Cystic fibrosis problem probed by proteolysis

The cystic fibrosis mutation, ΔF508, disrupts CFTR biosynthesis. ΔF508 protein resembles an early intermediate in wild type folding, but fails to adopt a mature conformation and hence cannot escape the ER.

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Correspondence to Michael J. Welsh.

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Welsh, M., Ostedgaard, L. Cystic fibrosis problem probed by proteolysis. Nat Struct Mol Biol 5, 167–169 (1998) doi:10.1038/nsb0398-167

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