Abnormal cortical development; towards elucidation of the LIS1 gene product function (review).

  • Authors:
    • O Reiner
    • T Sapir
  • View Affiliations

  • Published online on: May 1, 1998     https://doi.org/10.3892/ijmm.1.5.849
  • Pages: 849-902
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Abstract

Lissencephaly is a relatively common brain malformation. Lissencephaly type 1 is characterized by the smooth appearance of the cortex and the presence of four abnormally positioned layers instead of the normal six. Lissencephaly is considered to be an abnormality in neuronal migration. The gene mutated in type 1 lissencephaly was cloned by us and designated LIS1. Recently, several genes involved in cortical development have been cloned in the mouse. In human an additional X-linked lissencephaly gene has been identified. We summarize here our current knowledge on the LIS1 gene and its function. It has been identified as a non-catalytic subunit of PAF-acetylhydrolase, a heterotrimeric enzyme which inactivates the platelet-activating factor (PAF). In addition, we have demonstrated that LIS1 interacts with tubulin, and affects the dynamics properties of microtubles. LIS1 contains seven WD repeats and may structurally resemble the beta-subunit of heterotrimeric G proteins. Interestingly, the catalytic subunit of PAF-acetylhydrolase was found to resemble the alpha subunit of heterotrimeric G proteins. We raise the possibility that LIS1 is part of an intracellular signaling pathway involved in neuronal migration.

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May 1998
Volume 1 Issue 5

Print ISSN: 1107-3756
Online ISSN:1791-244X

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Spandidos Publications style
Reiner O and Reiner O: Abnormal cortical development; towards elucidation of the LIS1 gene product function (review).. Int J Mol Med 1: 849-902, 1998
APA
Reiner, O., & Reiner, O. (1998). Abnormal cortical development; towards elucidation of the LIS1 gene product function (review).. International Journal of Molecular Medicine, 1, 849-902. https://doi.org/10.3892/ijmm.1.5.849
MLA
Reiner, O., Sapir, T."Abnormal cortical development; towards elucidation of the LIS1 gene product function (review).". International Journal of Molecular Medicine 1.5 (1998): 849-902.
Chicago
Reiner, O., Sapir, T."Abnormal cortical development; towards elucidation of the LIS1 gene product function (review).". International Journal of Molecular Medicine 1, no. 5 (1998): 849-902. https://doi.org/10.3892/ijmm.1.5.849