Nerve conduction abnormalities in aging mice deficient for myelin-associated glycoprotein.
Nerve conduction abnormalities in aging mice deficient for myelin-associated glycoprotein.
复制标题
缺乏髓磷脂相关糖蛋白的衰老小鼠的神经传导异常。
DOI：
10.1002/mus.1159
复制
发表时间：
2001
期刊：
影响因子：
3.4
通讯作者：
Quarles,RH
中科院分区：
文献类型：
--
作者：
Weiss,MD;Luciano,CA;Quarles,RH
Ultrastructural, biochemical, and electrophysiological analyses were done on 12–14‐month‐old mice deficient for myelin‐associated glycoprotein (MAG) to further characterize the neuropathy that develops as they age. Electron microscopy demonstrated normal myelin compaction and axonal degeneration in a large number of myelinated nerve fibers. Western blots showed that the proteins of compact myelin, P0 glycoprotein, and myelin basic protein were not significantly altered in the mutants; however, the Schwann cell protein, 2′,3′‐cyclic nucleotide 3′‐phosphodiesterase, was reduced to less than half the control level. Also, both total and phosphorylated high‐molecular‐weight neurofilament proteins (TNFH and PNFH, respectively) were significantly decreased, as was the PNFH:TNFH ratio. Electrophysiological evaluation revealed a mild, but statistically significant, reduction of conduction velocity and a nonsignificant mild decrease in compound muscle action potential amplitudes. This constellation of findings in aging MAG‐null mice is consistent with an axonopathy that resembles axonal Charcot–Marie–Tooth (CMT2) disease in many respects. Thus, mutation of a myelin‐associated gene expressed by Schwann cells can induce axonal degeneration and cause a neuropathy with minimal signs of demyelination. © 2001 John Wiley & Sons, Inc. Muscle Nerve 24: 1380–1387, 2001
