1w70
From Proteopedia
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{{STRUCTURE_1w70| PDB=1w70 | SCENE= }} | {{STRUCTURE_1w70| PDB=1w70 | SCENE= }} | ||
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===SH3 DOMAIN OF P40PHOX COMPLEXED WITH C-TERMINAL POLYPROLINE REGION OF P47PHOX=== | ===SH3 DOMAIN OF P40PHOX COMPLEXED WITH C-TERMINAL POLYPROLINE REGION OF P47PHOX=== | ||
| + | {{ABSTRACT_PUBMED_15657040}} | ||
| - | + | ==Disease== | |
| + | [[http://www.uniprot.org/uniprot/NCF1_HUMAN NCF1_HUMAN]] Defects in NCF1 are the cause of chronic granulomatous disease autosomal recessive cytochrome-b-positive type 1 (CGD1) [MIM:[http://omim.org/entry/233700 233700]]. Chronic granulomatous disease is a genetically heterogeneous 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:2011585</ref><ref>PMID:11133775</ref> | ||
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| + | ==Function== | ||
| + | [[http://www.uniprot.org/uniprot/NCF1_HUMAN NCF1_HUMAN]] NCF2, NCF1, and a membrane bound cytochrome b558 are required for activation of the latent NADPH oxidase (necessary for superoxide production).<ref>PMID:19801500</ref> | ||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:015657040</ref><references group="xtra"/> | + | <ref group="xtra">PMID:015657040</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Brandolin, G.]] | [[Category: Brandolin, G.]] | ||
Revision as of 03:29, 25 March 2013
Contents |
SH3 DOMAIN OF P40PHOX COMPLEXED WITH C-TERMINAL POLYPROLINE REGION OF P47PHOX
Template:ABSTRACT PUBMED 15657040
Disease
[NCF1_HUMAN] Defects in NCF1 are the cause of chronic granulomatous disease autosomal recessive cytochrome-b-positive type 1 (CGD1) [MIM:233700]. Chronic granulomatous disease is a genetically heterogeneous 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][2]
Function
[NCF1_HUMAN] NCF2, NCF1, and a membrane bound cytochrome b558 are required for activation of the latent NADPH oxidase (necessary for superoxide production).[3]
About this Structure
1w70 is a 4 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Massenet C, Chenavas S, Cohen-Addad C, Dagher MC, Brandolin G, Pebay-Peyroula E, Fieschi F. Effects of p47phox C terminus phosphorylations on binding interactions with p40phox and p67phox. Structural and functional comparison of p40phox and p67phox SH3 domains. J Biol Chem. 2005 Apr 8;280(14):13752-61. Epub 2005 Jan 18. PMID:15657040 doi:10.1074/jbc.M412897200
- ↑ Casimir CM, Bu-Ghanim HN, Rodaway AR, Bentley DL, Rowe P, Segal AW. Autosomal recessive chronic granulomatous disease caused by deletion at a dinucleotide repeat. Proc Natl Acad Sci U S A. 1991 Apr 1;88(7):2753-7. PMID:2011585
- ↑ Noack D, Rae J, Cross AR, Ellis BA, Newburger PE, Curnutte JT, Heyworth PG. Autosomal recessive chronic granulomatous disease caused by defects in NCF-1, the gene encoding the phagocyte p47-phox: mutations not arising in the NCF-1 pseudogenes. Blood. 2001 Jan 1;97(1):305-11. PMID:11133775
- ↑ Kilpatrick LE, Sun S, Li H, Vary TC, Korchak HM. Regulation of TNF-induced oxygen radical production in human neutrophils: role of delta-PKC. J Leukoc Biol. 2010 Jan;87(1):153-64. doi: 10.1189/jlb.0408230. Epub 2009 Oct 2. PMID:19801500 doi:10.1189/jlb.0408230
