Absence of a trafficking defect in R1232W/T1620M, a double SCN5A mutant responsible for Brugada syndrome

Circ J. 2008 Jun;72(6):1018-9. doi: 10.1253/circj.72.1018.

Abstract

Background: A trafficking defect of mutant cardiac Na-channels (SCN5A) has been implicated in Brugada syndrome. Although R1232W polymorphism and T1620M mutation by themselves have little effect on Na-channel function, their combination has been reported to disrupt membrane trafficking, resulting in a non-functioning Na channel.

Methods and results: Contrary to previous findings, patch-clamp recordings of heterologously expressed R1232W/T1620M showed robust Na currents and confocal microscopy exhibited predominant expression in the plasma membrane, similar to the wild-type channel.

Conclusions: It is unlikely that an intragenic interaction between R1232W and T1620M of SCN5A causes a trafficking defect leading to a non-functioning Na channel.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Brugada Syndrome / genetics*
  • Brugada Syndrome / metabolism*
  • Brugada Syndrome / physiopathology
  • Cell Membrane / physiology
  • Humans
  • Muscle Proteins / genetics*
  • Muscle Proteins / metabolism*
  • Mutagenesis, Site-Directed
  • NAV1.5 Voltage-Gated Sodium Channel
  • Patch-Clamp Techniques
  • Plasmids
  • Protein Transport / physiology*
  • Sodium / metabolism
  • Sodium Channels / genetics*
  • Sodium Channels / metabolism*

Substances

  • Muscle Proteins
  • NAV1.5 Voltage-Gated Sodium Channel
  • SCN5A protein, human
  • Sodium Channels
  • Sodium