Literature search services are currently unavailable. During our hosting provider's UPS upgrade we experienced a hardware failure and are currently working to resolve the issue.

Preparing your results

Our searching services are busy right now. Your search will reload in five seconds.

Forgot Password

If you have forgotten your password you can enter your email here and get a temporary password sent to your email.

[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


  • Komada M
  • Soriano P


The Journal of cell biology

Publication Data

January 21, 2002

Associated Grants

  • Agency: NICHD NIH HHS, Id: HD24875

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