JCB logo
Avanti Polar Lipids, Inc.
  Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents
Published online 4 October 2004. doi:10.1083/jcb.200408007
The Rockefeller University Press, 0021-9525 $8.00
JCB, Volume 166, Number 7, 983-990
This Article
Right arrow Full Text
Right arrow Full Text (PDF, 8770K)
Right arrow PPT slides of all figures
Right arrow Alert me when this article is cited
Right arrow Citation Map
Services
Right arrow Email this article
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new content in the JCB
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Lacas-Gervais, S.
Right arrow Articles by Solimena, M.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Lacas-Gervais, S.
Right arrow Articles by Solimena, M.
Right arrowPubmed/NCBI databases
*Gene*GEO Profiles
*HomoloGene*UniGene
*Substance via MeSH
Related Collections
Right arrowRelated Article
Social Bookmarking
Add to CiteULike   Add to Complore   Add to Connotea   Add to Del.icio.us   Add to Digg   Add to Facebook   Add to Reddit   Add to Technorati   Add to Twitter  
What's this?

Report

ßIV{Sigma}1 spectrin stabilizes the nodes of Ranvier and axon initial segments



Sandra Lacas-Gervais1, Jun Guo3, Nicola Strenzke4, Eric Scarfone5, Melanie Kolpe1, Monika Jahkel2, Pietro De Camilli3, Tobias Moser4, Matthew N. Rasband6, and Michele Solimena1

1 Experimental Diabetology, University of Technology Dresden, 01307 Dresden, Germany
2 Department of Psychiatry, University of Technology Dresden, 01307 Dresden, Germany
3 Department of Cell Biology and Howard Hughes Medical Institute, Yale University, New Haven, CT 06510
4 Department of Otolaryngology, University of Goettingen, 37075 Goettingen, Germany
5 Centre de Recherches de Biochimie Macromoléculaire, Centre National de la Recherche Scientifique, 34293 Montpellier, France
6 Department of Neurosciences, University of Connecticut, Farmington, CT 06030

Address correspondence to M. Solimena, Medical School, University of Technology Dresden, Fetscherstrasse, 74, 01307 Dresden, Germany. Tel.: 49-351-4586611. Fax: 49-351-4586330. email: michele.solimena{at}mailbox.tu-dresden.de


Abstract

Saltatory electric conduction requires clustered voltage-gated sodium channels (VGSCs) at axon initial segments (AIS) and nodes of Ranvier (NR). A dense membrane undercoat is present at these sites, which is thought to be key for the focal accumulation of channels. Here, we prove that ßIV{Sigma}1 spectrin, the only ßIV spectrin with an actin-binding domain, is an essential component of this coat. Specifically, ßIV{Sigma}1 coexists with ßIV{Sigma}6 at both AIS and NR, being the predominant spectrin at AIS. Removal of ßIV{Sigma}1 alone causes the disappearance of the nodal coat, an increased diameter of the NR, and the presence of dilations filled with organelles. Moreover, in myelinated cochlear afferent fibers, VGSC and ankyrin G clusters appear fragmented. These ultrastructural changes can explain the motor and auditory neuropathies present in ßIV{Sigma}1 –/– mice and point to the ßIV{Sigma}1 spectrin isoform as a master-stabilizing factor of AIS/NR membranes.

Key Words: cytoskeleton; deafness; sciatic nerve; cochlea; cerebellum

Abbreviations used in this paper: ABR, auditory brainstem responses; AIS, axon initial segments; CNS, central nervous system; NR, nodes of Ranvier; PNS, peripheral nervous system; SD, specific domain; VGSC, voltage-gated sodium channel.


Add to CiteULike CiteULike   Add to Complore Complore   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us   Add to Digg Digg   Add to Facebook Facebook   Add to Reddit Reddit   Add to Technorati Technorati   Add to Twitter Twitter    What's this?

Related Article

Scaffolds from blood to brain
Nicole LeBrasseur
J. Cell Biol. 2004 166: 939. [Full Text] [PDF]



This article has been cited by other articles:



  Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents