Oligodendroglial pathology in the development of myelin breakdown in the dmy mutant rat
Oligodendroglial pathology in the development of myelin breakdown in the dmy mutant rat
复制标题
DOI：
10.1016/j.brainres.2011.03.009
复制
发表时间：
2011-05-10
期刊：
影响因子：
2.9
通讯作者：
Serikawa, Tadao
中科院分区：
文献类型：
--
作者：
Kuwamura, Mitsuru;Inumaki, Kazuo;Serikawa, Tadao
dmy 大鼠是一种常染色体隐性突变体，在整个中枢神经系统白质中表现出严重的髓磷脂破坏。最近，在dmy大鼠中发现了Mrs2内含子3的点突变。 Mrs2 编码酵母和人类细胞线粒体中主要电泳 Mg2+ 流入系统的重要组成部分。在这项研究中，我们检查了 dmy 大鼠脊髓髓磷脂破坏过程中少突胶质细胞的形态和数量变化。 dmy 大鼠中少突胶质细胞的数量从 7 周龄起根据髓鞘质分解迅速减少。经常观察到肥大的少突胶质细胞，并且发现细胞质中抑制素和细胞色素氧化酶（线粒体标记物）呈强阳性。这些数据表明线粒体功能障碍导致少突胶质细胞工作/代偿性肥大，导致直接细胞死亡并导致髓鞘质破坏。 (C) 2011 Elsevier B.V. 保留所有权利。
The dmy rat is an autosomal recessive mutant that exhibits severe myelin destruction throughout the white matter of the central nervous system. Recently, a point mutation in intron 3 of the Mrs2 has been found in the dmy rat. Mrs2 encodes an essential component of the major electrophoretic Mg2+ influx system in mitochondria of yeast as well as human cells. In this study, we examined the morphological and numerical changes of oligodendroctyes in the development of myelin destruction in the spinal cord of the dmy rat. The number of oligodendrocytes decreases rapidly from 7 weeks of age in the dmy rat in accordance with myelin breakdown. Hypertrophic oligodendrocytes were frequently observed, and the cytoplasm was found to be intensely positive for prohibitin and cytochrome oxidase, mitochondrial markers. These data suggest that mitochondrial dysfunction causes a work/compensatory hypertrophy of oligodendrocytes, resulting in direct cell death and leading to myelin destruction. (C) 2011 Elsevier B.V. All rights reserved.