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Myophosphorylase deficiency impairs muscle oxidative metabolism. 1985

R G Haller, and S F Lewis, and J D Cook, and C G Blomqvist

We studied oxidative metabolism during bicycle exercise in 4 patients with myophosphorylase deficiency. Maximal oxygen uptake (VO2max) was low (14.0 +/- 1.4 ml X kg-1 X min-1, mean +/- SE) compared with that in normal subjects (37.7 +/- 1.9; n = 12) and patients with myalgia (24.9 +/- 1.8; n = 10). Carbohydrate oxidation, as estimated by the respiratory exchange ratio (R), was low relative to workload (max R, mean +/- SE: McArdle's disease, 0.96 +/- 0.02; normal subjects, 1.13 +/- 0.02; myalgia, 1.09 +/- 0.02). Intravenous glucose administration increased maximal oxygen uptake about 20% in those with McArdle's disease, but both VO2max and R remained lower than in control subjects. These findings suggest that the capacity for dynamic exercise in McArdle's disease is limited by the availability of oxidative substrate, and indicate that blood glucose is unable to substitute fully for muscle glycogen as an oxidative fuel. We also found that exercise cardiac output (Q) was excessive relative to oxygen uptake in affected patients (delta Q/delta VO2, mean +/- SE: McArdle's disease, 11.6 +/- 1.7; normal subjects, 4.8 +/- 0.2; myalgia, 5.6 +/- 0.2). This hyperkinetic circulation in exercise may serve to increase the delivery of blood-borne oxidative substrate to working muscle.

UI MeSH Term Description Entries
D008297 Male Males
D009132 Muscles Contractile tissue that produces movement in animals. Muscle Tissue,Muscle,Muscle Tissues,Tissue, Muscle,Tissues, Muscle
D010101 Oxygen Consumption The rate at which oxygen is used by a tissue; microliters of oxygen STPD used per milligram of tissue per hour; the rate at which oxygen enters the blood from alveolar gas, equal in the steady state to the consumption of oxygen by tissue metabolism throughout the body. (Stedman, 25th ed, p346) Consumption, Oxygen,Consumptions, Oxygen,Oxygen Consumptions
D012119 Respiration The act of breathing with the LUNGS, consisting of INHALATION, or the taking into the lungs of the ambient air, and of EXHALATION, or the expelling of the modified air which contains more CARBON DIOXIDE than the air taken in (Blakiston's Gould Medical Dictionary, 4th ed.). This does not include tissue respiration ( Breathing
D002302 Cardiac Output The volume of BLOOD passing through the HEART per unit of time. It is usually expressed as liters (volume) per minute so as not to be confused with STROKE VOLUME (volume per beat). Cardiac Outputs,Output, Cardiac,Outputs, Cardiac
D005082 Physical Exertion Expenditure of energy during PHYSICAL ACTIVITY. Intensity of exertion may be measured by rate of OXYGEN CONSUMPTION; HEAT produced, or HEART RATE. Perceived exertion, a psychological measure of exertion, is included. Physical Effort,Effort, Physical,Efforts, Physical,Exertion, Physical,Exertions, Physical,Physical Efforts,Physical Exertions
D005260 Female Females
D006005 Phosphorylases A class of glucosyltransferases that catalyzes the degradation of storage polysaccharides, such as glucose polymers, by phosphorolysis in animals (GLYCOGEN PHOSPHORYLASE) and in plants (STARCH PHOSPHORYLASE). Glucan Phosphorylase,Phosphorylase,alpha-Glucan Phosphorylases
D006008 Glycogen Storage Disease A group of inherited metabolic disorders involving the enzymes responsible for the synthesis and degradation of glycogen. In some patients, prominent liver involvement is presented. In others, more generalized storage of glycogen occurs, sometimes with prominent cardiac involvement. Glycogenosis,Disease, Glycogen Storage,Diseases, Glycogen Storage,Glycogen Storage Diseases,Glycogenoses,Storage Disease, Glycogen,Storage Diseases, Glycogen
D006012 Glycogen Storage Disease Type V Glycogenosis due to muscle phosphorylase deficiency. Characterized by painful cramps following sustained exercise. Glycogenosis 5,McArdle's Disease,Deficiency, Muscle Phosphorylase,Glycogen Storage Disease Type 5,Glycogen Storage Disease V,McArdle Disease,McArdle Type Glycogen Storage Disease,Mcardle Syndrome,Muscle Glycogen Phosphorylase Deficiency,Muscle Phosphorylase Deficiency,Myophosphorylase deficiency,PYGM Deficiency,Deficiencies, Muscle Phosphorylase,Deficiencies, PYGM,Deficiency, PYGM,Disease, McArdle,Disease, McArdle's,Glycogenosis 5s,McArdles Disease,Mcardle Syndromes,Muscle Phosphorylase Deficiencies,Myophosphorylase deficiencies,PYGM Deficiencies,Phosphorylase Deficiencies, Muscle,Phosphorylase Deficiency, Muscle,Syndrome, Mcardle,Syndromes, Mcardle,deficiencies, Myophosphorylase,deficiency, Myophosphorylase

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