title: Cystic fibrosis: premature degradation of mutant proteins as a molecular disease mechanism.
authors.affiliation: Department of Biological Sciences, Stanford University, CA, USA.
authors.forename: Marina S
authors.initials: MS
authors.lastname: Gelman
authors.forename: Ron R
authors.initials: RR
authors.lastname: Kopito
mesh_terms: D003546:Cysteine Endopeptidases / Q000378:metabolism; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism* / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D009097:Multienzyme Complexes / Q000378:metabolism; D009154:Mutation; D046988:Proteasome Endopeptidase Complex; D017510:Protein Folding; D025801:Ubiquitin / Q000378:metabolism
chemical_list: C505032:CFTR protein, human; D009097:Multienzyme Complexes; D025801:Ubiquitin; C093349:cystic fibrosis transmembrane conductance regulator delta F508; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D003546:Cysteine Endopeptidases; D046988:Proteasome Endopeptidase Complex