Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis.

ABSTRACT:

Background

The underlying mechanisms of idiopathic pulmonary fibrosis (IPF) are unknown. This progressive disease has high mortality rates, and current models for prediction of mortality have limited value in identifying which patients will progress. We previously showed that the glycoprotein fibulin-1 is involved in enhanced proliferation and wound repair by mesenchymal cells and, thus, may contribute to lung fibrosis in IPF.

Methods

Serum, lung tissue, and lung function values were obtained from four independent locations (Sydney, NSW, and Perth, WA, Australia; San Francisco, CA; and Modena, Italy). Patients with IPF were followed for a minimum of 1 year and progression was defined as a significant decline in lung function or death. Primary parenchymal lung fibroblasts of

SUBMITTER: Jaffar J 

PROVIDER: S-EPMC4188142 | biostudies-literature | 2014 Oct

REPOSITORIES: biostudies-literature