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Mutations in Talpid3, a basal body protein essential for the assembly of primary cilia have been reported to be causative for Joubert Syndrome. Herein, we report prominent developmental defects in the hippocampus of a conditional knockout mouse lacking the conserved exons 11 and 12 of Talpid3. At early postnatal stages, the Talpid3 mutants exhibit a reduction in proliferation in the dentate gyrus and a disrupted glial scaffold. The occurrence of mis-localised progenitors in the GCL suggests a role for the disrupted glial scaffold in cell migration resulting in defective SPZ-to-hilar transition. Neurospheres derived from the hippocampus of Talpid3fl/flUbcCre mouse in which Talpid3 was conditionally deleted, lacked primary cilia and were smaller in size. In addition, neurosphere cells showed a disrupted actin cytoskeleton and defective migration. Our findings suggest a link between the hippocampal defects and the learning/memory deficits seen in JS patients.

Hippocampals neurogenesis is impaired in mice with a deletion in the coiled coil domain of Talpid3—implications for Joubert syndrome