Previous analysis of Zfhx1b mouse mutants has shed light on its functions in the development of cortical projection neurons (Miquelajauregui et al., 2007; Seuntjens et al., 2009). In humans, mutations of Zfhx1b result in Mowat-Wilson syndrome, a developmental disorder characterized by mental retardation, epilepsy and defects of neural crest-derived tissues, including craniofacial and enteric nervous system (Mowat et al., 2003). Our results that demonstrate Zfhx1b is required to generate cortical interneurons suggest a mechanism for the epilepsy observed in Mowat-Wilson syndrome.
