Article
Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites.
Cellular and molecular life sciences : CMLS - 27 Sept 2022
Saha Kusumika, Chevalier Benoit, Doly Stéphane, Baatallah Nesrine, Guilbert Thomas, Pranke Iwona, Scott Mark G H, Enslen Hervé, Guerrera Chiara, Chuon Cérina, Edelman Aleksander, Sermet-Gaudelus Isabelle, Hinzpeter Alexandre, Marullo Stefano
Abstract excerpt
The endoplasmic reticulum exit of some polytopic plasma membrane proteins (PMPs) is controlled by arginin-based retention motifs. PRAF2, a gatekeeper which recognizes these motifs, was shown to retain the GABAB-receptor GB1 subunit in the ER. We report that PRAF2 can interact on a stoichiometric basis with both wild type and mutant F508del Cystic Fibrosis (CF) Transmembrane Conductance Regulator (CFTR),...
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