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The Journal of Neuroscience, April 21, 2004, 24(16):4030-4042; doi:10.1523/JNEUROSCI.4139-03.2004

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Cellular/Molecular
Mice Lacking Sodium Channel 1 Subunits Display Defects in Neuronal Excitability, Sodium Channel Expression, and Nodal Architecture

Chunling Chen,¹ Ruth E. Westenbroek,⁴ Xiaorong Xu,⁵ Chris A. Edwards,² Dorothy R. Sorenson,² Yuan Chen,⁴ Dyke P. McEwen,¹ Heather A. O'Malley,¹ Vandana Bharucha,¹ Laurence S. Meadows,¹ Gabriel A. Knudsen,¹ Alex Vilaythong,⁶ Jeffrey L. Noebels,⁶ Thomas L. Saunders,³ Todd Scheuer,⁴ Peter Shrager,⁵ William A. Catterall,⁴ and Lori L. Isom¹

¹Department of Pharmacology, ²Microscopy and Image Analysis Laboratory, and ³Transgenic Animal Model Core Laboratory, The University of Michigan, Ann Arbor, Michigan 48109, ⁴Department of Pharmacology, University of Washington, Seattle, Washington 98195, ⁵Department of Neurobiology and Anatomy, University of Rochester Medical Center, Rochester, New York 14642, and ⁶Department of Neurology, Baylor College of Medicine, Houston, Texas 77030

Sodium channel 1 subunits modulate subunit gating and cell surface expression and participate in cell adhesive interactions in vitro. 1(-/-) mice appear ataxic and display spontaneous generalized seizures. In the optic nerve, the fastest components of the compound action potential are slowed and the number of mature nodes of Ranvier is reduced, but Na_v1.6, contactin, caspr 1, and K_v1 channels are all localized normally at nodes. At the ultrastructural level, the paranodal septate-like junctions immediately adjacent to the node are missing in a subset of axons, suggesting that 1 may participate in axo-glial communication at the periphery of the nodal gap. Sodium currents in dissociated hippocampal neurons are normal, but Na_v1.1 expression is reduced and Na_v1.3 expression is increased in a subset of pyramidal neurons in the CA2/CA3 region, suggesting a basis for the epileptic phenotype. Our results show that 1 subunits play important roles in the regulation of sodium channel density and localization, are involved in axo-glial communication at nodes of Ranvier, and are required for normal action potential conduction and control of excitability in vivo.

Key words: sodium; channel; epilepsy; node; adhesion;

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Received Sep 8, 2003; revised March 11, 2004; accepted March 11, 2004.

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