Sufu and Gli3 repressor mediate the temporal basal-to-apical progression of hair cell differentiation in mammalian cochleae

Abstract

The Sonic Hedgehog pathway plays important roles in mammalian inner ear development. Mutations of Shh, Smo and Gli3 lead to severe defects in mouse inner ear morphogenesis. However, knockout of Gli2 does not affect inner ear morphology or cochlear hair cell differentiation, suggesting that the Gli repressor function may be required for Hedgehog signaling during inner ear development. Sufu is a negative regulator of Hedgehog signaling and it functions to repress Gli activator and enhance Gli repressor ...