High frequency in the delay in primary tooth loss in X-linked chronic granulomatous disease

摘要

Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by mutations that render the NADPH oxidase complex incapable of producing the super-oxide anions that are necessary to kill bacterial and fungal microorganisms. Mutations in at least 5 different genes involved in the NADPH oxidase complex can provoke CGD. The CYBB gene on the X-chromosome that encodes the enzymatic centre of this oxidase (gp91phox) accounts for about two thirds of the cases [1]. NADPH oxidase complex catalytic component, responsible for the NADPH-driven reduction of oxygen to O_2~-, is flavocytochrome b_(559), located in the membrane and consisting of gp91phox and p22phox subunits [2].