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2j60
From Proteopedia
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| - | [[Image:2j60.png|left|200px]] | ||
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{{STRUCTURE_2j60| PDB=2j60 | SCENE= }} | {{STRUCTURE_2j60| PDB=2j60 | SCENE= }} | ||
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===H-FICOLIN COMPLEXED TO D-FUCOSE=== | ===H-FICOLIN COMPLEXED TO D-FUCOSE=== | ||
| + | {{ABSTRACT_PUBMED_17215869}} | ||
| - | + | ==Disease== | |
| + | [[http://www.uniprot.org/uniprot/FCN3_HUMAN FCN3_HUMAN]] Defects in FCN3 are the cause of ficolin 3 deficiency (FCN3D) [MIM:[http://omim.org/entry/613860 613860]]. FCN3D is a disorder characterized by immunodeficiency, recurrent infections, brain abscesses and recurrent warts on the fingers. Affected individuals have normal levels of lymphocytes, normal T-cell responses, and normal antibodies, but a selective deficient antibody response to pneumococcal polysaccharide vaccine.<ref>PMID:19535802</ref> | ||
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| + | ==Function== | ||
| + | [[http://www.uniprot.org/uniprot/FCN3_HUMAN FCN3_HUMAN]] May function in innate immunity through activation of the lectin complement pathway. Calcium-dependent and GlcNAc-binding lectin. Has affinity with GalNAc, GlcNAc, D-fucose, as mono/oligosaccharide and lipopolysaccharides from S.typhimurium and S.minnesota.<ref>PMID:11907111</ref> | ||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:017215869</ref><references group="xtra"/> | + | <ref group="xtra">PMID:017215869</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Gaboriaud, C.]] | [[Category: Gaboriaud, C.]] | ||
Revision as of 03:38, 25 March 2013
Contents |
H-FICOLIN COMPLEXED TO D-FUCOSE
Template:ABSTRACT PUBMED 17215869
Disease
[FCN3_HUMAN] Defects in FCN3 are the cause of ficolin 3 deficiency (FCN3D) [MIM:613860]. FCN3D is a disorder characterized by immunodeficiency, recurrent infections, brain abscesses and recurrent warts on the fingers. Affected individuals have normal levels of lymphocytes, normal T-cell responses, and normal antibodies, but a selective deficient antibody response to pneumococcal polysaccharide vaccine.[1]
Function
[FCN3_HUMAN] May function in innate immunity through activation of the lectin complement pathway. Calcium-dependent and GlcNAc-binding lectin. Has affinity with GalNAc, GlcNAc, D-fucose, as mono/oligosaccharide and lipopolysaccharides from S.typhimurium and S.minnesota.[2]
About this Structure
2j60 is a 3 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
See Also
Reference
- Garlatti V, Belloy N, Martin L, Lacroix M, Matsushita M, Endo Y, Fujita T, Fontecilla-Camps JC, Arlaud GJ, Thielens NM, Gaboriaud C. Structural insights into the innate immune recognition specificities of L- and H-ficolins. EMBO J. 2007 Jan 24;26(2):623-33. Epub 2007 Jan 11. PMID:17215869
- ↑ Munthe-Fog L, Hummelshoj T, Honore C, Madsen HO, Permin H, Garred P. Immunodeficiency associated with FCN3 mutation and ficolin-3 deficiency. N Engl J Med. 2009 Jun 18;360(25):2637-44. doi: 10.1056/NEJMoa0900381. PMID:19535802 doi:10.1056/NEJMoa0900381
- ↑ Matsushita M, Kuraya M, Hamasaki N, Tsujimura M, Shiraki H, Fujita T. Activation of the lectin complement pathway by H-ficolin (Hakata antigen). J Immunol. 2002 Apr 1;168(7):3502-6. PMID:11907111
