Impairment of chaperone-mediated autophagy leads to selective lysosomal degradation defects in the lysosomal storage disease cystinosis

EMBO Molecular Medicine

Volumn 7, Issue 2, 2015, Pages 158-174

  Napolitano, Gennaro ^a   Johnson, Jennifer L ^a   He, Jing ^a   Rocca, Celine J ^b   Monfregola, Jlenia ^a   Pestonjamasp, Kersi ^b   Cherqui, Stephanie ^b   Catz, Sergio D ^a  

^a SCRIPPS RESEARCH INSTITUTE   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Autophagy; CTNS; Cystinosis; Lysosomal storage disorder; Lysosomal trafficking

Indexed keywords

CYSTINE; LYSOSOME ASSOCIATED MEMBRANE PROTEIN 1; LYSOSOME ASSOCIATED MEMBRANE PROTEIN 2; LYSOSOME ASSOCIATED MEMBRANE PROTEIN 2A; MAMMALIAN TARGET OF RAPAMYCIN; PROTEIN P62; UNCLASSIFIED DRUG; AMINO ACID TRANSPORTER; CHAPERONE; CYSTINOSIN PROTEIN, MOUSE;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; AUTOPHAGOSOME; CELL MATURATION; CHAPERONE-MEDIATED AUTOPHAGY; CONFOCAL MICROSCOPY; CONTROLLED STUDY; CYSTINOSIS; DISEASE COURSE; FIBROBLAST; IMMUNOFLUORESCENCE; IN VIVO STUDY; LYSOSOME; MACROAUTOPHAGY; MOUSE; NONHUMAN; OXIDATIVE STRESS; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; ANIMAL; AUTOPHAGY; C57BL MOUSE; GENETICS; HUMAN; KNOCKOUT MOUSE; METABOLISM; PATHOPHYSIOLOGY;

AMINO ACID TRANSPORT SYSTEMS, NEUTRAL; ANIMALS; AUTOPHAGY; CYSTINE; CYSTINOSIS; HUMANS; LYSOSOMAL-ASSOCIATED MEMBRANE PROTEIN 2; LYSOSOMES; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MOLECULAR CHAPERONES;

EID: 84922063991     PISSN: 17574676     EISSN: 17574684     Source Type: Journal    
DOI: 10.15252/emmm.201404223     Document Type: Article

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