Skip to main content

Ribose administration during exercise: Effects on substrates and products of energy metabolism in healthy subjects and a patient with myoadenylate deaminase deficiency

Klinische Wochenschrift volume 69, pages 151–155 (1991)Cite this article

Summary

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.

Access options

Buy single article

Instant access to the full article PDF.

USD 39.95

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Abbreviations

FFA:

Free fatty acids

References

  1. 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

    Google Scholar 

  2. Atkinson DE (1968) The energy charge of the adenylate pool as a regulatory parameter. Interaction with feedback modifiers. Biochemistry 7:4030–4034

    Google Scholar 

  3. Fishbein WN, Armbrustmacher VW, Griffin JL (1978) Myoadenylate deaminase deficiency: A new disease of muscle. Science 200:545–548

    Google Scholar 

  4. 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

    Google Scholar 

  5. 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

    Google Scholar 

  6. 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

    Google Scholar 

  7. 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

    Google Scholar 

  8. 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

    Google Scholar 

  9. Patten BM (1982) Beneficial effect of D-ribose in patient with myoadenylate deaminase deficiency. Lancet 1:107

    Google Scholar 

  10. Patterson VH, Kaiser KK, Brooke MH (1983) Exercising muscle does not produce hypoxanthine in adenylate deaminase deficiency. Neurology 33:784–786

    Google Scholar 

  11. 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

    Google Scholar 

  12. 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

    Google Scholar 

  13. 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

    Google Scholar 

  14. 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

    Google Scholar 

  15. 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

    Google Scholar 

  16. 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

    Google Scholar 

  17. Sutton JR, Toews CJ, Ward GR, Fox IH (1980) Purine metabolism during strenuous muscular exercise in man. Metabolism 29:254–260

    Google Scholar 

  18. 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

    Google Scholar 

  19. 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

    Google Scholar 

  20. 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

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Medizinische Poliklinik der UniversitÄt München, FRG

    M. Gross, B. Kormann & N. Zöllner

Authors
  1. M. Gross
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. B. Kormann
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. N. Zöllner
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints 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

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01665856

Key words

  • Myoadenylate deaminase
  • AMP-deaminase
  • Deficiency
  • Ribose
  • Lactate
  • Ammonia
  • Hypoxanthine
  • Muscle
  • Metabolism