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Autophagy, as a process of lysosomal-dependent intracellular components degradation, is especially important in disorders where accumulation of the mutant protein is a hallmark, such as Machado-Joseph disease (MJD/SCA3;OMIM 109150), a late onset progressive polyglutamine ataxia, considered the worldwide most common autosomal dominant polyQ SCA caused by a CAG tract expansion in the ATXN3 gene (OMIM 607047).Beclin 1 is one of the autophagy-related proteins, defects in which were found to be correlated with neurodegenerative diseases.