Cloning and developmental expression of the murine homolog of doublecortin

While analyzing active genes in neonatal mouse hippocampus by quantitative 3'-cDNA collection, we identified a highly conserved murine homolog of doublecortin, the causative gene of X-linked lissencephaly (XLIS) and subcortical laminar heterotopia (SCLH) syndrome. The m-doublecortin cDNA contains nearly 8 kb 3' UTR homologous to hs-doublecortin and it was mapped to the X chromosome. The expression of m-doublecortin is 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. The m-doublecortin mRNA 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.