Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites.
Saha K, Chevalier B, Doly S, Baatallah N, Guilbert T, Pranke I, Scott MGH, Enslen H, Guerrera C, Chuon C, Edelman A, Sermet-Gaudelus I, Hinzpeter A, Marullo S.
Saha K, et al.
Cell Mol Life Sci. 2022 Sep 27;79(10):530. doi: 10.1007/s00018-022-04554-1.
Cell Mol Life Sci. 2022.
PMID: 36167862
Because of its lower abundance, compared to wild-type CFTR, CFTR-F508del recruitment into COPII vesicles is suppressed by the ER-resident PRAF2. We also demonstrate that some pharmacological chaperones that efficiently rescue CFTR-F508del loss of function in CF patients ta …
Because of its lower abundance, compared to wild-type CFTR, CFTR-F508del recruitment into COPII vesicles is suppressed by the ER-resident …