The role of chaperone mediated autophagy in neurodegenerative diseases

The role of chaperone mediated autophagy in neurodegenerative diseases

摘要

Chaperone mediated autophagy （CMA） is a selective lysosomal autophagy pathway for the degradation of individual cytosolic soluble proteins.It is different from other autophagic pathways due to its selectivity and distinctive mechanism by which substrates reach the lysosomal lumen.These characteristics of selective targeting and direct substrates translocation determine the contribution of CMA to different physiological functions and the type of pathological conditions associated with CMA dysfunction.Alterations in CMA have recently been shown to underlie some severe human disorders for which the decline with age in the activity of this pathway might become a major aggravating factor.In this review, we will briefly summarize recent findings on the molecular mechanisms behind CMA function, and describe the physiological relevance of the selective lysosomal degradation through this pathway.We also comment on the cellular consequences of CMA malfunction and the well established relationship between CMA dysfunction and neurodegenerative diseases.