Abnormal pro-gly-pro pathway and airway neutrophilia in pediatric cystic fibrosis.

ABSTRACT:

Background

Proline-glycine-proline (PGP) is a bioactive fragment of collagen generated by the action of matrix metalloproteinase-9 (MMP-9) and prolylendopeptidase (PE), and capable of eliciting neutrophil chemotaxis and epithelial remodelling. PGP is normally then degraded by leukotriene A₄ hydrolase (LTA₄H) to limit inflammation and remodelling. This study hypothesized that early and persistent airway neutrophilia in Cystic Fibrosis (CF) may relate to abnormalities in the PGP pathway and sought to understand underlying mechanisms.

Methods

Broncho-alveolar lavage (BAL) fluid was obtained from 38 CF (9 newborns and 29 older children) and 24 non-CF children. BAL cell differentials and levels of PGP, MMP-9, PE and LTA₄H were assessed.

Results

SUBMITTER: Turnbull AR 

PROVIDER: S-EPMC7001103 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature