OBJECTIVE: To determine the skeletal muscle metabolic manifestations of @entity1121 . DESIGN: A case-control study. SETTING: 31P magnetic resonance spectroscopy of the @entity291 muscle was performed on volunteers from a centre specialising in @entity1121 . @entity1 : Five @entity1 with abnormal beta myosin heavy chain protein in cardiac and skeletal muscle and five @entity1 with a troponin T abnormality in cardiac muscle were compared with healthy controls. RESULTS: High energy @entity1391 metabolism in vivo was examined in a non-invasive manner. In resting muscle, the beta myosin heavy chain group had a higher ratio of @entity1734 to @entity855 concentration (4.51 (SD 0.17)) than either the troponin T group (3.88 (0.42)) or controls (n = 16; 4.04 (0.40)). Exercise duration was reduced compared to controls, and during the fourth minute of exercise @entity1734 depletion and muscle acidification were greater in both @entity1 groups. After exercise, the recovery of @entity1734 -an index of oxidative metabolic capacity of the muscle-was slower in the beta myosin heavy chain group (mean half time 0.65 (0.08) minutes) than in the troponin T group (0.60 (0.17) minutes) or controls (0.48 (0.14) minutes). CONCLUSIONS: Exercise metabolism was abnormal in both groups of subjects, and the affected contractile protein determined the metabolic changes in muscle at rest and during recovery. In @entity1 with abnormal beta myosin heavy chain protein, there was a decrease in oxidative capacity consistent with the reduction in mitochondria reported in muscle biopsy studies of similar @entity1 .

Abnormal skeletal muscle bioenergetics in XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient', 'PATIENTS'] @entity1734 :: ('CHEBI:17287', 'Chemical') :: ['phosphocreatine'] @entity1391 :: ('CHEBI:26020', 'Chemical') :: ['phosphate'] @entity1121 :: ('MESH:D002312', 'Disease') :: ['familial hypertrophic cardiomyopathy'] @entity291 :: ('9913', 'Species') :: ['calf'] @entity855 :: ('MESH:D000255', 'Chemical') :: ['ATP']
@entity1121 :: (MESH:D002312,Disease) :: ['familial hypertrophic cardiomyopathy']
