The heredity chronic granulomatous disease (CGF) has been linked to mutations in p47-phox and p67-phox. The cytosolic proteins p47-phox and p67-phox, also designated neutrophil cytosol factor (NCF)1 and NCF2, respectively, are required for activation of the superoxide-producing NADPH oxidase in neutrophils and other phagocytic cells. During activation of the NADPH oxidase, p47-phox and p67-phox migrate to the plasma membrane where they associate with cytochrome b558 and the small G protein Rac to form the functional enzyme complex. Both p47-phox and p67-phox contain two Src homology 3 (SH3) domains. The C-terminal SH3 domain of p67-phox has been shown to interact with the proline-rich domain of p47-phox, suggesting that p47-phox may faciliate the transport of p67-phox to the membrane.
