Glucocerebroside inhibits NADPH oxidase activation in cell-free system

Patryk Moskwa, Anita Palicz, Marie Hélène Paclet, Marie Claire Dagher, Melinda Erdos, László Maródi, Erzsébet Ligeti

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10 Citations (Scopus)


We reported earlier that monocytes and macrophages from patients with type I Gaucher disease have a decreased capacity to generate superoxide anion (O 2-) on stimulation with opsonized S. aureus or formyl-methionyl-leucyl-phenylalanine. In this study, various forms of the cell-free assay system were used to probe the hypothesis that glucocerebroside (GC) accumulating in Gaucher patients' phagocytes may interfere with the activation of NADPH oxidase. Xanthine/xanthine oxidase assay was applied to explore the possibility that GC may scavenge O2-. We found that addition of GC to the crude, semirecombinant or fully purified cell-free systems inhibited activation of NADPH oxidase in a concentration-dependent manner. The inhibitory effect of GC could be overcome by increased concentrations of p47phox and p67phox. In contrast, O2- generation was not decreased by GC added to the assembled, catalytically active enzyme complex. In the xanthine/xanthine oxidase system, GC had no effect on the generation of O2 -. These data indicate that assembly of the respiratory burst oxidase of phagocytic cells may be a possible target of the pathologic actions of GC.

Original languageEnglish
Pages (from-to)197-203
Number of pages7
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Issue number3
Publication statusPublished - Apr 5 2004



  • Gaucher disease
  • Glucocerebroside
  • NADPH oxidase

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology

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