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21.
Human muscle phosphoglycerate mutase deficiency: newly discovered metabolic myopathy 总被引:7,自引:0,他引:7
Muscle phosphoglycerate mutase activity was decreased (5.7 percent of the lowest control value) in a 52-year-old man with intolerance for strenuous exercise and recurrent pigmenturia since adolescence. All of the other enzymes of glycolysis had normal activities, and glycogen concentration was normal. Electrophoretic, heat lability, and mercury inhibition studies showed that the small residual activity in the patient's muscle was represented by the brain (BB) isoenzyme of phosphoglycerate mutase, suggesting a genetic defect of the M subunit which predominates in normal muscle. The prevalence of the BB isoenzyme in other tissues, including muscle culture, may explain why symptoms were confined to muscle. 相似文献
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An x-ray survey of Centaurus A has given marginal evidence of its x-ray flux. If taken as an upper limit on inverse Compton x-rays generated by scattering interactions between relativistic electrons and cosmological background photons, the observation implies an upper limit of close to 3 degrees K for the background radiation temperature. 相似文献
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The relative migration ratio of plutonium in various rocks is approximately 100 micrometers per meter of waterflow; the corresponding migration ratio for americium is about 500 micrometers per meter of water flow. Under these conditions radioactive decay will have taken place to such an extent that little plutonium and americium can reach the external environment from a well-designed and isolated geological repository site. 相似文献
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Friedman S 《Science (New York, N.Y.)》1968,159(3810):110-111
The presence of trehalose enhances the rate of glucose-6-phosphate hydrolysis in extracts of fat body and other tissues of adult Phormia regina Meigen. The activation appears to be specific, and increasing the concentration of trehalose changes both V(max) and K(m) for glucose-6-phosphate. There is, however, no easily recognizable phosphotransferase in the extract. 相似文献
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Hoskins AA Friedman LJ Gallagher SS Crawford DJ Anderson EG Wombacher R Ramirez N Cornish VW Gelles J Moore MJ 《Science (New York, N.Y.)》2011,331(6022):1289-1295
The spliceosome is the complex macromolecular machine responsible for removing introns from precursors to messenger RNAs (pre-mRNAs). We combined yeast genetic engineering, chemical biology, and multiwavelength fluorescence microscopy to follow assembly of single spliceosomes in real time in whole-cell extracts. We find that individual spliceosomal subcomplexes associate with pre-mRNA sequentially via an ordered pathway to yield functional spliceosomes and that association of every subcomplex is reversible. Further, early subcomplex binding events do not fully commit a pre-mRNA to splicing; rather, commitment increases as assembly proceeds. These findings have important implications for the regulation of alternative splicing. This experimental strategy should prove widely useful for mechanistic analysis of other macromolecular machines in environments approaching the complexity of living cells. 相似文献
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An isotope dilution method using [1‐13C]sodium acetate was applied to determine the effect of feeding ensiled hop (Humulus lupulus L.) residues on plasma acetate turnover rate in six adult crossbred sheep. The sheep were fed 63 g/kg body weight (BW)0.75/day of either mixed hay of orchardgrass (Dactylis glomerata L.) and reed canarygrass (Phalaris arundinacea L.) and round bale silage at 3:1 ratio (Hay‐diet), or another where round bale silage was replaced by ensiled hop residues (Hop‐diet) with a crossover design each of a 3‐week period. The isotope dilution method was performed on day 21 of each dietary treatment. Dry matter digestibility was similar between diets, and nitrogen (N) digestibility was lower (P = 0.001) for Hop‐diet than Hay‐diet. However, N retention did not differ between diets. Plasma acetate concentration was lower (P = 0.04) for Hop‐diet than Hay‐diet, and the turnover rate of plasma acetate did not differ between diets. Plasma concentration of lactate and non‐esterified fatty acids were similar between diets. Hop‐diet was found almost comparable to Hay‐diet on plasma acetate turnover rate in the present experimental conditions. Therefore, it could be concluded that hop residues partially could be used as an alternative to traditionally used round bale silage for rearing sheep. 相似文献