scispace - formally typeset
Open AccessJournal ArticleDOI

Correction of metabolic deficiencies in the leukocytes of patients with chronic granulomatous disease

Robert L. Baehner, +2 more
- 01 May 1970 - 
- Vol. 49, Iss: 5, pp 865-870
Reads0
Chats0
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

Content maybe subject to copyright    Report

Citations
More filters
Journal Article

Cell biology of leukocyte abnormalities--membrane and cytoskeletal function in normal and defective cells. A review.

TL;DR: A series of diseases in which abnormalities of chemotaxis, phagocytosis, lysosomal degranulation, and/or oxidant generation can be explained directly or indirectly by abnormalities in dynamic properties of PMN membranes, microtubules, or microfilaments are selected.
Journal ArticleDOI

Oxidant injury of caucasian glucose-6-phosphate dehydrogenase—deficient red blood cells by phagocytosing leukocytes during infection

TL;DR: Phagocytizing polymorphonuclear leukocytes (PMN) can pose as a source of oxidant damage to G6PD-deficient RBC due to hydrogen peroxide generated during phagocyTosis.
Journal ArticleDOI

Extracellular and bacterial factors influencing staphylococcal phagocytosis and killing by human polymorphonuclear leukocytes.

TL;DR: In normal pooled serum, heat-labile factors were more important for effective phagocytosis than they were in immune serum, and although a saturation point for ingestion was reached, the percentage of ingested bacteria that remained alive within the leukocyte remained relatively fixed.
Journal ArticleDOI

The expanding network of redox signaling: new observations, complexities, and perspectives

TL;DR: In this corrigendum, the study of the transcription factor hypoxia-inducible factor 1 (HIF-1) has demonstrated its central role in regulating the body’s response to changing oxygen levels, and the list of nonstandard abbreviations used.
Journal ArticleDOI

Hydrogen peroxide production in chronic granulomatous disease. A cytochemical study of reduced pyridine nucleotide oxidases.

TL;DR: The ability of polymorphonuclear leukocytes (PMN) in response to phagocytic stimulation was examined cytochemically usingLeukocytes from normal individuals and patients with chronic granulomatous disease (CGD), finding that in CGD patients there are fewer cells capable of peroxide generation, and a majority of these reactive cells produce only reduced amounts of this bactericidal agent.
References
More filters
Journal Article

Protein Measurement with the Folin Phenol Reagent

TL;DR: Procedures are described for measuring protein in solution or after precipitation with acids or other agents, and for the determination of as little as 0.2 gamma of protein.
Journal ArticleDOI

In Vitro Bactericidal Capacity of Human Polymorphonuclear Leukocytes: Diminished Activity in Chronic Granulomatous Disease of Childhood

TL;DR: The deficiency of bactericidal capacity and the minimal degranulation after active phagocytosis by the PMN of these children with an inherited syndrome suggest that separate metabolic processes are involved in phagocytetosis and in intracellular digestion.
Journal ArticleDOI

Quantitative Nitroblue Tetrazolium Test in Chronic Granulomatous Disease

TL;DR: Chronic granulomatous disease is an X-linked defect in the killing of certain bacteria by peripheral blood granulocytes and may be detected with the nitroblue tetrazolium (NBT) test.
Journal ArticleDOI

Myeloperoxidase-Halide-Hydrogen Peroxide Antibacterial System

TL;DR: Myeloperoxidase, at high concentrations, exerted an antibacterial effect on L. acidophilus in the absence of added halide, which also was temperature- and catalase-sensitive, and suggests that, under the conditions employed, the antibacterial properties of a weak acid extract of guinea pig leukocytes is due, in part, to its peroxid enzyme content, particularly if a halide is present in the reaction mixture.
Related Papers (5)