Chaperone-mediated autophagy (CMA) activator that rescues LAMP2A lysosomal localization in cystinotic cells and reduces toxic α-syn accumulation in PD astrocytes.
QX77 is a chaperone-mediated autophagy (CMA) activator derived from the atypical retinoid AR7 that activates CMA by antagonizing retinoic acid receptor-α (RARα) signaling. QX77 rescues defective trafficking & lysosomal localization of the CMA receptor LAMP2A in cystinotic Ctns-/- MEFs and CTNS-KO human proximal tubule cells (PTCs) by restoring Rab11 expression and Rab11-positive vesicles trafficking to the level seen in wild-type cells (20 μM, 48 hrs). CMA activation by QX77 treatment (20 μM, 5 days) also significantly reduces toxic α-synuclein (α-syn) accumulation in PD patients iPSCs-derived astrocytes with LRRK2 G2019S mutation.
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Nature chemical biology, 9(6), 374-382 (2013-04-16)
Chaperone-mediated autophagy (CMA) contributes to cellular quality control and the cellular response to stress through the selective degradation of cytosolic proteins in lysosomes. A decrease in CMA activity occurs in aging and in age-related disorders (for example, neurodegenerative diseases and
Parkinson's disease (PD) is associated with the degeneration of ventral midbrain dopaminergic neurons (vmDAns) and the accumulation of toxic α-synuclein. A non-cell-autonomous contribution, in particular of astrocytes, during PD pathogenesis has been suggested by observational studies, but remains to be
Frontiers in endocrinology, 10, 21-21 (2019-02-19)
Cystinosis is a lysosomal storage disorder caused by defects in CTNS, the gene that encodes the lysosomal cystine transporter cystinosin. Patients with nephropathic cystinosis are characterized by endocrine defects, defective proximal tubule cell (PTC) function, the development of Fanconi syndrome
The Journal of biological chemistry, 292(25), 10328-10346 (2017-05-04)
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 are incompletely understood. Here
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