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棕色肠综合征:一种可能的平滑肌线粒体肌病?

The brown bowel syndrome: a possible smooth muscle mitochondrial myopathy?

作者信息

Foster C S

出版信息

Histopathology. 1979 Jan;3(1):1-17. doi: 10.1111/j.1365-2559.1979.tb02977.x.

Abstract

Two cases of lipofuscinosis of the gastrointestinal tract are described, and a mitochondrial origin of the pigment is proposed. The mechanism of formation of lipofuscin is discussed, with particular reference to the maintenance of structurally and functionally intact mitochondrial membranes. The central role of vitamin E is considered, and its biochemical significance to mitochondrial metabolism is emphasized. Comparisons are drawn between the abnormalities demonstrated within the smooth muscle cells of the two cases described, and the muscle cells observed in cases of skeletal muscle myopathies of mitochondrial origin. It is proposed that lipofuscinosis of the gastrointestinal tract, otherwise known as the 'brown bowel syndrome', may be regarded as a smooth muscle myopathy of mitochondrial origin.

摘要

本文描述了两例胃肠道脂褐质沉积症,并提出该色素的线粒体起源。文中讨论了脂褐质的形成机制,特别提及了维持结构和功能完整的线粒体膜。文中考虑了维生素E的核心作用,并强调了其对线粒体代谢的生化意义。对所描述的两例病例的平滑肌细胞内显示的异常情况,与线粒体起源的骨骼肌肌病病例中观察到的肌细胞进行了比较。本文提出,胃肠道脂褐质沉积症,即所谓的“棕色肠综合征”,可被视为线粒体起源的平滑肌肌病。

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