Article
Interactions between Sox10 and EdnrB modulate penetrance and severity of aganglionosis in the Sox10Dom mouse model of Hirschsprung disease.
Human molecular genetics - 1 Oct 2004
Cantrell V Ashley, Owens Sarah E, Chandler Ronald L, Airey David C, Bradley Kevin M, Smith Jeffrey R, Southard-Smith E Michelle
Abstract excerpt
Cumulative evidence suggests that Hirschsprung disease (HSCR) is the consequence of multiple gene interactions that modulate the ability of enteric neural crest (NC) cells to populate the developing gut. One of the essential genes for this process is the NC transcription factor Sox10. Sox10Dom mice on a mixed genetic background show variation in penetrance and expressivity of enteric aganglionosis that are...
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