Motor neuronal loss and neurofilament-ubiquitin alteration in MoMuLV-ts1 encephalopathy.
Motor neuronal loss and neurofilament-ubiquitin alteration in MoMuLV-ts1 encephalopathy.
复制标题
MoMuLV-ts1 脑病中的运动神经元丢失和神经丝泛素改变。
DOI：
10.1007/pl00007433
复制
发表时间：
2000
影响因子：
12.7
通讯作者：
Wong,PK
中科院分区：
文献类型：
--
作者：
Stoica,G;Tasca,SI;Wong,PK
莫洛尼鼠白血病病毒（MoMuLV-ts 1）的温度敏感突变体在易感新生小鼠中诱导免疫抑制和海绵状脑病相关的神经元变性主要涉及中枢神经系统（CNS）特定靶区域的运动神经元。神经元损失发生在没有直接病毒感染神经元的情况下，并且是感染小鼠CNS中最显著的病理变化。为了定量地证明神经元的损失，使用光学分割器方法进行了无偏的形态计量体视学研究。使用高度易感的FVB/N小鼠，在接种后20天和35天（dpi），在来自感染和未感染小鼠的脑干的组织切片中定量神经元损失。结果表明，20 dpi时神经元丢失不明显，35 dpi时神经元丢失明显（P< 0.05）。此外，组织学，透射电子显微镜和免疫组织化学显示路易体样包涵体的神经丝和细胞器的聚集体组成。退化的神经元和胶质细胞大量泛素化。总之，这些结果表明，显着的神经元损失发生在疾病过程的结束，路易体样形成和蛋白质泛素化的致病过程ints 1诱导的脑病的一部分。
A temperature-sensitive mutant of Moloney murine leukemia virus (MoMuLV-ts1) induces immunosuppression and spongiform encephalopathy in susceptible newborn mice. The associated neuronal degeneration primarily involves the motor neurons in specific target areas of the central nervous system (CNS). Neuronal loss occurs in the absence of direct viral infection of neurons and is the most dramatic pathological change in the CNS of infected mice. To quantitatively demonstrate neuronal loss, an unbiased morphometric stereological study was undertaken using the optical disector method. Using highly susceptible FVB/N mice, neuronal loss was quantitated in the tissue sections of brain stem from infected and noninfected mice at 20 and 35 days post inoculation (dpi). Results indicated that there was no significant neuronal loss at 20 dpi, but significant (P< 0.05) at 35 dpi. In addition, histology, transmission electron microscopy and immunohistochemistry revealed Lewy body-like inclusions consisting of aggregates of neurofilaments and cellular organelles. Degenerated neurons and glial cells were heavily ubiquitinated. Together, these results suggest that significant neuronal loss occurs at the end of the disease process and that Lewy body-like formation and protein ubiquitination are part of the pathogenic process ints1-induced encephalopathy.