作者: Christian S Lobsiger , Don W Cleveland
DOI: 10.1038/NN1988
关键词: Neuron 、 Neuroscience 、 Amyotrophic lateral sclerosis 、 Neurodegeneration 、 Oligodendrocyte 、 Spinocerebellar ataxia 、 Biology 、 Microglia 、 Central nervous system 、 Astrocyte
摘要: A lesson from dominantly inherited forms of diverse neurodegenerative diseases, including amyotrophic lateral sclerosis, spinocerebellar ataxia and Huntington's disease, is that the selective dysfunction or death neuronal population most at risk in each disease not mediated solely by damage mutant protein within target neurons. The disease-causing toxic process, which case caused mutation a gene widely ubiquitously expressed, involves done proteins non-neuronal glial cells central nervous system, especially astrocytes microglia. mechanism non-cell-autonomous, with toxicity derived glia as prominent contributor driving progression some instances even initiation.