Article
Correctors promote folding of the CFTR in the endoplasmic reticulum.
The Biochemical journal - 1 Jul 2008
Loo Tip W, Bartlett M Claire, Clarke David M
Abstract excerpt
Cystic fibrosis (CF) is most commonly caused by deletion of a residue (DeltaF508) in the CFTR (cystic fibrosis transmembrane conductance regulator) protein. The misfolded mutant protein is retained in the ER (endoplasmic reticulum) and is not trafficked to the cell surface (misprocessed mutant). Corrector molecules such as corr-2b or corr-4a are small molecules that increase the amount of functional CFTR at the...
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