Abstract
Adenylosuccinate lyase (EC 4.3.2.2; ASase) catalyses two steps in the synthesis of purine nucleotides: the conversion of succinylaminoimidazole carboxamide (SAICA) ribotide into aminoimidazole carboxamide (AICA) ribotide along the de novo pathway and the formation of AMP from adenylosuccinate in the conversion of IMP into adenine nucleotides. Both reactions involve the cleavage of a succinyl group, yielding fumarate.1
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
Similar content being viewed by others
References
M. R. Redinbo, S. M. Eide, R. L. Stone, J. E. Dixon, and T. O. Yeates, Crystallization and preliminary structural analysis of Bacillus subtilis adenylosuccinate lyase, an enzyme implicated in infantile autism. Protein Science 5: 786–788 (1996)
J. Jaeken and G. Van den Berghe. An infantile autistic syndrome characterized by the presence of succinylpurines in body fluids, Lancet 2: 1058–1061 (1984)
J. Jaeken, S. K. Wadman, M. Duran, F. J. Van Sprang, F. A. Beemer, R. A. Holl, P. M. Theunissen, P. De Cock, F. Van den Bergh, M. F. Vincent, and G. Van den Berghe. Adenylosuccinase deficiency: an inborn error of purine nucleotide synthesis. Eur. J. Pediatr. 148: 126–131 (1988)
C. Salerno, C. Crifó, and O. Giardini. Adenylosuccinase deficiency: a patient with impaired erythrocyte activity and anomalous response to intravenous fructose. J. Inher. Metab. Dis. 18: 602–608 (1995)
C. Salerno, S. Iotti, R. Lodi, C. Crifò, and B. Barbiroli. Failure of muscle energy metabolism in a patient with adenylosuccinate lyase deficiency: an in vivo study by phosphorus NMR spectroscopy. Biochim. Biophys. Acta (1997) in press
D. Arnold, P. M. Matthews, and G. K. Radda. Metabolic recovery after exercise and the assessment of mitochondrial function in human skeletal muscle in vivo by means of 31P-NMR. Magn. Reson. Med. 1: 307–315 (1984)
B. Chance, J. S. Leigh, J. Kent, K. K. McCully, S. Nioka, B. J. Clark, J. M. Maris, and T. Graham. Multiple controls of oxidative metabolism in living tissues as studied by phosphorus magnetic resonance. Proc. Natl. Acad. Sci. USA 83: 9458–9462 (1986)
S. Iotti, R. Lodi, G. Gottardi, P. Zaniol, and B. Barbiroli. Inorganic phosphate is transported into mitochondria in the absence of ATP biosynthesis. An in vivo 31P NMR study in the human skeletal muscle. Biochem. Biophys. Res. Commun. (1996) 225: 191–194
J. J. Aragon and J. M. Lowenstein. The purine-nucleotide cycle: comparison of the levels of citric acid cycle intermediates with the operation of the purine nucleotide cycle in rat skeletal muscle during exercise and recovery from exercise. Eur. J. Biochem. 110: 371–377 (1980)
M. E. Young, G. K. Radda, and B. Leighton. Activation of glycogen phosphorylase and glycogenolysis in rat skeletal muscle by AICAR—an activator of AMP-activated protein kinase. FEBS Letters 382: 43–47 (1996)
K. Tornheim and J. M. Lowenstein. The purine nucleotide cycle: control of phosphofructokinase and glycolytic oscillation in muscle extracts. J. Biol. Chem. 250: 6304–6308 (1975)
R. L. Sabina, J. L. Swain, B. M. Patten, T. Ashizawa, W. E. O’Brien, and E. W. Holmes. Disruption of the purine nucleotide cycle: a potential explanation for muscle dysfunction in myoadenylate deaminase deficiency. J. Clin. Invest. 66: 1419–1423 (1980)
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1998 Springer Science+Business Media New York
About this chapter
Cite this chapter
Salerno, C., Iotti, S., Lodi, R., Crifò, C., Barbiroli, B. (1998). Muscle Energy Metabolism in Human Adenylosuccinase Deficiency. In: Griesmacher, A., Müller, M.M., Chiba, P. (eds) Purine and Pyrimidine Metabolism in Man IX. Advances in Experimental Medicine and Biology, vol 431. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-5381-6_54
Download citation
DOI: https://doi.org/10.1007/978-1-4615-5381-6_54
Publisher Name: Springer, Boston, MA
Print ISBN: 978-1-4613-7456-5
Online ISBN: 978-1-4615-5381-6
eBook Packages: Springer Book Archive