Go to JCI Insight
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Publication alerts by email
  • Advertising
  • Job board
  • Contact
  • Clinical Research and Public Health
  • Current issue
  • Past issues
  • By specialty
    • COVID-19
    • Cardiology
    • Gastroenterology
    • Immunology
    • Metabolism
    • Nephrology
    • Neuroscience
    • Oncology
    • Pulmonology
    • Vascular biology
    • All ...
  • Videos
    • Conversations with Giants in Medicine
    • Video Abstracts
  • Reviews
    • View all reviews ...
    • Complement Biology and Therapeutics (May 2025)
    • Evolving insights into MASLD and MASH pathogenesis and treatment (Apr 2025)
    • Microbiome in Health and Disease (Feb 2025)
    • Substance Use Disorders (Oct 2024)
    • Clonal Hematopoiesis (Oct 2024)
    • Sex Differences in Medicine (Sep 2024)
    • Vascular Malformations (Apr 2024)
    • View all review series ...
  • Viewpoint
  • Collections
    • In-Press Preview
    • Clinical Research and Public Health
    • Research Letters
    • Letters to the Editor
    • Editorials
    • Commentaries
    • Editor's notes
    • Reviews
    • Viewpoints
    • 100th anniversary
    • Top read articles

  • Current issue
  • Past issues
  • Specialties
  • Reviews
  • Review series
  • Conversations with Giants in Medicine
  • Video Abstracts
  • In-Press Preview
  • Clinical Research and Public Health
  • Research Letters
  • Letters to the Editor
  • Editorials
  • Commentaries
  • Editor's notes
  • Reviews
  • Viewpoints
  • 100th anniversary
  • Top read articles
  • About
  • Editors
  • Consulting Editors
  • For authors
  • Publication ethics
  • Publication alerts by email
  • Advertising
  • Job board
  • Contact
Top
  • View PDF
  • Download citation information
  • Send a comment
  • Terms of use
  • Standard abbreviations
  • Need help? Email the journal
  • Top
  • Abstract
  • Version history
  • Article usage
  • Citations to this article (156)

Advertisement

Research Article Free access | 10.1172/JCI106871

Complete Deficiency of Leukocyte Glucose-6-Phosphate Dehydrogenase with Defective Bactericidal Activity

M. Robert Cooper, Lawrence R. DeChatelet, Charles E. McCall, Mariano F. La Via, Charles L. Spurr, and Robert L. Baehner

Department of Biochemistry, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Department of Medicine, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Division of Research Hematology, Department of Medicine, Children's Hospital Medical Center, Harvard Medical School, Boston, Massachusetts 02100

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

Department of Biochemistry, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Department of Medicine, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Division of Research Hematology, Department of Medicine, Children's Hospital Medical Center, Harvard Medical School, Boston, Massachusetts 02100

Find articles by DeChatelet, L. in: JCI | PubMed | Google Scholar

Department of Biochemistry, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Department of Medicine, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Division of Research Hematology, Department of Medicine, Children's Hospital Medical Center, Harvard Medical School, Boston, Massachusetts 02100

Find articles by McCall, C. in: JCI | PubMed | Google Scholar

Department of Biochemistry, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Department of Medicine, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Division of Research Hematology, Department of Medicine, Children's Hospital Medical Center, Harvard Medical School, Boston, Massachusetts 02100

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

Department of Biochemistry, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Department of Medicine, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Division of Research Hematology, Department of Medicine, Children's Hospital Medical Center, Harvard Medical School, Boston, Massachusetts 02100

Find articles by Spurr, C. in: JCI | PubMed | Google Scholar

Department of Biochemistry, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Department of Medicine, Bowman Gray School of Medicine, Winston-Salem, North Carolina 27103

Division of Research Hematology, Department of Medicine, Children's Hospital Medical Center, Harvard Medical School, Boston, Massachusetts 02100

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

Published April 1, 1972 - More info

Published in Volume 51, Issue 4 on April 1, 1972
J Clin Invest. 1972;51(4):769–778. https://doi.org/10.1172/JCI106871.
© 1972 The American Society for Clinical Investigation
Published April 1, 1972 - Version history
View PDF
Abstract

A 52 yr old Caucasian female (F. E.) had hemolytic anemia, a leukemoid reaction, and fatal sepsis due to Escherichia coli. Her leukocytes ingested bacteria normally but did not kill catalase positive Staphylococcus aureus, Escherichia coli, and Serratia marcescens. An H2O2-producing bacterium, Streptococcus faecalis, was killed normally. Granule myeloperoxidase, acid and alkaline phosphatase, and beta glucuronidase activities were normal, and these enzymes shifted normally to the phagocyte vacuole (light and electron microscopy). Intravacuolar reduction of nitroblue tetrazolium did not occur. Moreover, only minimal quantities of H2O2 were generated, and the hexose monophosphate shunt (HMPS) was not stimulated during phagocytosis.

These observations suggested the diagnosis of chronic granulomatous disease. However, in contrast to control and chronic granulomatous disease leukocytes, glucose-6-phosphate dehydrogenase activity was completely absent in F. E. leukocytes whereas NADH oxidase and NADPH oxidase activities were both normal. Unlike chronic granulomatous disease, methylene blue did not stimulate the hexose monophosphate shunt in F. E. cells. Thus, F. E. and chronic granulomatous disease leukocytes appear to share certain metabolic and bactericidal defects, but the metabolic basis of the abnormality differs. Chronic granulomatous disease cells lack oxidase activity which produces H2O2; F. E. cells had normal levels of oxidase activity but failed to produce NADPH due to complete glucose-6-phosphate dehydrogenase deficiency. These data indicate that a complete absence of leukocyte glucose-6-phosphate dehydrogenase with defective hexose monophosphate shunt activity is associated with low H2O2 production and inadequate bactericidal activity, and further suggest an important role for NADPH in the production of H2O2 in human granulocytes.

Images.

Browse pages

Click on an image below to see the page. View PDF of the complete article

icon of scanned page 769
page 769
icon of scanned page 770
page 770
icon of scanned page 771
page 771
icon of scanned page 772
page 772
icon of scanned page 773
page 773
icon of scanned page 774
page 774
icon of scanned page 775
page 775
icon of scanned page 776
page 776
icon of scanned page 777
page 777
icon of scanned page 778
page 778
Version history
  • Version 1 (April 1, 1972): No description

Article tools

  • View PDF
  • Download citation information
  • Send a comment
  • Terms of use
  • Standard abbreviations
  • Need help? Email the journal

Metrics

  • Article usage
  • Citations to this article (156)

Go to

  • Top
  • Abstract
  • Version history
Advertisement
Advertisement

Copyright © 2025 American Society for Clinical Investigation
ISSN: 0021-9738 (print), 1558-8238 (online)

Sign up for email alerts