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[贝克尔 - 金纳型肌营养不良症患者血浆和红细胞中游离氨基酸的分布]

[Distribution of free amino acid in blood plasma and erythrocytes in Becker-Kiner muscular dystrophy].

作者信息

Badalian L O, Grinio L P, Temin P A, Dzhutova E D, Kushelev A R

出版信息

Vopr Med Khim. 1984 Jul-Aug;30(4):132-6.

PMID:6506580
Abstract

Content of free amino acids was altered in blood plasma and erythrocytes of patients with Becker-Kiner progressive muscular dystrophy; these alterations were more distinct in blood plasma as compared with erythrocytes. Spectra of individual free amino acids were distinctly altered both in blood plasma and erythrocytes. Development of the myodystrophia process correlated with alterations in glycogen and branched-chain amino acids content. The data obtained suggest the important functions of erythrocytes in intertissue transport of free amino acids. Similarity between the alterations, observed in progressive muscular dystrophy and in other pathological states accompanied by accelerated metabolism of proteins, suggests that these impairments are among the components in the adaptation reactions to metabolic stresses.

摘要

贝克-金纳进行性肌营养不良患者血浆和红细胞中游离氨基酸的含量发生了改变;与红细胞相比,这些改变在血浆中更为明显。血浆和红细胞中单个游离氨基酸的光谱均有明显改变。肌营养不良过程的发展与糖原和支链氨基酸含量的改变相关。所获得的数据表明红细胞在游离氨基酸的组织间转运中具有重要功能。在进行性肌营养不良以及其他伴有蛋白质代谢加速的病理状态中观察到的改变之间的相似性,表明这些损伤是代谢应激适应反应的组成部分。

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