Possible dysregulation of chaperon and metabolic proteins in cystic fibrosis bronchial tissue |
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Authors: | Frischer Thomas Myung Jae-Kyung Maurer Gerald Eichler Irmgard Szepfalusi Zsolt Lubec Gert |
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Institution: | Department of Pediatrics, Medical University of Vienna, Vienna, Austria. |
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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. |
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Keywords: | Bronchial tissue Cystic fibrosis Mass spectrometry Protein derangement Two‐dimensional gel electrophoresis |
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