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The neuronal voltage-gated sodium channel, Scn8a, is essential for postnatal maturation of spinal, but not oculomotor, motor units.
Exp Neurol. 1996 Jun; 139(2):328-34.EN

Abstract

Mice with a nontargeted transgene insertion at the motor endplate disease (med) locus (med(tg)) contain a deletion of a novel gene encoding a neuronal voltage-gated sodium channel, designated Scn8a. We characterized severe skeletal muscle atrophy beginning by Postnatal Day 10 (P10) and death by P20 in the med(tg) mouse. Denervation was functional, rather than structural, since the Scn8a mutation was not accompanied by retraction of neuromuscular contacts, motoneuron death, or decreased motoneuron soma diameter. Although pathology consistent with denervation was seen in both hindlimb and forelimb musculature, the postnatal maturation of the extraocular muscles was not altered. The onset of paralysis is likely coincident with the time that the Scn8a sodium channel normally assumes a critical role in the initiation and/or propagation of action potentials in spinal motoneurons. By contrast, the lack of consequences for extraocular muscle suggests that the Scn8a voltage-gated sodium channel may be of relatively minor importance for oculomotor motoneurons.

Authors+Show Affiliations

Department of Anatomy, University of Kentucky Medical Center, Lexington, 40536, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, Non-P.H.S.
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

8654536

Citation

Porter, J D., et al. "The Neuronal Voltage-gated Sodium Channel, Scn8a, Is Essential for Postnatal Maturation of Spinal, but Not Oculomotor, Motor Units." Experimental Neurology, vol. 139, no. 2, 1996, pp. 328-34.
Porter JD, Goldstein LA, Kasarskis EJ, et al. The neuronal voltage-gated sodium channel, Scn8a, is essential for postnatal maturation of spinal, but not oculomotor, motor units. Exp Neurol. 1996;139(2):328-34.
Porter, J. D., Goldstein, L. A., Kasarskis, E. J., Brueckner, J. K., & Spear, B. T. (1996). The neuronal voltage-gated sodium channel, Scn8a, is essential for postnatal maturation of spinal, but not oculomotor, motor units. Experimental Neurology, 139(2), 328-34.
Porter JD, et al. The Neuronal Voltage-gated Sodium Channel, Scn8a, Is Essential for Postnatal Maturation of Spinal, but Not Oculomotor, Motor Units. Exp Neurol. 1996;139(2):328-34. PubMed PMID: 8654536.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The neuronal voltage-gated sodium channel, Scn8a, is essential for postnatal maturation of spinal, but not oculomotor, motor units. AU - Porter,J D, AU - Goldstein,L A, AU - Kasarskis,E J, AU - Brueckner,J K, AU - Spear,B T, PY - 1996/6/1/pubmed PY - 1996/6/1/medline PY - 1996/6/1/entrez SP - 328 EP - 34 JF - Experimental neurology JO - Exp. Neurol. VL - 139 IS - 2 N2 - Mice with a nontargeted transgene insertion at the motor endplate disease (med) locus (med(tg)) contain a deletion of a novel gene encoding a neuronal voltage-gated sodium channel, designated Scn8a. We characterized severe skeletal muscle atrophy beginning by Postnatal Day 10 (P10) and death by P20 in the med(tg) mouse. Denervation was functional, rather than structural, since the Scn8a mutation was not accompanied by retraction of neuromuscular contacts, motoneuron death, or decreased motoneuron soma diameter. Although pathology consistent with denervation was seen in both hindlimb and forelimb musculature, the postnatal maturation of the extraocular muscles was not altered. The onset of paralysis is likely coincident with the time that the Scn8a sodium channel normally assumes a critical role in the initiation and/or propagation of action potentials in spinal motoneurons. By contrast, the lack of consequences for extraocular muscle suggests that the Scn8a voltage-gated sodium channel may be of relatively minor importance for oculomotor motoneurons. SN - 0014-4886 UR - https://www.unboundmedicine.com/medline/citation/8654536/The_neuronal_voltage_gated_sodium_channel_Scn8a_is_essential_for_postnatal_maturation_of_spinal_but_not_oculomotor_motor_units_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0014-4886(96)90107-7 DB - PRIME DP - Unbound Medicine ER -