3a4u: Difference between revisions
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==Crystal structure of MCFD2 in complex with carbohydrate recognition domain of ERGIC-53== | ==Crystal structure of MCFD2 in complex with carbohydrate recognition domain of ERGIC-53== | ||
<StructureSection load='3a4u' size='340' side='right' caption='[[3a4u]], [[Resolution|resolution]] 1.84Å' scene=''> | <StructureSection load='3a4u' size='340' side='right'caption='[[3a4u]], [[Resolution|resolution]] 1.84Å' scene=''> | ||
== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[3a4u]] is a 2 chain structure with sequence from [ | <table><tr><td colspan='2'>[[3a4u]] 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=3A4U OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3A4U 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]] 1.84Å</td></tr> | ||
<tr id=' | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</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=3a4u FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3a4u OCA], [https://pdbe.org/3a4u PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3a4u RCSB], [https://www.ebi.ac.uk/pdbsum/3a4u PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3a4u ProSAT]</span></td></tr> | ||
</table> | </table> | ||
== Disease == | == Disease == | ||
[ | [https://www.uniprot.org/uniprot/LMAN1_HUMAN LMAN1_HUMAN] Defects in LMAN1 are THE cause of factor V and factor VIII combined deficiency type 1 (F5F8D1) [MIM:[https://omim.org/entry/227300 227300]; also known as multiple coagulation factor deficiency I (MCFD1). F5F8D1 is an autosomal recessive blood coagulation disorder characterized by bleeding symptoms similar to those in hemophilia or parahemophilia, that are caused by single deficiency of FV or FVIII, respectively. The most common symptoms are epistaxis, menorrhagia, and excessive bleeding during or after trauma. Plasma levels of coagulation factors V and VIII are in the range of 5 to 30% of normal.<ref>PMID:10090935</ref> | ||
== Function == | == Function == | ||
[ | [https://www.uniprot.org/uniprot/LMAN1_HUMAN LMAN1_HUMAN] Mannose-specific lectin. May recognize sugar residues of glycoproteins, glycolipids, or glycosylphosphatidyl inositol anchors and may be involved in the sorting or recycling of proteins, lipids, or both. The LMAN1-MCFD2 complex forms a specific cargo receptor for the ER-to-Golgi transport of selected proteins.<ref>PMID:13130098</ref> <ref>PMID:12717434</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> | ||
<scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/a4/3a4u_consurf.spt"</scriptWhenChecked> | <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/a4/3a4u_consurf.spt"</scriptWhenChecked> | ||
<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/ | <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 3a4u" style="background-color:#fffaf0;"></div> | <div class="pdbe-citations 3a4u" style="background-color:#fffaf0;"></div> | ||
==See Also== | |||
*[[ERGIC-53|ERGIC-53]] | |||
== References == | == References == | ||
<references/> | <references/> | ||
__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
[[Category: | [[Category: Homo sapiens]] | ||
[[Category: Fukui | [[Category: Large Structures]] | ||
[[Category: Hauri | [[Category: Fukui K]] | ||
[[Category: Kamiya | [[Category: Hauri HP]] | ||
[[Category: Kato | [[Category: Kamiya Y]] | ||
[[Category: McKay | [[Category: Kato K]] | ||
[[Category: Mizushima | [[Category: McKay AR]] | ||
[[Category: Nishio | [[Category: Mizushima T]] | ||
[[Category: Noda | [[Category: Nishio M]] | ||
[[Category: Sasakawa | [[Category: Noda M]] | ||
[[Category: Uchiyama | [[Category: Sasakawa H]] | ||
[[Category: Wakatsuki | [[Category: Uchiyama S]] | ||
[[Category: Yamamoto | [[Category: Wakatsuki S]] | ||
[[Category: Yamamoto K]] | |||