The extracellular matrix protein 1 (ECM1) in molecular-based skin biology
Faculty of Pharmaceutical, Biomedical and Veterinary Sciences . Biomedical Sciences
Berlin :Springer Verlag, 2015
Textbook of aging skin / Farage, M.A. [edit.]; et al.
University of Antwerp
Extracellular matrix protein 1 (ECM1) is an 85-kDa secreted glycoprotein that plays a pivotal role in the structural and homeostatic biology of the skin, particularly in the proliferation and differentiation of epidermal keratinocytes, reconstitution of basement membrane, angiogenesis, malignant transformation, and aging. The multifocal interaction of ECM1 with various extracellular matrix and structural molecules 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 now recognized as an immunogenetic disease counterpart sharing comparable skin pathology. A series of underlying insights for the in vivo ECM1 biology, as a binding core and/or a scaffolding protein, arose not only for wide-ranged differentiation properties of the epidermal keratinocytes, acquisition of immune tolerance and allergic responses via particular T cell subsets such as CD4+ CD25+ regulatory T cells and Th2 cells, and various cancers but also from intrinsic and extrinsic aging of the skin.