Heterozygous P0 knockout mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP).
Heterozygous P0 knockout mice develop a peripheral neuropathy that resembles chronic inflammatory demyelinating polyneuropathy (CIDP).
复制标题
杂合 P0 基因敲除小鼠会出现类似于慢性炎症性脱髓鞘性多发性神经病 (CIDP) 的周围神经病。
DOI：
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发表时间：
1997
影响因子：
3.2
通讯作者：
S. Scherer
中科院分区：
文献类型：
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作者：
M. Shy;E. Arroyo;J. Sladky;D. Menichella;H. Jiang;W. Xu;J. Kamholz;S. Scherer
脱髓鞘性周围神经病在临床上分为遗传性和获得性两类。遗传性脱髓鞘性神经病是由髓鞘化雪旺细胞表达的基因突变引起的，而获得性脱髓鞘性神经病，包括慢性炎症性脱髓鞘性多发性神经病（CIDP），可能是由自身免疫机制引起的。我们发现，杂合子P0基因敲除（P0+/-）小鼠发展的神经病变，类似CIDP。到一岁时，P0+/-小鼠出现严重的、不对称的运动神经减慢，伴有时间分散或传导阻滞，这是获得性脱髓鞘神经病（包括CIDP）的特征。此外，受影响的神经的形态学分析揭示了严重的和选择性的运动纤维脱髓鞘，脱髓鞘的焦点区域，和炎性细胞。这些数据表明，免疫介导的机制可能有助于P0+/-小鼠神经病变的发病机制。
Demyelinating peripheral neuropathies are clinically divided into inherited and acquired types. Inherited demyelinating neuropathies are caused by mutations in genes expressed by myelinating Schwann cells, whereas acquired ones, including chronic inflammatory demyelinating polyneuropathy (CIDP), are probably caused by autoimmune mechanisms. We find that heterozygous P0 knockout (P0+/-) mice develop a neuropathy that resembles CIDP. By one year of age, P0+/- mice develop severe, asymmetric slowing of motor nerves, with temporal dispersion or conduction block, which are features of acquired demyelinating neuropathies including CIDP. Moreover, morphological analysis of affected nerves reveals severe and selective demyelination of motor fibers, focal regions of demyelination, and inflammatory cells. These data suggest that immune-mediated mechanisms may contribute to the pathogenesis of the neuropathy in P0+/- mice.