Malignant hyperthermia: molecular defects in membrane permeability.
Malignant hyperthermia (MH), a genetically inherited disorder of skeletal muscle, is due to molecular defect in membrane permeability. The alteration in membrane permeability is suggested to be due to enhanced phospholipase A2 activity which is responsible for the increased level in sarcoplasmic Ca2+. The excess Ca2+ is responsible for muscle hyper-rigidity and enhanced rate of glycolysis, resulting in a rapid rate of lactic acid production and a low pH in MH muscle.
D000818:Animals; D002118:Calcium / Q000502:physiology*; D002451:Cell Compartmentation; D006801:Humans; D008305:Malignant Hyperthermia / Q000503:physiopathology*; D008931:Mitochondria, Muscle / Q000502:physiology; D009119:Muscle Contraction; D009132:Muscles / Q000503:physiopathology*; D010539:Permeability; D010741:Phospholipases A / Q000378:metabolism; D054467:Phospholipases A2; D012508:Sarcolemma / Q000502:physiology; D012519:Sarcoplasmic Reticulum / Q000502:physiology; D013552:Swine
D010741:Phospholipases A; D054467:Phospholipases A2; D002118:Calcium