Universität zu Köln

Bultmann-Mellin, Insa, Dinger, Katharina, Debuschewitz, Carolin, Loewe, Katharina M. A., Melcher, Yvonne, Plum, Miro T. W., Appel, Sarah, Rappl, Gunter, Willenborg, Sebastian, Schauss, Astrid C., Jungst, Christian ORCID: 0000-0002-6586-7129, Krueger, Marcus ORCID: 0000-0003-2008-4582, Dressler, Sven, Nakamura, Tomoyuki, Wempe, Frank, Alejandre Alcazar, Miguel A. and Sterner-Kock, Anja (2017). Role of LTBP4 in alveolarization, angiogenesis, and fibrosis in lungs. Am. J. Physiol.-Lung Cell. Mol. Physiol., 313 (4). S. L687 - 12. BETHESDA: AMER PHYSIOLOGICAL SOC. ISSN 1522-1504

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Abstract

Deficiency of the extracellular matrix protein latent transforming growth factor-beta (TGF-beta)-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-beta, 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-beta activity resulting in profibrotic processes in the lung.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Bultmann-Mellin, Insa UNSPECIFIED UNSPECIFIED UNSPECIFIED Dinger, Katharina UNSPECIFIED UNSPECIFIED UNSPECIFIED Debuschewitz, Carolin UNSPECIFIED UNSPECIFIED UNSPECIFIED Loewe, Katharina M. A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Melcher, Yvonne UNSPECIFIED UNSPECIFIED UNSPECIFIED Plum, Miro T. W. UNSPECIFIED UNSPECIFIED UNSPECIFIED Appel, Sarah UNSPECIFIED UNSPECIFIED UNSPECIFIED Rappl, Gunter UNSPECIFIED UNSPECIFIED UNSPECIFIED Willenborg, Sebastian UNSPECIFIED UNSPECIFIED UNSPECIFIED Schauss, Astrid C. UNSPECIFIED UNSPECIFIED UNSPECIFIED Jungst, Christian UNSPECIFIED orcid.org/0000-0002-6586-7129 UNSPECIFIED Krueger, Marcus UNSPECIFIED orcid.org/0000-0003-2008-4582 UNSPECIFIED Dressler, Sven UNSPECIFIED UNSPECIFIED UNSPECIFIED Nakamura, Tomoyuki UNSPECIFIED UNSPECIFIED UNSPECIFIED Wempe, Frank UNSPECIFIED UNSPECIFIED UNSPECIFIED Alejandre Alcazar, Miguel A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sterner-Kock, Anja UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-215159
DOI:	10.1152/ajplung.00031.2017
Journal or Publication Title:	Am. J. Physiol.-Lung Cell. Mol. Physiol.
Volume:	313
Number:	4
Page Range:	S. L687 - 12
Date:	2017
Publisher:	AMER PHYSIOLOGICAL SOC
Place of Publication:	BETHESDA
ISSN:	1522-1504
Language:	English
Faculty:	Faculty of Mathematics and Natural Sciences
Divisions:	Faculty of Mathematics and Natural Sciences > Department of Biology > Institute for Genetics
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language TRANSFORMING GROWTH-FACTOR-BETA-1; EXTRACELLULAR-MATRIX; PULMONARY-FIBROSIS; GENE-EXPRESSION; MOUSE LUNG; ELASTIN; MICE; ELASTOGENESIS; COLLAGEN; MODEL Multiple languages Physiology; Respiratory System Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/21515