期刊
EXPERT REVIEW OF PROTEOMICS
卷 7, 期 4, 页码 473-486出版社
TAYLOR & FRANCIS LTD
DOI: 10.1586/EPR.10.42
关键词
absolute quantification method; CFTR; CFTR biogenesis; cystic fibrosis; interactomics; proteolysis; proteomics; ubiquitination
资金
- National Institutes of Health [R01 H159410]
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, which encodes an ATP-dependent anion channel. Disease-causing mutations can affect channel biogenesis, trafficking or function, and result in reduced ion transport at the apical surface of many tissues. The most common CFTR mutation is a deletion of phenylalanine at position 508 (Delta F508), which results in a misfolded protein that is prematurely targeted for degradation. This article focuses on how proteomic approaches have been utilized to explore the mechanisms of premature proteolysis in CF. Additionally, we emphasize the potential for proteomic-based technologies in expanding our understanding of CF pathophysiology and therapeutic approaches.
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