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[进行性杜氏肌营养不良症中的线粒体单胺氧化酶]

[Mitochondrial monoamine oxidase in progressive Duchenne muscular dystrophy].

作者信息

Badalian L O, Amanova Z N, Temin P A, Grinio L P, Severina I S

出版信息

Vopr Med Khim. 1985 Jan-Feb;31(1):68-71.

PMID:3984276
Abstract

In children with progressive Duchenne muscular dystrophy distinct impairment in activity, of mitochondrial monoamine oxidase from skeletal muscles correlated with the step of myodystrophic process. The decrease in activity of monoamine oxidase in mitochondria of skeletal muscles, occurred with simultaneous increase in intensity of amines deamination in a medium surrounding the organelles, might de due to "leakage" of the enzyme as a result of deterioration of mitochondrial membranes permeability developed in the disease.

摘要

在进行性杜氏肌营养不良症患儿中,骨骼肌线粒体单胺氧化酶活性的明显损害与肌营养不良过程的阶段相关。骨骼肌线粒体中单胺氧化酶活性降低,同时细胞器周围介质中胺脱氨基强度增加,这可能是由于疾病中发生的线粒体膜通透性恶化导致酶“泄漏”所致。

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