
NCBI . NLM . NIH . GOV {
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Title[redir]:
Cystinosin, the small GTPase Rab11, and the Rab7 effector RILP regulate intracellular trafficking of the chaperone-mediated autophagy receptor LAMP2A - PMC
Description:
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 ...
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Keywords {🔍}
lampa, ctns, cells, cma, lysosomal, trafficking, cystinosis, rab, localization, expression, wildtype, cystinotic, doi, fig, google, scholar, pubmed, cell, membrane, lysosomes, analysis, pmc, rilp, article, lamp, activity, protein, analyzed, free, tfeb, defective, upregulation, substrate, cystine, performed, autophagy, vesicles, data, show, distribution, impaired, treatment, mechanism, lysosome, function, gapdh, images, biol, chaperonemediated, independent,
Topics {✒️}
endoplasmic reticulum quantitative rt-pcr confirmed dominant-negative gtpases induced dominant-negative deletion mutant qrt-pcr analysis confirmation rab7 dominant-negative gtpases internal targeting domain super-resolution microscopy analysis internal ha-tagged dominant-negative rab11 suggest deficient chaperone-mediated autophagy dominant-negative rab7 affected final super-resolution image quantitative rt-pcr chaperone-mediated autophagy leads retinoic acid receptor-α high-sensitivity microscopy approach hrp-conjugated secondary antibodies dimensional super-resolution imaging canonical tyrosine-based motif small-molecule cma activators tirf illumination module mouse β-actin forward tfeb-egfp-s3ar4a anti-mouse secondary antibodies rab11/rab11-fip4-dependent pathway mouse β-actin reverse dominant-negative rab11 tfeb-egfp-δ30 tlr9-initiated cellular responses tert-butyl hydroperoxide rilp-qpcr forward transcription factor eb rab27a effector jfc1/slp1 pmc beta search conjugated donkey anti-rabbit measuring β-hexosaminidase activity includes gdna-eliminator columns lentiviruses expressing gfp-ctns rilp-qpcr reverse oxidative-stress-induced death average log2-transformed signal enhanced trafficking abolished cystine-transporter activity c-terminal tag interferes rab-interacting lysosomal protein pmc copyright notice cma-mediated substrate processing lamp2a trafficking/localization-defective phenotype chaperone-mediated autophagy
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