2dyb
From Proteopedia
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{{STRUCTURE_2dyb| PDB=2dyb | SCENE= }} | {{STRUCTURE_2dyb| PDB=2dyb | SCENE= }} | ||
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===The crystal structure of human p40(phox)=== | ===The crystal structure of human p40(phox)=== | ||
| + | {{ABSTRACT_PUBMED_17290225}} | ||
| - | + | ==Disease== | |
| + | [[http://www.uniprot.org/uniprot/NCF4_HUMAN NCF4_HUMAN]] Defects in NCF4 are the cause of chronic granulomatous disease autosomal recessive cytochrome-b-positive type 3 (CGD3) [MIM:[http://omim.org/entry/613960 613960]]. CGD3 is a disorder characterized by the inability of neutrophils and phagocytes to kill microbes that they have ingested. Patients suffer from life-threatening bacterial/fungal infections.<ref>PMID:19692703</ref> | ||
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| + | ==Function== | ||
| + | [[http://www.uniprot.org/uniprot/NCF4_HUMAN NCF4_HUMAN]] Component of the NADPH-oxidase, a multicomponent enzyme system responsible for the oxidative burst in which electrons are transported from NADPH to molecular oxygen, generating reactive oxidant intermediates. It may be important for the assembly and/or activation of the NADPH-oxidase complex. | ||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:017290225</ref><references group="xtra"/> | + | <ref group="xtra">PMID:017290225</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Honbou, K.]] | [[Category: Honbou, K.]] | ||
[[Category: Nadph oxidase]] | [[Category: Nadph oxidase]] | ||
[[Category: Oxidoreductase]] | [[Category: Oxidoreductase]] | ||
Revision as of 09:20, 24 March 2013
Contents |
The crystal structure of human p40(phox)
Template:ABSTRACT PUBMED 17290225
Disease
[NCF4_HUMAN] Defects in NCF4 are the cause of chronic granulomatous disease autosomal recessive cytochrome-b-positive type 3 (CGD3) [MIM:613960]. CGD3 is a disorder characterized by the inability of neutrophils and phagocytes to kill microbes that they have ingested. Patients suffer from life-threatening bacterial/fungal infections.[1]
Function
[NCF4_HUMAN] Component of the NADPH-oxidase, a multicomponent enzyme system responsible for the oxidative burst in which electrons are transported from NADPH to molecular oxygen, generating reactive oxidant intermediates. It may be important for the assembly and/or activation of the NADPH-oxidase complex.
About this Structure
2dyb is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Honbou K, Minakami R, Yuzawa S, Takeya R, Suzuki NN, Kamakura S, Sumimoto H, Inagaki F. Full-length p40phox structure suggests a basis for regulation mechanism of its membrane binding. EMBO J. 2007 Feb 21;26(4):1176-86. Epub 2007 Feb 8. PMID:17290225
- ↑ Matute JD, Arias AA, Wright NA, Wrobel I, Waterhouse CC, Li XJ, Marchal CC, Stull ND, Lewis DB, Steele M, Kellner JD, Yu W, Meroueh SO, Nauseef WM, Dinauer MC. A new genetic subgroup of chronic granulomatous disease with autosomal recessive mutations in p40 phox and selective defects in neutrophil NADPH oxidase activity. Blood. 2009 Oct 8;114(15):3309-15. doi: 10.1182/blood-2009-07-231498. Epub 2009, Aug 19. PMID:19692703 doi:10.1182/blood-2009-07-231498
