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

Autor: Vowells SJ; Laboratory of Host Defenses, National Institute of Allergy and Infectious Disease, National Institutes of Health, Bethesda, Maryland 20892, USA., Fleisher TA, Sekhsaria S, Alling DW, Maguire TE, Malech HL
Jazyk: angličtina
Zdroj: The Journal of pediatrics [J Pediatr] 1996 Jan; Vol. 128 (1), pp. 104-7.
DOI: 10.1016/s0022-3476(96)70437-7
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.
Databáze: MEDLINE