Voltage-dependent block of normal and mutant muscle sodium channels by 4-Chloro-m-Cresol

Br J Pharmacol. 1999 Nov;128(6):1259-67. doi: 10.1038/sj.bjp.0702896.

Abstract

1 The effects of 4-Chloro-m-Cresol (4-CmC) were examined on heterologously expressed wild type (WT), Paramyotonia Congenita (R1448H) and Hyperkalemic Periodic Paralysis (M1360V) mutant alpha-subunits of human muscle sodium channels. 2 Block of rested sodium channels caused by 4-CmC was concentration-dependent with an ECR50 of 0.40 mM in WT, 0.45 mM in R1448H and 0.49 mM in M1360V. 3 Inactivation significantly promoted 4-CmC-induced sodium channel block in all clones indicated by 4-CmC-induced shifts of steady-state availability curves, reflecting a higher proportion of channel block at depolarized membrane potentials. Channel block was almost complete (>90%) at concentrations close to the ECR50 (0.5 mM) on application of an inactivating prepulse before the test pulse. 4 4-CmC accelerated the current decay following depolarization and prolonged recovery from inactivation in all clones. Of these, R1448H, the mutant which displayed severely impaired inactivation in the controls, responded to 4-CmC with the most pronounced acceleration of inactivation. Control experiments revealed enhanced recovery from inactivation in the mutants, which was restored to normal in 0.1 mM 4-CmC. 5 4-CmC induced no additional frequency-dependent block. 6 Our results clearly demonstrate that 4-CmC is as effective as lidocaine (Fan et al., 1996) in blocking muscle sodium channels. Low concentrations of the compound (</=ECR50) were able to restore pathologically accelerated recovery from inactivation and impaired inactivation in the mutants to the WT value.

MeSH terms

  • Amino Acid Substitution
  • Cell Line
  • Cresols / pharmacology*
  • Dose-Response Relationship, Drug
  • Electric Stimulation
  • Electrophysiology
  • Humans
  • Membrane Potentials / drug effects
  • Muscles / chemistry
  • Mutation
  • Patch-Clamp Techniques
  • Recombinant Fusion Proteins / antagonists & inhibitors
  • Recombinant Fusion Proteins / genetics
  • Recombinant Fusion Proteins / physiology
  • Sodium Channel Blockers*
  • Sodium Channels / genetics
  • Sodium Channels / physiology

Substances

  • Cresols
  • Recombinant Fusion Proteins
  • Sodium Channel Blockers
  • Sodium Channels
  • chlorocresol