Raymond J. Deshaies

This paper identifies PNPLA3-I148M as a neomorphic variant that impairs ApoB secretion and lipid metabolism, contributing to fatty liver disease.

This study investigates the cellular effects of the PNPLA3-I148M variant, revealing its role in altering lipid droplet-Golgi dynamics and inducing proteomic and transcriptomic changes associated with liver disease.

The paper quantifies MHC Class I peptide origins under UPS perturbation, revealing 30% of peptides are UPS-independent and 10% proteasome-independent, with implications for immune activation.

This study shows that inhibiting VCP activity rescues degenerating P23H rod cells and improves their function in retinal models of adRP.

This study identifies WIZ as a novel substrate of the CRL4CRBN E3 ubiquitin ligase complex that is degraded by lenalidomide and pomalidomide, highlighting the mechanism of IMiD-induced protein degradation.