Neutrophil cytosolic factor 2 explained

Neutrophil cytosol factor 2 is a protein that in humans is encoded by the NCF2 gene.

Function

This gene encodes neutrophil cytosolic factor 2, the 67-kilodalton cytosolic subunit of the multi-protein complex known as NADPH oxidase found in neutrophils. This oxidase produces a burst of superoxide which is delivered to the lumen of the neutrophil phagosome. Mutations in this gene, as well as in other NADPH oxidase subunits, can result in chronic granulomatous disease.^[1]

Further reading

• Wientjes FB, Segal AW . NADPH oxidase and the respiratory burst . Seminars in Cell Biology . 6 . 6 . 357–65 . December 1995 . 8748143 . 10.1016/S1043-4682(05)80006-6 .
• DeLeo FR, Quinn MT . Assembly of the phagocyte NADPH oxidase: molecular interaction of oxidase proteins . Journal of Leukocyte Biology . 60 . 6 . 677–91 . December 1996 . 8975869 . 10.1002/jlb.60.6.677. 17946755 .
• Dorseuil O, Gacon G . [Signal transduction by Rac small G proteins in phagocytes] . Comptes Rendus des Séances de la Société de Biologie et de ses Filiales . 191 . 2 . 237–46 . 1997 . 9255350 .
• Leto TL, Lomax KJ, Volpp BD, Nunoi H, Sechler JM, Nauseef WM, Clark RA, Gallin JI, Malech HL . Cloning of a 67-kD neutrophil oxidase factor with similarity to a noncatalytic region of p60c-src . Science . 248 . 4956 . 727–30 . May 1990 . 1692159 . 10.1126/science.1692159 . 1990Sci...248..727L .
• Kenney RT, Leto TL . A HindIII polymorphism in the human NCF2 gene . Nucleic Acids Research . 18 . 23 . 7193 . December 1990 . 1979859 . 332837 . 10.1093/nar/18.23.7193-a .
• Francke U, Hsieh CL, Foellmer BE, Lomax KJ, Malech HL, Leto TL . Genes for two autosomal recessive forms of chronic granulomatous disease assigned to 1q25 (NCF2) and 7q11.23 (NCF1) . American Journal of Human Genetics . 47 . 3 . 483–92 . September 1990 . 2393022 . 1683885 .
• Kenney RT, Malech HL, Epstein ND, Roberts RL, Leto TL . Characterization of the p67phox gene: genomic organization and restriction fragment length polymorphism analysis for prenatal diagnosis in chronic granulomatous disease . Blood . 82 . 12 . 3739–44 . December 1993 . 7903171 . 10.1182/blood.V82.12.3739.3739. free .
• Leto TL, Adams AG, de Mendez I . Assembly of the phagocyte NADPH oxidase: binding of Src homology 3 domains to proline-rich targets . Proceedings of the National Academy of Sciences of the United States of America . 91 . 22 . 10650–4 . October 1994 . 7938008 . 45079 . 10.1073/pnas.91.22.10650 . 1994PNAS...9110650L . free .
• Dusi S, Rossi F . Activation of NADPH oxidase of human neutrophils involves the phosphorylation and the translocation of cytosolic p67phox . The Biochemical Journal . 296 (Pt 2) . 2 . 367–71 . December 1993 . 8257426 . 1137705 . 10.1042/bj2960367.
• de Boer M, Hilarius-Stokman PM, Hossle JP, Verhoeven AJ, Graf N, Kenney RT, Seger R, Roos D . Autosomal recessive chronic granulomatous disease with absence of the 67-kD cytosolic NADPH oxidase component: identification of mutation and detection of carriers . Blood . 83 . 2 . 531–6 . January 1994 . 8286749 . 10.1182/blood.V83.2.531.531. free .
• Bonaldo MF, Lennon G, Soares MB . Normalization and subtraction: two approaches to facilitate gene discovery . Genome Research . 6 . 9 . 791–806 . September 1996 . 8889548 . 10.1101/gr.6.9.791 . free .
• Bonizzato A, Russo MP, Donini M, Dusi S . Identification of a double mutation (D160V-K161E) in the p67phox gene of a chronic granulomatous disease patient . Biochemical and Biophysical Research Communications . 231 . 3 . 861–3 . February 1997 . 9070911 . 10.1006/bbrc.1997.6204 .
• Sathyamoorthy M, de Mendez I, Adams AG, Leto TL . p40(phox) down-regulates NADPH oxidase activity through interactions with its SH3 domain . The Journal of Biological Chemistry . 272 . 14 . 9141–6 . April 1997 . 9083043 . 10.1074/jbc.272.14.9141 . free .
• Ahmed S, Prigmore E, Govind S, Veryard C, Kozma R, Wientjes FB, Segal AW, Lim L . Cryptic Rac-binding and p21(Cdc42Hs/Rac)-activated kinase phosphorylation sites of NADPH oxidase component p67(phox) . The Journal of Biological Chemistry . 273 . 25 . 15693–701 . June 1998 . 9624165 . 10.1074/jbc.273.25.15693 . free .
• Faris SL, Rinckel LA, Huang J, Hong YR, Kleinberg ME . Phagocyte NADPH oxidase p67-phox possesses a novel carboxylterminal binding site for the GTPases Rac2 and Cdc42 . Biochemical and Biophysical Research Communications . 247 . 2 . 271–6 . June 1998 . 9642115 . 10.1006/bbrc.1998.8775 .
• Rinckel LA, Faris SL, Hitt ND, Kleinberg ME . Rac1 disrupts p67phox/p40phox binding: a novel role for Rac in NADPH oxidase activation . Biochemical and Biophysical Research Communications . 263 . 1 . 118–22 . September 1999 . 10486263 . 10.1006/bbrc.1999.1334 .
• Patiño PJ, Rae J, Noack D, Erickson R, Ding J, de Olarte DG, Curnutte JT . Molecular characterization of autosomal recessive chronic granulomatous disease caused by a defect of the nicotinamide adenine dinucleotide phosphate (reduced form) oxidase component p67-phox . Blood . 94 . 7 . 2505–14 . October 1999 . 10498624 . 10.1182/blood.V94.7.2505.419k10_2505_2514.

Notes and References

1. Web site: Entrez Gene: NCF2 neutrophil cytosolic factor 2 (65kDa, chronic granulomatous disease, autosomal 2).