glycogen storage disease type Ⅱ (Ⅱ型糖原贮积症) -题库考试答案搜索网

glycogen storage disease type Ⅱ (Ⅱ型糖原贮积症)

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2022-01-02 03:24

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正确答案

是最早发现的贮积症。由于常染色体上的一个隐性基因突变，造成了溶酶体缺乏α；葡萄糖苷酶，缺少了这种酶的溶酶体不能把肝细胞中或肌细胞中过剩的糖原进行水解而大量积累在溶酶体内，造成溶酶体超载。此病多发于婴儿，表现为肌肉无力，心脏增大，心力衰竭，通常于两周内死亡。

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标签：真核细胞内膜系统大学试题

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