scholarly journals A calcium channel mutant mouse model of hypokalemic periodic paralysis

2012 ◽  
Vol 122 (12) ◽  
pp. 4580-4591 ◽  
Author(s):  
Fenfen Wu ◽  
Wentao Mi ◽  
Erick O. Hernández-Ochoa ◽  
Dennis K. Burns ◽  
Yu Fu ◽  
...  
Keyword(s):  
Mouse Model ◽  
Calcium Channel ◽  
Mutant Mouse ◽  
Periodic Paralysis ◽  
Hypokalemic Periodic Paralysis

A calcium channel mutation causing hypokalemic periodic paralysis

1994 ◽  
Vol 3 (8) ◽  
pp. 1415-1419 ◽  
Author(s):  
Karln Jurkat-Rott ◽  
Frank Lehmann-Horn ◽  
Alexis Elbaz ◽  
Roland Heine ◽  
Ronald G. Gregg ◽  
...  
Keyword(s):  
Calcium Channel ◽  
Periodic Paralysis ◽  
Hypokalemic Periodic Paralysis ◽  
Channel Mutation

scholarly journals Recovery from acidosis is a robust trigger for loss of force in murine hypokalemic periodic paralysis

2019 ◽  
Vol 151 (4) ◽  
pp. 555-566 ◽  
Author(s):  
Wentao Mi ◽  
Fenfen Wu ◽  
Marbella Quinonez ◽  
Marino DiFranco ◽  
Stephen C. Cannon
Keyword(s):  
Mouse Models ◽  
Muscle Force ◽  
Mutant Mouse ◽  
Prolonged Exposure ◽  
Periodic Paralysis ◽  
Resting Potential ◽  
Hypokalemic Periodic Paralysis ◽  
Vigorous Exercise ◽  
Exercise Induced ◽  
Cl Conductance

Periodic paralysis is an ion channelopathy of skeletal muscle in which recurrent episodes of weakness or paralysis are caused by sustained depolarization of the resting potential and thus reduction of fiber excitability. Episodes are often triggered by environmental stresses, such as changes in extracellular K+, cooling, or exercise. Rest after vigorous exercise is the most common trigger for weakness in periodic paralysis, but the mechanism is unknown. Here, we use knock-in mutant mouse models of hypokalemic periodic paralysis (HypoKPP; NaV1.4-R669H or CaV1.1-R528H) and hyperkalemic periodic paralysis (HyperKPP; NaV1.4-M1592V) to investigate whether the coupling between pH and susceptibility to loss of muscle force is a possible contributor to exercise-induced weakness. In both mouse models, acidosis (pH 6.7 in 25% CO2) is mildly protective, but a return to pH 7.4 (5% CO2) unexpectedly elicits a robust loss of force in HypoKPP but not HyperKPP muscle. Prolonged exposure to low pH (tens of minutes) is required to cause susceptibility to post-acidosis loss of force, and the force decrement can be prevented by maneuvers that impede Cl− entry. Based on these data, we propose a mechanism for post-acidosis loss of force wherein the reduced Cl− conductance in acidosis leads to a slow accumulation of myoplasmic Cl−. A rapid recovery of both pH and Cl− conductance, in the context of increased [Cl]in/[Cl]out, favors the anomalously depolarized state of the bistable resting potential in HypoKPP muscle, which reduces fiber excitability. This mechanism is consistent with the delayed onset of exercise-induced weakness that occurs with rest after vigorous activity.

A novel mutation in the calcium channel gene in a family with hypokalemic periodic paralysis

2011 ◽  
Vol 309 (1-2) ◽  
pp. 9-11 ◽  
Author(s):  
Makito Hirano ◽  
Yosuke Kokunai ◽  
Asami Nagai ◽  
Yusaku Nakamura ◽  
Kazumasa Saigoh ◽  
...  
Keyword(s):  
Calcium Channel ◽  
Novel Mutation ◽  
Periodic Paralysis ◽  
Hypokalemic Periodic Paralysis ◽  
Channel Gene
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