title: Possible dysregulation of chaperon and metabolic proteins in cystic fibrosis bronchial tissue.
abstract: Cystic fibrosis (CF) is an autosomal recessive disease due to mutations of the CF transmembrane conductance regulator gene. A systematic approach to generate a protein expressional pattern in CF bronchial tissue has not been performed so far. It was the aim of this hypothesis-generating study to construct differential proteomes of bronchial biopsies in controls (n = 8) and CF patients (n = 9). Biopsies (pools of three per patient) were taken; proteins were extracted and run on 2-DE with subsequent in-gel digestion and mass spectrometrical identification and quantification of proteins using specific software. Three hundred sixty-six protein spots were identified and compared between groups. Following an approach for multiple testing correction, the chaperone 75 kDa glucose-regulated protein and ubiquinol-cytochrome c reductase complex core protein I and one form of nidogen, a pseudogene of aconitase 2, were increased in CF (p < 0.005). Aberrant protein levels may reflect molecular changes of CF as well as CF-linked inflammation, infection and cellular stress response.
authors.affiliation: Department of Pediatrics, Medical University of Vienna, Vienna, Austria.
authors.forename: Thomas
authors.initials: T
authors.lastname: Frischer
authors.forename: Jae-Kyung
authors.initials: JK
authors.lastname: Myung
authors.forename: Gerald
authors.initials: G
authors.lastname: Maurer
authors.forename: Irmgard
authors.initials: I
authors.lastname: Eichler
authors.forename: Zsolt
authors.initials: Z
authors.lastname: Szepfalusi
authors.forename: Gert
authors.initials: G
authors.lastname: Lubec
mesh_terms: D000154:Aconitate Hydratase / Q000235:genetics; D000293:Adolescent; D000328:Adult; D000595:Amino Acid Sequence; D001706:Biopsy; D001980:Bronchi / Q000378:metabolism* / Q000473:pathology; D002648:Child; D003550:Cystic Fibrosis / Q000378:metabolism*; D014450:Electron Transport Complex III / Q000378:metabolism; D015180:Electrophoresis, Gel, Two-Dimensional; D005260:Female; D018840:HSP70 Heat-Shock Proteins / Q000378:metabolism; D006801:Humans; D008297:Male; D008562:Membrane Glycoproteins / Q000235:genetics / Q000378:metabolism; D008565:Membrane Proteins / Q000378:metabolism; D018832:Molecular Chaperones / Q000378:metabolism*; D008969:Molecular Sequence Data; D020543:Proteome / Q000378:metabolism*; D011544:Pseudogenes; D019032:Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization
chemical_list: D018840:HSP70 Heat-Shock Proteins; D008562:Membrane Glycoproteins; D008565:Membrane Proteins; D018832:Molecular Chaperones; D020543:Proteome; C018551:glucose-regulated proteins; C029967:nidogen; C476079:ACO2 protein, human; D000154:Aconitate Hydratase; D014450:Electron Transport Complex III