Bcl-2过表达不能保护神经元免受突变神经丝介导的运动神经元变性。
Bcl-2 overexpression does not protect neurons from mutant neurofilament-mediated motor neuron degeneration.
作者信息
Houseweart M K, Cleveland D W
机构信息
Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, California 92093, USA.
出版信息
J Neurosci. 1999 Aug 1;19(15):6446-56. doi: 10.1523/JNEUROSCI.19-15-06446.1999.
Abstract
Transgenic mice with a point mutation in the light neurofilament gene develop amyotrophic lateral sclerosis-like motor neuron disease characterized by selective spinal motor neuron loss, neurofilamentous accumulations, and severe muscle atrophy. To test whether the large motor neurons at risk in this disease could be protected from mutant neurofilament-mediated killing, these mice were bred to mice overexpressing the human Bcl-2 proto-oncogene. Elevated levels of Bcl-2 increased the numbers of motor and sensory axons surviving after the developmental period of naturally occurring cell death but did not greatly reduce the number of degenerating axons or protect the large motor neurons from mutant neurofilament-mediated death.
摘要
轻链神经丝基因发生点突变的转基因小鼠会患上肌萎缩侧索硬化样运动神经元疾病，其特征为选择性脊髓运动神经元丧失、神经丝积聚和严重的肌肉萎缩。为了测试这种疾病中处于危险的大型运动神经元是否能够免受突变神经丝介导的杀伤，这些小鼠与过表达人Bcl-2原癌基因的小鼠进行杂交。Bcl-2水平的升高增加了自然发生细胞死亡发育期后存活的运动和感觉轴突数量，但并没有显著减少退化轴突的数量，也没有保护大型运动神经元免受突变神经丝介导的死亡。
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