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βIV-spectrin regulates sodium channel clustering through ankyrin-G at axon initial segments and nodes of Ranvier

β-Spectrin and ankyrin are major components of the membrane cytoskeleton. We have generated mice carrying a null mutation in the βIV-spectrin gene using gene trapping in embryonic stem cells. Mice homozygous for the mutation exhibit tremors and contraction of hindlimbs. βIV-spectrin expression is mo...

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Detalles Bibliográficos
Autores principales: Komada, Masayuki, Soriano, Philippe
Formato: Texto
Lenguaje:English
Publicado: The Rockefeller University Press 2002
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2199236/
https://www.ncbi.nlm.nih.gov/pubmed/11807096
http://dx.doi.org/10.1083/jcb.200110003
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author Komada, Masayuki
Soriano, Philippe
author_facet Komada, Masayuki
Soriano, Philippe
author_sort Komada, Masayuki
collection PubMed
description β-Spectrin and ankyrin are major components of the membrane cytoskeleton. We have generated mice carrying a null mutation in the βIV-spectrin gene using gene trapping in embryonic stem cells. Mice homozygous for the mutation exhibit tremors and contraction of hindlimbs. βIV-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 βIV-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, βIV-spectrin is not localized to these sites. These results indicate that βIV-spectrin and ankyrin-G mutually stabilize the membrane protein cluster and the linked membrane cytoskeleton at AIS and NR.
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spelling pubmed-21992362008-05-01 βIV-spectrin regulates sodium channel clustering through ankyrin-G at axon initial segments and nodes of Ranvier Komada, Masayuki Soriano, Philippe J Cell Biol Article β-Spectrin and ankyrin are major components of the membrane cytoskeleton. We have generated mice carrying a null mutation in the βIV-spectrin gene using gene trapping in embryonic stem cells. Mice homozygous for the mutation exhibit tremors and contraction of hindlimbs. βIV-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 βIV-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, βIV-spectrin is not localized to these sites. These results indicate that βIV-spectrin and ankyrin-G mutually stabilize the membrane protein cluster and the linked membrane cytoskeleton at AIS and NR. The Rockefeller University Press 2002-01-21 /pmc/articles/PMC2199236/ /pubmed/11807096 http://dx.doi.org/10.1083/jcb.200110003 Text en Copyright © 2002, The Rockefeller University Press This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/4.0/).
spellingShingle Article
Komada, Masayuki
Soriano, Philippe
βIV-spectrin regulates sodium channel clustering through ankyrin-G at axon initial segments and nodes of Ranvier
title βIV-spectrin regulates sodium channel clustering through ankyrin-G at axon initial segments and nodes of Ranvier
title_full βIV-spectrin regulates sodium channel clustering through ankyrin-G at axon initial segments and nodes of Ranvier
title_fullStr βIV-spectrin regulates sodium channel clustering through ankyrin-G at axon initial segments and nodes of Ranvier
title_full_unstemmed βIV-spectrin regulates sodium channel clustering through ankyrin-G at axon initial segments and nodes of Ranvier
title_short βIV-spectrin regulates sodium channel clustering through ankyrin-G at axon initial segments and nodes of Ranvier
title_sort βiv-spectrin regulates sodium channel clustering through ankyrin-g at axon initial segments and nodes of ranvier
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2199236/
https://www.ncbi.nlm.nih.gov/pubmed/11807096
http://dx.doi.org/10.1083/jcb.200110003
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