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1l5r

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== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[1l5r]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1L5R OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1L5R FirstGlance]. <br>
<table><tr><td colspan='2'>[[1l5r]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1L5R OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1L5R FirstGlance]. <br>
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</td></tr><tr><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=700:[5-CHLORO-1H-INDOL-2-CARBONYL-PHENYLALANINYL]-AZETIDINE-3-CARBOXYLIC+ACID'>700</scene>, <scene name='pdbligand=MRD:(4R)-2-METHYLPENTANE-2,4-DIOL'>MRD</scene>, <scene name='pdbligand=NBG:1-N-ACETYL-BETA-D-GLUCOSAMINE'>NBG</scene>, <scene name='pdbligand=PLP:PYRIDOXAL-5-PHOSPHATE'>PLP</scene>, <scene name='pdbligand=RBF:RIBOFLAVIN'>RBF</scene><br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=700:[5-CHLORO-1H-INDOL-2-CARBONYL-PHENYLALANINYL]-AZETIDINE-3-CARBOXYLIC+ACID'>700</scene>, <scene name='pdbligand=MRD:(4R)-2-METHYLPENTANE-2,4-DIOL'>MRD</scene>, <scene name='pdbligand=NBG:1-N-ACETYL-BETA-D-GLUCOSAMINE'>NBG</scene>, <scene name='pdbligand=PLP:PYRIDOXAL-5-PHOSPHATE'>PLP</scene>, <scene name='pdbligand=RBF:RIBOFLAVIN'>RBF</scene></td></tr>
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<tr><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1l5q|1l5q]], [[1l5s|1l5s]], [[1l7x|1l7x]]</td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1l5q|1l5q]], [[1l5s|1l5s]], [[1l7x|1l7x]]</td></tr>
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<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Phosphorylase Phosphorylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.4.1.1 2.4.1.1] </span></td></tr>
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<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Phosphorylase Phosphorylase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=2.4.1.1 2.4.1.1] </span></td></tr>
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<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=1l5r FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1l5r OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1l5r RCSB], [http://www.ebi.ac.uk/pdbsum/1l5r PDBsum]</span></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=1l5r FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1l5r OCA], [http://www.rcsb.org/pdb/explore.do?structureId=1l5r RCSB], [http://www.ebi.ac.uk/pdbsum/1l5r PDBsum]</span></td></tr>
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<table>
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</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/PYGL_HUMAN PYGL_HUMAN]] Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6) [MIM:[http://omim.org/entry/232700 232700]]. A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected.<ref>PMID:9529348</ref>
[[http://www.uniprot.org/uniprot/PYGL_HUMAN PYGL_HUMAN]] Defects in PYGL are the cause of glycogen storage disease type 6 (GSD6) [MIM:[http://omim.org/entry/232700 232700]]. A metabolic disorder characterized by mild to moderate hypoglycemia, mild ketosis, growth retardation, and prominent hepatomegaly. Heart and skeletal muscle are not affected.<ref>PMID:9529348</ref>
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Phosphorylase]]
[[Category: Phosphorylase]]
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[[Category: Carty, M D.]]
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[[Category: Carty, M D]]
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[[Category: Culp, J.]]
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[[Category: Culp, J]]
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[[Category: Danley, D E.]]
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[[Category: Danley, D E]]
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[[Category: Day, Y S.N.]]
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[[Category: Day, Y S.N]]
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[[Category: Ekstrom, J L.]]
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[[Category: Ekstrom, J L]]
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[[Category: Fletterick, R J.]]
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[[Category: Fletterick, R J]]
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[[Category: Gibbs, E M.]]
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[[Category: Gibbs, E M]]
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[[Category: Hoover, D J.]]
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[[Category: Hoover, D J]]
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[[Category: Myszka, D G.]]
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[[Category: Myszka, D G]]
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[[Category: Pauly, T A.]]
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[[Category: Pauly, T A]]
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[[Category: Rath, V L.]]
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[[Category: Rath, V L]]
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[[Category: Soeller, W C.]]
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[[Category: Soeller, W C]]
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[[Category: Treadway, J L.]]
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[[Category: Treadway, J L]]
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[[Category: Phosphorylase]]
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[[Category: Purine site]]
[[Category: Purine site]]
[[Category: Transferase]]
[[Category: Transferase]]

Revision as of 15:16, 5 January 2015

Human liver glycogen phosphorylase a complexed with riboflavin, N-Acetyl-beta-D-Glucopyranosylamine and CP-403,700

1l5r, resolution 2.10Å

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