PCP Effector Proteins Inturned and Fuzzy Play Nonredundant Roles in the Patterning but Not Convergent Extension of Mammalian Neural Tube

PCP effector proteins Inturned (Intu) and Fuzzy (Fuz) play important roles in mammalian neural development and ciliogenesis, but the developmental defects in Intu and Fuz mutants are not the same as those with the complete loss of cilia. Furthermore, it remains unclear whether mouse Intu and Fuz play a role in convergent extension, a process regulated by PCP signaling. In the current study, we show that the functions of both Intu and Fuz in neural tube patterning are dependent on the presence of cilia. We further show that neither gene exhibits obvious genetic interaction with the core PCP regulator Vangl2 in convergent extension or patterning of the neural tube. Finally, we show in Intu; Fuz double mutants that the lack of convergent extension and more severe patterning defects in Intu and Fuz mutants does not result from a functional redundancy between these two proteins. Developmental Dynamics 240:1938-1948, 2011. (C) 2011 Wiley-Liss, Inc.