9e1t

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Current revision (06:25, 12 February 2025) (edit) (undo)
 
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'''Unreleased structure'''
 
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The entry 9e1t is ON HOLD until Paper Publication
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==CryoEM structure of LARGE1 bound to UDP==
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<StructureSection load='9e1t' size='340' side='right'caption='[[9e1t]], [[Resolution|resolution]] 3.00&Aring;' scene=''>
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Authors: Joesph, S., Spellmon, N., Campbell, K.P.
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== Structural highlights ==
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<table><tr><td colspan='2'>[[9e1t]] 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=9E1T OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=9E1T FirstGlance]. <br>
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Description: CryoEM structure of LARGE1 bound to UDP
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3&#8491;</td></tr>
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[[Category: Unreleased Structures]]
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=MN:MANGANESE+(II)+ION'>MN</scene>, <scene name='pdbligand=UDP:URIDINE-5-DIPHOSPHATE'>UDP</scene></td></tr>
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[[Category: Campbell, K.P]]
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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=9e1t FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=9e1t OCA], [https://pdbe.org/9e1t PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=9e1t RCSB], [https://www.ebi.ac.uk/pdbsum/9e1t PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=9e1t ProSAT]</span></td></tr>
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[[Category: Joesph, S]]
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</table>
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[[Category: Spellmon, N]]
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== Disease ==
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[https://www.uniprot.org/uniprot/LARG1_HUMAN LARG1_HUMAN] Congenital muscular dystrophy with intellectual disability;Walker-Warburg syndrome;Muscle-eye-brain disease. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry.
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== Function ==
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[https://www.uniprot.org/uniprot/LARG1_HUMAN LARG1_HUMAN] Bifunctional glycosyltransferase with both alpha-1,3-xylosyltransferase and beta-1,3-glucuronyltransferase activities involved in the maturation of alpha-dystroglycan (DAG1) by glycosylation leading to DAG1 binding to laminin G-like domain-containing extracellular proteins with high affinity (PubMed:15661757, PubMed:15752776, PubMed:21987822, PubMed:22223806, PubMed:23125099, PubMed:25279697, PubMed:25279699). Elongates the glucuronyl-beta-1,4-xylose-beta disaccharide primer structure initiated by B4GAT1 by adding repeating units [-3-Xylose-alpha-1,3-GlcA-beta-1-] to produce a heteropolysaccharide (PubMed:22223806, PubMed:23125099, PubMed:25138275, PubMed:25279697, PubMed:25279699, PubMed:32975514). Requires the phosphorylation of core M3 (O-mannosyl trisaccharide) by POMK to elongate the glucuronyl-beta-1,4-xylose-beta disaccharide primer (PubMed:21987822). Plays a key role in skeletal muscle function and regeneration (By similarity).[UniProtKB:Q9Z1M7]<ref>PMID:15661757</ref> <ref>PMID:15752776</ref> <ref>PMID:21987822</ref> <ref>PMID:22223806</ref> <ref>PMID:23125099</ref> <ref>PMID:25138275</ref> <ref>PMID:25279697</ref> <ref>PMID:25279699</ref> <ref>PMID:32975514</ref>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
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[[Category: Homo sapiens]]
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[[Category: Large Structures]]
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[[Category: Campbell KP]]
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[[Category: Joesph S]]
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[[Category: Spellmon N]]

Current revision

CryoEM structure of LARGE1 bound to UDP

PDB ID 9e1t

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