βIVΣ1 spectrin stabilizes the nodes of Ranvier and axon initial segments-Reference-Cited by-同舟云学术

βIVΣ1 spectrin stabilizes the nodes of Ranvier and axon initial segments

Published:2004-09-20 Issue:7 Volume:166 Page:983-990
ISSN:1540-8140
Container-title:Journal of Cell Biology
language:en
Short-container-title:

Author:

Lacas-Gervais Sandra¹,Guo Jun²,Strenzke Nicola³,Scarfone Eric⁴,Kolpe Melanie¹,Jahkel Monika⁵,De Camilli Pietro²,Moser Tobias³,Rasband Matthew N.⁶,Solimena Michele¹

Affiliation:

1. Experimental Diabetology, University of Technology Dresden, 01307 Dresden, Germany

2. Department of Cell Biology and Howard Hughes Medical Institute, Yale University, New Haven, CT 06510

3. Department of Otolaryngology, University of Goettingen, 37075 Goettingen, Germany

4. Centre de Recherches de Biochimie Macromoléculaire, Centre National de la Recherche Scientifique, 34293 Montpellier, France

5. Department of Psychiatry, University of Technology Dresden, 01307 Dresden, Germany

6. Department of Neurosciences, University of Connecticut, Farmington, CT 06030

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 βIVΣ1 spectrin, the only βIV spectrin with an actin-binding domain, is an essential component of this coat. Specifically, βIVΣ1 coexists with βIVΣ6 at both AIS and NR, being the predominant spectrin at AIS. Removal of βIVΣ1 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 βIVΣ1 −/− mice and point to the βIVΣ1 spectrin isoform as a master-stabilizing factor of AIS/NR membranes.

Publisher

Rockefeller University Press

Subject

Cell Biology

Link

http://rupress.org/jcb/article-pdf/166/7/983/1315849/jcb1667983.pdf

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