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Cystic fibrosis problem probed by proteolysis
Journal article

Cystic fibrosis problem probed by proteolysis

Michael J Welsh and Lynda S Ostedgaard
Nature structural biology, Vol.5(3), pp.167-169
03/1998
DOI: 10.1038/nsb0398-167
PMID: 9501902

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Abstract

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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