Pathophysiology of chaperone-mediated autophagy |
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Authors: | Massey Ashish Kiffin Roberta Cuervo Ana Maria |
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Institution: | Department of Anatomy and Structural Biology, Marion Bessin Liver Research Center, Albert Einstein College of Medicine, Ullmann Building Room 614, 1300 Morris Park Avenue, Bronx, NY 10461, USA |
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Abstract: | In contrast to the classically described “in bulk” lysosomal degradation, the first evidence for selective degradation of cytosolic proteins in lysosomes was presented more than 20 years ago. Throughout this time, we have gained a better understanding about this process, now known as chaperone-mediated autophagy (CMA). The identification of new substrates for CMA and novel components, in both the cytosol and the lysosomes, along with better insights on how CMA is regulated, have all helped to shape the possible physiological roles of CMA. We review here different intracellular functions of CMA that arise from its unique characteristics when compared to other forms of autophagy. In view of these functions, we discuss the relevance of the changes in CMA activity in aging and in different pathological conditions. |
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Keywords: | Lysosomes Aging Lysosomal storage disorders Nephropathy Neurodegeneration |
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