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3tvj
From Proteopedia
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| - | [[Image:3tvj.png|left|200px]] | ||
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{{STRUCTURE_3tvj| PDB=3tvj | SCENE= }} | {{STRUCTURE_3tvj| PDB=3tvj | SCENE= }} | ||
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===Catalytic fragment of MASP-2 in complex with its specific inhibitor developed by directed evolution on SGCI scaffold=== | ===Catalytic fragment of MASP-2 in complex with its specific inhibitor developed by directed evolution on SGCI scaffold=== | ||
| + | {{ABSTRACT_PUBMED_22511776}} | ||
| - | + | ==Disease== | |
| + | [[http://www.uniprot.org/uniprot/MASP2_HUMAN MASP2_HUMAN]] Defects in MASP2 are the cause of MASP2 deficiency (MASPD) [MIM:[http://omim.org/entry/613791 613791]]. MASPD is a disorder that results in autoimmune manifestations, recurrent severe infections, and chronic inflammatory disease.<ref>PMID:12904520</ref><ref>PMID:17252003</ref> | ||
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| + | ==Function== | ||
| + | [[http://www.uniprot.org/uniprot/MASP2_HUMAN MASP2_HUMAN]] Serum protease that plays an important role in the activation of the complement system via mannose-binding lectin. After activation by auto-catalytic cleavage it cleaves C2 and C4, leading to their activation and to the formation of C3 convertase.<ref>PMID:10946292</ref> | ||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
| - | <ref group="xtra">PMID:022511776</ref><references group="xtra"/> | + | <ref group="xtra">PMID:022511776</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Mannan-binding lectin-associated serine protease-2]] | [[Category: Mannan-binding lectin-associated serine protease-2]] | ||
Revision as of 06:23, 25 March 2013
Contents |
Catalytic fragment of MASP-2 in complex with its specific inhibitor developed by directed evolution on SGCI scaffold
Template:ABSTRACT PUBMED 22511776
Disease
[MASP2_HUMAN] Defects in MASP2 are the cause of MASP2 deficiency (MASPD) [MIM:613791]. MASPD is a disorder that results in autoimmune manifestations, recurrent severe infections, and chronic inflammatory disease.[1][2]
Function
[MASP2_HUMAN] Serum protease that plays an important role in the activation of the complement system via mannose-binding lectin. After activation by auto-catalytic cleavage it cleaves C2 and C4, leading to their activation and to the formation of C3 convertase.[3]
About this Structure
3tvj is a 3 chain structure with sequence from Homo sapiens and Schistocerca gregaria. Full crystallographic information is available from OCA.
Reference
- Heja D, Harmat V, Fodor K, Wilmanns M, Dobo J, Kekesi KA, Zavodszky P, Gal P, Pal G. Monospecific inhibitors show that both mannan-binding lectin-associated serine protease (MASP)-1 and -2 are essential for lectin pathway activation and reveal structural plasticity of MASP-2. J Biol Chem. 2012 Apr 16. PMID:22511776 doi:10.1074/jbc.M112.354332
- ↑ Stengaard-Pedersen K, Thiel S, Gadjeva M, Moller-Kristensen M, Sorensen R, Jensen LT, Sjoholm AG, Fugger L, Jensenius JC. Inherited deficiency of mannan-binding lectin-associated serine protease 2. N Engl J Med. 2003 Aug 7;349(6):554-60. PMID:12904520 doi:http://dx.doi.org/10.1056/NEJMoa022836
- ↑ Thiel S, Steffensen R, Christensen IJ, Ip WK, Lau YL, Reason IJ, Eiberg H, Gadjeva M, Ruseva M, Jensenius JC. Deficiency of mannan-binding lectin associated serine protease-2 due to missense polymorphisms. Genes Immun. 2007 Mar;8(2):154-63. Epub 2007 Jan 25. PMID:17252003 doi:10.1038/sj.gene.6364373
- ↑ Matsushita M, Thiel S, Jensenius JC, Terai I, Fujita T. Proteolytic activities of two types of mannose-binding lectin-associated serine protease. J Immunol. 2000 Sep 1;165(5):2637-42. PMID:10946292
