Chaperone-mediated autophagy degrades SERPINA1 E342K /α1-antitrypsin Z variant and alleviates cell stress

Abstract excerpt

Chaperone-mediated autophagy (CMA) is a specific form of autophagy that selectively targets proteins containing a KFERQ-like motif and relies on the chaperone protein HSPA8/HSC70 for substrate recognition. In SERPINA1/a1-antitrypsin deficiency (AATD), a disease characterized by the hepatic buildup of the SERPINA1E342K/ATZ, CMA’s role had been unclear. This work demonstrates the critical role that CMA plays in...

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