The Ectodysplasin and NFkappaB signalling pathways in odontogenesis.
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.
Courtney
Jo-Maree
JM
Craniofacial Development, Kings College London, Floor 28 Guy's Tower, Guy's Hospital, London Bridge, London SE1 9RT, UK.
Blackburn
James
J
Sharpe
Paul T
PT
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
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