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==The X-ray crystal structure of Mannose-binding lectin-associated serine proteinase-3 reveals the structural basis for enzyme inactivity associated with the 3MC syndrome== | ==The X-ray crystal structure of Mannose-binding lectin-associated serine proteinase-3 reveals the structural basis for enzyme inactivity associated with the 3MC syndrome== | ||
<StructureSection load='4kkd' size='340' side='right' caption='[[4kkd]], [[Resolution|resolution]] 2.60Å' scene=''> | <StructureSection load='4kkd' size='340' side='right'caption='[[4kkd]], [[Resolution|resolution]] 2.60Å' scene=''> | ||
== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[4kkd]] is a 2 chain structure with sequence from [ | <table><tr><td colspan='2'>[[4kkd]] is a 2 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4KKD OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4KKD FirstGlance]. <br> | ||
</td></tr><tr id=' | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.5991Å</td></tr> | ||
<tr id=' | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=IMD:IMIDAZOLE'>IMD</scene></td></tr> | ||
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[ | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4kkd FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4kkd OCA], [https://pdbe.org/4kkd PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4kkd RCSB], [https://www.ebi.ac.uk/pdbsum/4kkd PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4kkd ProSAT]</span></td></tr> | ||
</table> | </table> | ||
== Disease == | == Disease == | ||
[ | [https://www.uniprot.org/uniprot/MASP1_HUMAN MASP1_HUMAN] Defects in MASP1 are the cause of 3MC syndrome type 1 (3MC1) [MIM:[https://omim.org/entry/257920 257920]. 3MC1 is a disorder characterized by facial dysmorphism that includes hypertelorism, blepharophimosis, blepharoptosis and highly arched eyebrows, cleft lip and/or palate, craniosynostosis, learning disability and genital, limb and vesicorenal anomalies. The term 3MC syndrome includes Carnevale, Mingarelli, Malpuech, and Michels syndromes.<ref>PMID:21258343</ref> | ||
== Function == | == Function == | ||
[ | [https://www.uniprot.org/uniprot/MASP1_HUMAN MASP1_HUMAN] Functions in the lectin pathway of complement, which performs a key role in innate immunity by recognizing pathogens through patterns of sugar moieties and neutralizing them. The lectin pathway is triggered upon binding of mannan-binding lectin (MBL) and ficolins to sugar moieties which leads to activation of the associated proteases MASP1 and MASP2. Functions as an endopeptidase and may activate MASP2 or C2 or directly activate C3 the key component of complement reaction. Isoform 2 may have an inhibitory effect on the activation of the lectin pathway of complement or may cleave IGFBP5.<ref>PMID:11485744</ref> <ref>PMID:17182967</ref> | ||
<div style="background-color:#fffaf0;"> | <div style="background-color:#fffaf0;"> | ||
== Publication Abstract from PubMed == | == Publication Abstract from PubMed == | ||
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</div> | </div> | ||
<div class="pdbe-citations 4kkd" style="background-color:#fffaf0;"></div> | <div class="pdbe-citations 4kkd" style="background-color:#fffaf0;"></div> | ||
==See Also== | |||
*[[Mannan-binding lectin serine protease|Mannan-binding lectin serine protease]] | |||
== References == | == References == | ||
<references/> | <references/> | ||
__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
[[Category: | [[Category: Homo sapiens]] | ||
[[Category: Coetzer | [[Category: Large Structures]] | ||
[[Category: Pike | [[Category: Coetzer TH]] | ||
[[Category: Reeve | [[Category: Pike RN]] | ||
[[Category: Smith | [[Category: Reeve SB]] | ||
[[Category: Whisstock | [[Category: Smith AI]] | ||
[[Category: Wijeyewickrema | [[Category: Whisstock JC]] | ||
[[Category: Wilmann | [[Category: Wijeyewickrema LC]] | ||
[[Category: Yongqing | [[Category: Wilmann PG]] | ||
[[Category: Yongqing T]] | |||