title: The Ectodysplasin and NFkappaB signalling pathways in odontogenesis.
abstract: Hypohidrotic ectodermal dysplasia (HED) is a congenital disorder affecting organs of ectodermal origin including teeth, hair and sweat glands. Defects in Ectodysplasin (tabby), Edar (downless) and Edar associated death domain (Edaradd) (crinkled) cause HED in both humans and mice. Ectodysplasin is a tumour necrosis factor (TNF) superfamily member whose downstream signalling is transduced by the inhibitor of kappaB kinase (IKK) complex and inhibitors of kappaB (IkappaB) to activate the transcription factor NFkappaB. NFkappaB signalling is involved in a wide range of cellular processes and at each stage the different family members must be tightly regulated for each function. Recent data have demonstrated the importance of this signalling pathway in odontogenesis, particularly in the formation of cusps. Here we review recent advances in our understanding of Ectodysplasin/NFkappaB signalling in tooth development and in particular the central role of the IKK complex.
authors.affiliation: Craniofacial Development, Kings College London, Floor 28 Guy's Tower, Guy's Hospital, London Bridge, London SE1 9RT, UK.
authors.forename: Jo-Maree
authors.initials: JM
authors.lastname: Courtney
authors.forename: James
authors.initials: J
authors.lastname: Blackburn
authors.forename: Paul T
authors.initials: PT
authors.lastname: Sharpe
mesh_terms: D000818:Animals; D004476:Ectodermal Dysplasia / Q000235:genetics / Q000378:metabolism; D053331:Ectodysplasins; D053339:Edar Receptor; D015870:Gene Expression; D006801:Humans; D020672:I-kappa B Proteins / Q000235:genetics / Q000378:metabolism; D008565:Membrane Proteins / Q000235:genetics / Q000378:metabolism*; D016328:NF-kappa B / Q000235:genetics / Q000378:metabolism*; D009805:Odontogenesis / Q000502:physiology*; D011485:Protein Binding; D053338:Receptors, Ectodysplasin; D018124:Receptors, Tumor Necrosis Factor / Q000235:genetics / Q000378:metabolism; D015398:Signal Transduction / Q000502:physiology*; D047988:Tumor Necrosis Factor Receptor-Associated Peptides and Proteins / Q000235:genetics / Q000378:metabolism
chemical_list: C506018:EDA protein, human; C506008:EDAR protein, human; D053331:Ectodysplasins; D053339:Edar Receptor; D020672:I-kappa B Proteins; D008565:Membrane Proteins; D016328:NF-kappa B; D053338:Receptors, Ectodysplasin; D018124:Receptors, Tumor Necrosis Factor; D047988:Tumor Necrosis Factor Receptor-Associated Peptides and Proteins