Skeletal muscle mitochondrial respiration of malignant hyperthermia-susceptible patients. Ca2+-induced uncoupling and free fatty acids.
1. Skeletal muscle mitochondria of malignant hyperthermia (MH)-susceptible patients showed normal oxidative phosphorylation but were more easily uncoupled than normal by exogenous Ca2+. 2. Fatty acids, in stimulating the mitochondrial ATPase activity, are responsible for the enhanced State 4 respiration in MH-susceptible patients. 3. These results imply that skeletal muscle mitochondria and free fatty acids are associated with the development of MH syndrome.
Cheah
K S
KS
Department of Anesthesiology, Hahnemann University, Philadelphia, PA 19102.
Cheah
A M
AM
Fletcher
J E
JE
Rosenberg
H
H
D002118:Calcium / Q000502:physiology*; D004198:Disease Susceptibility; D005230:Fatty Acids, Nonesterified / Q000502:physiology*; D006801:Humans; D066298:In Vitro Techniques; D008305:Malignant Hyperthermia / Q000378:metabolism*; D008931:Mitochondria, Muscle / Q000378:metabolism*; D010084:Oxidation-Reduction; D010085:Oxidative Phosphorylation; D010101:Oxygen Consumption / Q000502:physiology*; D013386:Succinates / Q000378:metabolism
D005230:Fatty Acids, Nonesterified; D013386:Succinates; D002118:Calcium