![]() | Chris Dupont |
Prominent publications by Chris Dupont
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 |
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 |
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 |
The role of action potential changes in depolarization-induced failure of excitation contraction coupling in mouse skeletal muscle
[ PUBLICATION ]
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 |
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
Concept | World rank |
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mechanically induced myotonia | #7 |
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Key People For Myotonia Congenita
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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