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==Human Xylosyltransferase 1 in complex with peptide QEEEGSGGGQGG==
==Human Xylosyltransferase 1 in complex with peptide QEEEGSGGGQGG==
<StructureSection load='6ej8' size='340' side='right' caption='[[6ej8]], [[Resolution|resolution]] 2.09&Aring;' scene=''>
<StructureSection load='6ej8' size='340' side='right'caption='[[6ej8]], [[Resolution|resolution]] 2.09&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[6ej8]] 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=6EJ8 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6EJ8 FirstGlance]. <br>
<table><tr><td colspan='2'>[[6ej8]] 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=6EJ8 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6EJ8 FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=NA:SODIUM+ION'>NA</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</scene></td></tr>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.09&#8491;</td></tr>
<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">XYLT1, XT1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=NA:SODIUM+ION'>NA</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</scene></td></tr>
<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Protein_xylosyltransferase Protein xylosyltransferase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.4.2.26 2.4.2.26] </span></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=6ej8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6ej8 OCA], [https://pdbe.org/6ej8 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6ej8 RCSB], [https://www.ebi.ac.uk/pdbsum/6ej8 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6ej8 ProSAT]</span></td></tr>
<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=6ej8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6ej8 OCA], [http://pdbe.org/6ej8 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6ej8 RCSB], [http://www.ebi.ac.uk/pdbsum/6ej8 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6ej8 ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/XYLT1_HUMAN XYLT1_HUMAN]] XYLT1-CDG;Desbuquois syndrome. The disease is caused by mutations affecting the gene represented in this entry.  The gene represented in this entry acts as a disease modifier.  
[https://www.uniprot.org/uniprot/XYLT1_HUMAN XYLT1_HUMAN] XYLT1-CDG;Desbuquois syndrome. The disease is caused by mutations affecting the gene represented in this entry.  The gene represented in this entry acts as a disease modifier.
== Function ==
== Function ==
[[http://www.uniprot.org/uniprot/XYLT1_HUMAN XYLT1_HUMAN]] Catalyzes the first step in biosynthesis of glycosaminoglycan. Transfers D-xylose from UDP-D-xylose to specific serine residues of the core protein. Initial enzyme in the biosynthesis of chondroitin sulfate and dermatan sulfate proteoglycans in fibroblasts and chondrocytes.<ref>PMID:15461586</ref> [[http://www.uniprot.org/uniprot/AMBP_HUMAN AMBP_HUMAN]] Inter-alpha-trypsin inhibitor inhibits trypsin, plasmin, and lysosomal granulocytic elastase. Inhibits calcium oxalate crystallization.<ref>PMID:7676539</ref>  Trypstatin is a trypsin inhibitor (By similarity).<ref>PMID:7676539</ref> 
[https://www.uniprot.org/uniprot/XYLT1_HUMAN XYLT1_HUMAN] Catalyzes the first step in biosynthesis of glycosaminoglycan. Transfers D-xylose from UDP-D-xylose to specific serine residues of the core protein. Initial enzyme in the biosynthesis of chondroitin sulfate and dermatan sulfate proteoglycans in fibroblasts and chondrocytes.<ref>PMID:15461586</ref>  
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: Human]]
[[Category: Homo sapiens]]
[[Category: Protein xylosyltransferase]]
[[Category: Large Structures]]
[[Category: Briggs, D C]]
[[Category: Briggs DC]]
[[Category: Hohenester, E]]
[[Category: Hohenester E]]
[[Category: Glycosyltransferase]]
[[Category: Golgi]]
[[Category: Proteoglycan]]
[[Category: Transferase]]
[[Category: Xylosyltransferase]]
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