The motoneuron degeneration in the wobbler mouse is independent of the overexpression of a Bcl2 transgene in neurons
The motoneuron degeneration in the wobbler mouse is independent of the overexpression of a Bcl2 transgene in neurons
复制标题
wobbler 小鼠的运动神经元变性与神经元中 Bcl2 转基因的过度表达无关
DOI：
10.1016/0304-3940(95)12061-8
复制
发表时间：
1995
影响因子：
2.5
通讯作者：
François Rieger
中科院分区：
文献类型：
--
作者：
A. Aı̈t;M. Murawsky;B. Blondet;B. Blondet;D. Hantaz;D. Hantaz;J. Martinou;François Rieger
摇摆鼠突变，常染色体隐性突变，导致运动神经元变性在早期产后发育。产生了神经元过表达人bcl2转基因的转基因小鼠：在自然发生和实验诱导的细胞死亡过程中，bcl2的过表达减少了神经元的损失。在本研究中，我们产生了共同表达wobbler突变基因和bcl2转基因的小鼠，以确定bcl2过表达对wobbler小鼠神经退行性疾病的影响。该疾病的临床症状（虚弱、震颤、体型小）以及生化和组织学参数（肌肉胆碱乙酰转移酶（ChAT）活性、脊髓胶质变）在bcl2阳性和阴性摇摆小鼠中相似。这些结果表明，人类bcl2转基因的神经元特异性表达并不能纠正wobbler突变的影响。
The wobbler mouse mutation, an autosomal recessive mutation, leads to motoneuron degeneration in early post-natal development. Transgenic mice in which neurons overexpress human bcl2 transgene have been generated: the overexpression of bcl2 reduces the neuron loss during naturally occurring and experimentally-induced cell deaths. In the present study, we generate mice co-expressing the wobbler mutant gene and the bcl2 transgene in order to determine the effects of Bcl2 overexpression on the neurodegenerative disorders of the wobbler mouse. The clinical signs of the disease (weakness, tremor, small size) as well as biochemical and histological parameters (choline acetyltransferase (ChAT) activity in muscles, gliosis in spinal cord) are similar in bcl2 positive and negative wobbler mice. These results point to the fact that the neuron-specific expression of the human bcl2 transgene does not correct the effects of the wobbler mutation.