The causal genes of ectodermal dysplasia and the role in tooth developement

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  • 外胚葉異形成症の原因遺伝子群 : 歯の発生におけるその役割と最近のトピック

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Ectodermal dysplasia syndromes are human inheritance syndrome showing the affect of hairs, teeth, and glands, being classified to three types for the heredity pattern and the different gene loci, such as ectodermal dysplasia, anhidrotic (EDA/XL-HED), and hypohidrotic ectodermal dysplasia (HED). The recent cloning of the causal genes for the EDA and HED has let to know that the syndrome is caused by the abnormality or the disfunction of the ligand gene (Eda/Ta), receptor gene (Edar/dl), and adopter gene (Edaradd). Furthermore, Ectodysplasin A, the main product of Eda/Ta, is the new member of tumor necrosis factor family showing high specificity of binding to the receptor. The role of EDA and the receptor was considered to be involved in normal tooth development, i.e. (1) regulation of the tooth germ size during cap-early bell stage, and (2) determination of the location of tooth development as same as the case of the development of hair and fish scale.

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