from the rich and overpowering biodiversity of the rainforest. He speaks for the Children's Eternal Rainforest citizen's science as well as…

  6. Sample preparation of Medicago sativa L. hay for chemical analysis

    African Journals Online (AJOL)

    UFS Campus

    wavelength region (De Boever et al., 1996; Williams & Norris, 2001). Therefore, it could affect the predicted results of all the other parameters (CP, ADF, NDF, etc.). The grinding ... (September 2006 to May 2007). The samples represented lots that were selected at different stages of maturity. A moisture range as broad as ...

  7. The concept of suggestion in the early history of advertising psychology.

    Science.gov (United States)

    Kuna, D P

    1976-10-01

    As early as 1896, experimental psychologists began studying the mental processes involved in advertising. The first psychological theory of advertising maintained, in effect, that the consumer was a nonrational, suggestible creature under the hypnotic influence of the advertising copywriter. Walter Dill Scott was the major proponent of this theory, and it was largely through his writings that advertising men learned about the psychology of suggestion. Scott's theory was consistent with a growing trend in the advertising profession toward viewing consumer behavior as irrational. Scott's efforts might also be viewed as part of the trend in the advertising profession toward seeking a scientific basis for copywriting theory and practice.

  8. Natural mortality factors for African White-backed Vultures in Namibia?

    African Journals Online (AJOL)

    2007-09-02

    Sep 2, 2007 ... September 2007. Vulture News 57. 63. Figure 2. Lightning damage to the bark of the above camel thorn tree (photographer: Ann Scott). Figure 1. Carcass of an African White-backed Vulture below a nest in a camel thorn tree in the Kalahari Desert, Namibia (photographer: Ann Scott).

  9. Natural mortality factors for African White-backed Vultures in Namibia?

    African Journals Online (AJOL)

    Natural mortality factors for African White-backed Vultures in Namibia? A Scott, M Scott, P Bridgeford, M Bridgeford. Abstract. No Abstract. Vulture News Vol. 57 2007: pp. 62-64. Full Text: EMAIL FREE FULL TEXT EMAIL FREE FULL TEXT · DOWNLOAD FULL TEXT DOWNLOAD FULL TEXT · AJOL African Journals Online.

  10. Journal of Biosciences | Indian Academy of Sciences

    Indian Academy of Sciences (India)

    Home; Journals; Journal of Biosciences. Scott F Gilbert. Articles written in Journal of Biosciences. Volume 26 Issue 3 September 2001 pp 293-298. Commentary: New vistas for developmental biology · Scott F Gilbert Rocky S Tuan · More Details Fulltext PDF. Volume 27 Issue 5 September 2002 pp 445-446. Commentary: ...

  11. Engaging Students Regarding Special Needs in Technology and Engineering Education

    Science.gov (United States)

    White, David W.

    2015-01-01

    In 1984, James Buffer and Michael Scott produced the book "Special Needs Guide for Technology Education" (Buffer and Scott, 1984). This was a pivotal offering insofar as it set the stage for technology education educators, at the time, to think about and be provided with information regarding students with special needs in their…

  12. Seeking a Human Spaceflight Program Worthy of a Great Nation. Review of U.S. Human Spaceflight Plans Committee

    Science.gov (United States)

    2009-10-01

    Steve Metschan George E. Mueller Elon Musk Jack Mustard Clive Neal Scott Neish Benjamin J. Neumann Mike O’Brien Sean O’Keefe John Olson Scott Pace Anatoly...July 2009 Musk , Elon , “COTS Status Update & Crew Capabilities”, SpaceX, 16 June 2009 Musk , Elon , “COTS Status Update & Crew Capabilities”, SpaceX

  13. Sight-Reading Requirements at Concert Band Festivals: A National Survey

    Science.gov (United States)

    Paul, Timothy A.

    2010-01-01

    This study, a replication and extension of work by Norris (2004), examined sight-reading requirements at middle and high school large-group band festivals across the United States. As in the earlier investigation, answers to the following questions were solicited from all 50 states: (1) Are there ratings-based large-group band festivals? (2) Is…

  14. On the Influence of Naturalism on American Literature

    Science.gov (United States)

    Zhang, Xiaofen

    2010-01-01

    Naturalism was first proposed and formulated by French novelist Emile Zola, and it was introduced to America by American novelist Frank Norris. It is a new and harsher realism. It is a theory in literature emphasizing scientific observation of life without idealism or avoidance of the ugly. American literature naturalists dismissed the validity of…

  15. Chemical analyses of waters from geysers, hot springs, and pools in Yellowstone National Park, Wyoming from 1974 to 1978

    Energy Technology Data Exchange (ETDEWEB)

    Thompson, J.M.; Yadav, S.

    1979-01-01

    Waters from geysers, hot springs, and pools of Yellowstone National Park have been analyzed. We report 422 complete major ion analyses from 330 different locations of geysers, hot springs, and pools, collected from 1974 to 1978. Many of the analyses from Upper, Midway, Lower, and Norris Geyser Basin are recollections of features previously reported.

  16. Journal of Astrophysics and Astronomy | Indian Academy of Sciences

    Indian Academy of Sciences (India)

    2016-01-27

    Jan 27, 2016 ... Minnie Y. Mao1 2 3 Rob Sharp2 D. J. Saikia3 4 5 Ray P. Norris3 Melanie Johnston-Hollitt6 Enno Middelberg7 Jim E. J. Lovell1. University of Tasmania, Hobart, 7001, Australia. Australian Astronomical Observatory, Epping, NSW, 1710, Australia. CSIRO Australian Telescope National Facility, Epping, NSW, ...

  17. A novel NDP mutation in an infant with unilateral persistent fetal vasculature and retinal vasculopathy.

    Science.gov (United States)

    Aponte, Elisabeth P; Pulido, Jose S; Ellison, Jay W; Quiram, Polly A; Mohney, Brian G

    2009-06-01

    Mutations in the Norrie Disease gene, Norrie Disease Pseudoglioma (NDP) lead to a phenotypically heterogeneous group of retinopathies. We report a novel mutation in the NDP gene identified in a patient whose clinical presentation was suggestive of unilateral persistent fetal vasculature (PFV). Ophthalmic examinations, ocular ultrasounds and sequence analysis of the exons of the NDP gene on peripheral blood DNA were performed. A four-month-old boy was referred to our institution for presumed unilateral retinoblastoma. The clinical and ultrasonographic exams were consistent with PFV and retinal detachment of the left eye as well as retinal fibrovascular changes in the right eye. A vitrectomy of the left eye revealed the absence of a retrolenticular stalk and mutation analysis of the NDP gene of the proband and mother demonstrated a novel missense mutation at codon 66, designated as c. 196G > A at the cDNA level and E66K at the protein level. We report a novel mutation in the NDP gene in a patient whose presentation demonstrates the phenotypic heterogeneity of NDP-related disorders.

  18. Maternal xNorrin, a canonical Wnt signaling agonist and TGF-β antagonist, controls early neuroectoderm specification in Xenopus.

    Directory of Open Access Journals (Sweden)

    Suhong Xu

    Full Text Available Dorsal-ventral specification in the amphibian embryo is controlled by β-catenin, whose activation in all dorsal cells is dependent on maternal Wnt11. However, it remains unknown whether other maternally secreted factors contribute to β-catenin activation in the dorsal ectoderm. Here, we show that maternal Xenopus Norrin (xNorrin promotes anterior neural tissue formation in ventralized embryos. Conversely, when xNorrin function is inhibited, early canonical Wnt signaling in the dorsal ectoderm and the early expression of the zygotic neural inducers Chordin, Noggin, and Xnr3 are severely suppressed, causing the loss of anterior structures. In addition, xNorrin potently inhibits BMP- and Nodal/Activin-related functions through direct binding to the ligands. Moreover, a subset of Norrin mutants identified in humans with Norrie disease retain Wnt activation but show defective inhibition of Nodal/Activin-related signaling in mesoderm induction, suggesting that this disinhibition causes Norrie disease. Thus, xNorrin is an unusual molecule that acts on two major signaling pathways, Wnt and TGF-β, in opposite ways and is essential for early neuroectoderm specification.

  19. Novolak resin-based negative photoresists for deep UV lithography

    Indian Academy of Sciences (India)

    Administrator

    Presented at First International Conference of Scott Research. Forum, Scott Christian College, Nagercoil, Tamil Nadu, India on 19 April 2008 ..... The exposure was carried out on a DUV stepper operating on Hg-Xe lamp 500 W delivering a dosage of 3⋅2 mJ. Development was done using tetra methyl ammonium hydroxide ...

  20. 75 FR 62591 - Oral Argument

    Science.gov (United States)

    2010-10-12

    ... v. Office of Personnel Management, MSPB Docket Number AT-0731-09-0240-I-1; James A. Scott v. Office... a.m. Place: The United States Court of Appeals for the Federal Circuit, Room 201, 717 Madison Place... of Personnel Management, MSPB Docket Number AT-0731- 09-0240-I-1; James A. Scott v. Office of...

  1. Spectral Lag Evolution among γ-Ray Burst Pulses Lan-Wei Jia1 ...

    Indian Academy of Sciences (India)

    pulses with observations by CGRO/BATSE. No universal spectral lag evolution feature and pulse luminosity-lag relation within a GRB is observed. ... Key words. γ-rays: bursts—spectral lag—GRB pulse. 1. Introduction. It is found that soft photons lag behind the hard photons and is usually seen in long. GRBs (e.g., Norris et ...

  2. Naval Medical Research and Development News. Volume 7, Issue 1, January 2015

    Science.gov (United States)

    2015-01-01

    Laos, Malaysia , Thailand and the Philippines. Vietnam has set a 2030 malaria elimination goal and NAMRU-2 is partnering to identify malaria...death sort of way). Perhaps then, we’ll see true personal growth in ourselves and it will have nothing to do with a scale or the latest diet . From My family to Yours, Allison Norris

  3. Critical Information Literacy as Core Skill for Lifelong STEM Learning in the 21st Century: Reflections on the Desirability and Feasibility for Widespread Science Media Education

    Science.gov (United States)

    Storksdieck, Martin

    2016-01-01

    Grace Reid and the late Stephen Norris argue in this issue the urgent need for widespread Science Media Education (SME) as an integral part of formal and informal science education. SME is to achieve two goals: First, allow learners to critically evaluate any media as a source for scientific information by understanding the socio-economic and…

  4. Environmental Mycobiome Modifiers of Inflammation and Fibrosis in Systemic Sclerosis

    Science.gov (United States)

    2016-09-01

    autoimmune systemic sclerosis and cancer: disease stratification, co-expression networks and genetic polymorphisms” Cancer Mechanisms Program, Norris ...NOTES 14. ABSTRACT This project is focused on Systemic Sclerosis (SSc), a progressive fibrotic disease characterized by skin fibrosis and damage to...quantitative manner. Our studies suggest that disease pathogenesis includes a common environmental fungal trigger, Rhodotorula glutinis, which we

  5. A Simple Method to Find out when an Ordinary Differential Equation Is Separable

    Science.gov (United States)

    Cid, Jose Angel

    2009-01-01

    We present an alternative method to that of Scott (D. Scott, "When is an ordinary differential equation separable?", "Amer. Math. Monthly" 92 (1985), pp. 422-423) to teach the students how to discover whether a differential equation y[prime] = f(x,y) is separable or not when the nonlinearity f(x, y) is not explicitly factorized. Our approach is…

  6. 2006 Chemical Biological Individual Protection (CBIP) Conference and Exhibition

    Science.gov (United States)

    2006-03-09

    Requirements Office (JRO), MAJ W. Scott Smedley , Joint Requirements Office for Chemical, Biological, Radiological, and Nuclear Defense JPEO...Decker Director of Engineering 410-436-5600 www.ecbc.army.mil Gabe Patricio, JPEO 703 681-0808 Robert Wattenbarger, JPMOIP 703 432-3198 Canadian CBRN...UNCLASSIFIED Joint Requirements Office for Chemical, Biological, and Nuclear Defense MAJ W. Scott Smedley 8 March 2006 Individual Protection Conference

  7. Design, Test, and Evaluation of a Transonic Axial Compressor Rotor with Splitter Blades

    Science.gov (United States)

    2013-09-01

    INTRODUCTION A. MOTIVATION Over the course of turbomachinery history splitter vanes have been used extensively in centrifugal compressors . Axial...TEST, AND EVALUATION OF A TRANSONIC AXIAL COMPRESSOR ROTOR WITH SPLITTER BLADES by Scott Drayton September 2013 Dissertation Co...AXIAL COMPRESSOR ROTOR WITH SPLITTER BLADES 5. FUNDING NUMBERS 6. AUTHOR(S) Scott Drayton 7. PERFORMING ORGANIZATION NAME(S) AND ADDRESS(ES

  8. Research Design

    DEFF Research Database (Denmark)

    2019-01-01

    Gunnar Scott Reinbacher (editor) Antology.  Research Design : Validation in Social Sciences. Gunnar Scott Reinbacher: Introduction. Research design and validity. 15p Ole Riis: Creative Research design. 16 p Lennart Nørreklit: Validity in Research Design. 24p Gitte Sommer Harrits: Praxeological...... Scott Reinbacher: Multidisciplinary Research Designs in Problem Based Research. The case of an european project on chronical diseases, the Tandem project (Training Alternmative Networking Skills in Diabetes Management). 15p Niels Nørgaard Kristensen: A qualitative bottom up approach to post modern...... knowledge: An integrated strategy for combining "explaining" and "understanding". 22p Heidi Houlberg Salomonsen & Viola Burau: Comparative research designs. 40p Rasmus Antoft & Heidi Houlberg Salomonsen: Studying organizations by a Pragmatic Research Design: the case of qualitative case study  designs. 31p...

  9. Untitled

    Indian Academy of Sciences (India)

    Gouterman M. 1978 in The porphyrins (ed.) D Dolphin (New York: Academic Press) vol.3, pp. 157–231. Gouterman M, Schwarz F P, Smith PD and Dolphin D 1973 J. Chem. Phys. 59 676. Merkel P. P and Kearns D R 1972 J. Am. Chem. Soc. 94 7241. Norris Jr J R and Meiser D (eds) 1989 Photochemical emergy conversion ...

  10. Exploración o deporte: comparación entre los modelos britanico y noruego en la carrera por alcanzar el polo sur = Exploration or sport: comparision between british and norwegian models in the race to reach the south pole

    Directory of Open Access Journals (Sweden)

    Javier Gálvez González

    2013-03-01

    Full Text Available En este trabajo se analiza la carrera entre Roald Amundsen y Robert F. Scott por alcanzar el Polo Sur entre 1911 y 1912, tomando como base un planteamiento deportivo de organizar la exploración. La expedición de Amundsen fue considerada como moderna, por aplicar los avances existentes para llegar el primero a los 90º Sur. La expedición de Scott sin embargo se organizó según las tradiciones militares británicas. Al final, la victoria del noruego demostró una elección más adecuada de la los factores técnicos, tácticos, biológicos y psicológicos.--------------------------------------------------------------------------------This paper examines the race between Roald Amundsen and Robert F. Scott to reach the South Pole between 1911 and 1912, based on a Sports approach to organize the exploration. Amundsen's expedition was considered as modern to implement existing developments to be the first to get to 90 degrees south. Scott's expedition was organized, however, according to British military traditions. In the end, the Norwegian won, demonstrating a more suitable choice of the technical, tactical, biological and psychological factors.

  11. Design of Experiment Approach to Hydrogen Re-embrittlement Evaluation WP-2152

    Science.gov (United States)

    2015-04-01

    2152 by Scott M Grendahl, Hoang Nguyen, Franklin Kellogg , Shuying Zhu, and Stephen Jones Approved for public release...and Materials Research Directorate, ARL Hoang Nguyen and Franklin Kellogg Bowhead Science and Technology, LLC Shuying Zhu and Stephen Jones The...ELEMENT NUMBER 6. AUTHOR(S) Scott M Grendahl, Hoang Nguyen, Franklin Kellogg , Shuying Zhu, and Stephen Jones 5d. PROJECT NUMBER W74RDV20769717 5e

  12. ARO Summary Research Report (University of Michigan)

    Science.gov (United States)

    2014-10-03

    Federalism, and Cultural Evolution." , Cliodynamics: The Journal of Theoretical and Mathematical History (10 2011) Lu Hong, Scott Page, Maria Riolo...Priorot Conference on Political Economy and the Venice Conference on Behavioral Political Economy. Among other highlights, were presentations by...The Journal of Theoretical and Mathematical History 3(1):81-93, 2012. 4. Jenna Bednar, Yan Chen, and Xiao Liu and Scott Page,“Behavioral spillovers

  13. Operations Mercury and Husky: Contemporary Art of Operations and their Relevance for Operational Art

    Science.gov (United States)

    2016-04-07

    123 Cote, “OPERATION Husky: A critical Analysis,” 5-6. 124 Scott G. Hirshson and Paul J. Pugliese, Patton: A Soldier’s Life (New York: HarperCollins...prescriptive, 161 ADP 3-0, 9; introduced in section II, Gross, Mythos und Wirklichkeit, 17. 50 cherry ...MONTGOMERY: D-Day Commander. Washington: Potomac Books, 2007. Hirshson, Scott G. and Paul J. Pugliese. Patton: A Soldier’s Life. New York

  14. Developing a Common Metric for Evaluating Police Performance in Deadly Force Situations

    Science.gov (United States)

    2012-08-27

    Even police shootings that are morally, legally and procedurally justified can have potentially devastating consequences. As Geller and Scott (1992: 1...itself (for summaries, see Geller and Scott , 1992; Binder and Fridell, 1984; Alpert and Fridell, 1992; Fridell, 2005). Most of these studies have...Adrienne, Chris Berka, Ronald Stevens, Bryan Vila, Veasna Tan, Trysha Galloway , Robin Johnson, Giby Raphael. (2012) “Neurotechnology to Accelerate

  15. The Evolution of Centralized Operational Logistics

    Science.gov (United States)

    2012-05-17

    Lieutenant General Daniel G. Brown United States Army Deputy Commander in Chief, United States Transportation Command: An Oral History ( Scott Air Force Base...Printing Office, 2004. Fuson, Jack C. Transportation and Logistics: One Man’s Story. Washington, DC: Government Printing Office, 1997. Galloway ...History. Scott Air Force Base: United States Transportation Command, 2006. Mills, Patrick, Ken Evers, Donna Kinlin, and Robert S. Tripp. Supporting

  16. The Effect of Initial Public Offering (IPO) Firm Legitimacy on Cooperative Agreements and Performance

    Science.gov (United States)

    2000-04-04

    legitimacy A review of institutional theory (Meyer & Rowan, 1977; Scott, 1995; Zucker, 1983) suggests a set of institutional domains that Scott (1995:35...psychology (Berger & Luckman, 1967) and the cognitive school of institutional theory (Meyer & Rowan, 1977; Zucker, 1983). Organizations have to conform to...regression analysis for business and economics. Belmont, CA: Duxbury. DiMaggio, P.J. 1988. Interest and agency in institutional theory . In L.G

  17. Anonymous Agencies, Backstreet Businesses and Covert Collectives

    DEFF Research Database (Denmark)

    Krause Hansen, Hans; Schoeneborn, Dennis

    2015-01-01

    Book review of: Anonymous Agencies, Backstreet Businesses and Covert Collectives: rethinking Organizations in the 21st Century, C. R. Scott. Stanford, CA: Stanford University Press, 2013. 272 pp. £45.90. ISBN 9780804781381......Book review of: Anonymous Agencies, Backstreet Businesses and Covert Collectives: rethinking Organizations in the 21st Century, C. R. Scott. Stanford, CA: Stanford University Press, 2013. 272 pp. £45.90. ISBN 9780804781381...

  18. Decrease in N-acetylaspartate/creatine ratio in the motor area and the frontal lobe in amyotrophic lateral sclerosis

    International Nuclear Information System (INIS)

    Abe, K.; Takanashi, M.; Yanagihara, T.; Watanabe, Y.; Tanaka, H.; Fujita, N.; Hirabuki, N.

    2001-01-01

    We studied whether N-acetylaspartate (NAA), a neuronal marker, is reduced in the brain of 14 patients with clinically definite amyotrophic lateral sclerosis (ALS) and whether NAA levels in the motor area and frontal lobe correlate with the clinical features, including frontal lobe function. We also studied 14 normal controls were evaluated. We obtained peak integrals in 1 H magnetic resonance spectroscopy (MRS) for NAA, creatine (Cr), and choline-containing compounds (Cho). Severity of the disease was determined using the manual muscle strength test, and the Norris limb and bulbar scales. In the patients, the NAA/Cr ratio was reduced in the motor area and frontal lobe, while the Cho/Cr ratio was normal throughout the brain. There were significant correlations between the NAA/Cr ratio in the motor area and the Norris limb scale (r = 0.50; P < 0.01) and between the NAA/Cr ratio in the frontal lobe and the number of categories achieved in the Wisconsin Card Sorting test (r = 0.71; P < 0.05), implying frontal lobe dysfunction. These correlations suggest that a reduced NAA/Cr ratio is a marker of cortical neuronal loss and dysfunction in ALS. (orig.)

  19. Alternating current loss calculation in a high-TC superconducting transmission cable considering the magnetic field distribution

    International Nuclear Information System (INIS)

    Noji, H; Haji, K; Hamada, T

    2003-01-01

    We have calculated the alternating current (ac) losses of a 114 MVA high-T C superconducting (HTS) transmission cable using an electric-circuit (EC) model. The HTS cable is fabricated by Tokyo Electric Power Company and Sumitomo Electric Industries, Ltd. The EC model is comprised of a resistive part and an inductive part. The resistive part is obtained by the approximated Norris equation for a HTS tape. The Norris equation indicates hysteresis losses due to self-fields. The inductive part has two components, i.e. inductances related to axial fields and those related to circumferential fields. The layer currents and applied fields of each layer were calculated by the EC model. By using both values, the ac losses of the one-phase HTS cable were obtained by calculation considering the self-field, the axial field and the circumferential field of the HTS tape. The measured ac loss transporting 1 kA rms is 0.7 W m -1 ph -1 , which is equal to the calculation. The distribution of each layer loss resembles in shape the distribution of the circumferential field in each layer, which indicates that the circumferential fields strongly influence the ac losses of the HTS cable

  20. Decrease in N-acetylaspartate/creatine ratio in the motor area and the frontal lobe in amyotrophic lateral sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Abe, K.; Takanashi, M.; Yanagihara, T. [Dept. of Neurology, Osaka University Graduate School of Medicine (Japan); Watanabe, Y.; Tanaka, H.; Fujita, N.; Hirabuki, N. [Dept. of Radiology, Osaka University Graduate School of Medicine (Japan)

    2001-07-01

    We studied whether N-acetylaspartate (NAA), a neuronal marker, is reduced in the brain of 14 patients with clinically definite amyotrophic lateral sclerosis (ALS) and whether NAA levels in the motor area and frontal lobe correlate with the clinical features, including frontal lobe function. We also studied 14 normal controls were evaluated. We obtained peak integrals in {sup 1}H magnetic resonance spectroscopy (MRS) for NAA, creatine (Cr), and choline-containing compounds (Cho). Severity of the disease was determined using the manual muscle strength test, and the Norris limb and bulbar scales. In the patients, the NAA/Cr ratio was reduced in the motor area and frontal lobe, while the Cho/Cr ratio was normal throughout the brain. There were significant correlations between the NAA/Cr ratio in the motor area and the Norris limb scale (r = 0.50; P < 0.01) and between the NAA/Cr ratio in the frontal lobe and the number of categories achieved in the Wisconsin Card Sorting test (r = 0.71; P < 0.05), implying frontal lobe dysfunction. These correlations suggest that a reduced NAA/Cr ratio is a marker of cortical neuronal loss and dysfunction in ALS. (orig.)

  1. Linkage and candidate gene analysis of X-linked familial exudative vitreoretinopathy.

    Science.gov (United States)

    Shastry, B S; Hejtmancik, J F; Plager, D A; Hartzer, M K; Trese, M T

    1995-05-20

    Familial exudative vitreoretinopathy (FEVR) is a hereditary eye disorder characterized by avascularity of the peripheral retina, retinal exudates, tractional detachment, and retinal folds. The disorder is most commonly transmitted as an autosomal dominant trait, but X-linked transmission also occurs. To initiate the process of identifying the gene responsible for the X-linked disorder, linkage analysis has been performed with three previously unreported three- or four-generation families. Two-point analysis showed linkage to MAOA (Zmax = 2.1, theta max = 0) and DXS228 (Zmax = 0.5, theta max = 0.11), and this was further confirmed by multipoint analysis with these same markers (Zmax = 2.81 at MAOA), which both lie near the gene causing Norrie disease. Molecular genetic analysis further reveals a missense mutation (R121W) in the third exon of the Norrie's disease gene that perfectly cosegregates with the disease through three generations in one family. This mutation was not detected in the unaffected family members and six normal unrelated controls, suggesting that it is likely to be the pathogenic mutation. Additionally, a polymorphic missense mutation (H127R) was detected in a severely affected patient.

  2. Novel mutation in TSPAN12 leads to autosomal recessive inheritance of congenital vitreoretinal disease with intra-familial phenotypic variability.

    Science.gov (United States)

    Gal, Moran; Levanon, Erez Y; Hujeirat, Yasir; Khayat, Morad; Pe'er, Jacob; Shalev, Stavit

    2014-12-01

    Developmental malformations of the vitreoretinal vasculature are a heterogeneous group of conditions with various modes of inheritance, and include familial exudative vitreoretinopathy (FEVR), persistent fetal vasculature (PFV), and Norrie disease. We investigated a large consanguineous kindred with multiple affected individuals exhibiting variable phenotypes of abnormal vitreoretinal vasculature, consistent with the three above-mentioned conditions and compatible with autosomal recessive inheritance. Exome sequencing identified a novel c.542G > T (p.C181F) apparently mutation in the TSPAN12 gene that segregated with the ocular disease in the family. The TSPAN12 gene was previously reported to cause dominant and recessive FEVR, but has not yet been associated with other vitreoretinal manifestations. The intra-familial clinical variability caused by a single mutation in the TSPAN12 gene underscores the complicated phenotype-genotype correlation of mutations in this gene, and suggests that there are additional genetic and environmental factors involved in the complex process of ocular vascularization during embryonic development. Our study supports considering PFV, FEVR, and Norrie disease a spectrum of disorders, with clinical and genetic overlap, caused by mutations in distinct genes acting in the Norrin/β-catenin signaling pathway. © 2014 Wiley Periodicals, Inc.

  3. Determination of the Relationship between Information Capacity and Identification by Simulated Aerial Photography.

    Science.gov (United States)

    1978-06-27

    experiment by Frank Scott, Peter Hollanda , and Albert Harabedian 7 7Scott, F., Hollanda , P. A., and Harabedian, A., Phot. Sci. Eng., 14 1 pp 21-27...photographs of models of military tanks and trucks. The information capacity of these photos was varied by defocussing the taking camera and the simulated...photographic reconnaissance mission. This was done by photographing models of military targets, I processing and duplicating the resulting images, and

  4. Résultats de recherche | Page 450 | CRDI - Centre de recherches ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Scott Gilmore. Scott est entrepreneur social et rédacteur. Profile. -. Jean Lebel. Jean a été nommé président du CRDI le 1er mai 2013. À titre de président du CRDI, Jean dirige l'apport du Centre aux efforts que déploie le Canada en matière de développement, de politique étrangère et d'innovation mondiale. Pages.

  5. VAST Challenge 2015: Mayhem at Dinofun World

    Science.gov (United States)

    2015-10-25

    Crouser Abstract— A fictitious amusement park and a larger-than-life hometown football hero provided participants in the VAST Challenge 2015 with an...last year was a weekend tribute to Scott Jones, internationally renowned football (“soccer,” in US terminology) star. Scott Jones is from a town...Challenge: history , scope, and outcomes. Information Visualization, 13(4):301–312, 2014. [2] Visual Analytics Benchmark Repository, http://hcil2

  6. Preparing for the Future, Looking to the Past: History, Theory, and Doctrine in the U.S. Army

    Science.gov (United States)

    2013-05-23

    Construction, 10–11. 109 Scott Gorman, interviewed by author, Fort Leavenworth, KS, February 5, 2013. Dr. Schifferle recommended an interview with Dr...the End of the Cold War: Implications, Reconsiderations, Provocations. New York: Oxford University Press, USA, 1992. Galloway , Archie. “FM 100-5: Who...of Advanced Military Studies Faculty. Interview by author, Fort Leavenworth, KS, February 5, 2013. Gorman, Scott . Academic Director of the School of

  7. Collecting to Win: ISR for Strategic Effect

    Science.gov (United States)

    2014-02-13

    Cryptologic History (Ft. George G. Meade , MD: National Security Agency, 1992), 1-143; Matthew M. Aid, The Secret Sentry: The Untold History of the National...United States Cryptologic History. Ft. George G. Meade , MD: National Security Agency, 1992. Norris, Pat. Spies in the Sky: Surveillance Satellites in...Submitted to the Faculty In Partial Fulfillment of the Graduation Requirements Advisor: Dr. Herbert L. Frandsen, Jr. 13 February 2014

  8. A family with Wagner syndrome with uveitis and a new versican mutation

    OpenAIRE

    Rothschild, Pierre-Rapha?l; Br?zin, Antoine P.; Nedelec, Brigitte; des Roziers, Cyril Burin; Ghiotti, Tiffany; Orhant, Lucie; Boimard, Mathieu; Valleix, Sophie

    2013-01-01

    Purpose To report the clinical and molecular findings of a kindred with Wagner syndrome (WS) revealed by intraocular inflammatory features. Methods Eight available family members underwent complete ophthalmologic examination, including laser flare cell meter measurements. Collagen, type II, alpha 1, versican (VCAN), frizzled family receptor 4, low density lipoprotein receptor-related protein 5, tetraspanin 12, and Norrie disease (pseudoglioma) genes were screened with direct sequencing. Resul...

  9. Etiology of Depression Comorbidity in Combat-Related PTSD: A Review of the Literature

    Science.gov (United States)

    2014-01-01

    documented across disparate populations and in reaction to multiple types of trauma (Breslau, 2002; Kilpatrick et al., 2003; Norris , 1992), historically it...are aspects of a larger spectrumof a posttraumatic stress syndrome rather thandistinct disorders. If this were true, PTSD and depression should be...completely indis- tinct diagnoses (Hypothesis 7) or that they are simply subclusters of a larger syndrome of response to trauma (Hypothesis 8). Rather

  10. Three dimensional marine seismic survey has no measurable effect on species richness or abundance of a coral reef associated fish community

    International Nuclear Information System (INIS)

    Miller, Ian; Cripps, Edward

    2013-01-01

    Highlights: • A marine seismic survey was conducted at Scott Reef, North Western Australia. • Effects of the survey on demersal fish were gauged using underwater visual census. • There was no detectable impact of the seismic survey on species abundance. • There was no detectable impact of the seismic survey on species richness. -- Abstract: Underwater visual census was used to determine the effect of a three dimensional seismic survey on the shallow water coral reef slope associated fish community at Scott Reef. A census of the fish community was conducted on six locations at Scott Reef both before and after the survey. The census included small site attached demersal species belonging to the family Pomacentridae and larger roving demersal species belonging to the non-Pomacentridae families. These data were combined with a decade of historical data to assess the impact of the seismic survey. Taking into account spatial, temporal, spatio-temporal and observer variability, modelling showed no significant effect of the seismic survey on the overall abundance or species richness of Pomacentridae or non-Pomacentridae. The six most abundant species were also analysed individually. In all cases no detectable effect of the seismic survey was found on the abundance of these fish species at Scott Reef

  11. Résultats de recherche | Page 447 | CRDI - Centre de recherches ...

    International Development Research Centre (IDRC) Digital Library (Canada)

    Profile. Scott Gilmore. Scott est entrepreneur social et rédacteur. Profile. Jean Lebel. Jean a été nommé président du CRDI le 1er mai 2013. À titre de président du CRDI, Jean dirige l'apport du Centre aux efforts que déploie le Canada en matière de développement, de politique étrangère et d'innovation mondiale. Profile ...

  12. Statistical Inferences from the Topology of Complex Networks

    Science.gov (United States)

    2016-10-04

    stable, does not lose any information, has continuous and discrete versions, and obeys a strong law of large numbers and a central limit theorem. The...paper (with J.A. Scott) “Categorification of persistent homology” [7] in the journal Discrete and Computational Geome- try and the paper “Metrics for...Generalized Persistence Modules” (with J.A. Scott and V. de Silva) in the journal Foundations of Computational Math - ematics [5]. These papers develop

  13. Training sociolinguistic awareness in school pedagogy

    DEFF Research Database (Denmark)

    Fabricius, Anne

    2011-01-01

    Book review of "Affirming Students' Rights to Their Own Language: Bridging Language Policies and Pedagogical Practices,” by Jerrie Cobb Scott, Dolores Y. Straker and Laurie Katz. 2009. Routledge. pp 418. ISBN: 978-0-8058-6349-9.......Book review of "Affirming Students' Rights to Their Own Language: Bridging Language Policies and Pedagogical Practices,” by Jerrie Cobb Scott, Dolores Y. Straker and Laurie Katz. 2009. Routledge. pp 418. ISBN: 978-0-8058-6349-9....

  14. United States Air Force Summer Faculty Research Program 1989. Program Technical Report. Volume 2

    Science.gov (United States)

    1989-12-01

    968, June 1987. 22. E. Zielinski , H. Schweizer, S. Hausser, R. Stuber, M. H. Pilkuhn, and G. Weimann,"Systematics of laser operation in GaAs/AlGaAs...Mike Hinman, John Wagnon, Dave Froehlich, Scott Huse, Scott Shyne, and Ken Taylor. 79-3 I. Introduction Pattern Recognition (PR) is an important...many aspects of this research was also greatly appreciated. Finally, I want to thank Audrey Martinez, Al Leverette, Lt. Georke, Dave Fernald and

  15. A More Nuanced Approach to the Fourth Estate

    Science.gov (United States)

    2012-05-17

    and accurately.117 Veteran reporter Joe Galloway writing before the start of OIF, stated “Any reporter who has the [courage] to go into combat is...arguably crossing the line from objective reporter to active combatant.119 Boston Globe Journalist, Scott Bernard Nelson, also disclosed that he...www.brookings.edu/comm/events/20030617.pdf (accessed September 14, 2011). Donnelly, Mike, Mitch Gettle, Greg Scott , and Dana Warr. “Embedded Journalism: How War

  16. Social Behavior in Medulloblastoma: Functional Analysis of Tumor-Supporting Glial Cells

    Science.gov (United States)

    2015-10-01

    the manuscript with inputs from all authors. All authors reviewed the manuscript. Acknowledgements We thank Chris Doe, David Rowitch, and Praveen...201–208. Goodrich, L.V., Milenković, L., Higgins , K.M., and Scott, M.P. (1997). Altered Neural Cell Fates and Medulloblastoma in Mouse patched...Genes & Development 27, 98–115. Goodrich, L.V., Milenković, L., Higgins , K.M., and Scott, M.P. (1997). Altered Neural Cell Fates and Medulloblastoma in

  17. Flood Insurance Rate Map, Scott County, USA

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — The Digital Flood Insurance Rate Map (DFIRM) Database depicts flood risk information and supporting data used to develop the risk data. The primary risk...

  18. STAR TREK elab ja õilmitseb / Scott Abel

    Index Scriptorium Estoniae

    Abel, Scott

    2009-01-01

    Artikli autor käis Bonnis "Star Treki" (USA, 1966-1969) fännide iga-aastasel kokkutulekul. Artiklis sellest, kuidas kulttussari on mõjutanud inimesi, kultuuri, meediat. Meenutavad näitleja Nichelle Nichols ja teismelisena sarja looja Gene Roddenberry juures vabatahtliku abilisena töötanud Richard Arnold. Sarja algusest, järgprojektidest ("Star Trek : The Next Generation", "Deep Space Nine" jt.) kuni J. J. Abramsi filmini, mis esilinastus 8. mail

  19. OrthoImagery submittal for Scott County, Indiana

    Data.gov (United States)

    Federal Emergency Management Agency, Department of Homeland Security — Digital orthographic imagery datasets contain georeferenced images of the Earth's surface, collected by a sensor in which object displacement has been removed for...

  20. Sir Charles Scott Sherrington (1857–1952)

    Indian Academy of Sciences (India)

    IAS Admin

    Sherrington had the genius to see the real need to amalgamate the scattered ideas in neurophysiology at that time, to give a compre- hensive overview that changed our ... and desire for travel were all because of him. Despite Sherrington's.

  1. Update on eating disorders: current perspectives on avoidant/restrictive food intake disorder in children and youth

    OpenAIRE

    Norris, Mark L; Spettigue, Wendy J; Katzman, Debra K

    2016-01-01

    Mark L Norris,1 Wendy J Spettigue,2 Debra K Katzman3 1Division of Adolescent Medicine, Department of Pediatrics, Children’s Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada; 2Department of Psychiatry, Children’s Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada; 3Division of Adolescent Medicine, Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada Abstract: Avoidant/restrictive food intak...

  2. Women Aboard Navy Ships: A Comprehensive Health and Readiness Research Project

    Science.gov (United States)

    1996-03-01

    premenstrual syndrome during the past 90 days, and 16% reported that they lost two or more hours of work during the past 30 days due to menstrual...in diabetes mellitus. In Psychological Aspects of Diabetes in Children and Adolescents, Pediatric Adolescent Endocrinology (Edited by Laron , Z., and...13. Dingfelder, J.R. (1982). Treatment of dysmenorrhea. Hospital Physician. 73-78. 14. Norris, R.V., Sullivan, C. (1983). PMS/Perimenstrual Syndrome

  3. The Role of the Caspase-8 Inhibitor FLIP in Androgen-Withdrawal Induced Death of Prostate Epithelium

    Science.gov (United States)

    2007-01-01

    cells. Cancer Res 2000;60:2384-9. 20. Voelkel-Johnson C, King DL, Norris JS. Resistance of prostate cancer cells to soluble TNF-related apoptosis...Mukhopadhyay A, Bueso-Ramos C, Chatterjee D, Pantazis P, Aggarwal BB. Curcumin downregulates cell survival mechanisms in human prostate cancer cell...ethanol. LY294002 (Cayman) was dissolved in Androgens and FOXO3a regulate FLIP page 7 dimethylsulfoxide (DMSO). Soluble recombinant human TRAIL (Biomol

  4. Syndrome of Acute Anxiety Among Marines After Recent Arrival at High Altitude

    Science.gov (United States)

    2014-05-01

    Naval Health Research Center Syndrome of Acute Anxiety Among Marines After Recent Arrival at High Altitude Michael K. Sracic Darren Thomas...Allen Pate Jacob Norris Marc Norman, Jeffrey H. Gertsch Report No. 13-29 The views expressed in this article are those of the authors...MEDICINE, 179, 5:559, 2014 Syndrome of Acute Anxiety Among Marines After Recent Arrival at High Altitude LT Michael K. Sracic, MC USN*; LT Darren Thomas

  5. The Use of Bryophytes as Indicators of Hydric Soils and Wetland Hydrology during Wetland Delineations in the United States

    Science.gov (United States)

    2010-09-01

    readily observed without the aid of optical magnification, including all vascular plant species and mosses (e.g., Sphagnum spp.), as well as large algae ...in lakes, pools or ponds ) or rheophilous (occurring in streams or running water). In addition, many bryophytes are classified as obligate aquatics...Mosses and other bryophytes, an illustrated glossary. 2nd ed. New Zealand: Micro -Optics Press. Malcom, N., J. Shevock, and D. Norris. 2009

  6. The Shock and Vibration Digest. Volume 10, Number 12

    Science.gov (United States)

    1978-12-01

    programs Sho fArsae ah n ula nr. Univ. of Oklahoma, Norman , OK, J. Mech. Des., The reflection of a plane sound wave from a thick, i.e., Trans. ASME, 100 (2...1534 P Perkins, J...............1166 Norman , C.D ............. 866 Penl, E .................. 532 Norris, D.M .............. 197 Perreira, N.D...1499, 1867, 1868 Shende, R.W............. 488 Smallwood , D.O ........ 62,1422 Srinivasan, M.G ............ 98 Shepherd, R ............ 1745 Smigielski

  7. Website Redesign: A Case Study.

    Science.gov (United States)

    Wu, Jin; Brown, Janis F

    2016-01-01

    A library website redesign is a complicated and at times arduous task, requiring many different steps including determining user needs, analyzing past user behavior, examining other websites, defining design preferences, testing, marketing, and launching the site. Many different types of expertise are required over the entire process. Lessons learned from the Norris Medical Library's experience with the redesign effort may be useful to others undertaking a similar project.

  8. Hydrodynamic Performance of the Flippers of Large-bodied Cetaceans in Relation to Locomotor Ecology

    Science.gov (United States)

    2014-04-01

    ankylosed cervical vertebrae would limit maneuverability for active pursuit of prey (Norris and Møhl 1983, Rice 1989). The flipper shape of the killer...radius of 0.11 body lengths by using the flippers in combination with peduncle, and flukes (Fish 2002). Mature male killer whales have...Jeff Jacobsen of the Vertebrate Museum of Humboldt State University, California, the United States Coast Guard, and the United States Army Corps of

  9. The Influence of Multiple Host Contacts on the Acquisition and Transmission of Dengue-2 Virus

    Science.gov (United States)

    1993-01-01

    final push, Dr. Scott nudged , prodded and cajoled me into finishing. Dr. Scott also taught me that there’s always time and room for change , even within... change the behavior of their host. Sci. Am. 250: 108-115. Molyneux, D. H. and D. Jefferies. 1986. Feeding behaviour of pathogen-infected vectors...inadequate to account for changes in the incidence of dengue hemorrhagic fever." Since changes in adult female Af. agga. population size and life

  10. LWIR Microgrid Polarimeter for Remote Sensing Studies

    Science.gov (United States)

    2010-02-28

    Polarimeter for Remote Sensing Studies 5b. GRANT NUMBER FA9550-08-1-0295 5c. PROGRAM ELEMENT NUMBER 6. AUTHOR(S) 5d. PROJECT NUMBER 1. Scott Tyo 5e. TASK...and tested at the University of Arizona, and preliminary images are shown in this final report. 15. SUBJECT TERMS Remote Sensing , polarimetry 16...7.0 LWIR Microgrid Polarimeter for Remote Sensing Studies J. Scott Tyo College of Optical Sciences University of Arizona Tucson, AZ, 85721 tyo

  11. Stemcell Information: SKIP000849 [SKIP Stemcell Database[Archive

    Lifescience Database Archive (English)

    Full Text Available Technologies). 健常人由来iPS細胞。 human ES-like Research Grade Sendai virus Oct4, Sox2,Klf4, and c-Myc ... Yes MEF Hu...Chris M. Woodard ... The New York Stem Cell Foundation Research Institute The New York Stem Cell Foundation Research... Institute Scott A. Noggle ... Scott A. Noggle ... The New York Stem Cell Foundation Research Institute The N...ew York Stem Cell Foundation Research Institute Information Only ... 25456120

  12. Transport ac loss studies of YBCO coated conductors with nickel alloy substrates

    International Nuclear Information System (INIS)

    Duckworth, R C; Thompson, J R; Gouge, M J; Lue, J W; Ijaduola, A O; Yu, D; Verebelyi, D T

    2003-01-01

    Transport alternating current (ac) loss measurements were performed on a series of rolling-assisted biaxially textured substrate (RABiTS) processed YBa 2 Cu 3 O x (YBCO) coated conductors at 77 K. While each sample possessed a 1 μm layer of YBCO and a 3 μm silver cap layer, two different nickel alloy substrates were used and their impact on the ac loss was examined. Both substrates possessed a 75 μm Ni-5 at%W base, but one substrate also had a 2 μm nickel overlayer as part of the buffer layer architecture. The ac losses, which were determined by thermal and electrical measurements, contained two dominant contributions: superconductive hysteresis in the YBCO and ferromagnetic hysteresis in the substrates. The superconductive component followed the Norris elliptic model for the substrate with the nickel overlayer and the Norris thin strip model for the substrate without the nickel overlayer. The substrates' ferromagnetic loss was determined separately through magnetization measurements, which showed that this loss contribution was independent of the presence of the nickel overlayer for effective ac currents less than 50 A. While the overall loss was lower for the thin-strip-like conductor with no nickel overlayer, further research is necessary to strengthen this connection

  13. Development of 1 m HTS conductor using YBCO on textured metal substrate

    International Nuclear Information System (INIS)

    Yagi, M.; Sakamoto, H.; Mukoyama, S.; Yamamoto, K.; Amemiya, N.; Nagaya, S.; Kashima, N.; Shiohara, Y.

    2009-01-01

    We fabricated 1 m high temperature superconducting conductor (HTS conductor) using YBa 2 Cu 3 O 7-x coated conductors (YBCO tapes) on textured metal substrates, which are expected to be lower in cost than YBCO tapes using ion-beam assisted deposition. Those substrate and intermediate layers were manufactured by Furukawa Electric, and YBCO and a protective layer were applied to the intermediate layer by Chubu Electric Power. Before fabricating the conductor, a 0.1 mm thick copper tape was soldered to the YBCO tape, and 10 mm wide YBCO tape was divided into three strips by a YAG laser. To have sufficient current capacity for 1 kA, a two-layer conductor was fabricated, and its critical current (I c ) was 1976 A, but the magnetic properties of the textured metal substrates affected the increase in AC loss. In a low current region, the AC loss in this conductor was much higher than the Norris strip model, but approached the Norris strip model in the high current region because the magnetization was almost saturated. Low AC loss of 0.144 W/m at 1 kA rms was achieved even though the conductor had a small outer diameter of 20 mm and was composed of YBCO tapes with magnetic substrates.

  14. Overview of autoventing turbine technology development project

    International Nuclear Information System (INIS)

    Waldrop, W.R.

    1991-01-01

    This paper reports on low concentrations of dissolved oxygen (DO) in the discharge of hydro plants which represents one of the most significant environmental concerns confronting the hydropower industry. This is especially of concern to utilities attempting to relicense older plants and to build new facilities. One method which shows promise is the autoventing turbine (AVT). The concept of an AVT involves air to be aspirated into the water as it passes through the turbine whenever concentrations of DO are less than desired. Because of this simple and natural process of aeration, the AVT promises to be more cost efficient and reliable than any of the other techniques. This has been demonstrated through experimentation at TVA's Norris Dam. An applied research project is being conducted to develop experimental and numerical methods to allow for reliable design and deployment of this new environmentally improved hydroturbine. TVA is providing overall coordination for this research which is being performed cooperatively with the U.S. Army Corps of Engineers, the U.S. Bureau of Reclamation, and the Iowa Institute of Hydraulic Research. This applied research project is fully integrated with a scale model test program jointly supported and conducted by TVA and Voith Hydro to test alternative locations for venting air into replacement turbine runners for Norris Dam

  15. Miscalculated Ambiguity: The Effects of US Nuclear Declaratory Policy on Deterrence and Nonproliferation

    Science.gov (United States)

    2010-06-01

    Use. (Santa Monica, Calif.: RAND, 1995) 7. 5 Sagan , Scott. "The Case for No First Use." Survival 51, no. 3 (2009): 163-182. 6 The Stanley Foundation...America’s Nuclear Posture. (Cambridge, MA: Union of Concerned Scientists Global Security Program, 2010) 1. 60 Sagan , Scott. "The Case for No First...Foreign Policy for the 1970s: Building for Peace. S.l.: s.n., 1971. Nonproliferation--60 Years Later. DVD. Directed by Carla Robbins. Washington D.C

  16. Nation, region, and globe alternative definitions of place in world history

    OpenAIRE

    Little, Daniel

    2014-01-01

    The paper begins in the recognition of the importance of ‘world history’ and considers some of the current challenges this field faces. It considers several important contributions to the field that illuminate the value of fresh approaches: James Scott''s construction of ‘Zomia’, Emmanuel Todd''s historicization of ‘France’ as a nation, Bin Wong and Kenneth Pomeranz''s new approach to Eurasian economic history, and Victor Lieberman''s analysis of the strange synchrony between Southeast Asia a...

  17. Evaluating the Efficacy of ERG Targeted Therapy in vivo for Prostate Tumors

    Science.gov (United States)

    2016-06-01

    Arvin M. Gouw, Meital Gabay, Stacey J. Adam, David I. Bellovin, Phuoc T. Tran, William M. Philbricke, Adolfo Garcia-Ocanaf, Stephanie C. Casey, Yulin Li ...2016). In press. PMID: 27080744. 4. Abstracts (Year 5 only from a total of 47 since the beginning of the DoD PRTA): 1. Omar Y. Mian , Scott Robertson...Meeting. 2. Omar Y. Mian , Scott Robertson, Amol Narang, Sameer Agarwal, Hee Joon Bae, Todd McNutt, Phuoc Tran, Theodore L. DeWeese, Danny Y. Song

  18. Nanocrystal quantum dots

    CERN Document Server

    Klimov, Victor I

    2010-01-01

    ""Soft"" Chemical Synthesis and Manipulation of Semiconductor Nanocrystals, J.A. Hollingsworth and V.I. Klimov Electronic Structure in Semiconductor Nanocrystals: Optical Experiment, D.J. NorrisFine Structure and Polarization Properties of Band-Edge Excitons in Semiconductor Nanocrystals, A.L. EfrosIntraband Spectroscopy and Dynamics of Colloidal Semiconductor Quantum Dots, P. Guyot-Sionnest, M. Shim, and C. WangMultiexciton Phenomena in Semiconductor Nanocrystals, V.I. KlimovOptical Dynamics in Single Semiconductor Quantum Do

  19. The Operational Capability of the American Expeditionary Forces in the World War

    Science.gov (United States)

    2014-12-04

    attend the Advance Military Studies Program, COLs Michael Getchell, John Marr, and John Norris . Finally, to my college mentor, Dario Lorenzetti, killed...the United States Army and the War Department was selecting a general to lead the American Army in France. Since the end of the Spanish -American...formation since the end of the Spanish -American War. This proved to be a great challenge for the American Army in finding leaders to command the large

  20. Norris Bradbury and Edward Teller: A Fission-Fusion Reaction

    Energy Technology Data Exchange (ETDEWEB)

    Meade, Roger Allen [Los Alamos National Lab. (LANL), Los Alamos, NM (United States)

    2018-02-12

    On a bright, sunny day in October 1945, a boisterous and jubilant crowd assembled in front of Fuller Lodge to watch as the Army-Navy “E” Award, a prestigious national honor given for “Excellence in Production” during World War II, was presented to the Los Alamos Laboratory. Major General Leslie Groves, the commanding officer of the Manhattan Project; Navy Commodore William S. (Deak) Parsons, the wartime leader of the Laboratory’s Ordnance Division; Robert Sproul, the President of the University of California; and J. Robert Oppenheimer accepted the award on behalf of the Laboratory.

  1. Avian use of Norris Hill Wind Resource Area, Montana

    Energy Technology Data Exchange (ETDEWEB)

    Harmata, A.; Podruzny, K.; Zelenak, J. [Montana State Univ., Bozeman, MT (United States). Biology Dept.

    1998-07-01

    This document presents results of a study of avian use and mortality in and near a proposed wind resource area in southwestern Montana. Data collected in autumn 1995 through summer 1996 represented preconstruction condition; it was compiled, analyzed, and presented in a format such that comparison with post-construction data would be possible. The primary emphasis of the study was recording avian migration in and near the wind resource area using state-of-the-art marine surveillance radar. Avian use and mortality were investigated during the breeding season by employing traditional avian sampling methods, radiotelemetry, radar, and direct visual observation. 61 figs., 34 tabs.

  2. Origin and Evolution of the Elements

    Science.gov (United States)

    McWilliam, Andrew; Rauch, Michael

    2004-09-01

    Introduction; List of participants; 1. Mount Wilson Observatory contributions to the study of cosmic abundances of the chemical elements George W. Preston; 2. Synthesis of the elements in stars: B2FH and beyond E. Margaret Burbidge; 3. Stellar nucleosynthesis: a status report 2003 David Arnett; 4. Advances in r-process nucleosynthesis John J. Cowan and Christopher Sneden; 5. Element yields of intermediate-mass stars Richard B. C. Henry; 6. The impact of rotation on chemical abundances in red giant branch stars Corinne Charbonnel; 7. s-processing in AGB stars and the composition of carbon stars Maurizio Busso, Oscar Straniero, Roberto Gallino, and Carlos Abia; 8. Models of chemical evolution Francesca Matteucci; 9. Model atmospheres and stellar abundance analysis Bengt Gustafsson; 10. The light elements: lithium, beryllium, and boron Ann Merchant Boesgaard; 11. Extremely metal-poor stars John E. Norris; 12. Thin and thick galactic disks Poul E. Nissen; 13. Globular clusters and halo field stars Christopher Sneden, Inese I. Ivans and Jon P. Fulbright; 14. Chemical evolution in ω Centauri Verne V. Smith; 15. Chemical composition of the Magellanic Clouds, from young to old stars Vanessa Hill; 16. Detailed composition of stars in dwarf spheroidal galaxies Matthew D. Shetrone; 17. The evolutionary history of Local Group irregular galaxies Eva K. Grebel; 18. Chemical evolution of the old stellar populations of M31 R. Michael Rich; 19. Stellar winds of hot massive stars nearby and beyond the Local Group Fabio Bresolin and Rolf P. Kudritzki; 20. Presolar stardust grains Donald D. Clayton and Larry R. Nittler; 21. Interstellar dust B. T. Draine; 22. Interstellar atomic abundances Edward B. Jenkins; 23. Molecules in the interstellar medium Tommy Wiklind; 24. Metal ejection by galactic winds Crystal L. Martin; 25. Abundances from the integrated light of globular clusters and galaxies Scott C. Trager; 26. Abundances in spiral and irregular galaxies Donald R. Garnett; 27

  3. A comparison of the techniques of direct pars interarticularis repairs for spondylolysis and low-grade spondylolisthesis: a meta-analysis.

    Science.gov (United States)

    Mohammed, Nasser; Patra, Devi Prasad; Narayan, Vinayak; Savardekar, Amey R; Dossani, Rimal Hanif; Bollam, Papireddy; Bir, Shyamal; Nanda, Anil

    2018-01-01

    OBJECTIVE Spondylosis with or without spondylolisthesis that does not respond to conservative management has an excellent outcome with direct pars interarticularis repair. Direct repair preserves the segmental spinal motion. A number of operative techniques for direct repair are practiced; however, the procedure of choice is not clearly defined. The present study aims to clarify the advantages and disadvantages of the different operative techniques and their outcomes. METHODS A meta-analysis was conducted in accordance with the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. The following databases were searched: PubMed, Cochrane Library, Web of Science, and CINAHL ( Cumulative Index to Nursing and Allied Health Literature). Studies of patients with spondylolysis with or without low-grade spondylolisthesis who underwent direct repair were included. The patients were divided into 4 groups based on the operative technique used: the Buck repair group, Scott repair group, Morscher repair group, and pedicle screw-based repair group. The pooled data were analyzed using the DerSimonian and Laird random-effects model. Tests for bias and heterogeneity were performed. The I 2 statistic was calculated, and the results were analyzed. Statistical analysis was performed using StatsDirect version 2. RESULTS Forty-six studies consisting of 900 patients were included in the study. The majority of the patients were in their 2nd decade of life. The Buck group included 19 studies with 305 patients; the Scott group had 8 studies with 162 patients. The Morscher method included 5 studies with 193 patients, and the pedicle group included 14 studies with 240 patients. The overall pooled fusion, complication, and outcome rates were calculated. The pooled rates for fusion for the Buck, Scott, Morscher, and pedicle screw groups were 83.53%, 81.57%, 77.72%, and 90.21%, respectively. The pooled complication rates for the Buck, Scott, Morscher, and pedicle

  4. Cowboys et Indiens. Masculinités, métissage et queeritude chez Tomson Highway et Louis Hamelin

    Directory of Open Access Journals (Sweden)

    Corrie Scott

    2013-12-01

    Full Text Available Corrie Scott analyse tour à tour les masculinités autochtones construites par le romancier québécois Louis Hamelin dans Cowboy (1992 et celles proposées par l’écrivain cri Tomson Highway dans Champion et Ooneemeetoo [Kiss of the Fur Queen] (1998. Scott démontre que les deux auteurs jouent différemment du métissage et de son rapport avec le colonialisme : tandis que Hamelin met en scène des personnages autochtones dévirilisés et en perte d’authenticité, Highway s’appuie sur la notion amérindienne de bispiritualité pour créer des personnages queer qui transcendent les constructions binaires sur lesquelles se base la masculinité hégémonique. Corrie Scott examines the notion of métissage in the representation of Indigenous masculinities in Cowboy by Québécois novelist Louis Hamelin (1992 and in Kiss of the Fur Queen by Cree novelist Tomson Highway (1998. Scott shows the different ways in which the two authors approach the notion of métissage and its relationship with colonialism: while Hamelin depicts hybrid Indigenous characters as less authentic and virile, Highway embraces a queer poetics that questions hegemonic masculinities and gendered colonial stereotypes through hybrid fictional male identities. An intersectional theoretical framework proves essential to the analysis of hegemonic masculinities which function hand in hand with colonialism.

  5. Temperature Dependent Rubidium Helium Line Shapes and Fine Structure Mixing Rates

    Science.gov (United States)

    2015-09-01

    tube biased at 1,275 V exhibited a dark signal bias of 4-6 nA with noise fluctuation of 0.04 nA, as monitored on a Keithley model 386 picoammeter with...Journal of Physical Chemistry, 74(1):187–196, 1970. [79] Wu, Sheldon SQ, Thomas F Soules , Ralph H Page, Scott C Mitchell, V Keith Kanz, and Raymond J...Society for Optics and Photonics, 2008. [80] Wu, Sheldon SQ, Thomas F Soules , Ralph H Page, Scott C Mitchell, V Keith Kanz, and Raymond J Beach

  6. Temperature Dependent Rubidium-Helium Line Shapes and Fine Structure Mixing Rates

    Science.gov (United States)

    2015-09-17

    tube biased at 1,275 V exhibited a dark signal bias of 4-6 nA with noise fluctuation of 0.04 nA, as monitored on a Keithley model 386 picoammeter with...Journal of Physical Chemistry, 74(1):187–196, 1970. [79] Wu, Sheldon SQ, Thomas F Soules , Ralph H Page, Scott C Mitchell, V Keith Kanz, and Raymond J...Society for Optics and Photonics, 2008. [80] Wu, Sheldon SQ, Thomas F Soules , Ralph H Page, Scott C Mitchell, V Keith Kanz, and Raymond J Beach

  7. Camp Marmal Flood Study

    Science.gov (United States)

    2012-03-01

    was simulated by means of a broad - crested weir built into the topography of the mesh. There is 0.5 m of freeboard and the width of the weir is 30 m...ER D C/ CH L TR -1 2- 5 Camp Marmal Flood Study Co as ta l a nd H yd ra ul ic s La bo ra to ry Jeremy A. Sharp , Steve H. Scott...Camp Marmal Flood Study Jeremy A. Sharp , Steve H. Scott, Mark R. Jourdan, and Gaurav Savant Coastal and Hydraulics Laboratory U.S. Army Engineer

  8. Microwave phase locking of Josephson-junction fluxon oscillators

    DEFF Research Database (Denmark)

    Salerno, M.; Samuelsen, Mogens Rugholm; Filatrella, G.

    1990-01-01

    Application of the classic McLaughlin-Scott soliton perturbation theory to a Josephson-junction fluxon subjected to a microwave field that interacts with the fluxon only at the junction boundaries reduces the problem of phase locking of the fluxon oscillation to the study of a two-dimensional fun......Application of the classic McLaughlin-Scott soliton perturbation theory to a Josephson-junction fluxon subjected to a microwave field that interacts with the fluxon only at the junction boundaries reduces the problem of phase locking of the fluxon oscillation to the study of a two...

  9. Decay of reverberant sound in a spherical enclosure

    International Nuclear Information System (INIS)

    Carroll, M.M.; Chien, C.F.

    1977-01-01

    The assumption of diffuse reflection (Lambert's Law) leads to integral equations for the wall intensity in a reverberant sound field in the steady state and during decay. The latter equation, in the special case of a spherical enclosure with uniformly absorbent walls and uniform wall intensity, allows exponential decay with a decay time which agrees closely with the Norris--Eyring prediction. The sound-intensity and sound-energy density in the medium, during decay, are also calculated

  10. Deletion of MAOA and MAOB in a male patient causes severe developmental delay, intermittent hypotonia and stereotypical hand movements

    OpenAIRE

    Whibley, Annabel; Urquhart, Jill; Dore, Jonathan; Willatt, Lionel; Parkin, Georgina; Gaunt, Lorraine; Black, Graeme; Donnai, Dian; Raymond, F Lucy

    2010-01-01

    Monoamine oxidases (MAO-A and MAO-B) have a key role in the degradation of amine neurotransmitters, such as dopamine, norepinephrine and serotonin. We identified an inherited 240 kb deletion on Xp11.3–p11.4, which encompasses both monoamine oxidase genes but, unlike other published reports, does not affect the adjacent Norrie disease gene (NDP). The brothers who inherited the deletion, and thus have no monoamine oxidase function, presented with severe developmental delay, intermittent hypoton...

  11. Genetic heterogeneity in familial exudative vitreoretinopathy; exclusion of the EVR1 locus on chromosome 11q in a large autosomal dominant pedigree

    OpenAIRE

    Bamashmus, M; Downey, L; Inglehearn, C; Gupta, S; Mansfield, D

    2000-01-01

    BACKGROUND/AIMS—Familial exudative vitreoretinopathy (FEVR) is associated with mutations in the Norrie disease gene in X linked pedigrees and with linkage to the EVR1 locus at 11q13 in autosomal dominant cases. A large autosomal dominant FEVR family was studied, both clinically and by linkage analysis, to determine whether it differed from the known forms of FEVR.
METHODS—Affected members and obligate gene carriers from this family were examined by slit lamp biomicroscopy, indirect ophthalmos...

  12. VizieR Online Data Catalog: Boo-127 and Boo-980 high-resolution spectra (Frebel+, 2016)

    Science.gov (United States)

    Frebel, A.; Norris, J. E.; Gilmore, G.; Wyse, R. F. G.

    2016-09-01

    We observed Boo-980 and Boo-127 stars with the MIKE spectrograph on the Magellan-Clay telescope in 2010 March and 2011 March. Details of the MIKE observations and photometry taken from Norris et al. (2008ApJ...689L.113N) are given in Table 1. MIKE spectra have nearly full optical wavelength coverage over the range ~3500-9000Å (R~22000 in the red and ~28000 in the blue wavelength regime). (2 data files).

  13. ANAM4 TBI Reaction Time-Based Tests have Prognostic Utility for Acute Concussion

    Science.gov (United States)

    2013-07-01

    7:767. 2013 ANAM4 TBI Reaction Time-Based Tests Have Prognostic Utility for Acute Concussion LT Jacob N. Norris, MSC USN*; LCDR Waiter Carr, MSC USN...CDR Thomas Herzig, MSC USNf; CDR D. Waiter Labrie, MSC USNf; CDR Richard Sams, MC USN§ ABSTRACT The Concussion Restoration Care Center has used the...Work Unit No. N24LB. REFERENCES 1. Department of Defense: DoD Poiicy Guidance for Management of Mild Traumatic Brain Injury/Concussion in the Deployed

  14. A Historical Perspective on Light Infantry

    Science.gov (United States)

    1987-01-01

    which were scarce) expecting the enemy to use them in building fires. In another case, the CCF draped a large wire entanglement across a village...Jungle Frontier: 92 Special Air Service Regiment in the Borneo Campaign, 1963-1966 (London: Arms and Armour Press, 1985), 65, 57. Lieutenant Colonel P. E...1963-1966. London: Arms and Armour Press, 1985. Foxley-Norris, C. N., Air Vice Marshal, "Air Aspects of Operations Against ’Confrontation.’ " In

  15. Fission Product Yields for 14 MeV Neutrons on 235U, 238U and 239Pu

    International Nuclear Information System (INIS)

    Mac Innes, M.; Chadwick, M.B.; Kawano, T.

    2011-01-01

    We report cumulative fission product yields (FPY) measured at Los Alamos for 14 MeV neutrons on 235 U, 238 U and 239 Pu. The results are from historical measurements made in the 1950s–1970s, not previously available in the peer reviewed literature, although an early version of the data was reported in the Ford and Norris review. The results are compared with other measurements and with the ENDF/B-VI England and Rider evaluation. Compared to the Laurec (CEA) data and to ENDF/B-VI evaluation, good agreement is seen for 235 U and 238 U, but our FPYs are generally higher for 239 Pu. The reason for the higher plutonium FPYs compared to earlier Los Alamos assessments reported by Ford and Norris is that we update the measured values to use modern nuclear data, and in particular the 14 MeV 239 Pu fission cross section is now known to be 15–20% lower than the value assumed in the 1950s, and therefore our assessed number of fissions in the plutonium sample is correspondingly lower. Our results are in excellent agreement with absolute FPY measurements by Nethaway (1971), although Nethaway later renormalized his data down by 9% having hypothesized that he had a normalization error. The new ENDF/B-VII.1 14 MeV FPY evaluation is in good agreement with our data.

  16. On the negative resistance of double layers

    International Nuclear Information System (INIS)

    Raadu, M.A.; Silevitch, M.B.

    1982-08-01

    It is known that large amplitudes oscillations can occur in the current flowing through a plasma diode, typically when a constant potential is applied across the device. Burger (1965) suggested via a computer simulation that the oscillation characteristics was a function of the quantities T sub (e) and T sub (i), namely the respective time for an electron and an ion to cross the electric field region inside the diode. On the rapid time scale T sub (e) the self consistent equilibrium configuration, was unstable. Norris (1964) had previously arrived at the same conclusion using analytical arguments. In that work, it was concluded that the instability occurred since the diode acted as a negative resistance on the T sub (e) scale. A positive feedback effect forced the system away from equilibrium. Silevitch (1981) used the Burger mechanism to suggest an explanation for the flickering aurora phenomenon. He extended the Norris argument and showed by a variational method that a plausible analytic model for a double layer (DL) behaved as a negative resistance on the T sub (e) scale. In this present work we re-examine the negative resistance calculation by taking a more detailed account of the constraints which are imposed on the electron distributions that exist in the DL region. Specifically, we shall focus at the high potential side of the DL. (Authors)

  17. Non-conventional Frizzled ligands and Wnt receptors.

    Science.gov (United States)

    Hendrickx, Marijke; Leyns, Luc

    2008-05-01

    The Wnt family of secreted signaling factors plays numerous roles in embryonic development and in stem cell biology. In the adult, Wnt signaling is involved in tissue homeostasis and mutations that lead to the overexpression of Wnt can be linked to cancer. Wnt signaling is transduced intracellularly by the Frizzled (Fzd) family of receptors. In the canonical pathway, accumulation of beta-catenin and the subsequent formation of a complex with T cell factors (TCF) or lymphoid enhancing factors (Lef) lead to target gene activation. The identification of Ryk as an alternative Wnt receptor and the discovery of the novel Fzd ligands Norrie disease protein (NDP) and R-Spondin, changed the traditional view of Wnts binding to Fzd receptors. Mouse R-Spondin cooperates with Wnt signaling and Low density lipoprotein (LDL) receptor related protein (LRP) to activate beta-catenin dependent gene expression and is involved in processes such as limb and placental development in the mouse. NDP is the product of the Norrie disease gene and controls vascular development in the retina, inner ear and in the female reproductive system during pregnancy. In this review a functional overview of the interactions of the different Wnt and non-Wnt ligands with the Fzd receptors is given as well as a survey of Wnts binding to Ryk and we discuss the biological significance of these interactions.

  18. Wootz: Erroneous Transliteration of Sanskrit " Utsa" used for Indian Crucible Steel

    Science.gov (United States)

    Dube, R. K.

    2014-11-01

    The terminology Wootz for the legendary Indian crucible steel was first introduced by Helenus Scott in his letter to Joseph Banks, the then President of the Royal Society, London, in 1794. He stated several salient features of this steel in his letter. During the period 1794-1796, Banks received approximately 200 lbs. of this steel from Scott. Banks assigned several professionals to carry out experimental work on Indian crucible steel. One such important person was the famous surgical instrument maker, cutler and metallurgist of his time, James Stodart. Stodart experimented extensively with the Indian crucible steel, and was its great admirer. It has been shown, along with corroborative documentary evidence, that the original word for this steel was Sanskrit word " utsa". This was erroneously transliterated in Roman script as Wootz by Scott in his letter to Banks. It was James Stodart, who preserved the Sanskrit word " utsa" written in Devanāgarī script on his trade card for future generation. The reason for using this word for the Indian crucible steel has also been discussed.

  19. Bacteriocin-like substances of Lactobacillus curvatus P99: characterization and application in biodegradable films for control of Listeria monocytogenes in cheese.

    Science.gov (United States)

    Marques, Juliana de Lima; Funck, Graciele Daiana; Dannenberg, Guilherme da Silva; Cruxen, Claudio Eduardo Dos Santos; Halal, Shanise Lisie Mello El; Dias, Alvaro Renato Guerra; Fiorentini, Ângela Maria; Silva, Wladimir Padilha da

    2017-05-01

    The aim of this study was to evaluate the effectiveness of a biodegradable film, with antimicrobial metabolites produced by Lactobacillus curvatus P99 incorporated, targeting the control of Listeria monocytogenes in sliced "Prato" cheese. Tests were performed to evaluate the spectrum of action of cell-free supernatant (CFS) of P99 against different microorganisms, as well as to detect the minimum inhibitory (MIC) and bactericidal (MBC) concentrations against L. monocytogenes Scott A. The detection of genes that encode for the production of bacteriocins and evaluation of their expression were performed. Antimicrobial films were prepared, followed by in vitro and in situ analysis. The MIC and MBC of CFS against L. monocytogenes Scott A was 15.6 μL/mL and 62.5 μL/mL, respectively. Lactobacillus curvatus P99 presented two genes coding for the bacteriocins, which were expressed. Films with added MBC showed activity against different indicator microorganisms and were able to control L. monocytogenes Scott A when used in sliced "Prato" cheese. Copyright © 2016 Elsevier Ltd. All rights reserved.

  20. The relativistic Scott correction for atoms and molecules

    DEFF Research Database (Denmark)

    Solovej, Jan Philip; Sørensen, Thomas Østergaard; Spitzer, Wolfgang L.

    We prove the first correction to the leading Thomas-Fermi energy for the ground state energy of atoms and molecules in a model where the kinetic energy of the electrons is treated relativistically. The leading Thomas-Fermi energy, established in [25], as well as the correction given here are of s......We prove the first correction to the leading Thomas-Fermi energy for the ground state energy of atoms and molecules in a model where the kinetic energy of the electrons is treated relativistically. The leading Thomas-Fermi energy, established in [25], as well as the correction given here...... are of semi-classical nature. Our result on atoms and molecules is proved from a general semi-classical estimate for relativistic operators with potentials with Coulomb-like singularities. This semi-classical estimate is obtained using the coherent state calculus introduced in [36]. The paper contains...