Applications of proteomic technologies for understanding the premature proteolysis of CFTR

Mark J. Henderson, Om V. Singh, Pamela L. Zeitlin

Research output: Contribution to journalReview article

Abstract

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 (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.

Original languageEnglish (US)
Pages (from-to)473-486
Number of pages14
JournalExpert Review of Proteomics
Volume7
Issue number4
DOIs
StatePublished - Aug 1 2010

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Keywords

  • CFTR
  • CFTR biogenesis
  • absolute quantification method
  • cystic fibrosis
  • interactomics
  • proteolysis
  • proteomics
  • ubiquitination

ASJC Scopus subject areas

  • Biochemistry
  • Molecular Biology

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