Altered subthreshold sodium currents and disrupted firing patterns in Purkinje neurons of Scn8a mutant mice

Neuron

Volumn 19, Issue 4, 1997, Pages 881-891

  Raman, Indira M ^a   Sprunger, Leslie K ^b   Meisler, Miriam H ^b   Bean, Bruce P ^a  

^a HARVARD MEDICAL SCHOOL   (United States)

^b UNIVERSITY OF MICHIGAN MEDICAL SCHOOL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ION CHANNEL; MESSENGER RNA; SODIUM ION;

ACTION POTENTIAL; ANIMAL CELL; ANIMAL MODEL; ARTICLE; ATAXIA; CEREBELLUM; CONTROLLED STUDY; MEMBRANE ELECTROPHYSIOLOGY; MOUSE; NONHUMAN; PRIORITY JOURNAL; PURKINJE CELL; SODIUM CURRENT; STEADY STATE;

EID: 0030834501     PISSN: 08966273     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0896-6273(00)80969-1     Document Type: Article

References (44)

1. + current in pyramidal neurons from rat and cat sensorimotor cortex. J. Neurosci. 13, 660-673.
2. Beckh, S. (1990). Differential expression of sodium channel mRNAs in rat peripheral nervous system and innervated tissues. FEBS Lett. 262, 317-322.
3. Beckh, S., Noda, M., Lubbert, H., and Numa, S. (1989). Differential regulation of three sodium channel messenger RNAs in the rat central nervous system during development. EMBO J. 8, 3611-3616.
4. Black, J.A., Yokoyama, S., Higashida, H., Ransom, B.R., and Waxman, S.G. (1994). Sodium channel mRNAs I, II, and III in the CNS: cell-specific expression. Brain Res. Mol. Brain Res. 22, 275-289.
5. + currents is compatible with modal-gating hypothesis for sodium channels. J. Neurophysiol. 71, 2562-2565.
6. Burgess, D.L., Kohrman, D.C., Galt, J., Plummer, N.W., Jones, J.M., Spear, B., and Meisler, M.H. (1995). Mutation of anew sodium channel gene Scn8a in the mouse mutant 'motor endplate disease.' Nat. Genet. 10, 461-465.
7. Crill, W.E. (1996). Persistent sodium current in mammalian central neurons. Annu. Rev. Physiol. 58, 349-362.
8. + channel behaves like an open channel blocker. Neuron 7, 743-753.
9. Dick, D.J., Boakes, R.J., and Harris, J.B. (1985). A cerebellar abnormality in the mouse with motor endplate disease. Neuropath. Appl. Neurobiol. 11, 141-147.
10. Dick, D.J., Boakes, R.J., Candy, J.M., Harris, J.B., and Cullen, M.J. (1986). Cerebellar structure and function in the murine mutant "jolting." J. Neurol. Sci. 76, 255-267.
11. Dickie, M.M. (1965). Jolting. Mouse News Lett. 32, 44.
12. Duchen, L.W., and Searle, A.G. (1970). Hereditary motor endplate disease in the mouse: light and electron microscopic studies. J. Neurol. Neurosurg. Psychiat. 33, 238-250.
13. Duchen, L.W., and Stefani, E. (1971). Electrophysiological studies of neuromuscular transmission in hereditary motor endplate disease of the mouse. J. Physiol. 272, 535-548.
14. Eaholtz, G., Scheuer, T., and Catterall, W.A. (1994). Restoration of inactivation and block of open sodium channels by an inactivation gate peptide. Neuron 12, 1041-1048.
15. Felts, P.A., Yokoyama, S., Dib-Hajj, S., Black, J.A., and Waxman, S.G. (1997). Sodium channel α-subunit mRNAs I, II, III, NaG, Na6 and hNE (PN1): different expression patterns in developing rat nervous system. Brain Res. Mol. Brain Res. 45, 71-82.
16. French, C.R., Sah, P., Buckett, K.J., and Gage, P.W. (1990). A voltage-dependent persistent sodium current in mammalian hippocampal neurons. J. Gen. Physiol. 95, 1139-1157.
17. + channel mRNA in the rat brain. Brain Res. Mol. Brain Res. 17, 169-173.
18. Ganguly, A., Rock, M.J., and Prockop, D.J. (1993). Conformation-sensitive gel electrophoresis for rapid detection of single-base differences in double-stranded PCR products and DNA fragments: evidence for solvent-induced bends in DNA heteroduplexes. Proc. Natl. Acad. Sci. USA 90, 10325-10329.
19. Goldin, A.L. (1995). Voltage-gated sodium channels. In Handbook of Receptors and Channels. Ligand- and Voltage-Gated Ion Channels. R.A. North, ed. (Boca Raton: CRC Press), pp. 73-109.
20. Gruol, D.L., and Franklin, C.L. (1987). Morphological and physiological differentiation of Purkinje neurons in cultures of rat cerebellum. J. Neurosci. 7, 1271-1293.
21. Harris, J.B., and Pollard, S.L. (1986). Neuromuscular transmission in the murine mutants "motor end-plate disease" and "jolting." J. Neurol. Sci. 76, 239-253.
22. Harris, J.B., Boakes, R.J., and Court, J.A. (1992). Physiological and biochemical studies on the cerebellar cortex of the murine mutants "jolting" and "motor endplate disease." J. Neurol. Sci. 110, 186-194.
23. Hille, B. (1992). Ionic Channels of Excitable Membranes. (Sunderland, MA: Sinauer Associates).
24. Hounsgaard, J. (1979). Pacemaker properties of mammalian Purkinje neurons. Acta Physiologica Scandinavica 106, 91-92.
25. Kiskin, N.I., Chizhmakov, I.V., Tsyndrenko, A.Y., Krishtal, O.A., and Tegtmeier, F. (1993). R56865 and flunarizine as Na(+)-channel blockers in isolated Purkinje neurons of rat cerebellum. Neuroscience 54, 575-585.
26. Kohrman, D.C., Plummer, N.W., Schuster, T., Jones, J.M., Jang, W., Burgess, D.L., Galt, J., Spear, B.T., and Meisler, M.H. (1995). Insertional mutation of the motor endplate disease (med) locus on mouse chromosome 15. Genomics 26, 171-177.
27. J alleles of the sodium channel Scn8a: unusual patterns of exon skipping are influenced by a minor class AT-AC intron. J. Biol. Chem. 271, 17576-17581.
28. Kohrman, D.C., Smith, M.R., Goldin, A.L., Harris, J., and Meisler, M.H. (1996b). A missense mutation in the sodium channel Scn8a is responsible for cerebellar ataxia in the mouse mutant jolting. J. Neurosci. 16, 5993-5999.
29. Llinás, R (1988). The intrinsic electrophysiological properties of mammalian neurons: insights into central nervous system function. Science 242, 1654-1664.
30. Llinás, R., and Sugimori, M. (1980). Electrophysiological properties of in vitro Purkinje cell somata in mammalian cerebellar slices. J. Physiol. 305, 171-195.
31. Meisler, M.H., Sprunger, L.K., Plummer, N.W., Escayg, A., and Jones, J.M. (1997). Ion channel mutations in mouse models of inherited neurological disease. Ann. Med. (Helsinki), in press.
32. Mintz, I.M., Adams, M.E., and Bean, B.P. (1992). P-type calcium channels in rat central and peripheral neurons. Neuron 9, 85-95.
33. + channel currents in frog sartorius muscle. J. Gen. Physiol. 87, 305-326.
34. Patton, D.E., Isom, L.L., Catterall, W.A., and Goldin, A.L. (1994). The adult rat brain betal subunit modifies activation and inactivation gating of multiple sodium channel alpha subunits. J. Biol. Chem. 69, 17649-17655.
35. Plummer, N.W., McBurney, M.W., and Meisler, M.H. (1997). Alternative splicing of the sodium channel SCN8A predicts a truncated two-domain protein in fetal brain and non-neuronal cells. J. Biol. Chem. 272, 24008-24015.
36. Raman, I.M., and Bean, B.P. (1997). Resurgent sodium current and action potential formation in dissociated Purkinje neurons. J. Neurosci. 17, 4517-4526.
37. Regan, L.J. (1991). Voltage-dependent calcium currents in Purkinje cells from rat cerebellar vermis. J. Neurosci. 11, 2259-2269.
38. Schaller, K.L., Krzemien, D.M., Yarowsky, P.J., Krueger, B.K., and Caldwell, J.H. (1995). A novel, abundant sodium channel expressed in neurons and glia. J. Neurosci. 15, 3231-3242.
39. Song, J.H., and Narahashi, T. (1996). Modulation of sodium channels of rat cerebellar Purkinje neurons by the pyrethroid tetramethrin. J. Pharmacol. Exp. Ther. 277, 445-453.
40. + currents that fail to inactivate. Trends Neurosci. 116, 455-460.
41. Vandenberg, C.A., and Bezanilla, F. (1991). A sodium channel gating model based on single channel, macroscopic ionic and gating currents in the squid giant axon. Biophys J. 60, 1511-1533.
42. Vega-Saenz de Miera, E., Rudy, B., Sugimori, M., and Llinás, R. (1997). Molecular characterization of the sodium channel subunits expressed in mammalian cerebellar Purkinje cells. Proc. Natl. Acad. Sci. USA 94, 7059-7064.
43. West, J.W., Patton, D.E., Scheuer, T., Wang, Y., Goldin, A.L., and Catterall, W.A. (1992). A cluster of hydrophobic amino acid residues required for fast Na(+)-channel inactivation Proc. Natl. Acad. Sci. USA 89, 10910-10914.
44. + channel subtypes in central neurons. Neuron 3, 695-704.