| Type: | Family | Name: | Neutrophil cytosol factor 1 |
| Description: | Phagocytes form the first line of defence against invasion by micro-organisms. Engulfing of bacteria by neutrophils is accompanied by the consumption of large amounts of oxygen - a so-called respiratory burst. Defects in phagocytosis involving the lack of a respiratory burst give rise to chronic granulomatous disease (CGD), in which patients are pre-disposed to infection, often from otherwise non-pathogenic bacteria [].Regulation of the respiratory burst takes place at the phagocytic vacuole. The process is mediated by NADPH oxidase, which transports electrons across the plasma membrane to form superoxide (an oxygen molecule with an extra electron that is toxic to normal cells) in the vacuole interior. The electrons are carried across the membrane by a short electron transport chain in the form of an unusual flavocytochrome b [].To conserve NADPH and avoid the toxic effects of superoxide, the oxidase remains inactive until it receives an appropriate stimulus. Activation involves the participation of a number of cytosolic proteins, some of which attach to the flavocytochrome. p47phox, p67phox and p40phox are specialised components of phagocytic cells, all of which contain SH3 domains, by means of which they attach to proline-rich regions of other proteins. p40phox and p47phox share an additionnal domain, PX (phox). Upon activation, p47phox and p67phox are phosphorylated and, with p40phox, translocate to the region of the plasma membrane forming the phagocytic vacuole, where they associates with hydrophilic regions of the flavocytochrome [].This entry represents p67phox, also known as neutrophil cytosol factor 1. | Short Name: | P47PHOX |
