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Previous Article | Next Article
The Journal of Neuroscience, September 1, 2002, 22(17):7548-7557
Doublecortin Is Required in Mice for Lamination of the Hippocampus But Not the Neocortex
Joseph C. Corbo1, 2, Thomas A. Deuel1, Jeffrey M. Long3, Patricia LaPorte3, Elena Tsai1, Anthony Wynshaw-Boris3, and Christopher A. Walsh1 1 Department of Neurology, Beth Israel Deaconess Medical Center, and Programs in Neuroscience and Biological and Biomedical Sciences, Harvard Medical School, Boston, Massachusetts 02115, 2 Department of Pathology, Brigham and Women's Hospital, Boston, Massachusetts 02115, and 3 Departments of Pediatrics and Medicine, University of California, San Diego School of Medicine, San Diego, California 92093
Doublecortin (DCX) is a microtubule-associated protein that is required for normal neocortical and hippocampal development in humans. Mutations in the X-linked human DCX gene cause gross neocortical disorganization (lissencephaly or "smooth brain") in hemizygous males, whereas heterozygous females show a mosaic phenotype with a normal cortex as well as a second band of misplaced (heterotopic) neurons beneath the cortex ("double cortex syndrome"). We created a mouse carrying a targeted mutation in the Dcx gene. Hemizygous male Dcx mice show severe postnatal lethality; the few that survive to adulthood are variably fertile. Dcx mutant mice show neocortical lamination that is largely indistinguishable from wild type and show normal patterns of neocortical neurogenesis and neuronal migration. In contrast, the hippocampus of both heterozygous females and hemizygous males shows disrupted lamination that is most severe in the CA3 region. Behavioral tests show defects in context and cued conditioned fear tests, suggesting that deficits in hippocampal learning accompany the abnormal cytoarchitecture.
Key words: doublecortin; knock-out; mouse; cerebral cortex; hippocampus; lissencephaly; neuronal migration
Copyright © 2002 Society for Neuroscience 0270-6474/02/22177548-10$05.00/0
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