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