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The Proceedings of the American Thoracic Society 4:92-100 (2007)
© 2007 The American Thoracic Society
doi: 10.1513/pats.200607-147JG

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Using Mouse Genomics to Understand Idiopathic Interstitial Fibrosis

David M. Brass, John Tomfohr, Ivana V. Yang and David A. Schwartz

Laboratory of Respiratory Biology, National Institute of Environmental Health Sciences, Research Triangle Park, North Carolina

Correspondence and requests for reprints should be addressed to David M. Brass, Ph.D., National Institute of Environmental Health Sciences, Rall Building, Room C224, P.O. Box 12233 MD C2-15, 111 Alexander Drive, Research Triangle Park, NC 27709. E-mail: brassd{at}niehs.nih.gov

ABSTRACT

Idiopathic interstitial pneumonia represents a broad category of lung disorders characterized by scarring or fibrosis of the lung accompanied by varying degrees of inflammation. A number of important hypotheses based on clinical observations have substantially contributed to our understanding of the pathogenesis of the most insidious and devastating of the idiopathic interstitial pneumonias, idiopathic interstitial fibrosis (IIF). Patients with IIF usually present late in the course of their illness; thus, animal models of the early, preclinical stage of these diseases are needed. Although no model faithfully recapitulates the clinical course of disease or the histopathology observed in humans, all result in scarring of the lung and may therefore be used to understand the biological processes that contribute to this scarring. The purpose of this article is to summarize the application of mouse genetic and genomic tools to these models to advance our understanding of IIF and to describe emerging agnostic approaches to identifying genes important to the fibroproliferative component of IIF.

Key Words: fibrosis • QTL • genomics • microarray







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