title: Progress toward improving animal models for idiopathic pulmonary fibrosis.
abstract: Idiopathic pulmonary fibrosis (IPF) remains a disease with an unknown cause and a poor prognosis. Among attempts to define disease pathogenesis, animal models of experimental lung fibrosis have a prominent role. Commonly used models include exposure to bleomycin, silica, fluorescein isothiocyanate; irradiation; or expression of specific genes through a viral vector or transgenic system. These all have been instrumental in the study of lung fibrosis, but all have limitations and fall short of recapitulating a pattern of usual interstitial pneumonia, the pathologic correlate to IPF. A model of repetitive bleomycin lung injury has recently been reported that results in marked lung fibrosis, prominent alveolar epithelial cell hyperplasia, a pattern of temporal heterogeneity and persistence of aberrant remodeling well after stimulus removal, representing a significant addition to the collection of animal lung fibrosis models. Taken together, animal models remain a key component in research strategies to better define IPF pathogenesis.
authors: Division of Allergy, Pulmonary and Critical Care Medicine, Vanderbilt University School of Medicine, Nashville, TN 37232-2650, USA. amber.degryse@vanderbilt.edu
authors: Amber L
authors: AL
authors: Degryse
authors: William E
authors: WE
authors: Lawson
references: J Clin Invest. 2009 Jan;119(1):213-24
references: 19104148
references: J Clin Invest. 2001 Jun;107(12):1529-36
references: 11413160
references: Int J Radiat Oncol Biol Phys. 1995 Jan 15;31(2):361-9
references: 7836090
references: Respir Res. 2005 Oct 07;6:112
references: 16212659
references: Am J Respir Cell Mol Biol. 2009 Dec;41(6):661-70
references: 19265174
references: Am J Respir Cell Mol Biol. 2006 Aug;35(2):175-81
references: 16543609
references: J Clin Invest. 1994 Apr;93(4):1691-9
references: 8163670
references: Am J Pathol. 2005 Nov;167(5):1267-77
references: 16251411
references: Cell. 2005 Jun 17;121(6):823-35
references: 15960971
references: Curr Opin Pulm Med. 2009 Sep;15(5):463-9
references: 19550329
references: Cancer Treat Rev. 1983 Dec;10(4):221-43
references: 6198083
references: Am J Physiol Lung Cell Mol Physiol. 2010 Oct;299(4):L442-52
references: 20562227
references: Am J Respir Crit Care Med. 2000 Feb;161(2 Pt 1):646-64
references: 10673212
references: J Environ Pathol Toxicol Oncol. 1998;17(2):81-97
references: 9546745
references: N Engl J Med. 2001 Aug 16;345(7):517-25
references: 11519507
references: Cancer Res. 1997 Dec 1;57(23):5286-91
references: 9393751
references: Nature. 1990 Mar 15;344(6263):245-7
references: 2156165
references: Int J Biochem Cell Biol. 2008;40(3):362-82
references: 17936056
references: Am J Respir Crit Care Med. 2005 Apr 15;171(8):899-907
references: 15618458
references: Am J Respir Crit Care Med. 2002 Jan 15;165(2):277-304
references: 11790668
references: J Exp Med. 1989 Sep 1;170(3):655-63
references: 2475571
references: J Clin Invest. 1997 Aug 15;100(4):768-76
references: 9259574
references: Int J Radiat Oncol Biol Phys. 2000 Jul 1;47(4):1033-42
references: 10863076
references: J Clin Pathol. 2007 Mar;60(3):283-9
references: 16751304
references: Am J Physiol Lung Cell Mol Physiol. 2008 Feb;294(2):L152-60
references: 17993587
references: J Exp Med. 2004 Aug 2;200(3):377-89
references: 15289506
references: Am J Respir Crit Care Med. 2009 Oct 1;180(7):657-65
references: 19556518
references: Am J Physiol Lung Cell Mol Physiol. 2004 Apr;286(4):L741-9
references: 14660483
references: Am J Pathol. 1999 Nov;155(5):1773-9
references: 10550334
references: Proc Natl Acad Sci U S A. 2006 Aug 29;103(35):13180-5
references: 16924102
references: Am J Respir Cell Mol Biol. 2003 Sep;29(3 Pt 1):375-80
references: 12676806
references: J Clin Invest. 1995 Jul;96(1):250-9
references: 7542280