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The respiratory burst oxidase.

¹Division of Biochemistry, Research Institute of Scripps Clinic, La Jolla, California.

Hematology/Oncology Clinics of North America

|June 1, 1988

Summary

The respiratory burst oxidase produces superoxide in phagocytes. Defects in this enzyme cause chronic granulomatous disease, impairing the immune response.

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Area of Science:

Biochemistry
Immunology
Cell Biology

Background:

The respiratory burst oxidase is a critical enzyme in phagocytes.
It catalyzes the production of superoxide (O2-) from oxygen.
This process is essential for the innate immune response.

Purpose of the Study:

To elucidate the function of the respiratory burst oxidase.
To understand the biochemical basis of chronic granulomatous disease (CGD).
To identify the molecular defects leading to impaired superoxide production in CGD.

Main Methods:

Characterization of the membrane-bound flavo(hemo)protein.
Analysis of NADPH-dependent oxygen reduction.
Investigation of the oxidase activating system.

Main Results:

The respiratory burst oxidase was confirmed as an activatable, membrane-bound enzyme.
Its role in catalyzing NADPH-dependent reduction of oxygen to superoxide was established.
Biochemical lesions involving the oxidase or its system were identified as the cause of CGD.

Conclusions:

The respiratory burst oxidase is central to phagocyte function.
Deficiencies in this oxidase lead to chronic granulomatous disease.
Understanding these mechanisms is crucial for diagnosing and potentially treating CGD.

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