2 Works

The mechanism underlying transient weakness in myotonia congenita

Jessica Myers, Mark Rich, Kirsten Denman, Chris DuPont, Ahmed Hawash, Kevin Novak, Andrew Koesters, Manfred Grabner, Anamika Dayal & Andrew Voss
In addition to the hallmark muscle stiffness, patients with recessive myotonia congenita (Becker disease) experience debilitating bouts of transient weakness that remain poorly understood despite years of study. We made intracellular recordings from muscle of both genetic and pharmacologic mouse models of Becker disease to identify the mechanism underlying transient weakness. Our recordings reveal transient depolarizations (plateau potentials) of the membrane potential to -25 to -35 mV in the genetic and pharmacologic models of Becker...

The role of action potential changes in depolarization-induced failure of excitation contraction coupling in mouse skeletal muscle

Mark Rich
Excitation-contraction coupling (ECC) is the process by which electrical excitation of muscle is converted into force generation. Depolarization of skeletal muscle resting potential contributes to failure of ECC in diseases such as periodic paralysis, intensive care unit acquired weakness and possibly fatigue of muscle during vigorous exercise. When extracellular K+ is raised to depolarize the resting potential, failure of ECC occurs suddenly, over a narrow range of resting potentials. Intracellular recordings of action potentials (APs)...

Registration Year

  • 2021

Resource Types

  • Dataset


  • Wright State University
  • Wright-Patterson Air Force Base
  • University of Miami
  • Innsbruck Medical University