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Trends Cell Biol. 2001 Apr;11(4):155-60.

LIS1: cellular function of a disease-causing gene.

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Dept of Cell Biology, University of Massachusetts Medical School, 377 Plantation Street, Worcester, MA 06105, USA.


Brain development is severely defective in children with lissencephaly. The highly organized distribution of neurons within the cerebral cortex is disrupted, a condition that might arise from improper migration of neuronal progenitors to their cortical destinations. Type I lissencephaly results from mutations in the LIS1 gene, which has been implicated in the cytoplasmic dynein and platelet-activating factor pathways. Recent studies have identified roles for the product of LIS1 in nuclear migration, mitotic spindle orientation and chromosome alignment, where it appears to act in concert with cytoplasmic dynein. A unifying hypothesis for the subcellular function of LIS1 is presented.

[Indexed for MEDLINE]

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