background:
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 SLCterminal 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.
Function:
NCF2, NCF1, and a membrane bound cytochrome b558 are required for activation of the latent NADPH oxidase (necessary for superoxide production).
Subunit:
Interacts with NOXA1. Interacts with ADAM15. Interacts with TRAF4. Interacts with FASLG.
Subcellular Location:
Cytoplasm.
Post-translational modifications:
Phosphorylated by PRKCD; phosphorylation induces activation of NCF1 and NADPH oxidase activity.
DISEASE:
Defects in NCF1 are the cause of chronic granulomatous disease autosomal recessive cytochrome-b-positive type 1 (CGD1) [MIM:233700]. Chronic granulomatous disease is a genetically heterogeneous disorder characterized by the inability of neutrophils and phagocytes to kill microbes that they have ingested. Patients suffer from life-threatening bacterial/fungal infections.
Similarity:
Contains 1 PX (phox homology) domain.
Contains 2 SH3 domains.
Database links:
Entrez Gene: 281345 Cow
Entrez Gene: 653361 Human
Entrez Gene: 17969 Mouse
Entrez Gene: 100134857 Pig
Entrez Gene: 100001763 Rabbit
Entrez Gene: 114553 Rat
Omim: 608512 Human
SwissProt: O77774 Cow
SwissProt: P14598 Human
SwissProt: Q09014 Mouse
Unigene: 647047 Human
Unigene: 655201 Human
Unigene: 425296 Mouse
Unigene: 38575 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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