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Spinal muscular atrophy (SMA) is the leading genetic cause of death in young children, arising from homozygous deletion or mutation of the survival motor neuron 1 (SMN1) gene. SMN protein expressed from a paralogous gene, SMN2, is the primary genetic modifier of SMA; small changes in overall SMN levels cause dramatic changes in disease severity. Thus, deeper insight into mechanisms that regulate SMN protein stability should lead to better therapeutic outcomes. Here, we show that SMA patient-derived missense mutations in the Drosophila SMN Tudor domain exhibit a pronounced temperature sensitivity that affects organismal viability, larval locomotor function and adult longevity. These disease-related phenotypes are domain specific and result from decreased SMN stability at elevated temperature. This system was utilized to manipulate SMN levels during various stages of Drosophila development. Owing to a large maternal contribution of mRNA and protein, Smn is not expressed zygotically during embryogenesis. Interestingly, we find that only baseline levels of SMN are required during larval stages, whereas high levels of the protein are required during pupation. This previously uncharacterized period of elevated SMN expression, during which the majority of adult tissues are formed and differentiated, could be an important and translationally relevant developmental stage in which to study SMN function. Taken together, these findings illustrate a novel in vivo role for the SMN Tudor domain in maintaining SMN homeostasis and highlight the necessity for high SMN levels at crucial developmental time points that are conserved from Drosophila to humans.

The nature and causes of corrosive sulfur induced failures are examined in oil-filled transformers and shunt reactors. Copper sulfide, which is formed when the corrosive sulfur in a mineral oil reacts with the copper conductors, is likely to diffuse into the paper tapes insulating the conductors. Since copper sulfide is partially conducting, the dielectric losses of the contaminated oil-impregnated-paper tapes are markedly increased; paper tapes in close proximity to the copper conductors are found to attain tan [delta] values > 1.0 even at room temperature. It is highly likely that thermal instabilities develop at those sites at operating temperatures, leading to increased loss currents and, ultimately, short circuits between the turns. This sequence of events is substantiated by evidence from the field, which indicates large areas of thermally degraded insulations and charred breakdown regions along the coils, the extent of which becomes more pronounced at higher operating temperatures (toward the top of the windings). Index Terms--Corrosive sulfur, dibenzyl disulfide (DBDS), failures, oil insulation, standardization, transformers/shunt reactors.

Abstract Introduction Culturally validated neurocognitive measures for children in Low- and Middle-Income Countries are important in the timely and correct identification of neurocognitive impairments. Such measures can inform development of interventions for children exposed to additional vulnerabilities like HIV infection. The Battery for Neuropsychological Evaluation of Children (BENCI) is an openly available, computerized neuropsychological battery specifically developed to evaluate neurocognitive impairment. This study adapted the BENCI and evaluated its reliability and validity in Kenya. Methodology The BENCI was adapted using translation and back-translation from Spanish to English. The psychometric properties were evaluated in a case–control study of 328 children (aged 6 – 14 years) living with HIV and 260 children not living with HIV in Kenya. We assessed reliability, factor structure, and measurement invariance with respect to HIV. Additionally, we examined convergent validity of the BENCI using tests from the Kilifi Toolkit. Results Internal consistencies (0.49

Contamination of paper tapes by corrosive sulfur in insulating oils may cause shorting faults between turns. Typically, this occurs at higher temperature in the upper portions of the windings of shunt reactors and power transformers. In many of the tested oils, high amounts of dibenzyl-disulfide (DBDS) were found. Index Terms--Oil insulation, standardization, transformers.

Insulating mineral oils frequently contain additives to improve their inherent characteristics, such as oxidation stability (antioxidants), electrostatic charging tendency, and compatibility with other materials (passivators). Despite standard test methods are available for the detection of individual additives, none of them covers the simultaneous detection of additives of different kind. For the first time the simultaneous determination of antioxidants and passivators most frequently added to mineral insulating oils is provided. The compounds investigated included three inhibitors (N-phenyl-1-naphthylamine, 2,6-di-tert-butylphenol, and 2,6-di-tert-butyl-p-cresol) and two passivators (benzotriazole and Irgamet 39). A solid-phase extraction step, aimed at the reduction of matrix oil components was optimized. Due to the hydrophobic characteristics of the additives, a reversed-phase chromatographic separation was used and optimized. The method here developed was applied to the analysis of inhibited and passivated transformer oils, compared with Standard IEC60666 method, which requires the use of Fourier transform infrared (FT-IR) spectrophotometry (measurement of the adsorption corresponding to the stretching ν(OH) of the phenol group at 3650 cm−1), and finally applied inside a Proficiency Testing Program of the American Society for Testing and Materials International (ASTM). In a unique analysis, the method provides a tool to assess chemical composition and concentration of oil additivation, and it allows discrimination among phenolic inhibitors. [ABSTRACT FROM PUBLISHER]

Vascular disease – including coronary artery disease, carotid artery disease, and peripheral vascular disease – is a leading cause of morbidity and mortality worldwide. The standard of care for restoring patency or bypassing occluded vessels involves using autologous grafts, typically the saphenous veins or internal mammary arteries. Yet, many patients who need life- or limb-saving procedures have poor outcomes, and a third of patients who need vascular intervention have multivessel disease and therefore lack appropriate vasculature to harvest autologous grafts from. Given the steady increase in the prevalence of vascular disease, there is great need for grafts with the biological and mechanical properties of native vessels that can be used as vascular conduits. In this review, we present an overview of methods that have been employed to generate suitable vascular conduits, focusing on the advances in tissue engineering methods and current three-dimensional (3D) bioprinting methods. Tissue-engineered vascular grafts have been fabricated using a variety of approaches such as using preexisting scaffolds and acellular organic compounds. We also give an extensive overview of the novel use of 3D bioprinting as means of generating new vascular conduits. Different strategies have been employed in bioprinting, and the use of cell-based inks to create de novo structures offers a promising solution to bridge the gap of paucity of optimal donor grafts. Lastly, we provide a glimpse of our work to create scaffold-free, bioreactor-free, 3D bioprinted vessels from a combination of rat vascular smooth muscle cells and fibroblasts that remain patent and retain the tensile and mechanical strength of native vessels.

The objective of this study was to analyze lepidopterous insects with diurnal habits (butterflies) associated with a native forest portion. Eight traps with bananas installed at 1.0, 2.0, 3.0 and 4.0 m above soil surface were used. Samplings were obtained on a weekly basis from December 2005 to December 2006 in the county of São Sepé, RS. The faunistic indices represented by frequency, constancy, abundance and dominance were analyzed. Height of flight and species diversity were also evaluated. A total of 737 individuals of the Nymphalidae family were collected, which were classified into six subfamilies, 16 genus and 21 species. The most representative species were: Euptychia sp., Hamadryas feronia, Taygetis ypthima, Epiphile huebneri, Biblis hyperia, Taygetis sp.1 and Praepedaliodes phanias. The diversity indices of Shannon and Margalef indicate greater diversity at the height of 4.0 m and lower diversity at the height of 1.0 m, showing relative abundance as an important parameter in determining diversity. About 70% of the collected species were characterized as frequent, common, non-dominant and of accidental occurrence. Hamadryas feronia and Euptychia sp. were constant, dominant and very frequent. The species Euptychia sp. and Taygetis ypthima had height of flight close to the soil surface.

Com o objetivo de determinar qualitativamente e quantitativamente os pentatomídeos associados a diferentes espécies botânicas nativas, no período de setembro de 2005 a setembro de 2006, foram coletados insetos em nove espécies: Gochnatia polymorpha (Less.) (cambará), Eugenia uniflora Berg. (pitangueira), Acca selowiana (Berg) Burret (goiaba-da-serra), Psidium cattleianum Sabine (araçá), Baccharis spp., Solanaum mauritianum Scop. (fumo-bravo), Micanea cinerascens Miq. (passiquinho), Calliandra brevipes Bhent. (caliandra), Schinus molle L. (aroeira), localizadas na barragem Rodolfo da Costa e Silva, município de Itaara, RS. Foram realizadas coletas quinzenais, com o auxílio de um funil cônico confeccionado com folha de flandres (2mm), com 70cm de diâmetro na maior abertura e 63cm de altura. Retirou-se uma amostra por espécie botânica por data de coleta, obtida através de dez sacudidas dos ramos sobre o funil. Realizadas as coletas, o material foi levado ao Laboratório de Entomologia do Departamento de Defesa Fitossanitária da Universidade Federal de Santa Maria, onde foi realizada a triagem e identificação do material. Foram identificadas nove espécies de Pentatomidae. Edessa rufomarginata (De Geer, 1773) foi à espécie com maior ocorrência, seguida de Thyanta humilis Bergroth, 1891. A espécie botânica S. mauritianum foi a que apresentou o maior número de espécies de Pentatomidae coletados, representando 26,9% do total.

The present work was developed to observe the morphometric features and the morphometric relations for Cordia trichotoma trees in a planted forest, at Santa Maria municipality, state of RS, Brazil. The analised variables were: total height, comercial height of stem, diameter at breast height (dbh), crown diameter, crown length, crown proportion, crown projection area, slenderness rate, salience index, range index and crown formal. For the interdimensional relations study, the morphometric variables were related to diameter at breast height (dbh), by regression analysis. The results showed significative statistic relations between dbh and variables such as total height, crown diameter, crown length, crown proportion, slenderness rate and range index.

The objective of this study was to determine qualitatively and quantitatively the pentatomid fauna associated with the canopy of different native species during the period from September 2005 to September 2006. Insects were collected from among nine botanical species: Gochnatia polymorpha (Less.) (cambará), Eugenia uniflora Berg. (pitangueira), Acca selowiana (Berg) Burret (goiaba-da-serra), Psidium cattleianum Sabine (araçá), Baccharis spp., Solanaum mauritianum Scop. (fumo-bravo), Micanea cinerascens Miq. (passiquinho), Calliandra brevipes Bhent. (caliandra), and Schinus molle L. (aroeira) located at Rodolfo da Costa Dam in Itaara, RS, Brazil. Samples were taken every two weeks with a conical funnel made of tin plate (2mm), 70cm in diameter at the rim and 63cm in height. One sample per botanical species for each sampling date was taken, by shaking the branches, ten times over the funnel. Samples were sent to the Entomology Laboratory of the Crop Protection Department of the Federal University of Santa Maria, where the material was analyzed. A total of nine Pentatomidae species were identified. Edessa rufomarginata (De Geer, 1773) was the species of highest ocurrence followed by Thyanta humilis (Bergroth, 1891). The botanical species S. mauritianum presented the greatest number of Pentatomidae species, with an occurrence of 26.9%.

The objective of this study was to analyze lepidopterous insects with diurnal habits (butterflies) associated with a native forest portion. Eight traps with bananas installed at 1.0, 2.0, 3.0 and 4.0 m above soil surface were used. Samplings were obtained on a weekly basis from December 2005 to December 2006 in the county of São Sepé, RS. The faunistic indices represented by frequency, constancy, abundance and dominance were analyzed. Height of flight and species diversity were also evaluated. A total of 737 individuals of the Nymphalidae family were collected, which were classified into six subfamilies, 16 genus and 21 species. The most representative species were: Euptychia sp., Hamadryas feronia, Taygetis ypthima, Epiphile huebneri, Biblis hyperia, Taygetis sp.1 and Praepedaliodes phanias. The diversity indices of Shannon and Margalef indicate greater diversity at the height of 4.0 m and lower diversity at the height of 1.0 m, showing relative abundance as an important parameter in determining diversity. About 70% of the collected species were characterized as frequent, common, non-dominant and of accidental occurrence. Hamadryas feronia and Euptychia sp. were constant, dominant and very frequent. The species Euptychia sp. and Taygetis ypthima had height of flight close to the soil surface.

The present work was developed to observe the morphometric features and the morphometric relations for Cordia trichotoma trees in a planted forest, at Santa Maria municipality, state of RS, Brazil. The analised variables were: total height, comercial height of stem, diameter at breast height (dbh), crown diameter, crown length, crown proportion, crown projection area, slenderness rate, salience index, range index and crown formal. For the interdimensional relations study, the morphometric variables were related to diameter at breast height (dbh), by regression analysis. The results showed significative statistic relations between dbh and variables such as total height, crown diameter, crown length, crown proportion, slenderness rate and range index.