Abstract
The isoenzyme pattern of phosphorylase in human brain was studied by polyacrylamide slab gel electrophoresis and activity was revealed by iodine stain and by autoradiography: A major fast-migrating band was similar to isoenzyme I of the heart; an intermediate band migrated like the hybrid cardiac isoenzyme II; and a slow-migrating band, best revealed by autoradiography, migrated like the “muscle” isoenzyme III. The radioactivity in bands II and III accounted for 42% of total activity in the heart and 37% in the brain. Antibodies against mature muscle phosphorylase inhibited 41% of enzyme activity in heart extracts and 25% in brain extracts. These studies show that the “muscle” isoenzyme is a component of brain phosphorylase and suggest that partial phosphorylase deficiency occurs in the brain of patients with McArdle disease.
Similar content being viewed by others
References
Bonner W. M. and Laskey A. (1974) A film detection method for tritium-labelled protein and nucleic acids in polyacrylamide gels.Eur. J. Biochem. 46, 83–88.
Davis C. H., Schliselfeld L. H., Wolf D. P., Leavitt C. A., and Krebs E. G. (1967) Interrelationships among glycogen phosphorylase isozymes.J. Biol. Chem. 242, 4824–4833.
DiMauro S., and Bresolin N. (1984) Phosphorylase deficiency, inMyology (Engel A. G. and Banker B. Q., eds.). McGraw-Hill, New York, in press.
Dimauro S., Arnold S., Miranda A. F., and Rowland, L. P. (1978) McArdle disease: The mystery of reappearing phosphorylase activity in muscle culture: A fetal isoenzyme.Ann. Neurol. 3, 60–66.
Farmer T. W. (1973) Glycogenosis due to deficiency of muscle phosphorylase: recurrent myoglobinuria precipitated by grand mal convulsions, inClinical Studies in Myology (Kakulas B. A., ed.) pp 70–74, Excerpta Medica, Amsterdam.
Hanabusa K. and Kohno H. (1970) Isozymes of glycogen phosphorylase in pig brain.J. Biochem 68, 929–931.
Miranda A. F., Nette G., Hartlage P. L. and DiMauro S. (1979) Phosphorylase isoenzymes in normal and myophosphorylase-deficient, human heart.Neurology 29, 1538–1541.
Proux D. and Dreyfus J. C. (1973) Phosphorylase isoenzymes in tissues: Prevalence of the liver type in man.Clin. Chim. Acta 48, 167–172.
Salmon, S. E. and Turner C. E. (1965) McArdle’s disease presenting as convulsions and rhabdomyolysis.Am. J. Med. 39, 142–146.
Salter R. H., Adamson D. G., and Pearce G. W. (1967) McArdle’s syndrome (myophosphorylase deficiency).Quart. J. Med. 36, 566–578.
Sato, K., Satoh K., Sato T., Imai F., and Morris H. P. (1976) Isozymes patterns of glycogen phosphorylase in rat tissues and transplantable hepatomas.Cancer Res. 36, 487–495.
Turner C. E., and Waterhouse C. (1973) Glucose arteriovenous differences across muscle in McArdle’s disease.Am. J. Med. Sci. 265, 109–115.
Will H., Krause E. G., Bohm M., Guski H., and Wollenberger A. (1974) Kinetische Eigenschaften der Isoenzyme der Glycogen-phosphorylase b aus Herz- und Skelettmuskulatur des Menschen.Acta Biol. Med. Germ. 33, 149–160.
Yunis A. A., Fischer E. H., and Krebs E. G. (1962) Comparative studies on glycogen.J. Biol. Chem. 237, 2809–2815.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Bresolin, N., Miranda, A.F., Jacobson, M.P. et al. Phosphorylase isoenzymes of human brain. Neurochemical Pathology 1, 171–178 (1983). https://doi.org/10.1007/BF02834242
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02834242