Cystinosin, the small GTPase Rab11, and the Rab7 effector RILP regulate intracellular trafficking of the chaperone-mediated autophagy receptor LAMP2A

The lysosomal storage disease cystinosis, caused by cystinosin deficiency, is characterized by cell malfunction, tissue failure, and progressive renal injury despite cystine-depletion therapies. Cystinosis is associated with defects in chaperone-mediated autophagy (CMA), but the molecular mechanisms...

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Veröffentlicht in:	The Journal of biological chemistry 2017-06, Vol.292 (25), p.10328-10346
Hauptverfasser:	Zhang, Jinzhong, Johnson, Jennifer L., He, Jing, Napolitano, Gennaro, Ramadass, Mahalakshmi, Rocca, Celine, Kiosses, William B., Bucci, Cecilia, Xin, Qisheng, Gavathiotis, Evripidis, Cuervo, Ana María, Cherqui, Stephanie, Catz, Sergio D.
Format:	Artikel
Sprache:	eng
Schlagworte:	Adaptor Proteins, Signal Transducing - genetics Adaptor Proteins, Signal Transducing - metabolism Amino Acid Substitution Amino Acid Transport Systems, Neutral - genetics Amino Acid Transport Systems, Neutral - metabolism Animals autophagy Cell Biology Cystinosis - genetics Cystinosis - metabolism Cystinosis - pathology Enzyme Activators - pharmacology Gene Expression Regulation, Enzymologic - drug effects Gene Expression Regulation, Enzymologic - genetics lysosomal storage disease Lysosomal-Associated Membrane Protein 2 - genetics Lysosomal-Associated Membrane Protein 2 - metabolism lysosome Lysosomes - genetics Lysosomes - metabolism membrane trafficking Mice Mice, Knockout Point Mutation Protein Transport - genetics rab GTP-Binding Proteins - biosynthesis rab GTP-Binding Proteins - genetics rab GTP-Binding Proteins - metabolism
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