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

Détails

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Etat: Public
Version: Author's accepted manuscript
ID Serval
serval:BIB_DF90C801AF92
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Institution
Titre
Ectodysplasin A (EDA) - EDA receptor signalling and its pharmacological modulation.
Périodique
Cytokine and Growth Factor Reviews
Auteur⸱e⸱s
Kowalczyk-Quintas C., Schneider P.
ISSN
1879-0305 (Electronic)
ISSN-L
1359-6101
Statut éditorial
Publié
Date de publication
2014
Volume
25
Numéro
2
Pages
195-203
Langue
anglais
Résumé
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.
Mots-clé
Ectodysplasin, Ectodermal dysplasia, Hair, Tooth, TNF family
Pubmed
Web of science
Open Access
Oui
Création de la notice
20/06/2014 17:37
Dernière modification de la notice
20/08/2019 16:03
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