Chronic granulomatous disease: More than the lack of superoxide?

Research output: Contribution to journalReview article

56 Citations (Scopus)

Abstract

Chronic granulomatous disease (CGD) is an inherited disease characterized by severe and recurrent bacterial and fungal infections manifested in most cases in early childhood. Phagocytic cells of CGD patients are unable to produce superoxide anions, and their efficiency in bacterial killing is significantly impaired. Recent work has shown alterations in the electrophysiological properties of CGD granulocytes, which might contribute to the pathogenesis of the disease. The new aspects that we discuss in this review concern the proton channel function of gp91phox (the electron-transporting subunit of the NADPH oxidase) and the electrogenic activity of the active enzyme complex, which can affect the transmembrane trafficking of several ions. Based on the reviewed data, we also propose a hypothesis that the absence of a functional NADPH oxidase in CGD neutrophils could result in altered ion compositions within intracellular and intraphagosomal spaces during the process of phagocytosis.

Original languageEnglish
Pages (from-to)191-196
Number of pages6
JournalJournal of Leukocyte Biology
Volume69
Issue number2
Publication statusPublished - Feb 28 2001

Keywords

  • Calcium
  • Membrane potential
  • NADPH oxidase
  • Neutrophils
  • Proton channel

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology
  • Cell Biology

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