Autophagy is a major intracellular degradative process that delivers cytoplasmic materials to the lysosome for degradation. An increasing number of studies on the physiological and pathological roles of autophagy in a variety of autophagy knockout models and human diseases were carried out. Among them, the clearance of misfolded proteins is the important function of autophagy. Impairment at different steps of the autophagy system, such as the ubiquitin-proteasome and the autophagy-lysosome pathways, may result in the accumulation of misfolded proteins in insoluble aggregates. Abnormal accumulation of misfolded proteins in cells can lead to a variety of human diseases. Here, we review the major advances in autophagy and the metabolism of misfolding protein in human diseases. Current studies about the promising therapeutic strategy in autophagy-modulating are also summarized.
Keywords: Aggregation; Autophagy; Metabolism; Misfolding protein.