Article
Additive effect of multiple pharmacological chaperones on maturation of CFTR processing mutants.
The Biochemical journal - 1 Sept 2007
Wang Ying, Loo Tip W, Bartlett M Claire, Clarke David M
Abstract excerpt
The most common cause of CF (cystic fibrosis) is the deletion of Phe(508) (DeltaF508) in the CFTR [CF TM (transmembrane) conductance regulator] chloride channel. One major problem with DeltaF508 CFTR is that the protein is defective in folding so that little mature protein is delivered to the cel...
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