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

Details

Ressource 1Download: 24508088postcript.pdf (1021.55 [Ko])
State: Public
Version: Author's accepted manuscript
Serval ID
serval:BIB_DF90C801AF92
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Ectodysplasin A (EDA) - EDA receptor signalling and its pharmacological modulation.
Journal
Cytokine and Growth Factor Reviews
Author(s)
Kowalczyk-Quintas C., Schneider P.
ISSN
1879-0305 (Electronic)
ISSN-L
1359-6101
Publication state
Published
Issued date
2014
Volume
25
Number
2
Pages
195-203
Language
english
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
Ectodysplasin, Ectodermal dysplasia, Hair, Tooth, TNF family
Pubmed
Web of science
Open Access
Yes
Create date
20/06/2014 17:37
Last modification date
20/08/2019 16:03
Usage data