Acid sphingomyelinase deficiency attenuates bleomycin-induced lung inflammation and fibrosis in mice.

ABSTRACT:

Background/aims

The sphingomyelin/ceramide signaling pathway is an important component of many cellular processes implicated in the pathogenesis of lung disease. Acid sphingomyelinase (ASM) is a key mediator of this pathway, but its specific role in pulmonary fibrosis has not been previously investigated. Here we used the bleomycin model of pulmonary fibrosis to investigate fibrotic responses in normal and ASM knockout (ASM(-/-)) mice, and in NIH3T3 fibroblasts with and without ASM siRNA treatment.

Methods

Mice and cells with and without ASM activity were treated with bleomycin, and the effects on lung inflammation, formation of collagen producing myofibroblasts, and apoptosis were assessed.

Results

The development of bleomycin-induced inflammation and fibrosis in wil

SUBMITTER: Dhami R 

PROVIDER: S-EPMC3048941 | biostudies-literature | 2010

REPOSITORIES: biostudies-literature