title: Quantitative proteomic profiling reveals differentially regulated proteins in cystic fibrosis cells.
abstract: The most prevalent cause of cystic fibrosis (CF) is the deletion of a phenylalanine residue at position 508 in CFTR (ΔF508-CFTR) protein. The mutated protein fails to fold properly, is retained in the endoplasmic reticulum via the action of molecular chaperones, and is tagged for degradation. In this study, the differences in protein expression levels in CF cell models were assessed using a systems biology approach aided by the sensitivity of MudPIT proteomics. Analysis of the differential proteome modulation without a priori hypotheses has the potential to identify markers that have not yet been documented. These may also serve as the basis for developing new diagnostic and treatment modalities for CF. Several novel differentially expressed proteins observed in our study are likely to play important roles in the pathogenesis of CF and may serve as a useful resource for the CF scientific community.
authors.affiliation: Department of Chemical Physiology, ‡Department of Cell and Molecular Biology, The Scripps Research Institute , 10550 North Torrey Pines Road, La Jolla, California 92037, United States.
authors.forename: Navin
authors.initials: N
authors.lastname: Rauniyar
authors.forename: Vijay
authors.initials: V
authors.lastname: Gupta
authors.forename: William E
authors.initials: WE
authors.lastname: Balch
authors.forename: John R
authors.initials: JR
authors.lastname: Yates
mesh_terms: D015415:Biomarkers / Q000378:metabolism*; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D005786:Gene Expression Regulation / Q000235:genetics / Q000502:physiology*; D006801:Humans; D040901:Proteomics / Q000379:methods*; D049490:Systems Biology / Q000379:methods*
chemical_list: D015415:Biomarkers; C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: CFTR; Cystic Fibrosis; MudPIT; NSAF; cystic fibrosis transmembrane conductance regulator; label free; mass spectrometry; proteomics; spectral count