Journal
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
Volume 42, Issue 5, Pages 517-523Publisher
AMER THORACIC SOC
DOI: 10.1165/rcmb.2009-0101TR
Keywords
gremlin; pulmonary hypertension; bone morphogenetic protein
Funding
- Actelion
- Pfizer
- GlaxoSmithKline
- Lung Rx
- Sanofi
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Gremlin is an extracellular glycoprotein that was first identified over a decade ago through its important role in embryonic development, in which it acts as an antagonist of bone morphogenetic protein actions. It plays a critical role in the development of normal airways and the pulmonary circulation in the embryo. More recently, considerable evidence has been presented for a role for gremlin in the pathogenesis of lung diseases, particularly pulmonary hypertension and idiopathic pulmonary fibrosis. The purpose of this article is to review this evidence, consider the potential mechanisms and multicellular actions by which gremlin contributes to disease pathogenesis, and suggest future avenues of research.
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