To date, mutations on the genes correlated with proliferation of neural progenitors cause microcephaly, and associated with the neuronal migration induces lissencephaly.Therefore, these two congenital neurodevelopmental disorders have traditionally been classified into different forms of malformations of cerebral cortical development (MCD).However, most recent research reported that mutations in KATNB1, encoding the regulatory subunit of the microtubule-severing katanin, results in a rare congenital cortical malformations characterized by lissencephaly in combination with severe microcephaly (microlissencephaly).In this study, we attempted to clarify the mechanism of microlissencephaly stemming from the mutation of katanin P80.Previous work showed that Katanin P80 regulates the activity of katanin P60 via the C-terminal domain and targets it to the centrosome.Here, we found that the N-terminal WD40 repeats of katanin P80 interacts with cytoplasmic dynein and inhibits its microtubule gliding activity in vitro.Furthermore, katanin P80 not only localizes in the cytoplasm, but also in nuclei via its N-terminal region.siRNA experiments showed that loss of katanin P80 in Mef cells caused defects in cytokinesis.Taken together, mutations in katanin P80 couple neuronal migration defect and abnormal cell division cycle.