PMID- 29233876 OWN - NLM STAT- MEDLINE DCOM- 20180122 LR - 20220318 IS - 1744-1358 (Electronic) IS - 0071-1365 (Linking) VI - 61 IP - 6 DP - 2017 Dec 12 TI - Molecular control of chaperone-mediated autophagy. PG - 663-674 LID - 10.1042/EBC20170057 [doi] AB - Chaperone-mediated autophagy (CMA) is a selective form of autophagy in which cytosolic proteins bearing a pentapeptide motif biochemically related to the KFERQ sequence, are recognized by the heat shock protein family A member 8 (HSPA8) chaperone, delivered to the lysomal membrane, and directly translocated across the lysosomal membrane by a protein complex containing lysosomal associated membrane protein 2a (Lamp2a). Since its discovery over two decades ago, the importance of this pathway in cell proteostasis has been made increasingly apparent. Deregulation of this pathway has been implicated in a variety of diseases and conditions, including lysosomal storage diseases, cancer, neurodegeneration and even aging. Here, we describe the main molecular features of the pathway, its regulation, cross-talk with other degradation pathways and importance in disease. CI - (c) 2017 The Author(s). Published by Portland Press Limited on behalf of the Biochemical Society. FAU - Catarino, Steve AU - Catarino S AD - Institute for Biomedical Imaging and Life Sciences (IBILI), Faculty of Medicine, University of Coimbra, Coimbra, Portugal. AD - CNC.IBILI, University of Coimbra, Coimbra, Portugal. FAU - Pereira, Paulo AU - Pereira P AD - Institute for Biomedical Imaging and Life Sciences (IBILI), Faculty of Medicine, University of Coimbra, Coimbra, Portugal. AD - CNC.IBILI, University of Coimbra, Coimbra, Portugal. AD - Chronic Diseases Research Center (CEDOC), NOVA Medical School, Faculdade de Ciencias Medicas, Universidade NOVA de Lisboa, Lisboa, Portugal. FAU - Girao, Henrique AU - Girao H AD - Institute for Biomedical Imaging and Life Sciences (IBILI), Faculty of Medicine, University of Coimbra, Coimbra, Portugal hmgirao@fmed.uc.pt. AD - CNC.IBILI, University of Coimbra, Coimbra, Portugal. LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review DEP - 20171212 PL - England TA - Essays Biochem JT - Essays in biochemistry JID - 0043306 RN - 0 (HSC70 Heat-Shock Proteins) RN - 0 (Lysosomal-Associated Membrane Protein 2) RN - 0 (Molecular Chaperones) SB - IM MH - Animals MH - Autophagy/genetics/*physiology MH - HSC70 Heat-Shock Proteins/genetics/metabolism MH - Humans MH - Lysosomal-Associated Membrane Protein 2/genetics/metabolism MH - Lysosomes/metabolism MH - Molecular Chaperones/genetics/*metabolism OTO - NOTNLM OT - HSPA8 OT - Lamp2a OT - autophagy OT - chaperone-mediated autophagy EDAT- 2017/12/14 06:00 MHDA- 2018/01/23 06:00 CRDT- 2017/12/14 06:00 PHST- 2017/08/17 00:00 [received] PHST- 2017/10/23 00:00 [revised] PHST- 2017/11/01 00:00 [accepted] PHST- 2017/12/14 06:00 [entrez] PHST- 2017/12/14 06:00 [pubmed] PHST- 2018/01/23 06:00 [medline] AID - EBC20170057 [pii] AID - 10.1042/EBC20170057 [doi] PST - epublish SO - Essays Biochem. 2017 Dec 12;61(6):663-674. doi: 10.1042/EBC20170057. Print 2017 Dec 12.