Correction of metabolic deficiencies in the leukocytes of patients with chronic granulomatous disease
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Evidence is strengthened that the fundamental metabolic lesion in CGD cells during phagocytosis is indeed deficient production of hydrogen peroxide, probably, as previously shown, due to diminished oxidase for reduced nicotinamide adenine dinucleotide.Abstract:
Polymorphonuclear leukocytes from patients with chronic granulomatous disease (CGD) exhibit metabolic and bactericidal deficiencies that may be the result of inadequate production of H2O2. A hydrogen peroxide-generating system was, therefore, inserted into CGD leukocytes. This was accomplished by allowing the cells to phagocytize latex spherules coated with glucose oxidase. This produced an amelioration in the known metabolic deficiencies of these cells during phagocytosis: (a) intracellular (catalatic) formate oxidation dependent upon hydrogen peroxide production was enhanced fourfold; and (b) hexose monophosphate shunt activity, which other workers have shown to be at least partially dependent upon the availability of H2O2, was markedly stimulated. These data strengthen the evidence that the fundamental metabolic lesion in CGD cells during phagocytosis is indeed deficient production of hydrogen peroxide, probably, as previously shown, due to diminished oxidase for reduced nicotinamide adenine dinucleotide.read more
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References
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Biochemical Aspects of Phagocytosis
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Studies of the Metabolic Activity of Leukocytes from Patients with a Genetic Abnormality of Phagocytic Function
TL;DR: It appears that the stimulation of respiration with the formation of hydrogen peroxide and stimulation of the direct oxidative pathway of glucose metabolism are closely linked to degranulation and intracellular killing of bacteria by polymorphonuclear leukocytes.
Journal ArticleDOI
Leukocyte Oxidase: Defective Activity in Chronic Granulomatous Disease
TL;DR: The addition of nitroblue tetrazolium to a leukocyte suspension appears to provide a sensitive diagnostic screening test for chronic granulomatous disease.