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...

Registration Year

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Affiliations

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