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Model mouse for amyotrophic lateral sclerosis and / or anterior temporal lobe degeneration
Model mouse for amyotrophic lateral sclerosis and / or anterior temporal lobe degeneration
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机译：肌萎缩性侧索硬化和/或前颞叶变性的模型小鼠
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摘要
To generate a mouse model exhibiting symptoms similar to those of human amyotrophic lateral sclerosis and/or human frontotemporal lobar degeneration. A mouse model exhibiting symptoms similar to those of human amyotrophic lateral sclerosis and/or human frontotemporal lobar degeneration can be produced by means of generating a mutation in a vertebrate so that expression of a mutant protein with a substitution of tyrosine for alanine at a position corresponding to position 382 of SEQ ID NO. 1 or a mutant protein with a substitution of cysteine for glycine at a position corresponding to position 348 of SEQ ID NO. 1 is regulated by an endogenous TDP-43 gene promoter in at least one allele of an endogenous TDP-43 gene of the vertebrate.
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机译：为了产生表现出类似于人类肌萎缩性侧索硬化症和/或人类额颞叶变性的症状的小鼠模型。可以通过在脊椎动物中产生突变来产生表现出与人类肌萎缩性侧索硬化症和/或人类额颞叶变性相似的症状的小鼠模型，从而在相应位置上用酪氨酸替代丙氨酸的突变蛋白的表达SEQ ID NO.3的位置382。 1或在与SEQ ID NO.3的348位相对应的位置上用半胱氨酸替代甘氨酸的突变蛋白。在脊椎动物的内源性TDP-43基因的至少一个等位基因中，内源性TDP-43基因启动子调节图1所示的基因。
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