922 resultados para MCAD deficiency


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Detection of trace amounts of explosive materials is significantly important for security concerns and pollution control. Four multicomponent metal organic frameworks (MOFs-12, 13, 23, and 123) have been synthesized by employing ligands embedded with fluorescent tags. The multicomponent assembly of the ligands was utilized to acquire a diverse electronic behavior of the MOFs and the fluorescent tags were strategically chosen to enhance the electron density in the MOFs. The phase purity of the MOFs was established by PXRD, NMR spectroscopy, and finally by singlecrystal XRD. Single-crystal structures of the MOFs-12 and 13 showed the formation of three-dimensional porous networks with the aromatic tags projecting inwardly into the pores. These electron-rich MOFs were utilized for detection of ex- plosive nitroaromatic compounds (NACs) through fluorescence quenching with high selectivity and sensitivity. The rate of fluorescence quenching for all the MOFs follows the order of electron deficiency of the NACs. We also showed the detection of picric acid (PA) by luminescent MOFs is not always reliable and can be misleading. This attracts our attention to explore these MOFs for sensing picryl chloride (PC), which is as explosive as picric acid and used widely to prepare more stable explosives like 2,4,6-trinitroaniline from PA. Moreover, the recyclability and sensitivity studies indicated that these MOFs can be reused several times with parts per billion (ppb) levels of sensitivity towards PC and 2,4,6-trinitrotoluene (TNT).

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Layered transition metal dichalcogenides (TMDs), such as MoS2, are candidate materials for next generation 2-D electronic and optoelectronic devices. The ability to grow uniform, crystalline, atomic layers over large areas is the key to developing such technology. We report a chemical vapor deposition (CVD) technique which yields n-layered MoS2 on a variety of substrates. A generic approach suitable to all TMDs, involving thermodynamic modeling to identify the appropriate CVD process window, and quantitative control of the vapor phase supersaturation, is demonstrated. All reactant sources in our method are outside the growth chamber, a significant improvement over vapor-based methods for atomic layers reported to date. The as-deposited layers are p-type, due to Mo deficiency, with field effect and Hall hole mobilities of up to 2.4 cm(2) V-1 s(-1) and 44 cm(2) V-1 s(-1) respectively. These are among the best reported yet for CVD MoS2.

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A computational study of the interaction half-sandwich metal fragments (metal=Re/W, electron count=d(6)), containing linear nitrosyl (NO+), carbon monoxide (CO), trifluorophosphine (PF3), N-heterocyclic carbene (NHC) ligands with alkanes are conducted using density functional theory employing the hybrid meta-GGA functional (M06). Electron deficiency on the metal increases with the ligand in the order NHC < CO < PF3 < NO+. Electron-withdrawing ligands like NO+ lead to more stable alkane complexes than NHC, a strong electron donor. Energy decomposition analysis shows that stabilization is due to orbital interaction involving charge transfer from the alkane to the metal. Reactivity and dynamics of the alkane fragment are facilitated by electron donors on the metal. These results match most of the experimental results known for CO and PF3 complexes. The study suggests activation of alkane in metal complexes to be facile with strong donor ligands like NHC. (C) 2015 Wiley Periodicals, Inc.

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Melanosomes are a type of lysosome-related organelle that is commonly defective in Hermansky-Pudlak syndrome. Biogenesis of melanosomes is regulated by BLOC-1, -2, -3, or AP-1, -3 complexes, which mediate cargo transport from recycling endosomes to melanosomes. Although several Rab GTPases have been shown to regulate these trafficking steps, the precise role of Rab9A remains unknown. Here, we found that a cohort of Rab9A associates with the melanosomes and its knockdown in melanocytes results in hypopigmented melanosomes due to mistargeting of melanosomal proteins to lysosomes. In addition, the Rab9A-depletion phenotype resembles Rab38/ 32-inactivated or BLOC-3-deficient melanocytes, suggesting that Rab9A works in line with BLOC-3 and Rab38/ 32 during melanosome cargo transport. Furthermore, silencing of Rab9A, Rab38/ 32 or its effector VARP, or BLOC-3-deficiency in melanocytes decreased the length of STX13-positive recycling endosomal tubules and targeted the SNARE to lysosomes. This result indicates a defect in directing recycling endosomal tubules to melanosomes. Thus, Rab9A and its co-regulatory GTPases control STX13-mediated cargo delivery to maturing melanosomes.

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Resumen: Los estudios sobre los efectos del vecindario sobre los logros educativos han confirmado la existencia de estos efectos particularmente en la adolescencia. Una deficiencia común de la investigación empírica hasta la fecha, la falta de información en múltiples contextos, se aborda en este trabajo mediante el uso de encuestas escolares de datos para obtener una mayor comprensión sobre el efecto de la pobreza en los niños que cursan el nivel primario de enseñanza en los EE.UU. Este trabajo propone la utilización de un modelo jerárquico lineal de clasificación cruzada para tomar en cuenta en forma apropiada la estructura anidada de la información ya que los niños pertenecen simultáneamente a los dos grupos, el barrio y la escuela. Los resultados que se presentan, basados en la encuesta ECLS-K, una muestra de más de 20000 niños en aproximadamente 1.000 vecindarios y 1200 escuelas 1200, pone de manifiesto la asociación entre la composición socioeconómica del vecindario y los resultados académicos de los estudiantes. Este estudio proporciona evidencia a favor de las teorías de la socialización y epidémica. La presencia de adultos con buen nivel educativo en el vecindario así como la mediana de ingresos tienen impacto positivo en el logro del estudiante. De la misma forma, elevados niveles de pobreza tienen una influencia significativa, pero negativa en los resultados académicos. Sin embargo, el impacto se produce cuando se supera el umbral de 30% de hogares pobres en el vecindario. Los resultados agregados son invariantes a distintas especificaciones en términos de variables, esto no sucede cuando se analizan subgrupos clasificados según su origen étnico, género y estatus socio-económico.

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In this paper, equations calculating lift force of a rigid circular cyclinder at lock-in uniform flow are deduced in detail. Besides, equations calculating the lift force on a long flexible circular cyclinder at lock-in are deduced based on mode analysis of a multi-degree freedom system. The simplified forms of these equations are also given. Furthermore, an approximate method to predict the forces and response of rigid circular cyclinders and long flexible circular cyclinders at lock-in is introduced in the case of low mass-damping ratio. A method to eliminate one deficiency of these equations is introduced. Comparison with experimental results show the effectiveness of this approximate method.

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Astrocytes are fundamental for brain homeostasis and the progression and outcome of many neuropathologies including Alzheimer's disease (AD). In the triple transgenic mouse model of AD (3xTg-AD) generalised hippocampal astroglia atrophy precedes a restricted and specific beta-amyloid (A beta) plaque-related astrogliosis. Astrocytes are critical for CNS glutamatergic transmission being the principal elements of glutamate homeostasis through maintaining its synthesis, uptake and turnover via glutamate-glutamine shuttle. Glutamine synthetase (GS), which is specifically expressed in astrocytes, forms glutamine by an ATP-dependent amination of glutamate. Here, we report changes in GS astrocytic expression in two major cognitive areas of the hippocampus (the dentate gyrus, DG and the CA1) in 3xTg-AD animals aged between 9 and 18 months. We found a significant reduction in Nv (number of cell/mm(3)) of GS immunoreactive (GS-IR) astrocytes starting from 12 months (28.59%) of age in the DG, and sustained at 18 months (31.65%). CA1 decrease of GS-positive astrocytes Nv (33.26%) occurs at 18 months. This Nv reduction of GSIR astrocytes is paralleled by a decrease in overall GS expression (determined by its optical density) that becomes significant at 18 months (21.61% and 19.68% in DG and CA1, respectively). GS-IR Nv changes are directly associated with the presence of A beta deposits showing a decrease of 47.92% as opposed to 23.47% in areas free of A beta. These changes in GS containing astrocytes and GS-immunoreactivity indicate AD-related impairments of glutamate homeostatic system, at the advanced and late stages of the disease, which may affect the efficacy of glutamatergic transmission in the diseased brain that may contribute to the cognitive deficiency.

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[ES] Las Pymes colombianas, al igual que las de América Latina, afrontan una serie de barreras para la exportación asociadas a la carencia de recursos y capacidades organizacionales y directivas, las cuales hacen que su permanencia en los mercados internacionales sea cuestionable. Al no superar tales barreras y mostrar una posición competitiva más fuerte difícilmente éstas podrán penetrar y sobrevivir en los mercados internacionales y aprovechar las grandes oportunidades que ofrece el mercado mundial. Especialmente, aquéllas que surgen como resultado de los acuerdos de integración y de los sistemas de preferencia suscritos y otorgados a Colombia.

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Previous work has determined the age distribution from a sample of spotted dolphins (Stenella attenuata) killed in the eastern Pacific tuna purse-seine fishery. In this paper we examine the usefulness of this age distribution for estimating natural mortality rates. The observed age distribution has a deficiency of individuals from 5-15 years and cannot represent a stable age distribution. Sampling bias and errors in age interpretation are examined as possible causes of the "dip" in the observed age structure. Natural mortality rates are estimated for the 15+ age classes based on the assumption that these are sampled representatively. The resulting annual survival rate

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[ES]En las sociedades modernas existe una creciente preocupación por el aumento de la incidencia de la enfermedad renal crónica. Debido a la deficiencia de donantes de órganos y al elevado coste del tratamiento de diálisis, existe la necesidad de desarrollar nuevos tratamientos para estos pacientes. La medicina regenerativa basada en la aplicación de células iPS es una opción prometedora para el tratamiento de esta enfermedad. Sin embargo, la falta de conocimientos sobre el estado pluripotencial de las células y sobre su proceso de diferenciación, así como las limitaciones derivadas del propio procedimiento de reprogramación, impiden su aplicación clínica en un futuro inmediato. Para que se convierta en realidad, numerosas investigaciones se están llevando a cabo con el objetivo de mejorar el procedimiento y hacerlo adecuado para su aplicación clínica. En este trabajo se propone un método que permitiría obtener células iPS a partir de células mesangiales mediante la transfección con un vector no integrativo, el virus Sendai, portador de los genes Oct3/4, Sox2, Klf4 y c-Myc. Al tratarse de un vector no integrativo, se minimizaría el efecto del proceso de reprogramación sobre la estabilidad del genoma celular. Además, en este proyecto se estudiará la capacidad de las células iPS obtenidas para diferenciarse en células progenitoras de podocitos que puedan ser aplicadas específicamente en terapias regenerativas para enfermos renales crónicos.

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An overview is presented on the M74-Syndrome of Baltic salmon which is known since 1974 and which, since 1992, has caused considerable losses of artificially produced yolk-sac larvae in Swedish and Finnish hatcheries responsible for compensatory salmon stocking programmes. The syndrome only affects offspring of wild salmon ascending the rivers for spawning and not offspring derived from salmon broodstocks permanently kept in hatcheries. The syndrome seems to be restricted to the Baltic Sea where it has been recorded in all of the remaining Swedish (except the west coast) and Finnish salmon rivers as well as in populations of Estonian rivers and the Russian River Neva. In Sweden and in Finland, the syndrome has been recorded in recent years in offspring of up to 80 % of female salmon used for spawning and resulted in a larval mortality of up to 90 %. A nutrition-associated thiamine (vitamin B1) deficiency is considered as primary cause. However, other environmental factors seem to be involved in addition. There is concern that the syndrome constitutes a major threat with respect to the survival of the few still naturally reproducing populations of Baltic salmon.

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Germany is one of the iodine deficiency countries in Europe. Marine fish and its products can considerably contribute to the iodine supply via food. The short review gives an overview on the iodine content in fish and other marine species and discusses the influence of house hold cooking and other kitchen preparations. The iodine content in marine fish depends on the species and varies considerably at high level. Lean fish species have an average iodine content of more than 100 μg I / 100 g edible part. The recommended allowance for adults of dietary iodine of 180-200 μg can be covered by the consumption of one marine fish portion per day.

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This paper highlights some of the practices involved in integrated aquaculture such as poultry-cum-fish, pig-cum-fish, sheep and goat-cum-fish and grasscutter-cum-fish. Also the role of fisheries in alleviating protein deficiency was reviewed. Successful research findings on these practices in aquaculture at the Federal College of Forestry, Jericho, Ibadan (Nigeria) will eventually lead to alleviating protein deficiency of the inhabitants of the largest city in West African thus alleviating poverty in the nation