Neuromuscular Disorders
Volume 11, Issue 4 , Pages 370-375 , May 2001

ATP, phosphocreatine and lactate in exercising muscle in mitochondrial disease and McArdle’s disease

  • Mervi Löfberg

      Affiliations

    • Institute of Neurosciences, Department of Neurology, Helsinki University Hospital, 00029 HUS, Helsinki, Finland
    • Corresponding Author InformationCorresponding author. Tel.: +358-9-4717-2261; fax: +358-9-4717-4089
    • ,
  • Harri Lindholm

      Affiliations

    • Department of Medicine, Laboratory of Clinical Physiology, Helsinki University Hospital, Helsinki, Finland
    • ,
  • Hannu Näveri

      Affiliations

    • Department of Medicine, Division of Cardiology, Helsinki University Hospital, Helsinki, Finland
    • ,
  • Anna Majander

      Affiliations

    • Department of Medical Chemistry, Helsinki University Hospital, Helsinki, Finland
    • ,
  • Anu Suomalainen

      Affiliations

    • National Public Health Institute, Department of Human Molecular Genetics, Helsinki, Finland
    • ,
  • Anders Paetau

      Affiliations

    • Department of Pathology, Helsinki University Hospital, Helsinki, Finland
    • ,
  • Anssi Sovijärvi

      Affiliations

    • Department of Medicine, Laboratory of Clinical Physiology, Helsinki University Hospital, Helsinki, Finland
    • ,
  • Matti Härkönen

      Affiliations

    • Department of Clinical Chemistry, Helsinki University Hospital, Helsinki, Finland
    • ,
  • Hannu Somer

      Affiliations

    • Institute of Neurosciences, Department of Neurology, Helsinki University Hospital, 00029 HUS, Helsinki, Finland

Received 27 March 2000 ,Revised 19 September 2000 ,Accepted 11 October 2000.

References 

  1. Suomalainen A. Mitochondrial DNA and disease. Ann Med. 1997;29:235–246
  2. Näveri H, Rehunen S, Kuoppasalmi K, Tulikoura I, Härkönen M. Muscle metabolism during and after strenuous intermittent running. Scand J Clin Lab Invest. 1978;38:329–336
  3. Linderholm H, Essen-Gustavsson B, Thornell LE. Low succinate dehydrogenase (SDH) activity in a patient with a hereditary myopathy with paroxysmal myoglobinuria. J Intern Med. 1990;228:43–52
  4. Näveri HK, Leinonen H, Kiilavuori K, Härkönen M. Skeletal muscle lactate accumulation and creatine phosphate depletion during heavy exercise in congestive heart failure. Cause of limited exercise capacity?. Eur Heart J. 1997;18:1937–1945
  5. Arnold DL, Taylor DJ, Radda GK. Investigation of human mitochondrial myopathies by phosphorus magnetic resonance spectroscopy. Ann Neurol. 1985;18:189–196
  6. Argov Z, Bank WJ, Maris J, Peterson P, Chance B. Bioenergetic heterogeneity of human mitochondrial myopathies: phosphorus magnetic resonance spectroscopy study. Neurology. 1987;37:257–262
  7. Matthews PM, Allaire C, Shoubridge EA, Karpati G, Carpenter S, Arnold DL. In vivo muscle magnetic resonance spectroscopy in the clinical investigation of mitochondrial disease. Neurology. 1991;41:114–120
  8. Taylor DJ, Kemp GJ, Radda GK. Bioenergetics of skeletal muscle in mitochondrial myopathy. J Neurol Sci. 1994;127:198–206
  9. Suomalainen A, Kaukonen J, Amati P, et al.  An autosomal locus predisposing to deletions of mitochondrial DNA. Nat Genet. 1995;9:146–151
  10. Suomalainen A, Majander A, Wallin M, et al.  Autosomal dominant progressive external ophthalmoplegia with multiple deletions of mtDNA: clinical, biochemical, and molecular genetic features of the 10q-linked disease. Neurology. 1997;48:1244–1253
  11. Löfberg M, Jänkälä H, Paetau A, Härkönen M, Somer H. Metabolic causes of recurrent rhabdomyolysis. Acta Neurol Scand. 1998;98:268–275
  12. Borg G, Linderholm H. Perceived exertion and pulse rate during graded exercise in various age groups. Acta Med Scand. 1967;472:194–206
  13. Borg GA. Psychophysical bases of perceived exertion. Med Sci Sports Exerc. 1982;14:377–381
  14. Nordesjö LO, Landelius L. Clinical evaluation of physical work capacity. Scand J Clin Lab Invest. 1975;35:64
  15. Lowry OH, Passonneau JV, Hasselberger FX, Schultz DW. Effect of ishemia on known substrates and cofactors of the glycolytic pathway in brain. J Biol Chem. 1964;239:18
  16. Tarnopolsky MA, Parise G. Direct measurement of high-energy phosphate compounds in patients with neuromuscular disease. Muscle Nerve. 1999;22:1228–1233
  17. Kuhl CK, Layer G, Traber F, Zierz S, Block W, Reiser M. Mitochondrial encephalomyopathy: correlation of P-31 exercise MR spectroscopy with clinical findings. Radiology. 1994;192:223–230
  18. Rowland LP, Araki S, Carmel P. Contracture in McArdle’s disease. Arch Neurol. 1965;13:541–544
  19. Ross BD, Radda GK, Gadian DG, Rocker G, Esiri M, Falconer-Smith J. Examination of a case of suspected McArdle’s syndrome by 31P nuclear magnetic resonance. N Engl J Med. 1981;304:1338–1342
  20. Argov Z, Bank WJ, Maris J, Chance B. Muscle energy metabolism in McArdle’s syndrome by in vivo phosphorus magnetic resonance spectroscopy. Neurology. 1987;37:1720–1724
  21. De Stefano N, Argov Z, Matthews PM, Karpati G, Arnold DL. Impairment of muscle mitochondrial oxidative metabolism in McArdle’s disease. Muscle Nerve. 1996;19:764–769
  22. Duboc D, Jehenson P, Tran Dinh S, Marsac C, Syrota A, Fardeau M. Phosphorus NMR spectroscopy study of muscular enzyme deficiencies involving glycogenolysis and glycolysis. Neurology. 1987;37:663–671
  23. Bendahan D, Confort-Gouny S, Kozak-Ribbens G, Cozzone PJ. 31-P NMR characterization of the metabolic anomalies associated with the lack of glycogen phosphorylase activity in human forearm muscle. Biochem Biophys Res Commun. 1992;185:16–21

PII: S0960-8966(00)00205-4

Neuromuscular Disorders
Volume 11, Issue 4 , Pages 370-375 , May 2001

Article Tools