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2014 ; 184
(6
): 1643-51
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Matrix biology of idiopathic pulmonary fibrosis: a workshop report of the
national heart, lung, and blood institute
#MMPMID24726499
Thannickal VJ
; Henke CA
; Horowitz JC
; Noble PW
; Roman J
; Sime PJ
; Zhou Y
; Wells RG
; White ES
; Tschumperlin DJ
Am J Pathol
2014[Jun]; 184
(6
): 1643-51
PMID24726499
show ga
A hallmark of idiopathic pulmonary fibrosis (IPF) is excessive and disordered
deposition of extracellular matrix. Although the lung extracellular matrix
normally plays an essential role 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 tools 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.