Role of LTBP4 in alveolarization, angiogenesis, and fibrosis in lungs

Insa Bultmann-Mellin, Katharina Dinger, Carolin Debuschewitz, Katharina M.A. Loewe, Yvonne Melcher, Miro T.W. Plum, Sarah Appel, Gunter Rappl, Sebastian Willenborg, Astrid C. Schauss, Christian Jüngst, Marcus Krüger, Sven Dressler, Tomoyuki Nakamura, Frank Wempe, Miguel A.Alejandre Alcázar, Anja Sterner-Kock

Publikation: Beitrag in FachzeitschriftArtikelBegutachtung

19 Zitate (Scopus)

Abstract

Deficiency of the extracellular matrix protein latent transforming growth factor-β (TGF-β)-binding protein-4 (LTBP4) results in lack of intact elastic fibers, which leads to disturbed pulmonary development and lack of normal alveolarization in humans and mice. Formation of alveoli and alveolar septation in pulmonary development requires the concerted interaction of extracellular matrix proteins, growth factors such as TGF-β, fibroblasts, and myofibroblasts to promote elastogenesis as well as vascular formation in the alveolar septae. To investigate the role of LTBP4 in this context, lungs of LTBP4-deficient (Ltbp4-/-) mice were analyzed in close detail. We elucidate the role of LTBP4 in pulmonary alveolarization and show that three different, interacting mechanisms might contribute to alveolar septation defects in Ltbp4-/- lungs: 1) absence of an intact elastic fiber network, 2) reduced angiogenesis, and 3) upregulation of TGF-β activity resulting in profibrotic processes in the lung.

OriginalspracheEnglisch
Seiten (von - bis)L687-L698
FachzeitschriftAmerican Journal of Physiology - Lung Cellular and Molecular Physiology
Jahrgang313
Ausgabenummer4
DOIs
PublikationsstatusVeröffentlicht - Okt. 2017
Extern publiziertJa

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