Behavioral, electrophysiological, and histopathological characterization of a severe murine chronic demyelinating polyneuritis model.
Behavioral, electrophysiological, and histopathological characterization of a severe murine chronic demyelinating polyneuritis model.
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DOI：
10.1111/j.1529-8027.2012.00375.x
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发表时间：
2012-03
期刊：
影响因子：
--
通讯作者：
Sheikh KA
中科院分区：
文献类型：
--
作者：
Ubogu EE;Yosef N;Xia RH;Sheikh KA
本研究的目的是确定雌性B7-2缺陷非肥胖糖尿病（NOD）小鼠自发性自身免疫性周围多神经病变（SAPP）的行为、电生理和形态学特征。以18 ~ 40周龄77只雌性B7-2缺陷小鼠和31只野生型对照NOD小鼠为研究对象。在预先设定的时间点，进行背侧尾侧和坐骨运动神经传导研究（MNCS）。采集坐骨神经进行形态学评价。30周龄后，SAPP小鼠后肢和前肢出现缓慢进行性严重无力，无明显恢复。MNCS表现为24 ~ 27周龄平均复合动作电位幅度和传导速度逐渐降低，平均总波形持续时间增加，在32 ~ 35周龄达到峰值。甲苯胺蓝染色、半薄塑料包埋切片显示病灶性脱髓鞘在病程早期与单个核细胞浸润相关，在严重程度最高时，渐进性弥散性脱髓鞘和轴突丧失与更强烈的单个核浸润相关。免疫组织化学证实巨噬细胞为主的炎症。本研究证实SAPP是一种进行性、持续性慢性炎症性脱髓鞘性多神经病变伴轴突丧失。
The objective of this study was to define the behavioral, electrophysiological, and morphological characteristics of spontaneous autoimmune peripheral polyneuropathy (SAPP) in female B7-2 deficient non-obese diabetic (NOD) mice. A cohort of 77 female B7-2 deficient and 31 wild-type control NOD mice were studied from 18 to 40 weeks of age. At pre-defined time points, the dorsal caudal tail and sciatic motor nerve conduction studies (MNCS) were performed. Sciatic nerves were harvested for morphological evaluation. SAPP mice showed slowly progressive severe weakness in hind and forelimbs without significant recovery after 30 weeks of age. MNCS showed progressive reduction in mean compound motor action potential amplitudes and conduction velocities, and increase in mean total waveform duration from 24 to 27 weeks of age, peaking between 32 and 35 weeks of age. Toluidine blue-stained, semi-thin plastic-embedded sections demonstrated focal demyelination associated with mononuclear cell infiltration early in the disease course, with progressively diffuse demyelination and axonal loss associated with more intense mononuclear infiltration at peak severity. Immunohistochemistry confirmed macrophage-predominant inflammation. This study verifies SAPP as a progressive, unremitting chronic inflammatory demyelinating polyneuropathy with axonal loss.
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影响因子：
12.7
作者：
Rizzuto, N;Morbin, M;Rizzuto, SG
通讯作者：
Rizzuto, SG
影响因子：
3.3
作者：
Xia, Robin H.;Yosef, Nejla;Ubogu, Eroboghene E.
通讯作者：
Ubogu, Eroboghene E.
DOI：
10.1084/jem.20082113
发表时间：
2009-03-16
期刊：
The Journal of experimental medicine
影响因子：
--
作者：
Louvet C;Kabre BG;Davini DW;Martinier N;Su MA;DeVoss JJ;Rosenthal WL;Anderson MS;Bour-Jordan H;Bluestone JA
通讯作者：
Bluestone JA
影响因子：
9.9
作者：
Kieseier, BC;Dalakas, MC;Hartung, HP
通讯作者：
Hartung, HP
影响因子：
3.4
作者：
Xia, Robin H.;Yosef, Nejla;Ubogu, Eroboghene E.
通讯作者：
Ubogu, Eroboghene E.