BetaIVSigma1 spectrin stabilizes the nodes of Ranvier and axon initial segments

The Journal of Cell Biology
Sandra Lacas-GervaisMichele Solimena

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 betaIVSigma1 spectrin, the only betaIV spectrin with an actin-binding domain, is an essential component of this coat. Specifically, betaIVSigma1 coexists with betaIVSigma6 at both AIS and NR, being the predominant spectrin at AIS. Removal of betaIVSigma1 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 betaIVSigma1 -/- mice and point to the betaIVSigma1 spectrin isoform as a master-stabilizing factor of AIS/NR membranes.

References

Jan 1, 1985·Annual Review of Cell Biology·V T Marchesi
Nov 22, 2000·The Journal of Comparative Neurology·M N Rasband, J S Trimmer
Jun 8, 2001·Proceedings of the National Academy of Sciences of the United States of America·S BerghsM Solimena
Aug 31, 2001·Nature Genetics·N J ParkinsonB L Tempel
Sep 3, 2003·Nature Reviews. Neuroscience·Ueli Suter, Steven S Scherer
Oct 15, 2003·Neuron·James L Salzer
Dec 20, 2003·Nature Reviews. Neuroscience·Sebastian Poliak, Elior Peles

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Citations

Nov 9, 2006·The Journal of Membrane Biology·Ronald R Dubreuil
Mar 27, 2009·Neuropsychopharmacology : Official Publication of the American College of Neuropsychopharmacology·Dianne A CruzDavid A Lewis
Oct 9, 2013·Proceedings of the National Academy of Sciences of the United States of America·Crystal F KlinePeter J Mohler
Sep 11, 2008·The Journal of Biological Chemistry·Shane R Cunha, Peter J Mohler
May 12, 2010·Cold Spring Harbor Perspectives in Biology·Vann Bennett, Jane Healy
Sep 5, 2008·American Journal of Physiology. Cell Physiology·Paul R StabachJon S Morrow
Sep 30, 2010·The Journal of Clinical Investigation·Thomas J HundPeter J Mohler
Jul 22, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Waheeda A HossainD Kent Morest
Jul 8, 2011·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Anna LysakowskiRuth Anne Eatock
Oct 23, 2009·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Dorothy P SchaferMatthew N Rasband
Feb 14, 2006·Proceedings of the National Academy of Sciences of the United States of America·Maria Carmen IndaAlberto Muñoz
Jun 4, 2013·Brain Structure & Function·Barbara Le BrasFrançois Couraud
Dec 2, 2008·Revue neurologique·P-M MartinJ-A Girault
May 28, 2013·Current Translational Geriatrics and Experimental Gerontology Reports·Xiao-Xin YanA Jeromin
Feb 7, 2007·The Journal of Cell Biology·Yang YangMatthew N Rasband
Feb 20, 2016·Nature Reviews. Neurology·Tobias Moser, Arnold Starr
Dec 30, 2015·Cell Reports·Christophe LeterrierBénédicte Dargent
Oct 12, 2010·Seminars in Cell & Developmental Biology·Matthew N Rasband
Oct 12, 2010·Seminars in Cell & Developmental Biology·Christophe LeterrierHelene Vacher
Jun 12, 2010·Current Opinion in Neurobiology·Matthew S Grubb, Juan Burrone
Oct 17, 2009·Neuroscience Letters·Setareh Shams'iliPeter Sillevis Smitt
Sep 20, 2008·Current Opinion in Neurobiology·Yasuhiro Ogawa, Matthew N Rasband
Dec 18, 2007·Trends in Molecular Medicine·Vann Bennett, Jane Healy
Jan 22, 2009·Hippocampus·Raphael WinkelsStephan W Schwarzacher
Jun 22, 2006·Journal of Neurochemistry·Kristian L Hedstrom, Matthew N Rasband
Jun 6, 2007·Journal of Neuroscience Research·Anilkumar M PillaiManzoor A Bhat
Jul 28, 2011·IUBMB Life·Lida ZoupiDomna Karagogeos
Feb 14, 2013·Journal of Neuroscience Research·Elizabeth D ButtermoreManzoor A Bhat
Dec 15, 2010·Developmental Neurobiology·David A Lewis
Sep 2, 2006·Current Opinion in Neurobiology·Dorothy P Schafer, Matthew N Rasband
Nov 14, 2015·Annual Review of Cell and Developmental Biology·Daniel R ZollingerMatthew N Rasband
Jul 6, 2010·Molecular and Cellular Neurosciences·Elisa M RamserThomas Tilling

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Methods Mentioned

BETA
electron microscopy
PCR

Software Mentioned

MetaMorph

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