Different effects of mexiletine on two mutant sodium channels causing paramyotonia congenita and hyperkalemic periodic paralysis

Neuromuscular Disorders

Volumn 10, Issue 1, 2000, Pages 31-39

  Weckbecker, K ^a,b   Wurz A ^a   Mohammadi, B ^a   Mansuroglu, T ^a   George Jr , A L ^c   Lerche, H ^b   Dengler, R ^a   Lehmann Horn, F ^b   Mitrovic, N ^b  

^a HANNOVER MEDICAL SCHOOL   (Germany)

^b UNIVERSITY OF ULM   (Germany)

^c VANDERBILT UNIVERSITY MEDICAL CENTER   (United States)

Author keywords

Channelopathies; Local anaesthetics; Myotonia; Patch clamp

Indexed keywords

MEXILETINE; MUTANT PROTEIN; SODIUM CHANNEL;

ACTION POTENTIAL; ARTICLE; ELECTROPHYSIOLOGY; HUMAN; HUMAN CELL; IN VITRO STUDY; KIDNEY CELL CULTURE; MUSCLE STIFFNESS; PATCH CLAMP; PERIODIC PARALYSIS; PRIORITY JOURNAL; PROTEIN EXPRESSION; SITE DIRECTED MUTAGENESIS; SODIUM CURRENT; THOMSEN DISEASE;

CELL LINE; HOMEOSTASIS; HUMANS; MEXILETINE; MODELS, MOLECULAR; MUTATION; MYOTONIC DISORDERS; PARALYSIS, HYPERKALEMIC PERIODIC; PATCH-CLAMP TECHNIQUES; SODIUM CHANNEL BLOCKERS; SODIUM CHANNELS;

EID: 0342615025     PISSN: 09608966     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0960-8966(99)00060-7     Document Type: Article

References (29)

1. Lehmann-Horn F., Rüdel R. Molecular pathophysiology of voltage-gated ion channels. Rev Physiol Biochem Pharmacol. 128:1996;195-268.
2. Ricker K., Moxley R.T., Heine R., Lehmann-Horn F. Myotonia fluctuans. A third type of muscle sodium channel disease. Arch Neurol. 51:1994;1095-1102.
3. Lehmann-Horn F., Rüdel R., Ricker R. Membrane defects in paramyotonia congenita (Eulenburg). Muscle Nerve. 10:1987;633-641.
4. + channel mutations identified in families with periodic paralysis. Neuron. 10:1993;317-326.
5. + channel mutation causing hyperkalemic periodic paralysis. Neuron. 10:1993;667-678.
6. Chahine M., George A.L. Jr, Zhou M., Ji S., Sun W., Barchi R.L., Horn R. Sodium channel mutations in paramyotonia congenita uncouple inactivation from activation. Neuron. 12:1994;281-294.
7. + channel by the V1589M mutation. J Physiol. 478:1994;395-402.
8. Mitrovic N., George A.L. Jr, Lerche H., Wagner S., Fahlke C.H., Lehmann-Horn F. Different effects on gating of three myotonia-causing mutations in the inactivation gate of the human muscle sodium channel. J Physiol. 487:1995;107-114.
9. Witt T.N., Lane R.J.M. Myotonias. Brandt T., Caplan L.R., Dichgans J., Diener H.C., Kennard C., Neurological Disorders. Course and Treatment. 1996;997-1002 Academic Press, San Diego, CA.
10. Ricker K., Böhlen R., Rohkamm R. Different effectiveness of tocainide and hydrochlorothiazide in paramyotonia congenita with hyperkalemic episodic paralysis. Neurology. 33:1983;1615-1618.
11. Kwiecinski H., Ryniewicz B., Ostrzycki A. Treatment of myotonia with antiarrhythmic drugs. Acta Neurol Scand. 86:1992;371-375.
12. Ricker K., Rohkamm R., Böhlen R. Adynamia episodica and paralysis periodica paramyotonica. Neurology. 36:1986;682-686.
13. Hille B. Mechanisms of block. Hille B., Ionic channels of excitable membranes, second ed. 1992;390-422 Sinauer, Sunderland.
14. + channels by local anesthetics. Science. 265:1994;1724-1728.
15. + channels expressed in a mammalian cell line. J Physiol. 496:1996;275-286.
16. Wagner S., Mitrovic N., Heine R., Lerche H., George A.L., Lehmann-Horn F. A new single point mutation in human muscle sodium channel causing hyperkalemic periodic paralysis. Neurology. 49:1997;1018-1025.
17. Hamill O.P., Marty A., Neher E., Sakmann B., Sigworth F.J. Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches. Pflügers Arch. 391:1981;85-100.
18. Noda M., Shimizu S., Tanabe T., et al. Primary structure of Electrophorus electricus sodium channel deduced from cDNA sequence. Nature. 312:1984;121-127.
19. Catterall W.A. Structure and function of voltage-gated ion channels. Annu Rev Biochem. 64:1995;493-531.
20. + channels. Neuron. 16:1996;113-122.
21. Bean B., Cohen C.J., Tsien R.W. Lidocain block of cardiac sodium channels. J Gen Physiol. 81:1983;613-642.
22. Yang N., Ji S., Zhou M., Ptacek L.J., Barchi R.L., Horn R., George A.L. Jr. Sodium channel mutations in paramyotonia congenita exhibit similar biophysical phenotypes in vitro. Proc Natl Acad Sci USA. 91:1994;12785-12789.
23. + channel. Neurosci Lett. 214:1996;9-12.
24. + channel mutation in adult human skeletal muscle. Ann Neurol. 39:1996;599-608.
25. Cummins T.R., Sigworth F.J. Impaired slow inactivation in mutant sodium channels. Biophys J. 71:1996;227-236.
26. + channels associated with myotonia and periodic paralysis. Biophys J. 72:1997;1204-1219.
27. Cereghino J.J., Brock J.T., Van-Meter J.C., Penry J.K., Kupferberg H.J., Smith L.D., White B.G. A multiple-dose study of mexiletine (Ko 1173). Epilepsia. 16:1975;673-677.
28. + channel. Modification of block by alterations in the alpha-subunit III-IV interdomain. Circ Res. 77:1995;584-592.
29. Lehmann-Horn F., Engel A.G., Ricker K., Rüdel R. The periodic paralyses and paramyotonia congenita. Engel A.G., Franzini-Armstrong, . Myology, second ed. 1994;1303-1334 McGraw-Hill, New York.