This old version of Proteopedia is provided for student assignments while the new version is undergoing repairs. Content and edits done in this old version of Proteopedia after March 1, 2026 will eventually be lost when it is retired in about June of 2026.
Apply for new accounts at the new Proteopedia. Your logins will work in both the old and new versions.
1uec
From Proteopedia
m (Protected "1uec" [edit=sysop:move=sysop]) |
|||
| Line 1: | Line 1: | ||
| - | [[Image:1uec.png|left|200px]] | ||
| - | |||
{{STRUCTURE_1uec| PDB=1uec | SCENE= }} | {{STRUCTURE_1uec| PDB=1uec | SCENE= }} | ||
| - | |||
===Crystal structure of autoinhibited form of tandem SH3 domain of p47phox=== | ===Crystal structure of autoinhibited form of tandem SH3 domain of p47phox=== | ||
| + | {{ABSTRACT_PUBMED_15147273}} | ||
| - | + | ==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> | ||
| + | |||
| + | ==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== | ||
| Line 11: | Line 13: | ||
==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:015147273</ref><references group="xtra"/> | + | <ref group="xtra">PMID:015147273</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Fujioka, Y.]] | [[Category: Fujioka, Y.]] | ||
Revision as of 01:47, 25 March 2013
Contents |
Crystal structure of autoinhibited form of tandem SH3 domain of p47phox
Template:ABSTRACT PUBMED 15147273
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
1uec is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Yuzawa S, Suzuki NN, Fujioka Y, Ogura K, Sumimoto H, Inagaki F. A molecular mechanism for autoinhibition of the tandem SH3 domains of p47phox, the regulatory subunit of the phagocyte NADPH oxidase. Genes Cells. 2004 May;9(5):443-56. PMID:15147273 doi:10.1111/j.1356-9597.2004.00733.x
- ↑ 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
