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Two forms of glutathione synthetase deficiency have been described. While one form is mild, causing hemolytic anemia, the other more severe form causes 5-oxo-prolinuria with secondary neurological involvement. Despite the existence of two deficiency phenotypes, Southern blots hybridized with a glutathione synthetase cDNA suggest that there is a single glutathione synthetase gene in the human genome. Analysis of somatic cell hybrids showed the human glutathione synthetase gene (GSS) to be located on chromosome 20, and this assignment has been refined to subband 20q11.2 using in situ hybridization.  相似文献   
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Molecular analysis of cellulose biosynthesis in Arabidopsis   总被引:1,自引:0,他引:1  
Cellulose, an abundant, crystalline polysaccharide, is central to plant morphogenesis and to many industries. Chemical and ultrastructural analyses together with map-based cloning indicate that the RSW1 locus of Arabidopsis encodes the catalytic subunit of cellulose synthase. The cloned gene complements the rsw1 mutant whose temperature-sensitive allele is changed in one amino acid. The mutant allele causes a specific reduction in cellulose synthesis, accumulation of noncrystalline beta-1,4-glucan, disassembly of cellulose synthase, and widespread morphological abnormalities. Microfibril crystallization may require proper assembly of the RSW1 gene product into synthase complexes whereas glucan biosynthesis per se does not.  相似文献   
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The objective of this study was to determine the effects of fat supplementation from canola seed (CS) on ruminal fermentation and postruminal digestion of OM, carbohydrates, and energy of diets containing different levels of forage. Six ruminally and duodenally cannulated beef steers (354 kg +/- 18) were given ad libitum access to six isonitrogenous diets that were offered twice daily in a 6 x 6 Latin square design. Treatments were arranged as a 2 x 3 factorial with two forage levels (70 vs 30% of dietary DM as corn silage) and three forms of CS supplementation including no CS or CS added at 10% of dietary DM as whole CS treated with alkaline hydrogen peroxide or untreated crushed CS. Fat from CS provided 5% of dietary DM. The remaining dietary ingredients were corn, canola meal, molasses, and urea. No interactions (P > .05) between dietary forage level and CS supplementation were observed for ruminal characteristics or digestion of OM, carbohydrates, and energy in the rumen, postruminally, or in the total tract. Fat supplementation from CS did not affect (P > .05) DMI. With few exceptions, fat supplementation did not affect (P > .05) ruminal, postruminal, or total tract digestibilities of OM, structural and nonstructural carbohydrates, and GE. Ruminal disappearance of GE was decreased (P < .05) when diets were supplemented with fat from whole treated CS, and total tract digestibilities of OM and GE were decreased (P < .05) when diets were supplemented with fat from CS in either form. Ruminal pH, concentrations of NH3 N and total VFA, and molar proportions of acetate, propionate, and butyrate were not affected (P > .05) by fat supplementation. Results suggest that fat supplementation from CS (at 5% of dietary DM) as whole treated or untreated crushed had no negative effects on ruminal fermentation of OM, carbohydrates, or energy when steers were given ad libitum access to diets containing high or low forage.  相似文献   
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Linear and nonlinear methods for brain-computer interfaces   总被引:7,自引:0,他引:7  
At the recent Second International Meeting on Brain-Computer Interfaces (BCIs) held in June 2002 in Rensselaerville, NY, a formal debate was held on the pros and cons of linear and nonlinear methods in BCI research. Specific examples applying EEG data sets to linear and nonlinear methods are given and an overview of the various pros and cons of each approach is summarized. Overall, it was agreed that simplicity is generally best and, therefore, the use of linear methods is recommended wherever possible. It was also agreed that nonlinear methods in some applications can provide better results, particularly with complex and/or other very large data sets.  相似文献   
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The protein encoded by the Drosophila cGMP-dependent protein kinase gene, DG1, was expressed in Sf9 cells. cGMP (10 microM) stimulated histone H2B phosphorylation by the DG1 protein kinase 20-fold. Maximal activity was observed at 40-50 mM Mg2+. The concentrations of cGMP, cAMP, cIMP, 8-bromo-cGMP, and 8-bromo-cAMP that gave 50% activation were 0.19 +/- 0.06, 11.7 +/- 2.8, 5.3 +/- 1.5, 0.04 +/- 0. 01, and 0.62 +/- 0.06 microM, respectively. cGMP activation was cooperative with a Hill coefficient (nH) of 1.28 +/- 0.10, whereas activation by cAMP was not cooperative. DG1 kinase expressed in Sf9 cells was found to be a dimer with an amino-terminal dimerization domain. It also autophosphorylated in a reaction stimulated by cGMP and cAMP. Immunoadsorbed DG1 protein from fly extracts was also capable of autophosphorylation, and this assay was used to quantitate the DG1 kinase in extracts from heads and bodies of adults and whole embryos. Activity was highest in heads of either sex and male bodies, intermediate in female bodies, and lowest in embryos. These results were in accord with DG1 mRNA abundance. Tissue distribution of the DG1 kinase was investigated by immunohistochemistry. In embryos, specific immunoreactivity was observed in large cells scattered along the anterior-posterior axis at stage 13. Prominent staining of adult heads was restricted to the proximal level of the lamina cortex.  相似文献   
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