Genetic Defects of Phagocyte Nadph Oxidase Activity and Activation

Abstract

NADPH oxidase is the key enzyme of the free radical-generating oxidative matabolism of phagocytes. Work from our and other's laboratories has recently established that the oxidase is not a single molecular entity, but it is a multicomponent system including a NADPH-binding protein, a flavoprotein, a b-type cytochrome and other unidentified factors. A working model of the molecular nature and of the activation mechanism of phagocyte NADPH oxidase is here proposed. This model is suitable for the study and the classification of the molecular pathology of the oxidase system. The various genetic defects of the NADPH oxidase, that are the cause of chronic granulomatous disease, (CGD) are here presented and discussed.