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.
003 Detection of serum autoantibodies to extracellular matrix protein 1 (ECM1) and relevant abnormal expression of hemidesmosomal antigens in lichen sclerosus
