Correction of metabolic deficiencies in the leukocytes of patients with chronic granulomatous disease
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
Citations
More filters
Journal ArticleDOI
Defective polymorphonuclear leukocyte metabolism and function in canine cyclic neutropenia.
TL;DR: In this paper, a study of grey collie dogs with cyclic neutropenia was conducted and the results showed that the leukocyte cells had significant metabolic and functional abnormalities, such as abnormally increased postphagocytic C1-glucose oxidation, decreased iodination of trichloroacetic acid-precipitable protein in the resting and phagocytizing state, decreased levels of intracellular myeloperoxidase, and a bactericidal defect against a variety of bacteria.
Journal ArticleDOI
O2- and host defense: the production and fate of O2- in neutrophils.
TL;DR: O2‐ serves as a precursor of OH, another powerful oxidant which may be employed by the neutrophil as an antimicrobial agent, and Singlet oxygen may also be formed in neutrophils.
Generation of Chemiluminescence by a Particulate Fraction Isolated from Human Neutrophils
TL;DR: Results suggest that the energy responsible for the Luminescence of particulate fractions and, presumably, the intact cell, is derived from more than one oxygen species and that luminescence is a product of the interaction of these species and excitable substrates within the cell.
Journal ArticleDOI
Utilization of liposomes for correction of the metabolic and bactericidal deficiencies in chronic granulomatous disease.
TL;DR: It is possible to restore the oxidative metabolic capacities and to obtain moderate improvement in the bactericidal functions in vitro of CGD leukocytes by providing the cells ingestible liposomes containing GO, as well as employing drugs that generate H2O2.
Journal ArticleDOI
Phagocytosis: The normal process and its clinically significant abnormalities.
TL;DR: Phagocytosis, one of the many mechanisms available to the host in its struggle against invading microorganisms, plays an important role in the critical early stages of bacterial infections and is thus a significant determinant of the eventual outcome of these infections.
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
The biochemical basis of phagocytosis. I. Metabolic changes during the ingestion of particles by polymorphonuclear leukocytes.
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.