Article
Proteomic interaction profiling reveals KIFC1 as a factor involved in early targeting of F508del-CFTR to degradation.
Cellular and molecular life sciences : CMLS - 1 Dec 2018
Canato Sara, Santos João D, Carvalho Ana S, Aloria Kerman, Amaral Margarida D, Matthiesen Rune, Falcao André O, Farinha Carlos M
Abstract excerpt
Misfolded F508del-CFTR, the main molecular cause of the recessive disorder cystic fibrosis, is recognized by the endoplasmic reticulum (ER) quality control (ERQC) resulting in its retention and early degradation. The ERQC mechanisms rely mainly on molecular chaperones and on sorting motifs, whose presence and exposure determine CFTR retention or exit through the secretory pathway. Arginine-framed tripeptides...
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