Nine healthy men and a patient with myoadenylate deaminase deficiency were exercised on a bicycle ergometer (30 minutes, 125 Watts) with and without oral ribose administration at a dose of 2 g every 5 minutes of exercise. Plasma or serum levels of glucose, free fatty acids, lactate, ammonia and hypoxanthine and the urinary hypoxanthine excretion were determined. After 30 minutes of exercise without ribose intake the healthy subjects showed significant increases in plasma lactate (p<0.05), ammonia (p<0.01) and hypoxanthine (p<0.05) concentrations and a decrease in serum glucose concentration (p<0.05). When ribose was administered, the plasma lactate concentration increased significantly higher (p< 0.05) and the increase in plasma hypoxanthine concentration was no longer significant. The patient showed the same pattern of changes in serum or plasma concentrations with exercise with the exception of hypoxanthine in plasma which increased higher when ribose was administered.
This is a preview of subscription content, access via your institution.
Buy single article
Instant access to the full article PDF.
Price excludes VAT (USA)
Tax calculation will be finalised during checkout.
Free fatty acids
Aragon JJ, Tornheim K, Goodman MN, Lowenstein JM (1981) Replenishment of citric acid cycle intermediates by the purine nucleotide cycle in rat skeletal muscle. Curr Top Cell Regul 18:131–149
Atkinson DE (1968) The energy charge of the adenylate pool as a regulatory parameter. Interaction with feedback modifiers. Biochemistry 7:4030–4034
Fishbein WN, Armbrustmacher VW, Griffin JL (1978) Myoadenylate deaminase deficiency: A new disease of muscle. Science 200:545–548
Graham TE (1984) Measurement and interpretation of lactate. In: Löllgen H, Mellerowicz H (eds) Progress in ergometry: quality control and test criteria. Springer, Berlin Heidelberg New York Tokyo, pp 51–66
Gross M, Reiter S, Zöllner N (1989) Metabolism of Dribose administered continuously to healthy persons and to patients with myoadenylate deaminase deficiency. Klin Wochenschr 67:1205–1213
Gross M, Zöllner N (1991) Serum levels of glucose, insulin, and c-peptide during long term D-ribose administration in man. Klin Wochenschr 69:31–36
Harkness RA, Simmonds RJ, Coade SB (1983) Purine transport and metabolism in man: the effect of exercise on concentrations of purine bases, nucleosides and nucleotides in plasma, urine, leucocytes and erythrocytes. Clin Science 64:333–340
Manfredi JP, Holmes EW (1984) Control of the purine nucleotide cycle in extracts of rat skeletal muscle: Effects of energy state and concentrations of cycle intermediates. Archives Biochem Biophys 233:515–529
Patten BM (1982) Beneficial effect of D-ribose in patient with myoadenylate deaminase deficiency. Lancet 1:107
Patterson VH, Kaiser KK, Brooke MH (1983) Exercising muscle does not produce hypoxanthine in adenylate deaminase deficiency. Neurology 33:784–786
Sabina RL, Swain JL, Holmes EW (1982) Functional and biochemical evidence of the importance of the purine nucleotide cycle in muscle. J Clin Chem Clin Biochem 20:414
Sabina RL, Swain JL, Olanow CW, Bradley WG, Fishbein WN, DiMauro S, Holmes EW (1984) Myoadenylate deaminase deficiency. Functional and metabolic abnormalities associated with disruption of the purine nucleotide cycle. J Clin Invest 73:720–730
Sinkeler SPT, Joosten EMG, Wevers RA, Binkhorst RA, Oerlemans FT, van Bennekom CA, Coerwinkel MM, Oei TL (1986) Ischaemic exercise test in myoadenylate deaminase deficiency and McArdle's disease: measurement of plasma adenosine, inosine and hypoxanthine. Clin Science 70:399–401
Sinkeler SPT, Wevers RA, Joosten EMG, Binkhorst RA, Oei LT, Hof MAvt, Haan AF (1986) Improvement of screening in exertional myalgia with a standardized ischemic forearm test. Muscle Nerve 9:731–737
Sinkeler SPT, Binkhorst RA, Joosten EMG, Wevers RA, Coerwinkel MM, Oei TL (1987) AMP deaminase deficiency: study of the human skeletal muscle purine metabolism during ischaemic isometric exercise. Clin Science 72:475–482
Sinkeler SPT, Joosten EMG, Wevers RA, Oei TL, Jacobs AEM, Veerkamp JH, Hamel BCJ (1988) Myoadenylate deaminase deficiency: A clinical, genetic, and biochemical study in nine families. Muscle Nerve 2:312–317
Sutton JR, Toews CJ, Ward GR, Fox IH (1980) Purine metabolism during strenuous muscular exercise in man. Metabolism 29:254–260
Swain JL, Sabina RL, Holmes EW (1983) Myoadenylate deaminase deficiency. In: Stanbury JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS (eds) The metabolic basis of inherited disease, 5th edn. McGraw-Hill, New York, pp 1184–1191
Zimmer HG, Ibel H (1984) Ribose accelerates the repletion of the ATP pool during recovery from reversible ischemia of the rat myocardium. J Mol Cell Cardiol 16:863–866
Zöllner N, Reiter S, Gross M, Pongratz D, Reimers CD, Gerbitz K, Paetzke I, Deufel T, Hübner G (1986) Myoadenylate deaminase deficiency: Successful symptomatic therapy by high dose oral administration of ribose. Klin Wochenschr 64:1281–1290
Rights and permissions
About this article
Cite this article
Gross, M., Kormann, B. & Zöllner, N. Ribose administration during exercise: Effects on substrates and products of energy metabolism in healthy subjects and a patient with myoadenylate deaminase deficiency. Klin Wochenschr 69, 151–155 (1991). https://doi.org/10.1007/BF01665856
- Myoadenylate deaminase