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Journal of Neuroscience, Vol 15, 3730-3738, Copyright © 1995 by Society for Neuroscience
Lissencephaly gene (LIS1) expression in the CNS suggests a role in neuronal migration
O Reiner, U Albrecht, M Gordon, KA Chianese, C Wong, O Gal-Gerber, T Sapir, LD Siracusa, AM Buchberg and CT Caskey
Department of Molecular Genetics and Virology, Weizmann Institute of Science, Rehovot, Israel.
Miller-Dieker lissencephaly syndrome (MDS) is a human developmental brain
malformation caused by neuronal migration defects resulting in abnormal
layering of the cerebral cortex. LIS1, the gene defective in MDS, encodes a
subunit of brain platelet-activating factor (PAF) acetylhydrolase which
inactivates PAF, a neuroregulatory molecule. We have isolated murine cDNAs
homologous to human LIS1 and mapped these to three different chromosomal
loci (Lis1, Lis3, Lis4). The predicted sequences of murine Lis1 protein and
its human homolog LIS1 are virtually identical. In the developing mouse and
human, Lis1 and LIS1 genes were strongly expressed in the cortical plate.
In the adult mouse Lis1 transcripts were abundant in cortex and
hippocampus. The direct correlation between cortical defects in MDS
patients and Lis1 expression in the murine cortex suggest that the mouse is
a model system suitable to study the mechanistic basis of this intriguing
genetic disease.
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