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Genetic Disorders of Granulocyte Function: What they Tell Us about Normal Mechanisms

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Host Defenses to Intracellular Pathogens

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 162))

Abstract

Clinically significant genetic defects of phagocyte function have been most clearly defined for polymorphonuclear leukocytes (PMN). Several important lessons have been learned from an analysis of these defects. These include:

  1. 1)

    Defining the role of oxidative metabolism in the microbicidal activity of PMNs;

  2. 2)

    determining the existence and activity of “back-up” killing mechanisms when deletions in normal pathways occur, and;

  3. 3)

    discerning the importance of PMNs relative to other cells in host defense against different microorganisms.

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References

  1. Schimpff, S.C. (1977). Therpay of infection in patients with granulocytopenia. Med. Clin. N. Amer. 61:1101.

    PubMed  CAS  Google Scholar 

  2. Johnston, R.B., Jr. and Baehner, R.L. (1971). Chronic granulomatous disease: correlation between pathgenesis and clinical findings. Pediatrics 48:730–379.

    PubMed  Google Scholar 

  3. Cohen, M.S., Isturiz, R.E., Malech, H.L., et al. (1981). Fungal infection in chronic granulomatous disease: the importance of the phagocyte in defense against fungi. Am. J. Med. 71:59–66.

    Article  PubMed  CAS  Google Scholar 

  4. Blume, R.S. and Wolff, S.M. (1972). The Chediak-Higashi syndrome, studies in four patients and a review of the literature. Medicine 51:247–280.

    Article  PubMed  CAS  Google Scholar 

  5. Lehrer, R.I. and Cline, M.J. (1969). Leukocyte myeloperoxidase deficiency and disseminated candidiasis: the role of myeloperoxidase in resistance to Candida infection. J. Clin. Invest. 48:1478–88.

    Article  PubMed  CAS  Google Scholar 

  6. Stossel, T.P., Root, R.K. and Vaughan, M. (1972). Phagocytosis in chronic granulomatous disease and the Chediak-Higashi syndrome. N. Engl. J. Med. 286:120–123.

    Article  PubMed  CAS  Google Scholar 

  7. Rausch, P.G., Pryzwansky, K.B. and Spitznagel, J.K (1978). Immunocytochemical identification of azurophilic and specific granule markers in the giant granules of Chediak Higashi neutrophils. N. Engl. J. Med. 298: 693–698.

    Article  PubMed  CAS  Google Scholar 

  8. Oliver, J.M. and Zurier, R.B. (1976). Correction of characteristic abnormalities of microtubule function and granule morphology in Chediak-Higashi syndrome with cholinergic agonists: studies in vitro in man and in vivo in the beige mouse. J. Clin. Invest. 57:1239–1247.

    Article  PubMed  CAS  Google Scholar 

  9. Root, R.K., Rosenthal, A.S. and Balestra, D.J. (1972). Abnormal bactericidal, metabolic and lysosomal functions of Chediak-Higashi syndrome leukocytes. J. Clin. Invest. 51:649–665.

    Article  PubMed  CAS  Google Scholar 

  10. Clark, R.A. and Kimball, H.R. (1971). Defective granulocyte Chemotaxis in the Chediak-Higashi syndrome. J. Clin. Invest. 50:2645–2652.

    Article  PubMed  CAS  Google Scholar 

  11. Gallin, J.I., Klimerman, J.A., Padgett, G.A., et al. (1975). Defective mononuclear leukocyte Chemotaxis in the Chediak-Higashi syndrome of humans, mink and cattle. Blood 45:863–870.

    PubMed  CAS  Google Scholar 

  12. Root, R.K. (1975). Comparison of other defects of granulocyte oxidative killing mechanisms with chronic granulomatous disease in J. Bellanti and D.H. Dayton (ed). The Phagocytic Cell in Host Resistance. Raven Press, New York, p. 201–226.

    Google Scholar 

  13. Boxer, L.A., Watanabe, A.M., Rister, M., et al. (1976). Correction of leukocyte function in Chediak-Higashi syndrome by ascorbate. N. Engl. J. Med. 295:1041–1045.

    Article  PubMed  CAS  Google Scholar 

  14. Mills, E.L. and Quie, P.G. (1980). Congenital disorders of the functions of polymorphonuclear neutrophils. Rev. Inf. Dis. 2:505–517.

    Article  CAS  Google Scholar 

  15. Windhorst, D.B., Page, A.R., Holmes, B., et al. (1968). The pattern of genetic transmission of the leukocyte defect in fatal granulomatous disease of childhood. J. Clin. Invest. 47:1026–1034.

    Article  PubMed  CAS  Google Scholar 

  16. Holmes, B., Park, B.H., Malawista, S.E., et al. (1970). Chronic granulomatous disease in females: a deficiency of leukocyte glutathione peroxidase. N. Engl. J. Med. 282:217–221.

    Article  Google Scholar 

  17. Quie, P.G., White, J.G., Holmes, B., et al. (1967). In vitro bactericidal capacity of human polymorphonuclear leukocytes: diminished activity in chronic granulomatous disease of childhood. J. Clin. Invest. 46:668–679.

    Article  PubMed  CAS  Google Scholar 

  18. Holmes, B., Page, A.R. and Good, R.A. (1967). Studies of the metabolic activity of leukocytes from patients with a genetic abnormality of phagocytic function. J. Clin. Invest. 46:1422–1432.

    Article  PubMed  CAS  Google Scholar 

  19. Rodey, G.E., Park, B.H., Windhorst, D.B., et al. (1969). Defective bactericidal activity of monocytes in fatal granulomatous disease. Blood 33:813–820.

    PubMed  CAS  Google Scholar 

  20. Baehner, R.L. and Nathan, D.G. (1968). Quantitative nitroblue tetrazolium test in chronic granulomatous disease. N. Engl. J. Med. 278:971–976.

    Article  PubMed  CAS  Google Scholar 

  21. Badwey, J.A., Curnutte, J.T. and Kamorsky, M.C. (1979). The enzyme of granulocytes that produces superoxide and peroxide: an elusive pimpernel. N. Engl. J. Med. 300:1157–1160.

    Article  PubMed  CAS  Google Scholar 

  22. Johnston, R.B., Jr., Keele, B.B., Jr., Misra, H.P., et al. (1972). The role of superoxide anion generation in phagocytic bactericidal activity: studies with normal and chronic granulomatous disease leukocytes. J. Clin. Invest. 55:1357–1372.

    Article  Google Scholar 

  23. Drath, D.B., Karnovsky, M.L. (1975). Superoxide production by phagocytic leukocytes. J. Exp. Med. 141:257–262.

    Article  PubMed  CAS  Google Scholar 

  24. Nathan, C.F. and Root, R.K. (1977). Hydrogen peroxide release from mouse peritoneal macrophages: depending on sequential activation and triggering. J. Exp. Med. 146:1648–1662.

    Article  PubMed  CAS  Google Scholar 

  25. Dilworth, J.A. and Mandell, G.L. (1977). Adults with chronic granulomatous disease of childhood. Am. J. Med. 63:233.

    Article  PubMed  CAS  Google Scholar 

  26. Schultz, J. and Kaminker, K. (1962). Myeloperoxidase of the leucocyte of normal human blood. I. Content and localization. Arch. Biochem. Biophys. 96:465.

    Article  PubMed  CAS  Google Scholar 

  27. Klebanoff, J.J. (1967). Iodination of bacteria: a bactericidal mechanism. J. Exp. Med. 126:1063–1078.

    Article  PubMed  CAS  Google Scholar 

  28. Root, R.K. and Cohen, M.S. (1981). The microbicidal mechanisms of human neutrophils and eosinophils. Rev. Inf. Dis. 3:565–598.

    Article  CAS  Google Scholar 

  29. Klebanoff, S.J. and Clark, R.A. (1978). The neutrophil: Function and clinical disorders. Amsterdam, North Holland Publishing Co. 1978.

    Google Scholar 

  30. Klebanoff, S.J. and Clark, R.A. (1977). Iodination by human polymorphonuclear leukocytes: a reevaluation. J. Lab. Clin. Med. 89:675–686.

    PubMed  CAS  Google Scholar 

  31. Klebanoff, S.J. and Hamon, C.B. (1972). Role of myeloperoxidase-mediated antimicrobial systems in intact leukocytes. J. Reticuloendothel. Soc. 12:170–196.

    PubMed  CAS  Google Scholar 

  32. Parry, M.F., Root, R.K., Metcalf, J.A., et al. (1981). Myeloperoxidase deficiency: prevalence and clinical significance. Ann. Int. Med. 95:293–301.

    PubMed  CAS  Google Scholar 

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© 1983 Plenum Press, New York

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Root, R.K. (1983). Genetic Disorders of Granulocyte Function: What they Tell Us about Normal Mechanisms. In: Eisenstein, T.K., Actor, P., Friedman, H. (eds) Host Defenses to Intracellular Pathogens. Advances in Experimental Medicine and Biology, vol 162. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-4481-0_5

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  • DOI: https://doi.org/10.1007/978-1-4684-4481-0_5

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4684-4483-4

  • Online ISBN: 978-1-4684-4481-0

  • eBook Packages: Springer Book Archive

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