title: Cystic fibrosis transmembrane conductance regulator as a model substrate to study endoplasmic reticulum protein quality control in mammalian cells.
abstract: Components of the ubiquitin-proteasome system function on the surface of the endoplasmic reticulum (ER) to select misfolded proteins for degradation. Herein we describe methods that allow for the study of the pathway for proteasomal degradation of the cystic fibrosis transmembrane conductance regulator (CFTR). The experimental system described employs transiently transfected HEK-293 cells and is utilized to monitor the biogenesis of CFTR by Western blot and pulse-chase analysis.
authors.affiliation: Department of Cell and Developmental Biology, University of North Carolina at Chapel Hill, USA.
authors.forename: J Michael
authors.initials: JM
authors.lastname: Younger
authors.forename: Chun-Yang
authors.initials: CY
authors.lastname: Fan
authors.forename: Liling
authors.initials: L
authors.lastname: Chen
authors.forename: Meredith F N
authors.initials: MF
authors.lastname: Rosser
authors.forename: Cam
authors.initials: C
authors.lastname: Patterson
authors.forename: Douglas M
authors.initials: DM
authors.lastname: Cyr
mesh_terms: D002460:Cell Line; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D004721:Endoplasmic Reticulum / Q000201:enzymology*; D015870:Gene Expression; D006801:Humans; D008958:Models, Molecular; D046988:Proteasome Endopeptidase Complex / Q000378:metabolism*; D017510:Protein Folding; D014162:Transfection
chemical_list: C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D046988:Proteasome Endopeptidase Complex