The fibrillin microfibril/elastic fibre network: A critical extracellular supramolecular scaffold to balance skin homoeostasis.

Autor: Adamo CS; Center for Biochemistry, Medical Faculty, University of Cologne, Cologne, Germany.; Department of Pediatrics and Adolescent Medicine, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany., Zuk AV; Center for Biochemistry, Medical Faculty, University of Cologne, Cologne, Germany., Sengle G; Center for Biochemistry, Medical Faculty, University of Cologne, Cologne, Germany.; Department of Pediatrics and Adolescent Medicine, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany.; Center for Molecular Medicine Cologne (CMMC), University of Cologne, Cologne, Germany.; Cologne Center for Musculoskeletal Biomechanics (CCMB), Cologne, Germany.
Jazyk: angličtina
Zdroj: Experimental dermatology [Exp Dermatol] 2021 Jan; Vol. 30 (1), pp. 25-37. Date of Electronic Publication: 2020 Oct 01.
DOI: 10.1111/exd.14191
Abstrakt: 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.
(© 2020 The Authors. Experimental Dermatology published by John Wiley & Sons Ltd.)
Databáze: MEDLINE
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