Universität zu Köln

Zigrino, Paola ORCID: 0000-0002-7470-0064 and Sengle, Gerhard (2019). Fibrillin microfibrils and proteases, key integrators of fibrotic pathways. Adv. Drug Deliv. Rev., 146. S. 3 - 17. AMSTERDAM: ELSEVIER. ISSN 1872-8294

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Abstract

Supramolecular networks composed of multi-domain ECM proteins represent intricate cellular microenvironments which are required to balance tissue homeostasis and direct remodeling. Structural deficiency in ECM proteins results in imbalances in ECM-cell communication resulting often times in fibrotic reactions. To understand how individual components of the ECM integrate communication with the cell surface by presenting growth factors or providing fine-tuned biomechanical properties is mandatory for gaining a better understanding of disease mechanisms in the quest for new therapeutic approaches. Here we provide an overview about what we can learn from inherited connective tissue disorders caused primarily by mutations in fibrillin-1 and binding partners as well as by altered ECM processing leading to defined structural changes and similar functional knock-in mouse models. We will utilize this knowledge to propose new molecular hypotheses which should be tested in future studies. (C) 2018 Elsevier B.V. All rights reserved.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Zigrino, Paola UNSPECIFIED orcid.org/0000-0002-7470-0064 UNSPECIFIED Sengle, Gerhard UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-138307
DOI:	10.1016/j.addr.2018.04.019
Journal or Publication Title:	Adv. Drug Deliv. Rev.
Volume:	146
Page Range:	S. 3 - 17
Date:	2019
Publisher:	ELSEVIER
Place of Publication:	AMSTERDAM
ISSN:	1872-8294
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language GROWTH-FACTOR-BETA; TIGHT-SKIN MOUSE; COLLAGENASE CLOSTRIDIUM-HISTOLYTICUM; THORACIC AORTIC-ANEURYSM; VERSUS-HOST-DISEASE; MAST-CELL CHYMASE; BINDING-PROTEIN 4; TGF-BETA; EXTRACELLULAR-MATRIX; MEPRIN-BETA Multiple languages Pharmacology & Pharmacy Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/13830