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Gene Review

EDA2R  -  ectodysplasin A2 receptor

Homo sapiens

Synonyms: EDA-A2 receptor, EDA-A2R, EDAA2R, TNFRSF27, Tumor necrosis factor receptor superfamily member 27, ...
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Disease relevance of EDA2R


High impact information on EDA2R

  • This insertion functions to determine receptor binding specificity, such that EDA-A1 binds only the receptor EDAR, whereas EDA-A2 binds only the related, but distinct, X-linked ectodysplasin-A2 receptor (XEDAR) [2].
  • In this report, we demonstrate that although XEDAR lacks a death domain, it nevertheless induces apoptosis in an EDA-A2-dependent fashion [1].
  • Deletion and point mutagenesis studies delineate two distinct regions in the cytoplasmic domain of XEDAR, which are involved in binding to TRAF3 and -6, respectively, and play a major role in the activation of the NF-kappaB and JNK pathways [3].

Biological context of EDA2R

  • The role of an alternative EDA receptor, localised on the X chromosome (XEDAR) in the developmental control of the differentiation of skin appendages, is discussed [4].
  • XEDAR may represent an early stage in the evolution of death receptors prior to the emergence of the death domain and may play a role in the induction of apoptosis during embryonic development and adult life [1].


  1. Induction of apoptosis by X-linked ectodermal dysplasia receptor via a caspase 8-dependent mechanism. Sinha, S.K., Chaudhary, P.M. J. Biol. Chem. (2004) [Pubmed]
  2. Two-amino acid molecular switch in an epithelial morphogen that regulates binding to two distinct receptors. Yan, M., Wang, L.C., Hymowitz, S.G., Schilbach, S., Lee, J., Goddard, A., de Vos, A.M., Gao, W.Q., Dixit, V.M. Science (2000) [Pubmed]
  3. Role of TRAF3 and -6 in the activation of the NF-kappa B and JNK pathways by X-linked ectodermal dysplasia receptor. Sinha, S.K., Zachariah, S., Quiñones, H.I., Shindo, M., Chaudhary, P.M. J. Biol. Chem. (2002) [Pubmed]
  4. Recent advances in understanding of the molecular basis of anhidrotic ectodermal dysplasia: discovery of a ligand, ectodysplasin A and its two receptors. Wiśniewski, S.A., Kobielak, A., Trzeciak, W.H., Kobielak, K. J. Appl. Genet. (2002) [Pubmed]
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