Correction of metabolic deficiencies in the leukocytes of patients with chronic granulomatous disease
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TLDR
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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Improvement of Polymorphonuclear Leucocyte Oxidative and Bactericidal Functions in Chronic Granulomatous Disease with 4‐Amino‐4′‐4 Hyndroxylaminodiphenyl Sulphone
TL;DR: In this paper, a dapsone derivative, 4-amino-4'-hydroxylaminodiphenyl sulphone (DDS-NOH), was used to restore oxidant-dependent capabilities to chronic granulomatous disease (CGD) polymorphonuclear leucocytes (PMN).
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Acid phosphatase cytochemistry of phagocytizing leukocytes from patients with chronic granulomatous disease.
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References
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Protein Measurement with the Folin Phenol Reagent
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In Vitro Bactericidal Capacity of Human Polymorphonuclear Leukocytes: Diminished Activity in Chronic Granulomatous Disease of Childhood
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Quantitative Nitroblue Tetrazolium Test in Chronic Granulomatous Disease
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Myeloperoxidase-Halide-Hydrogen Peroxide Antibacterial System
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