5nn6
From Proteopedia
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| - | '''Unreleased structure''' | ||
| - | + | ==Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-hydroxyethyl-1-deoxynojirimycin== | |
| - | + | <StructureSection load='5nn6' size='340' side='right' caption='[[5nn6]], [[Resolution|resolution]] 2.00Å' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[5nn6]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5NN6 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5NN6 FirstGlance]. <br> | |
| - | + | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=BMA:BETA-D-MANNOSE'>BMA</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=EDO:1,2-ETHANEDIOL'>EDO</scene>, <scene name='pdbligand=FUC:ALPHA-L-FUCOSE'>FUC</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=MIG:(2R,3R,4R,5S)-1-(2-HYDROXYETHYL)-2-(HYDROXYMETHYL)PIPERIDINE-3,4,5-TRIOL'>MIG</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=PGE:TRIETHYLENE+GLYCOL'>PGE</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr> | |
| - | [[Category: | + | <tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=CSO:S-HYDROXYCYSTEINE'>CSO</scene></td></tr> |
| + | <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[5nn3|5nn3]], [[5nn4|5nn4]], [[5nn5|5nn5]]</td></tr> | ||
| + | <tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Alpha-glucosidase Alpha-glucosidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.20 3.2.1.20] </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=5nn6 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5nn6 OCA], [http://pdbe.org/5nn6 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5nn6 RCSB], [http://www.ebi.ac.uk/pdbsum/5nn6 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5nn6 ProSAT]</span></td></tr> | ||
| + | </table> | ||
| + | == Disease == | ||
| + | [[http://www.uniprot.org/uniprot/LYAG_HUMAN LYAG_HUMAN]] Glycogen storage disease due to acid maltase deficiency, infantile onset;Glycogen storage disease due to acid maltase deficiency, juvenile onset;Glycogen storage disease due to acid maltase deficiency, adult onset. The disease is caused by mutations affecting the gene represented in this entry. | ||
| + | == Function == | ||
| + | [[http://www.uniprot.org/uniprot/LYAG_HUMAN LYAG_HUMAN]] Essential for the degradation of glygogen to glucose in lysosomes. | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Alpha-glucosidase]] | ||
| + | [[Category: Bourne, Y]] | ||
| + | [[Category: Cobucci-Ponzano, B]] | ||
| + | [[Category: Ferrara, M C]] | ||
[[Category: Germany, S]] | [[Category: Germany, S]] | ||
| - | [[Category: Ferrara, M.C]] | ||
[[Category: Iacono, R]] | [[Category: Iacono, R]] | ||
| + | [[Category: Moracci, M]] | ||
| + | [[Category: Parenti, G]] | ||
[[Category: Roig-Zamboni, V]] | [[Category: Roig-Zamboni, V]] | ||
| - | [[Category: | + | [[Category: Glycogen catabolism]] |
| - | [[Category: | + | [[Category: Glycoside hydrolase]] |
| - | [[Category: | + | [[Category: Hydrolase]] |
| - | [[Category: | + | [[Category: Lysosome]] |
| + | [[Category: Pompe disease]] | ||
Revision as of 07:13, 25 October 2017
Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-hydroxyethyl-1-deoxynojirimycin
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