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Updated: Jan 22, 2026

Use of pHluorin to Assess the Dynamics of Axon Guidance Receptors in Cell Culture and in the Chick Embryo
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Use of pHluorin to Assess the Dynamics of Axon Guidance Receptors in Cell Culture and in the Chick Embryo

Published on: January 12, 2014

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Inactivation of Lgi1 in murine neuronal precursor cells leads to dysregulation of axon guidance pathways

Jeane Silva1, Lesleyann Hawthorn1, John K Cowell1

  • 1The Georgia Cancer Center, Augusta University, 1410 Laney Walker Blvd., Augusta, GA 30912, United States of America.

Genomics
|July 6, 2019
PubMed

Insights

Mutations in LGI1 cause rare epilepsy. In LGI1-deficient mice, neuronal precursor cells exhibit altered gene expression, particularly in axon guidance pathways, leading to increased cell motility and cortical dysplasia.

Area of Science:

  • Neuroscience
  • Developmental Biology
  • Genetics

Background:

  • Leucine-rich glioma inactivated 1 (LGI1) mutations are linked to rare epilepsy syndromes.
  • Lgi1 inactivation in mice results in early-onset seizures and premature death.
  • Histopathology reveals cortical dysplasia and hypercellularity in Lgi1 null mice.

Purpose of the Study:

  • To investigate the molecular mechanisms underlying cortical dysplasia in Lgi1 null mice.
  • To identify gene expression changes in neuronal precursor cells (NPCs) of Lgi1 null mice.

Main Methods:

  • Comparative gene expression analysis of NPCs from Lgi1 null and wild-type mice.
  • In vitro cell motility assays.

Main Results:

  • Significant dysregulation of canonical axon guidance signaling pathways was observed in Lgi1 null NPCs.
  • Multiple networks involved in cell movement, adhesion, and invasion, related to actin cytoskeleton reorganization, were affected.
  • Lgi1 null NPCs demonstrated increased cell motility in vitro compared to wild-type controls.

Conclusions:

  • Dysregulation of genes crucial for cell movement and migration, along with key transcription factors for early neuronal development, is a prominent feature in Lgi1 null mice.
  • These findings provide a mechanistic link between LGI1 deficiency and the observed cortical abnormalities in developing brains.
Keywords:
Axon guidanceCell migrationGene expressionLGI1

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