Bcl-2: Prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosis
Bcl-2: Prolonging life in a transgenic mouse model of familial amyotrophic lateral sclerosis
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DOI：
10.1126/science.277.5325.559
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发表时间：
1997-07-25
期刊：
影响因子：
56.9
通讯作者：
Przedborski, S
中科院分区：
文献类型：
--
作者：
Kostic, V;JacksonLewis, V;Przedborski, S
编码铜/锌超氧化物歧化酶的基因的突变产生了家族性肌萎缩侧索硬化症（FALS）的动物模型，这是一种以瘫痪为特征的致命疾病。原癌基因bcl-2的过表达延迟了运动神经元疾病的发作，并延长了转基因小鼠的生存期，这些转基因小鼠表达了93位甘氨酸被丙氨酸取代的cDNAs连锁突变。然而，它并没有改变疾病的持续时间。bcl-2的过表达也减弱了转基因小鼠脊髓运动神经元变性的程度。
Mutations in the gene encoding copper/zinc superoxide dismutase enzyme produce an animal model of familial amyotrophic lateral sclerosis (FALS), a fatal disorder characterized by paralysis. Overexpression of the proto-oncogene bcl-2 delayed onset of motor neuron disease and prolonged survival in transgenic mice expressing the FALS-linked mutation in which glycine is substituted by alanine at position 93. it did not, however, alter the duration of the disease. Overexpression of bcl-2 also attenuated the magnitude of spinal cord motor neuron degeneration in the FALS-transgenic mice.