Skip to main content

Advertisement

Log in

Deoxyadenosine metabolism in the erythrocytes of children with sever, combined immunodeficiency

  • 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. Enzyme Defects and Immune Dysfunction. (1979) Ciba Symposium 68 (new series), Excerpta Medica, Amsterdam.

  2. Hirschhorn R (1979) inInborn Errors of Specific Immunity, (Pollard B, Pickering RJ, Meuwissen HJ & Porter IH, eds), pp 5–15, Academic Press, New York.

    Google Scholar 

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

    Google Scholar 

  4. Hirschhorn 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 

  5. Rich KC, Richman CM, Mejias E & Daddona P (1980) Immunoreconstitution by peripheral blood leukocytes in adenosine deaminase-deficient severe combined immunodeficiency. J. Clin. Invest.66, 389–395.

    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, 1619–1626.

    Google Scholar 

  7. 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 

  8. Ziegler JB, Lee CH, Van der Weyden MB, Bagnara AS & Beveridge J (1980) Severe combined immunodeficiency and adenosine deaminase deficiency: failure of enzyme replacement therapy. Arch. Dis. Child55, 452–457.

    Google Scholar 

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

    Google Scholar 

  10. Simmonds HA, Panayi GS & Corrigall V (1978) A role for purine metabolism in the immune response: adenosine deaminase activity and deoxyadenosine catabolism. Lanceti, 60–63.

    Google Scholar 

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

    Google Scholar 

  12. Snyder FF & Henderson JF (1973) Alternative routes of deoxyadenosine and adenosine metabolism. J. Biol. Chem.248, 5899–5904.

    Google Scholar 

  13. Agarwal RP, Crabtree GW, Parks RE Jr, Nelson JA, Keightley R, Parkman R, Rosen FS, Stern RC & Polman SH (1976) Purine nucleoside metabolism in the erythrocytes of patients with adenosine deaminase deficiency and severe combined immunodeficiency. J. Clin. Invest.57, 1025–1035.

    Google Scholar 

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

    Google Scholar 

  15. Sahota A, Simmonds HA, Potter CF, Watson JG, Hugh-Jones K & Perrett D (1979) Abnormal purine metabolism in adenosine deaminase deficiency. Correction following marrow transplantation. InPurine Metabolism in Man, IIIA, (Rapado A, De Bruyn C & Watts RWE, eds), pp 397–403, Plenum Press, New York.

    Google Scholar 

  16. Dean BM, Perrett D & Sensi M (1978) Changes in nucleotide concentrations in the erythrocytes of man, rabbit and rat during short-term storage. Biochem. Biophys. Res. Commun.80, 147–154.

    Google Scholar 

  17. Dean BM & Perrett D (1976) Studies on adenine and adenosine metabolism by intact human erythrocytes using high performance liquid chromatography. Biochim. Biophys. Acta437, 1–15.

    Google Scholar 

  18. Perrett D (1979) Application of high performace liquid chromatography (HPLC) to biochemical anslysis. InTechniques in Metabolic Research, (Kornberg HL, Metcalfe JC, Northcote DH, Pogson CI & Tipton KF, eds), pp 1–22, B 215, Elsevier, North Holland.

    Google Scholar 

  19. Schrader WP, Pollara B & Meuwissen HJ (1978) Characterisation of the residual adenosine deaminating activity in the spleen of a patient with combined immunodeficiency disease and adenosine deaminase deficiency (enzyme deficiency). Proc. Natl. Acad. Sci. U. S. A.75, 446–450.

    Google Scholar 

  20. 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 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Perrett, D., Sahota, A., Simmonds, H.A. et al. Deoxyadenosine metabolism in the erythrocytes of children with sever, combined immunodeficiency. Biosci Rep 1, 933–944 (1981). https://doi.org/10.1007/BF01114963

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01114963

Keywords

Navigation