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Cytokine Growth Factor Rev. 2014 Apr;25(2):195-203. doi: 10.1016/j.cytogfr.2014.01.004. Epub 2014 Jan 23.

Ectodysplasin A (EDA) - EDA receptor signalling and its pharmacological modulation.

Author information

1
Department of Biochemistry, University of Lausanne, CH-1066 Epalinges, Switzerland.
2
Department of Biochemistry, University of Lausanne, CH-1066 Epalinges, Switzerland. Electronic address: pascal.schneider@unil.ch.

Abstract

The TNF family ligand ectodysplasin A (EDA) regulates the induction, morphogenesis and/or maintenance of skin-derived structures such as teeth, hair, sweat glands and several other glands. Deficiencies in the EDA - EDA receptor (EDAR) signalling pathway cause hypohidrotic ectodermal dysplasia (HED). This syndrome is characterized by the absence or malformation of several skin-derived appendages resulting in hypotrychosis, hypodontia, heat-intolerance, dry skin and dry eyes, susceptibility to airways infections and crusting of various secretions. The EDA-EDAR system is an important effector of canonical Wnt signalling in developing skin appendages. It functions by stimulating NF-κB-mediated transcription of effectors or inhibitors of the Wnt, Sonic hedgehog (SHH), fibroblast growth factor (FGF) and transforming growth factor beta (TGFβ) pathways that regulate interactions within or between epithelial and mesenchymal cells and tissues. In animal models of Eda-deficiency, soluble EDAR agonists can precisely correct clinically relevant symptoms with low side effects even at high agonist doses, indicating that efficient negative feedback signals occur in treated tissues. Hijacking of the placental antibody transport system can help deliver active molecules to developing foetuses in a timely manner. EDAR agonists may serve to treat certain forms of ectodermal dysplasia.

KEYWORDS:

Ectodermal dysplasia; Ectodysplasin; Hair; TNF family; Tooth

PMID:
24508088
DOI:
10.1016/j.cytogfr.2014.01.004
[Indexed for MEDLINE]
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