نتایج جستجو برای: oculodentodigital dysplasia

تعداد نتایج: 28629  

2016
John J. Kelly Jessica L. Esseltine Qing Shao Ethylin Wang Jabs Jacinda Sampson Mari Auranen Donglin Bai Dale W. Laird

Oculodentodigital dysplasia (ODDD) is a rare genetic disease that affects the development of multiple organs in the human body. More than 70 mutations in the gap junction connexin43 (Cx43) gene, GJA1, are associated with ODDD, most of which are inherited in an autosomal dominant manner. Many patients exhibit similar clinical presentations. However, there is high intrafamilial and interfamilial ...

Journal: :Disease models & mechanisms 2009
Dan Tong Deanne Colley Renee Thoo Tony Y Li Isabelle Plante Dale W Laird Donglin Bai Gerald M Kidder

The essential role of connexin43 (Cx43) during oogenesis has been demonstrated by the severe germ cell deficiency and arrested folliculogenesis observed in Cx43 knockout mice. Recently, another mutant mouse strain became available (Gja1(Jrt)/+) that carries the dominant loss-of-function Cx43 mutation, Cx43(G60S). Gja1(Jrt)/+ mice display features of the human disease oculodentodigital dysplasia...

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