Neurofilament distribution is altered in the Mnd (motor neuron degeneration) mouse.
Neurofilament distribution is altered in the Mnd (motor neuron degeneration) mouse.
复制标题
Mnd（运动神经元变性）小鼠的神经丝分布发生改变。
DOI：
10.1097/00005072-199107000-00009
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发表时间：
1991
影响因子：
3.2
通讯作者：
Mazurkiewicz,JE
中科院分区：
文献类型：
--
作者：
Callahan,LM;Wylen,EL;Messer,A;Mazurkiewicz,JE
运动神经元变性（Mnd）是小鼠的遗传性神经变性疾病，其特征在于运动功能障碍的进行性增加，从后肢移动到前肢，导致瘫痪。使用针对不同磷酸化程度的不同神经丝亚基的抗体，对疾病所有阶段（包括症状前）的Mnd小鼠脊髓运动神经元中神经丝分布进行免疫细胞化学分析。磷酸化神经丝抗体染色的胞体存在于Mnd和对照脊髓中，但Mnd中染色的胞体数量与对照无显著差异。在某些运动神经元的胞质内，神经丝有明显的重新分布。在Mnd而不是对照组中，免疫反应产物出现边缘化，在细胞质中留下免疫染色缺失的区域。这些区域在疾病的所有阶段都可以观察到，但在症状前不太容易预测。这些区域的大小和包含这些区域的运动神经元的数量似乎随着疾病的严重程度而增加。Mnd脊髓L4节段第IX层后肢区前角神经元数目较对照组减少，提示Mnd神经元缺失。
Motor neuron degeneration (Mnd) is a genetic neurodegenerative disease of the mouse that is characterized by a progressive increase in motor dysfunction, moving from hind to fore limbs, leading to paralysis. An immunocytochemical analysis of the neurofilament distribution in spinal motor neurons inMndmice from all stages of the disease, including the presymptomatic, was performed using antibodies to different neurofilament subunits with different degrees of phosphorylation. Perikarya that stained with antibodies to phosphorylated neurofilaments were present inMndand control spinal cords, but the number of stained perikarya inMndwas not significantly different from controls. There was a marked redistribution of neurofilaments within the cytoplasm of some motor neurons inMndcords. InMndbut not controls, the immunoreaction product appeared marginated, leaving areas in the cytoplasm absent of immunostaining. These areas were observed in all stages of the disease, but less predictably in presymptomatics. Both the size of the areas and the number of motoneurons containing these areas appeared to increase with the severity of the disease. The number of anterior horn neurons in the hind limb region of lamina IX in spinal segment L4 ofMndwas lower than in controls, suggesting there is a loss of neurons inMnd.