A global defect in scaling relationship between electrical activity and availability of muscle sodium channels in hyperkalemic periodic paralysis

Pflugers Archiv European Journal of Physiology

Volumn 438, Issue 2, 1999, Pages 213-217

  Melamed Frank, Meira ^a   Marom, Shimon ^a  

^a TECHNION ISRAEL INSTITUTE OF TECHNOLOGY   (Israel)

Author keywords

Electrophysiology; Excitability; Hyperkalemic periodic paralysis; Inactivation; Power law scaling; Sodium channel

Indexed keywords

SODIUM; SODIUM CHANNEL;

ANIMAL CELL; ARTICLE; ELECTRIC ACTIVITY; ELECTROPHYSIOLOGY; GENE MUTATION; HUMAN; MUSCLE WEAKNESS; MYOTONIA; PERIODIC PARALYSIS; PRIORITY JOURNAL; SODIUM CURRENT;

CELL LINE; ELECTROPHYSIOLOGY; HUMANS; ION CHANNEL GATING; KINETICS; MUSCLE, SKELETAL; MUTATION; PARALYSES, FAMILIAL PERIODIC; PLASMIDS; SODIUM CHANNELS;

EID: 0033006954     PISSN: 00316768     EISSN: None     Source Type: Journal    
DOI: 10.1007/s004240050900     Document Type: Article

References (14)

1. 1. Bassingthwaighte JB, Liebovitch LS, West BJ (1994) Fractal physiology. Oxford University Press, New York
2. 2. Cannon SC (1996) Sodium channel defects in myotonia and periodic paralysis. [Review]. Annu Rev Neurosci 19:141-164
3. 3. Cannon SC, Strittmatter SM (1993) Functional expression of sodium channel mutations identified in families with periodic paralysis. Neuron 10:317-326
4. 4. Cummins TR, Sigworth FJ (1996) Impaired slow inactivation in mutant sodium channels [see comments]. Biophys J 71:227-236
5. + channel mutation causing hyperkalemic periodic paralysis. Neuron 10:667-678
6. 6. Featherstone DE, Richmond JE, Ruben PC (1996) Interaction between fast and slow inactivation in Skm1 sodium channels. Biophys J 71:3098-3109
7. 7. Hayward LJ, Brown RHJ, Cannon SC (1997) Slow inactivation differs among mutant Na channels associated with myotonia and periodic paralysis. Biophys J 72:1204-1219
8. 8. Kalman K, Nguyen A, Tseng-Crank J, Dukes ID, Chandy G, Hustad CM, Copeland NG, Jenkins NA, Mohrenweiser H, Brandriff B, Cahalan M, Gutman GA, Chandy KG (1998) Genomic organization, chromosomal localization, tissue distribution, and biophysical characterization of a novel mammalian Shaker-related voltage-gated potassium channel, Kv1.7. J Biol Chem 273:5851-5857
9. 9. Marom S (1998) Slow changes in the availability of voltage-gated ion channels: effects on the dynamics of excitable membranes. [Review]. J Membr Biol 161:105-113
10. 10. Ptacek LJ, George ALJ, Griggs RC, Tawil R, Kallen RG, Barchi RL, Robertson M, Leppert MF (1991) Identification of a mutation in the gene causing hyperkalemic periodic paralysis. Cell 67:1021-1027
11. 11. Rudel R, Lehmann-Horn F (1985) Membrane changes in cells from myotonia patients. [Review]. Physiol Rev 65:310-356
12. + channel inactivation must be disrupted to evoke prolonged depolarization-induced paralysis. Biophys J 66:542-545
13. + channels. J Neurosci 18:1893-1903
14. 14. Yang N, Ji S, Zhou M, Ptacek LJ, Barchi RL, Horn R, George-AL J (1994) Sodium channel mutations in paramyotonia congenita exhibit similar biophysical phenotypes in vitro. Proc Natl Acad Sci USA 91:12785-12789