Glucocerebroside inhibits NADPH oxidase activation in cell-free system

Patryk Moskwa, Anita Palicz, Marie Hélène Paclet, Marie Claire Dagher, M. Erdős, L. Máródi, E. Ligeti

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

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
Volume1688
Issue number3
DOIs
Publication statusPublished - Apr 5 2004

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Glucosylceramides
Cell-Free System
NADPH Oxidase
Xanthine
Xanthine Oxidase
Phagocytes
methionyl-leucyl-phenylalanine
Cohort Effect
Gaucher Disease
Superoxides
Monocytes
Macrophages
Enzymes

Keywords

  • Gaucher disease
  • Glucocerebroside
  • NADPH oxidase

ASJC Scopus subject areas

  • Molecular Biology
  • Molecular Medicine
  • Biophysics

Cite this

Glucocerebroside inhibits NADPH oxidase activation in cell-free system. / Moskwa, Patryk; Palicz, Anita; Paclet, Marie Hélène; Dagher, Marie Claire; Erdős, M.; Máródi, L.; Ligeti, E.

In: Biochimica et Biophysica Acta - Molecular Basis of Disease, Vol. 1688, No. 3, 05.04.2004, p. 197-203.

Research output: Contribution to journalArticle

Moskwa, Patryk ; Palicz, Anita ; Paclet, Marie Hélène ; Dagher, Marie Claire ; Erdős, M. ; Máródi, L. ; Ligeti, E. / Glucocerebroside inhibits NADPH oxidase activation in cell-free system. In: Biochimica et Biophysica Acta - Molecular Basis of Disease. 2004 ; Vol. 1688, No. 3. pp. 197-203.
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