Elsevier

Metabolism

Volume 30, Issue 6, June 1981, Pages 616-634
Metabolism

Metabolic basis for disorders of purine nucleotide degradation

https://doi.org/10.1016/0026-0495(81)90142-6Get rights and content

Abstract

Purine nucleotide degradation refers to a regulated series of reactions by which human purine ribonucleotides and deoxyribonucleotides are degraded to uric acid in humans. Two major types of disorders occur in this pathway. A block of degradation occurs with syndromes involving immune deficiency, myopathy or renal calculi. Increased degradation of nucleotides occurs with syndromes characterized by hyperuricemia and gout, renal calculi, anemia or acute hypoxia. Management of disorders of purine nucleotide degradation is dependent upon modifying the specific molecular pathology underlying each disease state.

References (237)

  • Y Woo et al.

    5′-Nucleotidase. An ecto-enzyme of frog skeletal muscle

    Biochim Biophys Acta

    (1975)
  • V Stefanovic et al.

    Ecto-5′-nucleotidase of intact cultured C6 rat glioma cells

    J Biol Chem

    (1976)
  • G.P. Frick et al.

    Studies of 5′-nucleotidase in the perfused rat heart

    J Biol Chem

    (1976)
  • G.P. Frick et al.

    Vectorial production of adenosine by 5′-nucleotidase in the perfused rat heart

    J Biol Chem

    (1978)
  • R Itoh et al.

    5′-Nucleotidase of chicken liver

    Biochim Biophys Acta

    (1967)
  • Y Naito et al.

    Cytosol 5′-nucleotidase from chicken liver

    Biochim Biophys Acta

    (1976)
  • P Fritzson

    Nucleotidase activities on the soluble fraction of rat liver homogenate. Partial purification and properties of 5′-nucleotidase with pH optimum 6.3

    Biochim Biophys Acta

    (1969)
  • P Fritzson

    Regulation of nucleotidase activities in animal tissues

    Adv Enz Reg

    (1978)
  • H Hers et al.

    Enzyme defect in primary gout

    The Lancet

    (1979)
  • T.J. Wheeler et al.

    Adenylate deaminase from rat muscle: regulation by purine nucleotides and orthophosphate in the presence of 150 mM KCL

    J Biol Chem

    (1979)
  • T Palella et al.

    Human placental adenosine kinase: Kinetic mechanism and inhibition

    J Biol Chem

    (1980)
  • V Krygier et al.

    Mammalian deoxynucleoside kinases. II. Deoxyadenosine kinase: Purification and properties

    J Biol Chem

    (1971)
  • C.M. Andres et al.

    Purification and properties of human placental adenosine kinase

    J Biol Chem

    (1979)
  • B Ullman et al.

    Deoxyadenosine metabolism and cytotoxicity in cultured mouse T-lymphoma cells: A model of immunodeficiency disease

    Cell

    (1978)
  • N.L. Edwards et al.

    Lymphocyte 5′-nucleotidase deficiency: Clinical characteristics of the associated hypogammaglobulinemia

    Clin Immunol Immnopathol

    (1980)
  • S.M. Johnson et al.

    Lymphocyte purine 5′-nucleotidase deficiency in primary hypogammaglobulinemia

    Lancet

    (1977)
  • K LaMantia et al.

    Lymphocyte 5′-nucleotidase absence of detectable protein in chronic lymphocytic leukemia

    Blood

    (1977)
  • E.R. Giblett et al.

    Adenosine-deaminase deficiency in two patients with severely impaired cellular immunity

    Lancet

    (1972)
  • C.H. Lee et al.

    In vitro platelet abnormality in adenosine deaminase deficiency and severe combined immunodeficiency

    Blood

    (1979)
  • H.A. Simmonds et al.

    A role for purine metabolism in the immune response: Adenosine deaminase activity and deoxyadenosine catabolism

    Lancet

    (1978)
  • G.L. Mills et al.

    Urinary excretion of purines, purine nucleoside and pseudouridine in adenosine deaminase deficiency

    Biochem Med

    (1978)
  • F.C. Schmalstieg et al.

    Increased purine nucleotides in adenosine-deaminase deficient lymphocytes

    J Pediatr

    (1977)
  • M.B. Van der Weyden et al.

    Molecular form of adenosine deaminase in severe combined immunodeficiency

    Biochem Biophys Res Commun

    (1974)
  • L.J. Gudas et al.

    Deoxyguanosine toxicity in a mouse T-lymphoma: Relationship to purine nucleoside phosphorylase-associated immune dysfunction

    Cell

    (1978)
  • T Chan

    Deoxyguanosine toxicity on lymphoid cells as a cause for immunosuppression in purine nucleoside phosphorylase deficiency

    Cell

    (1978)
  • C.A. Lomax et al.

    Adenosine formation and metabolism during adenosine triphosphate catabolism in Ehrlich ascites tumor cells

    Cancer Res

    (1973)
  • C.A. Lomax et al.

    Studies of regulation of purine nucleotide catabolism

    Can J Biochem

    (1975)
  • R.B. McComb et al.

    Metabolism of ascites tumor cells IV. Enzymatic reaction involved in adenosine triphosphate degradation induced by 2-deoxyglucose

    Cancer Res

    (1964)
  • J.C. Bode et al.

    Depletion of liver adenosine phosphates and metabolic effects of intravenous infusion of fructose or sorbital in man and in the rat

    Europ J Clin Invest

    (1973)
  • J.P. Knochel et al.

    Heat, stress, exercise and muscle injury: Effects on urate metabolism and renal function

    Ann Int Med

    (1974)
  • I.H. Fox

    Degradation of purine nucleotides

  • A Solyom et al.

    Enzyme markers in characterization of isolated plasma membranes

    Enzyme

    (1972)
  • M.G. Farquhar et al.

    Cytochemistry of golgi fractions prepared from rat liver

    J Cell Biol

    (1974)
  • D.N. Misra et al.

    Lymphocyte plasma membranes II. Cyto-chemical localization of 5′-nucleotidase in rat lymphocytes

    Biochim Biophys Acta

    (1974)
  • I.H. Fox et al.

    Purine catabolism in man: Inhibition of 5′-phosphomonoesterase activities from placental microsomes

    Can J Biochem

    (1976)
  • N.L. Edwards et al.

    Roles of cytoplasmic and plasma membrane 5′-nucleotidase in regulating intracellular nucleotide levels

    Clin Res

    (1980)
  • G Van Den Derghe et al.

    A kinetic study of the soluble 5′-nucleotidase of rat liver

    Biochem J

    (1977)
  • G Van Den Berghe et al.

    The mechanism of adenosine triphosphate depletion in the liver after a loadof fructose: a kinetic study of adenylate deaminase

    Biochem J

    (1977)
  • F Snyder et al.

    Effects of elevated intracellular ATP and GTP concentrations on purine ribonucleotide synthesis and interconversions

    Canad J Biochem

    (1973)
  • J Barankiewicz et al.

    Effect of lowered intracellular ATP and GTP concentrations on purine ribonucleotide synthesis and interconversions

    Canad J Biochem

    (1977)
  • Cited by (0)

    This work supported in part by USPHS grants AM 19674 and 5M01RR42, and grants from the Michigan Heart Association and the Warner Lambert Company.

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