title: EDA signaling and skin appendage development.
abstract: The same morphogenetic signals are often involved in the development of different organs. For developing skin appendages, a model for tissue-specific regulation of signaling is provided by the EDA pathway, which accesses the otherwise ubiquitous NFkappaB transcription factors. EDA signaling is mediated by ectodysplasin, EDAR and EDARADD, which form a new TNF ligand-receptor-adaptor family that is restricted to skin appendages in vertebrates from fish to human. The critical function of the pathway was demonstrated in the hereditary genetic disorder Anhidrotic Ectodermal Dysplasia (EDA), which is characterized by defective formation of hair follicles, sweat glands and teeth. The pathway does not appear to initiate the development of the appendages, but is regulated by and regulates the course of further morphogenesis. In mice, transgenic and knockout strains have increasingly revealed features of the mechanism, and suggest possible non-invasive interventions to alleviate EDA deficiency, especially in sweat glands and eyes.
authors.affiliation: Laboratory of Genetics, National Institute on Aging, National Institutes of Health, Baltimore, Maryland 21224, USA.
authors.forename: Chang-Yi
authors.initials: CY
authors.lastname: Cui
authors.forename: David
authors.initials: D
authors.lastname: Schlessinger
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D004476:Ectodermal Dysplasia / Q000235:genetics; D053331:Ectodysplasins / Q000737:chemistry / Q000235:genetics* / Q000502:physiology*; D018507:Gene Expression Regulation, Developmental; D006801:Humans; D008024:Ligands; D051379:Mice; D018345:Mice, Knockout; D008822:Mice, Transgenic; D008954:Models, Biological; D008969:Molecular Sequence Data; D017386:Sequence Homology, Amino Acid; D015398:Signal Transduction; D012867:Skin / Q000196:embryology* / Q000378:metabolism*
chemical_list: D053331:Ectodysplasins; D008024:Ligands