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[Beta]IV-spectrin regulates sodium channel clustering through ankyrin-G at axon initial segments and nodes of Ranvier.

beta-Spectrin and ankyrin are major components of the membrane cytoskeleton. We have generated mice carrying a null mutation in the betaIV-spectrin gene using gene trapping in embryonic stem cells. Mice homozygous for the mutation exhibit tremors and contraction of hindlimbs. betaIV-spectrin expression is mostly restricted to neurons, where it colocalizes with and binds to ankyrin-G at axon initial segments (AISs) and nodes of Ranvier (NR). In betaIV-spectrin-null neurons, neither ankyrin-G nor voltage-gated sodium channels (VGSC) are correctly clustered at these sites, suggesting that impaired action potential caused by mislocalization of VGSC leads to the phenotype. Conversely, in ankyrin-G-null neurons, betaIV-spectrin is not localized to these sites. These results indicate that betaIV-spectrin and ankyrin-G mutually stabilize the membrane protein cluster and the linked membrane cytoskeleton at AIS and NR.

Pubmed ID: 11807096 RIS Download

Mesh terms: Animals | Ankyrins | Axons | Base Sequence | Brain | Cells, Cultured | Cytoskeleton | Gene Deletion | Gene Expression Profiling | Hindlimb | Ion Channel Gating | Mice | Mice, Knockout | Mice, Mutant Strains | Molecular Sequence Data | Nerve Tissue Proteins | Phenotype | Protein Isoforms | Protein Transport | RNA, Messenger | Ranvier's Nodes | Retroviridae | Sodium Channels | Spectrin | Tremor

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Associated grants

  • Agency: NICHD NIH HHS, Id: R01 HD024875
  • Agency: NICHD NIH HHS, Id: HD24875

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