Abstract
The ectodermal dysplasias (EDs) are a heterogeneous group of genetic disorders which are identified by the absent or deficient function of at least two derivatives of ectoderm (e.g., skin, nails, sweat glands, or teeth) (1). More than 150 different types of EDs have been identified, and the combined incidence of these disorders may be as high as 7 per 10,000 births. However, a large number of cases go undetected due to the relatively mild phenotype and the apparent ability of the affected individuals to cover up their disease by application of cosmetics, wigs, or dentures. Although Charles Darwin wrote one of the earliest descriptions of an ED involving “the toothless men of Sind” (2), the diagnosis of these disorders may be extremely difficult, partly because there can be many different permutations of ectodermal defects. EDs have been broadly classified into two major subgroups based on the absence or presence of sweat gland function—hidrotic ectodermal dysplasia (or Clouston syndrome) and hypohidrotic ectodermal dysplasias (HEDs). Recent studies suggest an important role of signaling via the tumor necrosis factor receptor (TNFR) family in the pathogenesis of hypohidrotic ectodermal dysplasias, and that will be the focus of this discussion.
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© 2005 Humana Press Inc., Totowa, NJ
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Chaudhary, P.M. (2005). Death Receptor Signaling in Embryonic Ectodermal Development. In: El-Deiry, W.S. (eds) Death Receptors in Cancer Therapy. Cancer Drug Discovery and Development. Humana Press. https://doi.org/10.1385/1-59259-851-X:083
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DOI: https://doi.org/10.1385/1-59259-851-X:083
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