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, 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:
CreatorsEmailORCIDORCID Put Code
Bultmann-Mellin, InsaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dinger, KatharinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Debuschewitz, CarolinUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Loewe, Katharina M. A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Melcher, YvonneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Plum, Miro T. W.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Appel, SarahUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rappl, GunterUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Willenborg, SebastianUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schauss, Astrid C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Jungst, ChristianUNSPECIFIEDorcid.org/0000-0002-6586-7129UNSPECIFIED
Krueger, MarcusUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dressler, SvenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nakamura, TomoyukiUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wempe, FrankUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Alejandre Alcazar, Miguel A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Sterner-Kock, AnjaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
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: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
TRANSFORMING GROWTH-FACTOR-BETA-1; EXTRACELLULAR-MATRIX; PULMONARY-FIBROSIS; GENE-EXPRESSION; MOUSE LUNG; ELASTIN; MICE; ELASTOGENESIS; COLLAGEN; MODELMultiple languages
Physiology; Respiratory SystemMultiple languages
Refereed: Yes
URI: http://kups.ub.uni-koeln.de/id/eprint/21515

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