Skip to main content

Advertisement

Log in

Formation and degradation of deoxyadenosine nucleotides in inherited adenosine deaminase deficiency

  • Published:
Bioscience Reports

This is a preview of subscription content, log in via an institution to check access.

Access this article

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

Instant access to the full article PDF.

References

  1. Carson DA, Kaye J, Matsumoto S, Seegmiller JE & Thompson L (1979) Biochemical basis for the enhanced toxicity of deoxyribonucleosides toward malignant human T-cell lines. Proc. Natl. Acad. Sci. U.S.A.76, 2430–2433.

    Google Scholar 

  2. Hershfield MS & Kredich NM (1980) Resistance of an adenosine kinase-deficient human lymphoblast cell line to effects of deoxyadenosine on growth, S-adenosyl homocysteine hydrolase inactivation and dATP accumulation. Proc. Natl. Acad. Sci. U.S.A.77, 4292–4296.

    Google Scholar 

  3. Ballet J-J, Insel R, Merler E & Rosen FS (1976) Inhibition of maturation of human precursor lymphocytes by coformycin, an inhibitor of the enzyme adenosine deaminase. J. Exper. Med.143, 1271–1276.

    Google Scholar 

  4. Simmonds HA, Sahota A, Potter CF, Perrett D, Hugh-Jones K & Watson JG (1979) Purine metabolism in adenosine deaminase deficiency. InEnzyme Defects and Immune Dysfunction, Ciba Symposium 68 (new series). Excerpta Medica, 255–262.

  5. Cohen A, Hirshorn R, Horowitz SD, Rubenstein A, Polmar SH, Hong R & Martin DW (1978) Deoxyadenosine triphosphate as a potentially toxic metabolite in adenosine deaminase deficiency. Proc. Natl. Acad. Sci. U.S.A.75, 472–476.

    Google Scholar 

  6. Coleman MS, Donofrio J, Hutton JJ, Hahn L, Daoud A, Lampkin B & Dyminski J (1978) Identification and quantitation of adenine deoxynucleotides in erythrocytes of a patient with adenosine deaminase deficiency and severe combined immunodeficiency. J. Biol. Chem.253, 1619–1628.

    Google Scholar 

  7. Schmalstieg FC, Mills GC, Nelson JA, May LT, Goldman AS & Goldblum RM (1978) Limited effect of erythrocyte and plasma infusions in adenosine deaminase deficiency. J. Pediatrics93, 597–603.

    Google Scholar 

  8. Chen S-H, Ochs HD & Scott CR (1978) Adenosine deaminase deficiency. Disappearance of adenine deoxynucleotides from a patient's erythrocytes after successful marrow transplantation. J. Clin. Invest.62, 1386–1389.

    Google Scholar 

  9. Hirschorn R & Roegner V (1980) Plasma deoxyadenosine, adenosine and erythrocyte deoxy-ATP are elevated at birth in an adenosine deaminase deficient child. J. Clin. Invest.65, 768–771.

    Google Scholar 

  10. Simmonds HA, Levinsky RJ, Perrett D & Webster DR (1982) Reciprocal relationship between erythrocyte ATP and deoxy-ATP levels in inherited ADA deficiency. Biochem. Pharmacol.31, 947–951.

    Google Scholar 

  11. Perrett D, Sahota A, Simmonds HA & Hugh-Jones K (1981) Deoxyadenosine metabolism in the erythrocytes of children with severe combined immunodeficiency. Bioscience Reports1, 933–944.

    Google Scholar 

  12. Goday A, Webster DR, Simmonds HA, Perrett D, Brown Z, Reiter S, Watson AR, and Levinsky RJ (in press) Nucleotide levels in peripheral blood mononuclear cells of immunodeficient children: Problems of measurement. IV International Symposium on Human Purine and Pyrimidine Metabolism. J. Clin. Chem. Clin. Biochem. (abstract); and Proceedings, Plenum (full paper).

  13. Webster DR, Simmonds HA, Barry DMJ & Becroft DMO (1981) Pyrimidine and purine metabolites in ornithine transcarbamylase deficiency. J. Inher. Metab. Dis.4, 27–33.

    Google Scholar 

  14. Hutton JJ, Wiginton DA, Coleman MS, Fuller SA, Limouze S & Lampkin BC (1981) Biochemical and functional abnormalities in lymphocytes from an adenosine deaminase deficient patient during enzyme replacement therapy. J. Clin. Invest.68, 413–421.

    Google Scholar 

  15. Agarwal RP & Parks RE (1975) A possible association between the nucleoside transport system of human erythrocytes and adenosine deaminase. Biochem. Pharmac.24, 547–550.

    Google Scholar 

  16. Mager J, Dvilansky A, Razin A, Wind E & Izak G (1966) Turnover of purine nucleotides in human red blood cells. Israel. J. Med. Sci.2, 297–301.

    Google Scholar 

  17. Perrett D & Dean BM (1977) The function of adenosine deaminase in the human erythrocyte. Biochem. Biophys. Res. Commun.77, 374–378.

    Google Scholar 

  18. Meyskens FL & Williams HE (1971) Adenosine metabolism in human erythrocytes. Biochim. Biophys. Acta240, 170–179.

    Google Scholar 

  19. Dean BM, Perrett D, Simmonds HA, Sahota A & Van Acker KJ (1978) Adenine and adenosine metabolism in intact erythrocytes deficient in adenosine monophosphate-pyrophosphate phosphoribosyltransferase: a study of two families. Clin. Sci. Mol. Med.55, 407–412.

    Google Scholar 

  20. Siaw MFE, Mitchell BS, Koller CA, Coleman MS & Hutton JJ (1980) ATP depletion as a consequence of adenosine deaminase inhibition in man. Proc. Natl. Acad. Sci. U.S.A.77, 6157–6161.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Simmonds, H.A., Webster, D.R., Perrett, D. et al. Formation and degradation of deoxyadenosine nucleotides in inherited adenosine deaminase deficiency. Biosci Rep 2, 303–314 (1982). https://doi.org/10.1007/BF01115116

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01115116

Keywords

Navigation