Universität zu Köln

Przyklenk, Matthias, Georgieva, Veronika S. ORCID: 0000-0002-9234-2504, Metzen, Fabian ORCID: 0000-0002-5248-8218, Mostert, Sebastian, Kobbe, Birgit, Callewaert, Bert, Sengle, Gerhard, Brachvogel, Bent ORCID: 0000-0002-3923-0554, Mecham, Robert P., Paulsson, Mats, Wagener, Raimund, Koch, Manuel ORCID: 0000-0002-2962-7814 and Schiavinato, Alvise (2022). LTBP1 promotes fibrillin incorporation into the extracellular matrix. Matrix Biol., 110. S. 60 - 76. AMSTERDAM: ELSEVIER. ISSN 1569-1802

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Abstract

LTBP1 is a large extracellular matrix protein and an associated ligand of fibrillin-microfibrils. Knowledge of LTBP1 functions is largely limited to its role in targeting and sequestering TGF beta growth factors within the extracellular matrix, thereby regulating their bioavailability. However, the recent description of a wide spectrum of phenotypes in multiple tissues in patients harboring LTBP1 pathogenic variants suggests a multifaceted role of the protein in the homeostasis of connective tissues. To better understand the human pathology caused by LTBP1 deficiency it is important to investigate its functional role in extracellular matrix formation. In this study, we show that LTBP1 coordinates the incorporation of fibrillin-1 and -2 into the extracellular matrix in vitro. We also demonstrate that this function is differentially exerted by the two isoforms, the short and long forms of LTBP1. Thereby our findings uncover a novel TGF beta-independent LTBP1 function potentially contributing to the development of connective tissue disorders. (C) 2022 Elsevier B.V. All rights reserved.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Przyklenk, Matthias UNSPECIFIED UNSPECIFIED UNSPECIFIED Georgieva, Veronika S. UNSPECIFIED orcid.org/0000-0002-9234-2504 UNSPECIFIED Metzen, Fabian UNSPECIFIED orcid.org/0000-0002-5248-8218 UNSPECIFIED Mostert, Sebastian UNSPECIFIED UNSPECIFIED UNSPECIFIED Kobbe, Birgit UNSPECIFIED UNSPECIFIED UNSPECIFIED Callewaert, Bert UNSPECIFIED UNSPECIFIED UNSPECIFIED Sengle, Gerhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Brachvogel, Bent UNSPECIFIED orcid.org/0000-0002-3923-0554 UNSPECIFIED Mecham, Robert P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Paulsson, Mats UNSPECIFIED UNSPECIFIED UNSPECIFIED Wagener, Raimund UNSPECIFIED UNSPECIFIED UNSPECIFIED Koch, Manuel UNSPECIFIED orcid.org/0000-0002-2962-7814 UNSPECIFIED Schiavinato, Alvise UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-679675
DOI:	10.1016/j.matbio.2022.04.004
Journal or Publication Title:	Matrix Biol.
Volume:	110
Page Range:	S. 60 - 76
Date:	2022
Publisher:	ELSEVIER
Place of Publication:	AMSTERDAM
ISSN:	1569-1802
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language BETA BINDING-PROTEIN; N-TERMINAL DOMAINS; TGF-BETA; MARFAN-SYNDROME; LONG FORM; MUTATIONS; FIBRONECTIN; GENE; TROPOELASTIN; MICROFIBRILS Multiple languages Biochemistry & Molecular Biology; Cell Biology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/67967