Article
Emergent properties of proteostasis in managing cystic fibrosis.
Cold Spring Harbor perspectives in biology - 1 Feb 2011
Balch William E, Roth Daniela M, Hutt Darren M
Abstract excerpt
Cystic fibrosis (CF) is a consequence of defective recognition of the multimembrane spanning protein cystic fibrosis conductance transmembrane regulator (CFTR) by the protein homeostasis or proteostasis network (PN) (Hutt and Balch (2010). Like many variant proteins triggering misfolding diseases, mutant CFTR has a complex folding and membrane trafficking itinerary that is managed by the PN to maintain proteome...
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