Impaired slow inactivation in mutant sodium channels

Biophysical Journal

Volumn 71, Issue 1, 1996, Pages 227-236

  Cummins, Theodore R ^a   Sigworth, Frederick J ^a  

^a YALE UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

MUTANT PROTEIN; SODIUM CHANNEL; SODIUM ION;

ANIMAL CELL; ARTICLE; AUTOSOMAL DOMINANT DISORDER; CONTROLLED STUDY; HYPERPOLARIZATION; ION CURRENT; MUSCLE EXCITATION; MUSCLE FIBER MEMBRANE POTENTIAL; NONHUMAN; PERIODIC PARALYSIS; RAT; SKELETAL MUSCLE;

ANIMALIA;

EID: 0029976727     PISSN: 00063495     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0006-3495(96)79219-6     Document Type: Article

References (25)

1. Almers, W., P. R. Stanfield, and W. Stühmer. 1983. Slow changes in currents through sodium channels in frog muscle membrane. J. Physiol. 339:253-271.
2. Barchi, R. L. 1995. Molecular pathology of the skeletal muscle sodium channel. Annu. Rev. Physiol. 57:355-385.
3. Cannon, S. C., and S. M. Strittmatter. 1993. Functional expression of sodium channel mutations identified in families with periodic paralysis. Neuron. 10:317-326.
4. Chahine, M., A. L. George, M. Zhou, S. Ji, W. Sun, R. L. Barchi, and R. Horn. 1994. Sodium channel mutations in paramyotonia congenita uncouple inactivation from activation. Neuron. 12:281-294.
5. + channel mutation causing hyperkalemic periodic paralysis. Neuron. 10: 667-668.
6. Fahlke, Ch., and R. Rüdel. 1992. Giga-seal formation alters properties of sodium channels of human myoballs. Pflügers Arch. 420:248-254.
7. Hodgkin, A. L., and A. F. Huxley. 1952. The dual effect of membrane potential on sodium conductance in the giant axon of Loligo. J. Physiol. 116:497-506.
8. Kirsch, G. E., and M. F. Anderson. 1986. Sodium channel kinetics in normal and denervated rabbit muscle membrane. Muscle Nerve. 9:738-747.
9. Lehmann-Horn, F., G. Küther, K. Ricker, P. Grafe, K. Ballanyi, and R. Rüdel. 1987. Adynamia episodica hereditaria with myotonia: a non-inactivating sodium current and the effect of extracellular pH. Muscle Nerve. 10:363-374.
10. Lehmann-Horn, F., R. Rüdel, K. Ricker, H. Lorkovic, R. Dengler, and H. C. Hopf. 1983. Two cases of adynamia episodica hereditaria: in vitro investigation of muscle cell membrane and contraction parameters. Muscle Nerve. 6:113-121.
11. McArdle, B. 1962. Adynamia episodica hereditaria and its treatment. Brain. 85:121-148.
12. Ptacek, L. J., A. L. George, R. C. Griggs, R. Tawil, R. G. Kallen, R. L. Barchi, M. Robertson, and M. F. Leppert. 1991. Identification of a mutation in the gene causing hyperkalemic periodic paralysis. Cell. 67:1021-1027.
13. Ricker, K., L. M. Camacho, P. Grafe, F. Lehmann-Horn, and R. Rüdel. 1989. Adynamia episodica hereditaria: what causes the weakness? Muscle Nerve. 12:883-891.
14. Rüdel, R., and F. Lehmann-Horn. 1985. Membrane changes in cells from myotonia patients. Physiol. Rev. 65:310-356.
15. Rüdel, R., K. Ricker, and F. Lehmann-Horn. 1993. Genotype-phenotype correlations in human skeletal muscle sodium channel diseases. Arch. Neurol. 50:1241-1248.
16. Ruben, P. C., J. G. Starkus, and M. D. Rayner. 1992. Steady-state availability of sodium channels: interactions between activation and slow inactivation. Biophys. J. 61:941-955.
17. Rudy, B. 1978. Slow inactivation of the sodium conductance in squid giant axons: pronase resistance. J. Physiol. 283:1-21.
18. + channel inactivation must be disrupted to evoke prolonged depolarization-induced paralysis. Biophys. J. 66:542.
19. Ruff, R. L., L. Simoncini, and W. Stühmer. 1987. Comparison between slow sodium channel inactivation in rat slow- and fast-twitch muscle. J. Physiol. 383:339-348.
20. + current densities and voltage dependence in human intercostal muscle fibres. J. Physiol. 458:85-97.
21. Simoncini, L., and W. Stühmer. 1987. Slow sodium channel inactivation in rat and fast-twitch muscle. J. Physiol. 383:327-337.
22. Streib, E. W. 1987. AAEE minimonograph #27. Differential diagnosis of myotonic syndromes. Muscle Nerve. 10:603-615.
23. + channels expressed transiently in human embryonic kidney cells: biochemical and biophysical properties. Neuron. 8:663-676.
24. + channel inactivation. Proc. Natl. Acad. Sci. USA. 89:910-914.
25. Yang, N., S. Ji, M. Zhou, L. J. Ptacek, R. L. Barchi, R. Horn, and A. L. George. 1994. Sodium channel mutations in paramyotonia congenita exhibit similar biophysical phenotypes in vitro. Proc. Natl. Acad. Sci. USA. 91:12,785-12,789.