Neutrophil elastase and matrix metalloproteinase 12 in cystic fibrosis lung disease.

ABSTRACT: Chronic lung disease remains the major cause of morbidity and mortality in patients with cystic fibrosis (CF). Recent studies in young children with CF diagnosed by newborn screening identified neutrophil elastase (NE), a major product released from neutrophils in inflamed airways, as a key risk factor for the onset and early progression of CF lung disease. However, the understanding of how NE and potentially other proteases contribute to the complex in vivo pathogenesis of CF lung disease remains limited. In this review, we summarize recent progress in this area based on studies in βENaC-overexpressing (βENaC-Tg) mice featuring CF-like lung disease and novel protease-specific Förster resonance energy transfer (FRET) sensors for localization and quantification of protease activity in the l

SUBMITTER: Wagner CJ 

PROVIDER: S-EPMC4960106 | biostudies-literature | 2016 Dec

REPOSITORIES: biostudies-literature