Abstract
In humans an inherited deficiency of the enzyme purine nucleoside Phosphorylase (PNP; EC 2.4.2.1) is associated with a T cell deficiency and apparently normal B cells (1,2). Although a deficiency of PNP is present in all cells of deficient individuals, only the cells within the T lymphocyte lineage are affected. The immunodeficiency state associated with PNP deficiency is thought to arise from block(s) in T lymphocyte differentiation resulting from the accumulation of toxic metabolites of the enzyme’s substrate(s) (3-5).
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References
Giblett, E.R., A.J. Amman, D.W. Wara and L.K. Diamond. 1975. Lancet 1: 1010.
Stoop, J.W., B.J.M. Zegers, G.F.M. Hendricks, L.H. Siegenbeek van Heukelom, G.E.J. Staal, P.K. DeBree, S.K. Wadman and R.E. Ballieux. 1977. New Engl. J. Med. 296: 651.
Polmar, S.H. 1980. Sem. Hematol. 17: 30.
Osborne, W.R.A. 1981. Trends Biochem. Sci. 63: 80.
Hirschhorn, R. 1983. Birth Defects 19: 73
Mitchell, B.S., E. Mejias, P.E. Dadonna and W.N. Kelly. 1978. Proc. Nat. Acad. Sci. 75: 5011.
Carson, D.A., J. Kaye and J.E. Seegmiller. 1978. J. Immunol. 121: 1726.
Cohen, A., J.W.W. Lee, H.-M. Dosch and E.W. Gelfand. 1980. J. Immunol. 121: 1726.
Fox, R.M., E.H. Tripp, S.K. Piddington and M.H.N. Tattersall. 1980. Cancer Res. 40: 3383.
Ullman, B., L.J. Gudas, S.M. Clift and D.W. Martin. 1979. Proc. Nat. Acad. Sci. 76: 1074.
Cohen, A., L.F. Gudas, A.J. Amman, G.E.J. Staal and D.W. Martin. 1978. J. Clin. Invest. 61: 1405.
Gelfand, E.W., J.W. Lee and H.-M. Dosch. 1979. Proc. Nat. Acad. Sci. 76: 1998.
Chan, T. 1978. Cell 14: 523.
Osborne, W.R.A. and C.R. Scott. 1983. Biochem. J. 214: 711.
Stoeckler, J.D., C. Cambor, V. Kuhns, S.-H. Chu and R.E. Parks. 1982. Biochem. Pharmacol. 31: 163.
Kazmers, I.S., B.S. Mitchell, P.E. Dadonna, L.L. Wotring, L.B. Townsend and W.N. Kelly. 1981. Science 214: 1138.
Barton, R.W., F. Martinuik, R. Hirschhorn and I. Goldschneider. 1980. Cell. Immunol. 49: 208.
Beaudoin, B.J. and I. Goldschneider. 1985.
Garrett, C. and D.V. Santi. 1979. Anal. Biochem. 99: 268.
Osborne, W.R.A., W.P. Hammond and D.C. Dale. 1983. J. Clin. Invest. 71: 1348.
Gudas, L.J., B. Ullman, A. Cohen and D.W. Martin, Jr. Cell 14: 531.
Goldschneider, I. 1980. Curr. Top. Dev. Biol. 14: 33.
Metcalf, D. 1966. in The Thymus: Experimental and Clinical Studies. Edited by G.E.W. Wolstenholme and R. Porter. Ciba Foundation Symposium, Churchill, Ltd., London, pp. 242–263.
Shortman, K. and R. Scollay. 1985. In Recognition and Regulation in Cell-Mediated Immunity. Edited by J. Watson. Marcel Dekker, New York.
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© 1986 Plenum Press, New York
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Barton, R.W., Osborne, W.R.A. (1986). The Effects of PNP Inhibition on Rat Lymphoid Cell Populations. In: Nyhan, W.L., Thompson, L.F., Watts, R.W.E. (eds) Purine and Pyrimidine Metabolism in Man V. Advances in Experimental Medicine and Biology, vol 195B. Springer, New York, NY. https://doi.org/10.1007/978-1-4684-1248-2_67
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DOI: https://doi.org/10.1007/978-1-4684-1248-2_67
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