Universität zu Köln

Adamo, Christin S. ORCID: 0000-0002-8560-6153, Zuk, Alexandra V. and Sengle, Gerhard (2021). The fibrillin microfibril/elastic fibre network: A critical extracellular supramolecular scaffold to balance skin homoeostasis. Exp. Dermatol., 30 (1). S. 25 - 38. HOBOKEN: WILEY. ISSN 1600-0625

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Abstract

Supramolecular networks composed of fibrillins (fibrillin-1 and fibrillin-2) and associated ligands form intricate cellular microenvironments which balance skin homoeostasis and direct remodelling. Fibrillins assemble into microfibrils which are not only indispensable for conferring elasticity to the skin, but also control the bioavailability of growth factors targeted to the extracellular matrix architecture. Fibrillin microfibrils (FMF) represent the core scaffolds for elastic fibre formation, and they also decorate the surface of elastic fibres and form independent networks. In normal dermis, elastic fibres are suspended in a three-dimensional basket-like lattice of FMF intersecting basement membranes at the dermal-epidermal junction and thus conferring pliability to the skin. The importance of FMF for skin homoeostasis is illustrated by the clinical features caused by mutations in the human fibrillin genes (FBN1,FBN2), summarized as fibrillinopathies. In skin, fibrillin mutations result in phenotypes ranging from thick, stiff and fibrotic skin to thin, lax and hyperextensible skin. The most plausible explanation for this spectrum of phenotypic outcomes is that FMF regulate growth factor signalling essential for proper growth and homoeostasis of the skin. Here, we will give an overview about the current understanding of the underlying pathomechanisms leading to fibrillin-dependent fibrosis as well as forms of cutis laxa caused by mutational inactivation of FMF-associated ligands.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Adamo, Christin S. UNSPECIFIED orcid.org/0000-0002-8560-6153 UNSPECIFIED Zuk, Alexandra V. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sengle, Gerhard UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-317548
DOI:	10.1111/exd.14191
Journal or Publication Title:	Exp. Dermatol.
Volume:	30
Number:	1
Page Range:	S. 25 - 38
Date:	2021
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1600-0625
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language ABNORMAL ELASTIC FIBERS; BETA-BINDING-PROTEINS; AMYLOID-P COMPONENT; TGF-BETA; WEILL-MARCHESANI; MARFAN-SYNDROME; RICH MICROFIBRILS; INTEGRIN BINDING; CELL-ADHESION; GROWTH Multiple languages Dermatology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/31754