ABSTRACT

This enzyme generates, together with other components such as cytochrome b558 and several GTP-binding proteins, reactive

oxygen metabolites

. This multicomponent enzyme forms an electron-transport chain, using NADPH as a donor to reduce oxygen to

superoxide anion

(O

), which is then dismuted to

hydrogen peroxide

(H

O

), and thereafter metabolized to hydroxyl radicals (OH

). Defective cytochrome b558 results in a disease with severe infections (X-linked chronic granulomatous disease).