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5nn4

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'''Unreleased structure'''
 
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The entry 5nn4 is ON HOLD until Paper Publication
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==Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-acetyl-cysteine==
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<StructureSection load='5nn4' size='340' side='right' caption='[[5nn4]], [[Resolution|resolution]] 1.83&Aring;' scene=''>
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Authors: Roig-Zamboni, V., Cobucci-Ponzano, B., Iacono, R., Ferrara, M.C., Germany, S., Parenti, G., Bourne, Y., Moracci, M.
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== Structural highlights ==
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<table><tr><td colspan='2'>[[5nn4]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5NN4 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5NN4 FirstGlance]. <br>
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Description: Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-acetyl-cysteine
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</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=MAN:ALPHA-D-MANNOSE'>MAN</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=PGE:TRIETHYLENE+GLYCOL'>PGE</scene>, <scene name='pdbligand=SC2:N-ACETYL-L-CYSTEINE'>SC2</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
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[[Category: Unreleased Structures]]
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[5nn3|5nn3]]</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/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>
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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=5nn4 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5nn4 OCA], [http://pdbe.org/5nn4 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5nn4 RCSB], [http://www.ebi.ac.uk/pdbsum/5nn4 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5nn4 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[[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.
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== Function ==
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[[http://www.uniprot.org/uniprot/LYAG_HUMAN LYAG_HUMAN]] Essential for the degradation of glygogen to glucose in lysosomes.
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__TOC__
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</StructureSection>
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[[Category: Alpha-glucosidase]]
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[[Category: Bourne, Y]]
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[[Category: Cobucci-Ponzano, B]]
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[[Category: Ferrara, M C]]
[[Category: Germany, S]]
[[Category: Germany, S]]
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[[Category: Ferrara, M.C]]
 
[[Category: Iacono, R]]
[[Category: Iacono, R]]
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[[Category: Moracci, M]]
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[[Category: Parenti, G]]
[[Category: Roig-Zamboni, V]]
[[Category: Roig-Zamboni, V]]
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[[Category: Parenti, G]]
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[[Category: Glycogen catabolism]]
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[[Category: Bourne, Y]]
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[[Category: Glycoside hydrolase]]
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[[Category: Cobucci-Ponzano, B]]
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[[Category: Hydrolase]]
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[[Category: Moracci, M]]
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[[Category: Lysosome]]
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[[Category: Pompe disease]]

Revision as of 07:12, 25 October 2017

Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-acetyl-cysteine

5nn4, resolution 1.83Å

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