Abstract

While analyzing active genes in neonatal mouse hippocampus by quantitative 3′-cDNA collection, we identified a highly conserved murine homolog ofdoublecortin,the causative gene of X-linked lissencephaly (XLIS) and subcortical laminar heterotopia (SCLH) syndrome. Them-doublecortincDNA contains nearly 8 kb 3′ UTR homologous tohs-doublecortinand it was mapped to the X chromosome. The expression ofm-doublecortinis limited to the developing CNS, especially the cortical plate, supporting that XLIS/SCLH syndrome is associated with an arrest of neuronal migration in the cerebral cortex. Them-doublecortinmRNA was absent in the ventricular zone where neuronal precursors proliferate, and interestingly it was found in various brain structures that are not typically affected in patients with this syndrome.

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