Journal
AMERICAN JOURNAL OF PATHOLOGY
Volume 184, Issue 6, Pages 1643-1651Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.ajpath.2014.02.003
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Funding
- NHLBI NIH HHS [R01 HL060539, T32 HL007749, P01 HL108793, R01 HL085083, P01 HL114470, R01 HL105489, R01 HL109118] Funding Source: Medline
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A hallmark of idiopathic pulmonary fibrosis (IPF) is excessive and disordered deposition of extracellular matrix. Although the Lung extracellular matrix normally plays an essential rote in development and maintenance of lung tissue through reciprocal interactions with resident cells, the disordered matrix in the diseased lung is increasingly recognized as an active and important contributor to IPF pathogenesis. This working group summary from a recently conducted National Heart, Lung, and Blood Institute strategic planning workshop for IPF research highlights recent advances, challenges, and opportunities in the study of matrix biology in IPF. Particular attention is given to the composition and mechanical properties of the matrix in normal and diseased lungs, and the biochemical and biomechanical influences exerted by pathological matrix. Recently developed model systems are also summarized as key toots for advancing our understanding of matrix biology in IPF. Emerging approaches to therapeutically target the matrix in preclinical and clinical settings are discussed, as are important concepts, such as alterations of the matrix with aging and the potential for the resolution of fibrosis. Specific recommendations for future studies in matrix biology of IPF are also proposed.
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