نتایج جستجو برای: myofibrillar myopathy
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Nemaline myopathy (NM) is a rare congenital myopathy characterised by hypotonia, muscle weakness, and often skeletal muscle deformities with the presence of nemaline bodies (rods) in the muscle biopsy. The nebulin (NEB) gene is the most commonly mutated and is thought to account for approximately 50% of genetically diagnosed cases of NM. We undertook a detailed muscle morphological analysis of ...
Increases in free Mg from 0.04 to 10.0 mM with constant pH 7.0, 0.10 M ionic strength, and 2 m.M MgATP" caused a rightward shift of the free Ca-relative ATPase relation for both cardiac and skeletal myofibrils. The specific activity of cardiac myofibrillar ATPase over a wide range of free Ca was, however, depressed in 0.04 vs. 1.0 mM Mg" , whereas a similar decrease in free Mg r slightly enhanc...
how to cite this article: nilipour y. lysosomal myopathies. iran j child neurol autumn 2012; 6:4 (suppl. 1):11. pls see pdf.
Digital Rights Management is a term used for technologies that control how digital content is used. It is the restriction put on digital media such as high definition video and audio formats to counteract privacy and copyright infringement. Digital Rights Management can be ensured in many ways such as region coding, copy-protection on media. If fully realised, it will create a significant negat...
In this paper we present a symmetry of a toroidally compactified type II string theory. This symmetry has the interpretaion that it interchanges the left and the right-moving worldsheet coordinates and reverses the orientations of some of the spatial coordinates. We also identify another discrete symmetry of the type II theory which is related to the above one by a nontrivial U-duality element ...
A sequestered fraction of myofibrillar magnesium presumably bound to thin filaments during polymerization of actin was used to determine myofibrillar mass in rat left ventricles and rabbit hearts. Myofibrillar volume was also determined by stereological measurements on electron micrographs of rat ventricles. Myafibrillar Mg (1.11 mmoles/kg dry ventricle) can be determined in glycerinated ventri...
A sequestered fraction of myofibrillar magnesium presumably bound to thin filaments during polymerization of actin was used to determine myofibrillar mass in rat left ventricles and rabbit hearts. Myofibrillar volume was also determined by stereological measurements on electron micrographs of rat ventricles. Myafibrillar Mg (1.11 mmoles/kg dry ventricle) can be determined in glycerinated ventri...
Regulation of myofibrillar protein turnover during maturation in normal and undernourished rat pups.
The study tested the hypothesis that a higher rate of myofibrillar than sarcoplasmic protein synthesis is responsible for the rapid postdifferentiation accumulation of myofibrils and that an inadequate nutrient intake will compromise primarily myofibrillar protein synthesis. Myofibrillar (total and individual) and sarcoplasmic protein synthesis, accretion, and degradation rates were measured in...
Increases in free Mg2+ from 0.04 to 10.0 mM with constant pH 7.0 TO 0.10 M ionic strength, and 2 mM MgATP2- caused a rightward shift of the free Ca-relative ATPase relation for both cardiac skeletal myofibrils. The specific activity of cardiac myofibrillar ATPase over a wide range of free Ca2+ was, however, depressed in 0.04 vs. 1.0 mM Mg2+, whereas a similar decrease in free Mg2+ slightly enha...
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