Ataxic phenotype and neurodegeneration are triggered by the impairment of chaperone‐mediated autophagy in cerebellar neurons
Ataxic phenotype and neurodegeneration are triggered by the impairment of chaperone‐mediated autophagy in cerebellar neurons
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小脑神经元伴侣介导的自噬损伤可触发共济失调表型和神经退行性变
DOI：
10.1111/nan.12649
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发表时间：
2020-07
影响因子：
5
通讯作者：
Masahiro Sato;Tomoko Ohta;Yuria Morikawa;Ayumu Konno;H. Hirai;Y. Kurauchi;A. Hisatsune;H. Katsuki-H.-K
中科院分区：
文献类型：
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作者：
Masahiro Sato;Tomoko Ohta;Yuria Morikawa;Ayumu Konno;H. Hirai;Y. Kurauchi;A. Hisatsune;H. Katsuki-H.-K
伴侣介导的自噬（CMA）是一种参与自噬溶酶体蛋白降解系统的途径。由于CMA参与致病蛋白的降解，它作为神经退行性疾病的一个促成因素而受到关注。我们之前的研究表明，CMA通常在表达引起脊髓小脑共济失调（SCAs）的蛋白质的细胞中受损。因此，我们通过微RNA （miRNA）介导的溶酶体相关蛋白2A （LAMP2A）的敲低，研究了CMA损伤对小脑神经元运动功能和神经存活的影响。
Chaperone‐mediated autophagy (CMA) is a pathway involved in the autophagy lysosome protein degradation system. CMA has attracted attention as a contributing factor to neurodegenerative diseases since it participates in the degradation of disease‐causing proteins. We previously showed that CMA is generally impaired in cells expressing the proteins causing spinocerebellar ataxias (SCAs). Therefore, we investigated the effect of CMA impairment on motor function and the neural survival of cerebellar neurons using the micro RNA (miRNA)‐mediated knockdown of lysosome‐associated protein 2A (LAMP2A), a CMA‐related protein.