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Published online 4 October 2004. doi:10.1083/jcb.200408007
 The Rockefeller University Press, 0021-9525 $8.00
The Journal of Cell Biology
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 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 and 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.


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