Article

Small molecule correctors divert CFTR-F508del from ERAD by stabilizing sequential folding states

2023-09-16

Abstract excerpt

<h4>ABSTRACT</h4> Over 80% of people with cystic fibrosis (CF) carry the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR), a chloride ion channel at the apical plasma membrane (PM) of epithelial cells. F508del impairs CFTR folding causing it to be destroyed by endoplasmic reticulum associated degradation (ERAD). Small molecule correctors, which act as pharmacological chaperones t...

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Literature Corpus work: cb024da9-adcc-5232-91f5-7770d07c4a7e
DOI: 10.1101/2023.09.15.556420

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