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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)
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
Interactions
molecular events
Related
chronic granulomatous disease
NCF1 gene mutation
General
phosphoprotein
regulatory molecule
Properties
SIZE: entity length = 390 aa
MW = 45 kD
COMPARTMENT: cytoplasm
CELL: neutrophil
T-cell
B lymphocyte
MOTIF: PX domain {4-125}
MOTIF: SH3-binding site
NAME: SH3-binding site
src homology 3 [SH3] domain
SITE: 156-215
acidic region {211-254}
MOTIF: acidic residue (SEVERAL)
src homology 3 [SH3] domain
SITE: 226-285
lysine-rich region {292-390}
MOTIF: lysine residue (SEVERAL)
Ser phosphorylation site {S303}
Ser phosphorylation site {S304}
Ser phosphorylation site {S320}
Ser phosphorylation site {S328}
Ser phosphorylation site {S345}
Ser phosphorylation site {S348}
Database Correlations
OMIM correlations
MORBIDMAP 608512
UniProt P14598
PFAM correlations
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: 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: 8488557
- Pawson T
Protein modules and signalling networks
Nature 373:573 1995
PMID: 7531822
- NCF1base; NCF1 deficiency database
http://bioinf.uta.fi/NCF1base/
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=NCF1
- UniProt :accession P14598
Component-of
molecular complex