Nature Structural Biology
5, 167 - 169 (1998)
doi:10.1038/nsb0398-167
Cystic fibrosis problem probed by proteolysisMichael J. Welsh1, 2
& Lynda S. Ostedgaard1
1Michael J. Welsh and Lynda S. Ostedgaard are in the Howard Hughes Medical Institute, Departments of Internal Medicine and Physiology and Biophysics, University of Iowa College of Medicine, Iowa City, Iowa 52245, USA.
2email: mjwelsh@blue.weeg.uiowa.edu The cystic fibrosis mutation,  F508, disrupts CFTR biosynthesis. F508 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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