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Ataxic phenotype and neurodegeneration are triggered by the impairment of chaperone‐mediated autophagy in cerebellar neurons

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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… Click to show full abstract

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.

Keywords: mediated autophagy; chaperone mediated; ataxic phenotype; cma; cerebellar neurons

Journal Title: Neuropathology and Applied Neurobiology
Year Published: 2020

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