Dystrophin deficient mdx muscle is not prone to MH susceptibility: an in vitro study.
The association between malignant hyperthermia (MH) and neuromuscular disorders is controversial. An association between MH and Duchenne muscular dystrophy, a common and lethal disorder caused by deficiency of dystrophin, has been reported sporadically but is still not proved. To examine this problem, we performed halothane and caffeine in vitro contracture tests on skeletal muscles from dystrophin deficient mdx mice, an animal model for human Duchenne muscular dystrophy. As neither halothane nor caffeine triggered abnormal responses in mdx muscles, we conclude that dystrophin deficiency per se is not the primary cause of MH-like crises, as reported for patients with Duchenne muscular dystrophy.
Mader
N
N
Neuromuscular Research Department, University of Vienna, Austria.
Gilly
H
H
Bittner
R E
RE
D018685:Anesthetics, Inhalation / Q000494:pharmacology; D000818:Animals; D002110:Caffeine / Q000494:pharmacology; D004198:Disease Susceptibility; D016189:Dystrophin / Q000172:deficiency*; D006221:Halothane / Q000494:pharmacology; D008297:Male; D008305:Malignant Hyperthermia / Q000209:etiology*; D051379:Mice; D018101:Mice, Inbred mdx; D009119:Muscle Contraction / Q000187:drug effects; D018482:Muscle, Skeletal / Q000187:drug effects; D009137:Muscular Dystrophy, Animal / Q000150:complications*
D018685:Anesthetics, Inhalation; D016189:Dystrophin; D002110:Caffeine; D006221:Halothane