Glucocerebroside inhibits NADPH oxidase activation in cell-free system.
Biochim Biophys Acta
; 1688(3): 197-203, 2004 Apr 05.
Article
en En
| MEDLINE
| ID: mdl-15062869
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 O(2)(-). 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 p47(phox) and p67(phox). In contrast, O(2)(-) 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 O(2)(-). These data indicate that assembly of the respiratory burst oxidase of phagocytic cells may be a possible target of the pathologic actions of GC.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
NADPH Oxidasas
/
Glucosilceramidas
/
Neutrófilos
Límite:
Humans
Idioma:
En
Revista:
Biochim Biophys Acta
Año:
2004
Tipo del documento:
Article
País de afiliación:
Hungria
Pais de publicación:
Países Bajos