A human muscle Na+ channel mutation in the voltage sensor IV/S4 affects channel block by the pentapeptide KIFMK

Journal of Physiology

Volumn 518, Issue 1, 1999, Pages 13-22

  Peter, W ^a   Mitrovic, N ^a   Schiebe, M ^a   Lehmann Horn, F ^a   Lerche, H ^a  

^a UNIVERSITY OF ULM   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

PENTAPEPTIDE; RECEPTOR; SODIUM CHANNEL; SODIUM CHANNEL BLOCKING AGENT; LYSYLISOLEUCYLPHENYLALANYLMETHIONYLLYSINE; UNCLASSIFIED DRUG;

ANIMAL CELL; ARTICLE; CLONE; CONTROLLED STUDY; DEPOLARIZATION; GENE MUTATION; GENETIC TRANSFECTION; MYOTONIA; NONHUMAN; PATCH CLAMP; PRIORITY JOURNAL; REPOLARIZATION; ACTION POTENTIAL; CARBOXY TERMINAL SEQUENCE; DISSOCIATION; ENZYME INACTIVATION; EXPRESSION VECTOR; HIGH PERFORMANCE LIQUID CHROMATOGRAPHY; HUMAN; HUMAN CELL; HYPERPOLARIZATION; MEDICAL PARAMETERS; MEMBRANE DEPOLARIZATION; MEMBRANE POTENTIAL; MUTATION; VOLTAGE SENSOR IV S4;

ALGORITHMS; ELECTROPHYSIOLOGY; HUMANS; ION CHANNEL GATING; MEMBRANE POTENTIALS; MUSCLE, SKELETAL; MUTAGENESIS, SITE-DIRECTED; MUTATION; OLIGOPEPTIDES; PATCH-CLAMP TECHNIQUES; SODIUM; SODIUM CHANNEL BLOCKERS; SODIUM CHANNELS;

EID: 0033166236     PISSN: 00223751     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1469-7793.1999.0013r.x     Document Type: Article

References (28)

1. CAHALAN, M. D. & ALMERS, W. (1979). Block of sodium conductance and gating current in squid giant axons poisoned with quaternary strychnine. Biophysical Journal 27, 57-74.
2. CATTERALL, W. A. (1995). Structure and function of voltage-gated ion channels. Animal Review of Biochemistry 64, 493-531.
3. + channel mutations in paramyotonia congenita uncouple inactivation from activation. Neuron 12, 281-294.
4. EAHOLTZ, G., SCHEUER, T. & CATTERALL, W. A. (1994). Restoration of inactivation and block of open sodium channels by an inactivation gate peptide. Neuron 12, 1041-1048.
5. EAHOLTZ, G., ZAGOTTA, W. N. & CATTERALL, W. A. (1998). Kinetic analysis of block of open sodium channels by a peptide containing the isoleucine, phenylalanine and methionine (IFM) motif from the inactivation gate. Journal of General Physiology 111, 75-82.
6. FEATHERSTONE, D. E., FUJIMOTO, E. & RUBEN, P. C. (1998). A defect in skeletal muscle sodium channel deactivation exacerbates hyperexcitability in human paramyotonia congenita. Journal of Physiology 506, 627-638.
7. FILATOV, G. N., NGUYEN, T. P., KRANER, S. D. & BARCHI, R. L. (1998). Inactivation and secondary structure in the D4/S4-5 region of the SkM1 sodium channel. Journal of General Physiology 111, 703-715.
8. HAMILL, O. P., MARTY, A., NEHER, E., SAKMANN, B. & SIGWORTH, F. J. (1981). Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches. Pflügers Archiv 391, 85-100.
9. HAYWARD, L. J., BROWN, R. H. JR & CANNON, S. C. (1996). Inactivation defects caused by myotonia-associated mutations in the sodium channel III-IV linker. Journal of General Physiology 107, 559-576.
10. HODGKIN, A. L. & HUXLEY, A. F. (1952). A quantitative description of membrane current and its application to conduction and excitation in nerve. Journal of Physiology 117, 500-544.
11. KELLENBERGER, S., SCHEUER, T. & CATTERALL, W. A. (1996). Movement of the sodium channel inactivation gate during inactivation. Journal of Biological Chemistry 271, 30971-30979.
12. LEHMANN-HORN, F. & RÜDEL, R. (1996). Molecular pathophysiology of voltage-gated ion channels. Reviews in Physiology, Biochemistry and Pharmacology 128, 195-268.
13. + channel myotonia: slowed channel inactivation due to substitutions for a glycine within the III-IV linker. Journal of Physiology 470, 13-22.
14. + channel mutation in adult human skeletal muscle. Annals of Neurology 39, 599-608.
15. + channel probed by cysteine mutagenesis. Journal of Physiology 505, 345-352.
16. MCPHEE, J. C., RAGSDALE, D. S., SCHEUER, T. & CATTERALL, W. A. (1998). A critical role for the S4-S5 intracellular loop in domain IV of the sodium channel α-subunit in fast inactivation. Journal of Biological Chemistry 273, 1121-1129.
17. + channel by the V1589M mutation. Journal of Physiology 478, 395-402.
18. MITROVIC, N., GEORGE, A. L. JR, LERCHE, H., WAGNER, S., FAHLKE, C. & LEHMANN-HORN, F. (1995). Different effects on gating of three myotonia-causing mutations in the inactivation gate of the human muscle sodium channel. Journal of Physiology 487, 107-114.
19. + current in paramyotonia congenita muscle. Brain (in the Press).
20. O'LEARY, M. E., CHEN, L. Q., KALLEN, R. G. & HORN, R. (1995). A molecular link between activation and inactivation of sodium channels. Journal of General Physiology 106, 641-658.
21. O'LEARY, M. E. & HORN, R. (1994). Internal block of human heart sodium channels by symmetrical tetraalkylammoniums. Journal of General Physiology 104, 507-522.
22. 1649 in the S4-S5 cytoplasmic loop of domain 4 removes the voltage sensitivity of fast inactivation in the human heart sodium channel. Journal of General Physiology 111, 639-652.
23. TANG, L., KALLEN, R. G. & HORN, R. (1996). Role of an S4-S5 linker in sodium channel inactivation probed by mutagenesis and a peptide blocker. Journal of General Physiology 108, 89-104.
24. VEDANTHAM, V. & CANNON, S. C. (1998). Slow inactivation does not affect movement of the fast inactivation gate in voltage-gated sodium channels. Journal of General Physiology 111, 83-93.
25. WANG, J., DUBOWITZ, V., LEHMANN-HORN, F., RICKER, K., PTACEK, L. & HOFFMAN, E. P. (1995). In vivo structure/function studies: consecutive Arg1448 changes to Cys, His, and Pro at the extracellular surface of IVS4. Society of General Physiology Series 50, 77-88.
26. + channel inactivation. Proceedings of the National Academy of Sciences of the USA 91, 12785-12789.
27. WOODHULL, A. M. (1973). Ionic blockage of sodium channels in nerve. Journal of General Physiology 61, 687-708.
28. YANG, N., GEORGE, A. L. JR & HORN, R. (1996). Molecular basis of charge movement in voltage-gated sodium channels. Neuron 16, 113-122.