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Lymphocyte 5′-Nucleotidase Deficiency: Clinical and Metabolic Characteristics of the Associated Hypogammaglobulinemia

  • N. Lawrence Edwards
  • James T. Cassidy
  • Irving H. Fox
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 122B)

Abstract

Decreased activity of 5′-nucleotidase on the surface of peripheral blood mononuclear cells (PBM’s) has recently been observed in young males with congenital agammaglobulinemia1, primary non-familial hypogammaglobulinemia1–3, and selective IgA deficiency3,4. A partial deficiency of the ecto-enzyme occurred in 12 of 12 patients with congenital agammaglobulinemia, in 4 of 15 with acquired (common variable) immunodeficiency, and in 2 of 10 with selective IgA deficiency. The enzyme values and clinical classification of the 18 enzyme deficient subjects with primary hypogammaglobulinemia are shown in Table 1.

Keywords

Carbonyl Iron Purine Metabolism Common Variable Immunodeficiency Nucleotidase Activity Peripheral Mononuclear Cell 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. 1.
    N. L. Edwards, D. B. Magilavy, J. T. Cassidy, and I. H. Fox, Lymphocyte ecto-5′-nucleotidase deficiency in agammaglobulinemia, Science 201:628 (1978).PubMedCrossRefGoogle Scholar
  2. 2.
    S. M. Johson, G. L. Asherson, R. W. E. Watts, M. E. North, J. Allsop, and A. D. B. Webster, Lymphocyte purine 5′-nucleotidase deficiency in primary hypogammaglobulinemia, Lancet 1:168 (1977).CrossRefGoogle Scholar
  3. 3.
    N. L. Edwards, J. T. Cassidy, and I. H. Fox, Lymphocyte 5′-nucleotidase deficiency: Clinical characteristics of the associated hypogammaglobulinemia. Clin. Res. 27:324A (1979).Google Scholar
  4. 4.
    A. D. B. Webster, M. North, J. Allsop, G. L. Asherson, and R. W. E. Watts, Purine metabolism in lymphocytes from patients with primary hypogammaglobulinemia. Clin. Exp. Immunol. 31:456 (1978).PubMedGoogle Scholar
  5. 5.
    N. L. Edwards, E. W. Gelfand, L. Burk, H. M. Dosch, and I. H. Fox, Distribution of 5′-nucleotidase in human lymphoid tissues. Proc. Natl. Acad. Sci. USA 76:(In Press, July) (1979).Google Scholar

Copyright information

© Plenum Press, New York 1980

Authors and Affiliations

  • N. Lawrence Edwards
    • 1
  • James T. Cassidy
    • 1
  • Irving H. Fox
    • 1
  1. 1.Human Purine Research Center, Departments of Internal Medicine and Biological ChemistryUniversity of MichiganAnn ArborUSA

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