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Research Article Free access | 10.1172/JCI105967

Degranulation of leukocytes in chronic granulomatous disease

Robert L. Baehner, Morris J. Karnovsky, and Manfred L. Karnovsky

Department of Biological Chemistry, Harvard Medical School, Boston, Massachusetts 02115

Department of Pathology, Harvard Medical School, Boston, Massachusetts 02115

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Division of Hematology of the Department of Medicine, Childrens Hospital Medical Center, Boston, Massachusetts 02115

Find articles by Baehner, R. in: JCI | PubMed | Google Scholar

Department of Biological Chemistry, Harvard Medical School, Boston, Massachusetts 02115

Department of Pathology, Harvard Medical School, Boston, Massachusetts 02115

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Division of Hematology of the Department of Medicine, Childrens Hospital Medical Center, Boston, Massachusetts 02115

Find articles by Karnovsky, M. in: JCI | PubMed | Google Scholar

Department of Biological Chemistry, Harvard Medical School, Boston, Massachusetts 02115

Department of Pathology, Harvard Medical School, Boston, Massachusetts 02115

Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Division of Hematology of the Department of Medicine, Childrens Hospital Medical Center, Boston, Massachusetts 02115

Find articles by Karnovsky, M. in: JCI | PubMed | Google Scholar

Published January 1, 1969 - More info

Published in Volume 48, Issue 1 on January 1, 1969
J Clin Invest. 1969;48(1):187–192. https://doi.org/10.1172/JCI105967.
© 1969 The American Society for Clinical Investigation
Published January 1, 1969 - Version history
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Abstract

Quantitative chemical analyses of the subcellular distribution patterns for acid and alkaline phosphatase, beta glucuronidase and peroxidase were obtained for human peripheral blood leukocytes of four patients with chronic granulomatous disease (CGD). Five young adults with acute infections served as controls. The observations were made on fractions obtained by homogenization and centrifugation of leukocytes previously incubated with or without particles for ingestion. Distributions in resting CGD and normal cells were very similar for acid and alkaline phosphatase and peroxidase, but the proportion of beta glucuronidase in the granule fraction of CGD cells was depressed, with an increased proportion in the soluble fraction. Release of granule-bound enzymes during phagocytosis of a variety of particles was the same for CGD and control cells, except that release of beta glucuronidase was less marked in CGD cells. Total enzymatic activity of CGD cells for the hydrolases studied was normal. The data indicated that granular enzymes are released in a normal fashion in phagocytizing CGD cells. Supportive evidence of release of enzymes into the phagocytic vacuole of CGD cells was obtained by an electron microscopic study of myeloperoxidase.

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