SOX10 and Hirschsprung disease: Whereas Sox10 deletion leads to ENS developmental disorders, manifesting as Hirschsprung’s disease in humans, Sox10 overexpression in enteric neural crest stem cells or enteric progenitors cells results in the maintenance of a progenitor state and inhibition of neuronal or glial differentiation (Kim et al. 2003; Bondurand et al. 2006).