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)
Defining the role of oxidative metabolism in the microbicidal activity of PMNs;
-
2)
determining the existence and activity of “back-up” killing mechanisms when deletions in normal pathways occur, and;
-
3)
discerning the importance of PMNs relative to other cells in host defense against different microorganisms.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Schimpff, S.C. (1977). Therpay of infection in patients with granulocytopenia. Med. Clin. N. Amer. 61:1101.
Johnston, R.B., Jr. and Baehner, R.L. (1971). Chronic granulomatous disease: correlation between pathgenesis and clinical findings. Pediatrics 48:730–379.
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.
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.
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.
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.
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.
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.
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.
Clark, R.A. and Kimball, H.R. (1971). Defective granulocyte Chemotaxis in the Chediak-Higashi syndrome. J. Clin. Invest. 50:2645–2652.
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.
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.
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.
Mills, E.L. and Quie, P.G. (1980). Congenital disorders of the functions of polymorphonuclear neutrophils. Rev. Inf. Dis. 2:505–517.
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.
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.
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.
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.
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.
Baehner, R.L. and Nathan, D.G. (1968). Quantitative nitroblue tetrazolium test in chronic granulomatous disease. N. Engl. J. Med. 278:971–976.
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.
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.
Drath, D.B., Karnovsky, M.L. (1975). Superoxide production by phagocytic leukocytes. J. Exp. Med. 141:257–262.
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.
Dilworth, J.A. and Mandell, G.L. (1977). Adults with chronic granulomatous disease of childhood. Am. J. Med. 63:233.
Schultz, J. and Kaminker, K. (1962). Myeloperoxidase of the leucocyte of normal human blood. I. Content and localization. Arch. Biochem. Biophys. 96:465.
Klebanoff, J.J. (1967). Iodination of bacteria: a bactericidal mechanism. J. Exp. Med. 126:1063–1078.
Root, R.K. and Cohen, M.S. (1981). The microbicidal mechanisms of human neutrophils and eosinophils. Rev. Inf. Dis. 3:565–598.
Klebanoff, S.J. and Clark, R.A. (1978). The neutrophil: Function and clinical disorders. Amsterdam, North Holland Publishing Co. 1978.
Klebanoff, S.J. and Clark, R.A. (1977). Iodination by human polymorphonuclear leukocytes: a reevaluation. J. Lab. Clin. Med. 89:675–686.
Klebanoff, S.J. and Hamon, C.B. (1972). Role of myeloperoxidase-mediated antimicrobial systems in intact leukocytes. J. Reticuloendothel. Soc. 12:170–196.
Parry, M.F., Root, R.K., Metcalf, J.A., et al. (1981). Myeloperoxidase deficiency: prevalence and clinical significance. Ann. Int. Med. 95:293–301.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 1983 Plenum Press, New York
About this paper
Cite this paper
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
Download citation
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