RGD Reference Report - Doublecortin is required in mice for lamination of the hippocampus but not the neocortex. - Rat Genome Database

RGD Reference Report - Doublecortin is required in mice for lamination of the hippocampus but not the neocortex. - Rat Genome Database

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Doublecortin is required in mice for lamination of the hippocampus but not the neocortex.
Authors:	Corbo, JC Deuel, TA Long, JM LaPorte, P Tsai, E Wynshaw-Boris, A Walsh, CA
Citation:	Corbo JC, etal., J Neurosci 2002 Sep 1;22(17):7548-57.
RGD ID:	727744
Pubmed:	PMID:12196578 (View Abstract at PubMed)
PMCID:	PMC6757990 (View Article at PubMed Central)
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.

Objects referenced in this article

Gene	Dcx	doublecortin	Mus musculus
Gene	Dcx	doublecortin	Rattus norvegicus

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