NADPH oxidase deficiency in X-linked chronic granulomatous disease.

Höhn, D.; Lehrer, Robert I.

doi:10.1172/jci107980

Cited by 242 publications

(94 citation statements)

References 23 publications

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“…Previously, we and others have found that granule fractions isolated from phagocytizing cells had higher NADPH oxidase activity than granule fractions isolated from resting cells (1,3,4,13). This suggested that the oxidase could be the enzyme responsible for initiation of the respiratory burst.…”

Section: Resultsmentioning

confidence: 73%

“…A possible explanation for the difference is that Paul's group studied guinea pig cells, while our studies and those of Patriarca utilized human cells. This hypothesis is further weakened by the presence of normal NADPH oxidase activity in the PMNL of patients deficient in myeloperoxidase (3,18).…”

Section: Resultsmentioning

confidence: 99%

“…These events include increases in oxygen consumption, hexose monophosphate shunt activity, and the production of both hydrogen peroxide (3)(4)(5), consistent with the enzyme being the initiator. However, the use of Mn2' in the assay for the oxidase has been viewed somewhat skeptically.…”

Section: Introductionmentioning

confidence: 99%

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Further characterization of NADPH oxidase activity of human polymorphonuclear leukocytes.

McPhail¹,

DeChatelet²,

Shirley³

1976

J. Clin. Invest.

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A B S T R A C T Mn2+ was shown to catalyze a nonenzymatic oxidation of NADPH in the presence of superoxide anion by means of an isotopic assay for measurement of the oxidation of NADPH to NADP+. Human polymorphonuclear leukocyte granule NADPH oxidase activity was evaluated in the absence of Mn2" and was found to be higher in granules from phagocytizing cells than in granules from resting cells. The drug phorbol myristate acetate, which affects the oxidative metabolism of the neutrophil like phagocytosis, was found to activate granule NADPH oxidase activity. Superoxide dismutase was shown to inhibit NADPH oxidase activity both in the presence and absence of added Mn2'. The NADPH oxidase reaction in the absence of Mn2" was optimal at pH 5.5, and was more linear with increasing time and protein concentration than in the presence of Mn2+. No activity was measurable in granules isolated from resting cells until the level of NADPH added was above 0.25 mM. Activity was present in granules isolated from cells challenged with opsonized zymosan, even at 0.05 mM NADPH, and was higher than the activity found in granule fractions from resting cells at all levels of NADPH tested. The addition of as little as 0.1 /AM NADH to the reaction mixture was found to inhibit granular NADPH oxidase activity, indicating a possible regulatory role for NADH. These results suggest that NADPH oxidase may be the enzyme that initiates the metabolic events accompanying phagocytosis.

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Section: Resultsmentioning

confidence: 73%

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Further characterization of NADPH oxidase activity of human polymorphonuclear leukocytes.

McPhail¹,

DeChatelet²,

Shirley³

1976

J. Clin. Invest.

View full text Add to dashboard Cite

show abstract

“…Data obtained so far, however, have provided only equivocal support for this hypothesis. The proposal that the lesion in CGD is a defect in the soluble granulocyte NADH oxidase has been supported by studies from one laboratory (24) but not by studies from two other laboratories (19,25). Very recently a defect in the particulate NADPH oxidase of CGD granulocytes has been reported (25).…”

Section: Discussionmentioning

confidence: 99%

“…The proposal that the lesion in CGD is a defect in the soluble granulocyte NADH oxidase has been supported by studies from one laboratory (24) but not by studies from two other laboratories (19,25). Very recently a defect in the particulate NADPH oxidase of CGD granulocytes has been reported (25). In view of the rather inconclusive nature of the evidence concerning defects in oxygen-metabolizing enzymes, it has been suggested that the metabolic lesion in CGD may involve an enzyme system physiologically connected to, but not identical with, the oxygen-consuming enzymes directly responsible for the respiratory burst (1).…”

Section: Discussionmentioning

confidence: 99%