Publication
Title
The extracellular matrix protein 1 (ECM1) in skin biology : an update for the pleiotropic action
Author
Abstract
Extracellular matrix protein 1 (ECM1) is a secreted glycoprotein that plays a pivotal role in the structural and homeostatic biology of the skin, particularly in angiogenesis, reconstitution of basement membrane, proliferation and differentiation of epidermal keratinocytes and dermal fibroblasts, and malignant transformation. This rationale is substantiated by loss-of-function mutations in the ECM1 gene in an autosomal recessive genodermatosis lipoid proteinosis and circulating IgG autoantibodies to this molecule in a humoral autoimmune condition lichen sclerosus, both of which are counterpart disease conditions sharing comparable skin pathology. In the recent decade, considerable progress has been made in determining the in vivo function of ECM1 in animal model studies. Furthermore, underlying insights arose for the genetic predisposition in inflammatory bowel disease ulcerative colitis, acquisition of immune tolerance and allergic responses via particular T cell subsets such as CD4+CD25+ regulatory T cells and Th2 cells, regeneration of certain organs, and clinical advantages for diagnostic and prognostic significance in various cancers. Following our latest review in 2009, we now update the most recent evidences for the pleiotropic action of ECM1 in skin research, and also highlight the novel pathogenic relevance of this molecule in a variety of human disorders.
Language
English
Source (journal)
The open dermatology journal
Publication
2013
ISSN
1874-3722
Volume/pages
7(2013), p. 29-41
Full text (Publishers DOI)
Full text (open access)
UAntwerpen
Faculty/Department
Research group
Publication type
Subject
Affiliation
Publications with a UAntwerp address
External links
Record
Identification
Creation 07.01.2014
Last edited 08.01.2014
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