1gd5

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[[Image:1gd5.png|left|200px]]
 
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{{STRUCTURE_1gd5| PDB=1gd5 | SCENE= }}
{{STRUCTURE_1gd5| PDB=1gd5 | SCENE= }}
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===SOLUTION STRUCTURE OF THE PX DOMAIN FROM HUMAN P47PHOX NADPH OXIDASE===
===SOLUTION STRUCTURE OF THE PX DOMAIN FROM HUMAN P47PHOX NADPH OXIDASE===
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{{ABSTRACT_PUBMED_11373621}}
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{{ABSTRACT_PUBMED_11373621}}
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==Disease==
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[[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==
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[[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==
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<ref group="xtra">PMID:011373621</ref><references group="xtra"/>
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<ref group="xtra">PMID:011373621</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Ago, T.]]
[[Category: Ago, T.]]

Revision as of 09:29, 24 March 2013

Template:STRUCTURE 1gd5

Contents

SOLUTION STRUCTURE OF THE PX DOMAIN FROM HUMAN P47PHOX NADPH OXIDASE

Template:ABSTRACT PUBMED 11373621

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

1gd5 is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA.

Reference

  • Hiroaki H, Ago T, Ito T, Sumimoto H, Kohda D. Solution structure of the PX domain, a target of the SH3 domain. Nat Struct Biol. 2001 Jun;8(6):526-30. PMID:11373621 doi:10.1038/88591
  1. ↑ 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
  2. ↑ 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
  3. ↑ 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

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