6kal

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Current revision (12:48, 6 November 2024) (edit) (undo)
 
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<StructureSection load='6kal' size='340' side='right'caption='[[6kal]], [[Resolution|resolution]] 2.60&Aring;' scene=''>
<StructureSection load='6kal' size='340' side='right'caption='[[6kal]], [[Resolution|resolution]] 2.60&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[6kal]] is a 4 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=6KAL OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6KAL FirstGlance]. <br>
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<table><tr><td colspan='2'>[[6kal]] is a 4 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=6KAL OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6KAL 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=C5P:CYTIDINE-5-MONOPHOSPHATE'>C5P</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</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]] 2.6&#8491;</td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">FKRP ([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=C5P:CYTIDINE-5-MONOPHOSPHATE'>C5P</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</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=6kal FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6kal OCA], [http://pdbe.org/6kal PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6kal RCSB], [http://www.ebi.ac.uk/pdbsum/6kal PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6kal ProSAT]</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=6kal FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6kal OCA], [https://pdbe.org/6kal PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6kal RCSB], [https://www.ebi.ac.uk/pdbsum/6kal PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6kal ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/FKRP_HUMAN FKRP_HUMAN]] Congenital muscular dystrophy with intellectual disability;Walker-Warburg syndrome;Autosomal recessive limb-girdle muscular dystrophy type 2I;Congenital muscular dystrophy with cerebellar involvement;Muscle-eye-brain disease;Congenital muscular dystrophy without intellectual disability. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry.
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[https://www.uniprot.org/uniprot/FKRP_HUMAN FKRP_HUMAN] Congenital muscular dystrophy with intellectual disability;Walker-Warburg syndrome;Autosomal recessive limb-girdle muscular dystrophy type 2I;Congenital muscular dystrophy with cerebellar involvement;Muscle-eye-brain disease;Congenital muscular dystrophy without intellectual disability. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry.
== Function ==
== Function ==
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[[http://www.uniprot.org/uniprot/FKRP_HUMAN FKRP_HUMAN]] Catalyzes the transfer of CDP-ribitol to ribitol 5-phosphate previously attached by FKTN/fukutin of to the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) (PubMed:25279699, PubMed:26923585, PubMed:29477842). This constitutes the second step in the formation of the ribose 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1 (PubMed:25279699, PubMed:26923585, PubMed:29477842).<ref>PMID:25279699</ref> <ref>PMID:26923585</ref> <ref>PMID:29477842</ref>
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[https://www.uniprot.org/uniprot/FKRP_HUMAN FKRP_HUMAN] Catalyzes the transfer of CDP-ribitol to ribitol 5-phosphate previously attached by FKTN/fukutin of to the phosphorylated O-mannosyl trisaccharide (N-acetylgalactosamine-beta-3-N-acetylglucosamine-beta-4-(phosphate-6-)mannose), a carbohydrate structure present in alpha-dystroglycan (DAG1) (PubMed:25279699, PubMed:26923585, PubMed:29477842). This constitutes the second step in the formation of the ribose 5-phosphate tandem repeat which links the phosphorylated O-mannosyl trisaccharide to the ligand binding moiety composed of repeats of 3-xylosyl-alpha-1,3-glucuronic acid-beta-1 (PubMed:25279699, PubMed:26923585, PubMed:29477842).<ref>PMID:25279699</ref> <ref>PMID:26923585</ref> <ref>PMID:29477842</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>
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[[Category: Human]]
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[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Large Structures]]
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[[Category: Kuwabara, N]]
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[[Category: Kuwabara N]]
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[[Category: Glycosyltranferase]]
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[[Category: Phospho ribitoyl tranferase]]
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[[Category: Transferase]]
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Current revision

Crystal structure of FKRP in complex with Mg ion and CMP

PDB ID 6kal

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