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线粒体疾病中中枢和外周髓鞘的损伤

Impairment of central and peripheral myelin in mitochondrial diseases.

作者信息

Kalman B, Lublin F D, Alder H

机构信息

Department of Neurology, Thomas Jefferson University, Philadelphia, USA.

出版信息

Mult Scler. 1997 Jan;2(6):267-78. doi: 10.1177/135245859700200602.

DOI:10.1177/135245859700200602
PMID:9065917
Abstract

Clinical or sub-clinical impairment of central and peripheral myelin is often part of the overlapping multisystem disorders associated with a variety of mitochondrial (mt)DNA abnormalities. Suboptimal energy metabolism of the oligodendrocytes and Schwann cells carrying mitochondrial defects may cause insufficient production of myelin. Further, edema, vascular and toxic factors may directly damage myelin. The recognition that certain mtDNA point mutations are associated with inflammatory demyelination of the central nervous system suggests that additional mechanisms besides degeneration need to be considered in the development of some forms of myelin damage.

摘要

中枢和外周髓鞘的临床或亚临床损伤通常是与多种线粒体(mt)DNA异常相关的重叠多系统疾病的一部分。携带线粒体缺陷的少突胶质细胞和施万细胞的能量代谢欠佳可能导致髓鞘生成不足。此外,水肿、血管和毒性因素可能直接损伤髓鞘。某些mtDNA点突变与中枢神经系统炎性脱髓鞘相关这一认识表明,在某些形式的髓鞘损伤发展过程中,除了变性之外还需要考虑其他机制。

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