p47phox (neutrophil cytosol factor-1, NCF-1, NCF-47K, 47 kD autosomal chronic granulomatous disease protein, Nox-organizing protein 2, SH3 & PX domain-containing protein 1A, NCF1, NOXO2, SH3PXD1A)
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Function
- NCF2, NCF1, & a membrane bound cytochrome b558 are required for activation of the latent NADPH oxidase (necessary for superoxide production)
- upon activation, SH3 domain(s) bind(s) to proline-rich region of membrane-bound p22phox
Structure
- contains 1 PX (phox homology) domain
- contains 2 SH3 domains
Compartment
- cytoplasm
- translocates to plasma membrane & endosomal membrane upon phosphorylation &/or leukocyte activation
Pathology
- defects in NCF1 are the cause of autosomal cytochrome-b- positive chronic granulomatous disease type 1
More general terms
Additional terms
Component of
References
- ↑ Rotrosen D, Yeung CL, Leto TL, Malech HL, Kwong CH. Cytochrome b558: the flavin-binding component of the phagocyte NADPH oxidase. Science. 1992 Jun 5;256(5062):1459-62. PMID: https://www.ncbi.nlm.nih.gov/pubmed/1318579
- ↑ Segal AW, Abo A. The biochemical basis of the NADPH oxidase of phagocytes. Trends Biochem Sci. 1993 Feb;18(2):43-7. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/8488557
- ↑ Pawson T Protein modules and signalling networks Nature 373:573 1995 PMID: https://www.ncbi.nlm.nih.gov/pubmed/7531822
- ↑ NCF1base; NCF1 deficiency database http://bioinf.uta.fi/NCF1base/
- ↑ GeneReviews https://www.genecards.org/cgi-bin/carddisp.pl?gene=NCF1
- ↑ UniProt http://www.uniprot.org/uniprot/P14598.html