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.
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27 September 2004
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September 20 2004
βIVΣ1 spectrin stabilizes the nodes of Ranvier and axon initial segments
Sandra Lacas-Gervais,
Sandra Lacas-Gervais
1Experimental Diabetology, University of Technology Dresden, 01307 Dresden, Germany
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Jun Guo,
Jun Guo
3Department of Cell Biology and Howard Hughes Medical Institute, Yale University, New Haven, CT 06510
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Nicola Strenzke,
Nicola Strenzke
4Department of Otolaryngology, University of Goettingen, 37075 Goettingen, Germany
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Eric Scarfone,
Eric Scarfone
5Centre de Recherches de Biochimie Macromoléculaire, Centre National de la Recherche Scientifique, 34293 Montpellier, France
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Melanie Kolpe,
Melanie Kolpe
1Experimental Diabetology, University of Technology Dresden, 01307 Dresden, Germany
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Monika Jahkel,
Monika Jahkel
2Department of Psychiatry, University of Technology Dresden, 01307 Dresden, Germany
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Pietro De Camilli,
Pietro De Camilli
3Department of Cell Biology and Howard Hughes Medical Institute, Yale University, New Haven, CT 06510
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Tobias Moser,
Tobias Moser
4Department of Otolaryngology, University of Goettingen, 37075 Goettingen, Germany
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Matthew N. Rasband,
Matthew N. Rasband
6Department of Neurosciences, University of Connecticut, Farmington, CT 06030
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Michele Solimena
Michele Solimena
1Experimental Diabetology, University of Technology Dresden, 01307 Dresden, Germany
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Sandra Lacas-Gervais
1Experimental Diabetology, University of Technology Dresden, 01307 Dresden, Germany
Jun Guo
3Department of Cell Biology and Howard Hughes Medical Institute, Yale University, New Haven, CT 06510
Nicola Strenzke
4Department of Otolaryngology, University of Goettingen, 37075 Goettingen, Germany
Eric Scarfone
5Centre de Recherches de Biochimie Macromoléculaire, Centre National de la Recherche Scientifique, 34293 Montpellier, France
Melanie Kolpe
1Experimental Diabetology, University of Technology Dresden, 01307 Dresden, Germany
Monika Jahkel
2Department of Psychiatry, University of Technology Dresden, 01307 Dresden, Germany
Pietro De Camilli
3Department of Cell Biology and Howard Hughes Medical Institute, Yale University, New Haven, CT 06510
Tobias Moser
4Department of Otolaryngology, University of Goettingen, 37075 Goettingen, Germany
Matthew N. Rasband
6Department of Neurosciences, University of Connecticut, Farmington, CT 06030
Michele Solimena
1Experimental Diabetology, University of Technology Dresden, 01307 Dresden, Germany
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: [email protected]
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.
Received:
August 02 2004
Accepted:
August 12 2004
Online ISSN: 1540-8140
Print ISSN: 0021-9525
The Rockefeller University Press
2004
J Cell Biol (2004) 166 (7): 983–990.
Article history
Received:
August 02 2004
Accepted:
August 12 2004
Citation
Sandra Lacas-Gervais, Jun Guo, Nicola Strenzke, Eric Scarfone, Melanie Kolpe, Monika Jahkel, Pietro De Camilli, Tobias Moser, Matthew N. Rasband, Michele Solimena; βIVΣ1 spectrin stabilizes the nodes of Ranvier and axon initial segments . J Cell Biol 27 September 2004; 166 (7): 983–990. doi: https://doi.org/10.1083/jcb.200408007
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