Prominent publications by Chris Dupont

KOL Index score: 7353

Patients with myotonia congenita suffer from muscle stiffness caused by muscle hyperexcitability. Although loss-of-function mutations in the ClC-1 muscle chloride channel have been known for 25 years to cause myotonia congenita, this discovery has led to little progress on development of therapy. Currently, treatment is primarily focused on reducing hyperexcitability by blocking Na+ current. However, other approaches such as increasing K+ currents might also be effective. For example, ...

Known for Myotonia Congenita |  Action Potentials |  Muscle Excitability |  Potassium Channels |  Membrane Potential
KOL Index score: 3124

OBJECTIVE: Myotonia is caused by involuntary firing of skeletal muscle action potentials and causes debilitating stiffness. Current treatments are insufficiently efficacious and associated with side effects. Myotonia can be triggered by voluntary movement (electrically induced myotonia) or percussion (mechanically induced myotonia). Whether distinct molecular mechanisms underlie these triggers is unknown. Our goal was to identify ion channels involved in mechanically induced myotonia and ...

Known for Myotonia Congenita |  Ion Channels |  Skeletal Muscle |  Genetic Pharmacologic |  Mouse Models
KOL Index score: 1777

It is generally thought that muscle excitability is almost exclusively controlled by currents responsible for generation of action potentials. We propose that smaller ion channel currents that contribute to setting the resting potential and to subthreshold fluctuations in membrane potential can also modulate excitability in important ways. These channels open at voltages more negative than the action potential threshold and are thus termed subthreshold currents. As subthreshold currents ...

Known for Action Potential |  Neuromuscular Junction |  Ion Channels |  Muscle Excitability |  Ann Neurol
KOL Index score: 1620

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. ...

Known for Action Potential |  Muscle Skeletal |  Extracellular Raised
KOL Index score: 277

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 performed 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 ...


Chris Dupont: Influence Statistics

Sample of concepts for which Chris Dupont is among the top experts in the world.
Concept World rank
mechanically induced myotonia #7

Key People For Myotonia Congenita

Top KOLs in the world
Frank Lehmann‐Horn
malignant hyperthermia skeletal muscle paramyotonia congenita
Kenneth W Ricker
paramyotonia congenita myotonic dystrophy type myasthenia gravis
Alfred L George
sodium channel paramyotonia congenita skeletal muscle
Manuela C Koch
myotonic dystrophy myotonia congenita genetic counseling
Thomas J Jentsch
chloride channels potassium channel xenopus oocytes
Klaus Steinmeyer
chloride channel xenopus oocytes cell cycle

Chris Dupont:Expert Impact

Concepts for whichChris Duponthas direct influence:Myotonia congenita,  Transient weakness,  Mechanically induced myotonia,  Subthreshold currents,  Plateau potentials,  Underlying transient weakness,  Induced myotonia,  Becker disease.

Chris Dupont:KOL impact

Concepts related to the work of other authors for whichfor which Chris Dupont has influence:Electrical myotonia,  Transient weakness,  Mitochondrial potassium channels,  Skeletal muscle fibers,  Subthreshold currents,  Carbonic anhydrase inhibitors,  Myotonic dystrophy type.



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Wright State University, Department of Neuroscience, Cell Biology, and Physiology, Dayton, United States | Department of Neuroscience, Cell Biology and Physiology, Wright State University, Dayton, United States | Department of Neuroscience, Cell Biol

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