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Fibulin-1 predicts disease progression in patients with idiopathic pulmonary fibrosis.
Chest
October 2014
The Woolcock Institute of Medical Research, The University of Sydney, Glebe, NSW, Australia; Discipline of Pharmacology, The University of Sydney, Sydney, NSW, Australia; Sydney Medical School, The University of Sydney, and Royal Prince Alfred Hospital (Sydney Local Health District), Sydney, NSW, Australia.
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.
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