Influences of primary cilia on cortical morphogenesis and neuronal subtype maturation

Recognition that virtually every neuronal progenitor cell and neuron in the cerebral cortex is ciliated has triggered intense interest in neuronal cilia function. Here, we review recent studies that suggest the primary cilia of cortical progenitor cells are required for establishing and maintaining the organization within pools of proliferative cells. In addition, signaling via primary cilia differentially influence the migration and differentiation of excitatory and inhibitory neurons in the developing cortex. Specifically, the primary cilia of excitatory neurons appear to play a significant role in regulating the post-migratory differentiation of these neurons whereas cilia of inhibitory neurons appear to be required for the proper migration and positioning of those cells in cortex. Given the recently discovered functions of cilia in proliferation, neuronal migration, and differentiation, it is likely that further studies of cilia signaling will improve our understanding of how these basic developmental processes are regulated and may provide insight into how mutations in specific cilia genes linked to ciliopathies lead to the many neurological deficits associated with these diseases.