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Mutations in the  Aristaless-related homeobox  ( ARX ) gene are found in a spectrum of epilepsy and X-linked intellectual disability disorders. During development  Arx  is expressed in pallial ventricular zone (VZ) progenitor cells where the excitatory projection neurons of the cortex are born.  Arx −/Y   mice were shown to have decreased proliferation in the cortical VZ resulting in smaller brains; however, the basis for this reduced proliferation was not established. To determine the role of ARX on cell cycle dynamics in cortical progenitor cells, we generated cerebral cortex-specific  Arx  mouse mutants (cKO). The loss of pallial  Arx  resulted in the reduction of cortical progenitor cells, particularly the proliferation of intermediate progenitor cells (IPCs) was affected. Later in development and postnatally cKO brains showed a reduction of upper layer but not deeper layer neurons consistent with the IPC defect. Transcriptional profile analysis of E14.5  Arx -ablated cortices compared with control revealed that CDKN1C, an inhibitor of cell cycle progression, is overexpressed in the cortical VZ and SVZ of  Arx  KOs throughout corticogenesis. We also identified ARX as a direct regulator of  Cdkn1c  transcription. Together these data support a model where ARX regulates the expansion of cortical progenitor cells through repression of  Cdkn1c .

ARX Regulates Cortical Intermediate Progenitor Cell Expansion and Upper Layer Neuron Formation Through Repression of Cdkn1c