Genotype-dependent variability in flow cytometric evaluation of reduced nicotinamide adenine dinucleotide phosphate oxidase function in patients with chronic granulomatous disease

Autor: Vowells, S.J., Fleisher, T.A., Sekhsaria, S., Alling, D.W., Maguire, T.E., Malech, H.L.
Zdroj: The Journal of Pediatrics; January 1996, Vol. 128 Issue: 1 p104-107, 4p
Abstrakt: We studied phagocyte reduced nicotinamide adenine dinucleotide phosphate function to evaluate production of reactive oxygen species in both X-linked and autosomal forms of chronic granulomatous disease. We found a consistent and significant difference between the activated granulocyte response of the X-linked (gp91-phagocyte oxidase) form of chronic granulomatous disease (n = 18) and that of the most common autosomal recessive (p47-phagocyte oxidase) form of the disease (n = 17). The data indicate that mutations in the p47-phagocyte oxidase component of the reduced nicotinamide adenine dinucleotide phosphate oxidase component do not completely prevent oxidation despite severe defects in superoxide generation. (J PEDIATR 1996;128:104-7)
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