Neurodegeneration in Progressive Multiple Sclerosis
Neurodegeneration in Progressive Multiple Sclerosis
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DOI：
10.1101/cshperspect.a028985
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发表时间：
2018-10-01
影响因子：
5.4
通讯作者：
Mahad, Don
中科院分区：
文献类型：
--
作者：
Campbell, Graham;Mahad, Don
该神经元是多发性硬化症（MS）中炎性脱髓鞘过程的目标。然而，在进展性MS中，有越来越多的证据表明神经元细胞体内的分子变化。所有这些内在神经元的分子变化都集中在线粒体上，最常见的变化与线粒体呼吸链复合物的缺乏有关。神经元细胞体中产生ATP的能力受损与脱髓鞘轴突对能量的需求增加相结合，这与长束如具有长投射轴突的皮质脊髓束特别相关。我们实验室和合作者最近的工作表明，在实验疾病模型中，固有神经元的分子变化反映有限。神经元室内的线粒体变化是进展性MS的一个未被认识的方面，可能为改善神经元功能和神经保护提供新的靶点。
The neuron is the target of inflammatory demyelinating processes in multiple sclerosis (MS). In progressive MS, however, there is a gathering body of evidence indicating molecular changes within neuronal cell bodies. All of these molecular changes to intrinsic neurons converge on mitochondria, and the most reproduced change relates to mitochondrial respiratory chain complex deficiency. This compromise in the capacity to generate ATP in the neuronal cell body is coupled with an increased demand for energy by the demyelinated axon, which is particularly relevant to the long tracts such as corticospinal tracts with long projection axons. Recent work in our laboratory and that of our collaborators indicate limited reflection of the molecular changes that are intrinsic neurons in the experimental disease models. The mitochondrial changes within neuronal compartments are an under-recognized aspect of progressive MS and likely to offer novel targets for the improvement of neuronal function as well as neuroprotection.