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==Crystal structure of the catalytic region of human MASP-1==
==Crystal structure of the catalytic region of human MASP-1==
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<StructureSection load='3gov' size='340' side='right' caption='[[3gov]], [[Resolution|resolution]] 2.55&Aring;' scene=''>
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<StructureSection load='3gov' size='340' side='right'caption='[[3gov]], [[Resolution|resolution]] 2.55&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[3gov]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3GOV OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3GOV FirstGlance]. <br>
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<table><tr><td colspan='2'>[[3gov]] 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=3GOV OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3GOV FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.55&#8491;</td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">CRARF, CRARF1, MASP1, PRSS5 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3gov FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3gov OCA], [http://pdbe.org/3gov PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=3gov RCSB], [http://www.ebi.ac.uk/pdbsum/3gov PDBsum]</span></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3gov FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3gov OCA], [https://pdbe.org/3gov PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3gov RCSB], [https://www.ebi.ac.uk/pdbsum/3gov PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3gov ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/MASP1_HUMAN MASP1_HUMAN]] Defects in MASP1 are the cause of 3MC syndrome type 1 (3MC1) [MIM:[http://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>
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[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 ==
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[[http://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>
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[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>
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
Check<jmol>
Check<jmol>
<jmolCheckbox>
<jmolCheckbox>
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<scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/go/3gov_consurf.spt"</scriptWhenChecked>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/go/3gov_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview03.spt</scriptWhenUnchecked>
<text>to colour the structure by Evolutionary Conservation</text>
<text>to colour the structure by Evolutionary Conservation</text>
</jmolCheckbox>
</jmolCheckbox>
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</div>
</div>
<div class="pdbe-citations 3gov" style="background-color:#fffaf0;"></div>
<div class="pdbe-citations 3gov" style="background-color:#fffaf0;"></div>
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==See Also==
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*[[Mannan-binding lectin serine protease|Mannan-binding lectin serine protease]]
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Human]]
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[[Category: Homo sapiens]]
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[[Category: Beinrohr, L]]
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[[Category: Large Structures]]
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[[Category: Dobo, J]]
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[[Category: Beinrohr L]]
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[[Category: Gal, P]]
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[[Category: Dobo J]]
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[[Category: Harmat, V]]
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[[Category: Gal P]]
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[[Category: Sebestyen, E]]
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[[Category: Harmat V]]
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[[Category: Zavodszky, P]]
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[[Category: Sebestyen E]]
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[[Category: Beta barrel]]
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[[Category: Zavodszky P]]
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[[Category: Coagulation]]
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[[Category: Complement]]
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[[Category: Complement pathway]]
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[[Category: Disulfide bond]]
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[[Category: Egf-like domain]]
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[[Category: Glycoprotein]]
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[[Category: Hydrolase]]
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[[Category: Hydroxylation]]
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[[Category: Immune response]]
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[[Category: Innate immunity]]
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[[Category: Protease]]
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[[Category: Serine protease]]
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[[Category: Sushi]]
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Current revision

Crystal structure of the catalytic region of human MASP-1

PDB ID 3gov

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