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| | <StructureSection load='2vcc' size='340' side='right'caption='[[2vcc]], [[Resolution|resolution]] 2.00Å' scene=''> | | <StructureSection load='2vcc' size='340' side='right'caption='[[2vcc]], [[Resolution|resolution]] 2.00Å' scene=''> |
| | == Structural highlights == | | == Structural highlights == |
| - | <table><tr><td colspan='2'>[[2vcc]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/"bacillus_perfringens"_veillon_and_zuber_1898 "bacillus perfringens" veillon and zuber 1898]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2VCC OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2VCC FirstGlance]. <br> | + | <table><tr><td colspan='2'>[[2vcc]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Clostridium_perfringens Clostridium perfringens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2VCC OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2VCC FirstGlance]. <br> |
| - | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</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]] 2Å</td></tr> |
| - | <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[2vc9|2vc9]], [[2vca|2vca]], [[2vcb|2vcb]]</div></td></tr> | + | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr> |
| - | <tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[https://en.wikipedia.org/wiki/Alpha-N-acetylglucosaminidase Alpha-N-acetylglucosaminidase], with EC number [https://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.50 3.2.1.50] </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=2vcc FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2vcc OCA], [https://pdbe.org/2vcc PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2vcc RCSB], [https://www.ebi.ac.uk/pdbsum/2vcc PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2vcc ProSAT]</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=2vcc FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2vcc OCA], [https://pdbe.org/2vcc PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2vcc RCSB], [https://www.ebi.ac.uk/pdbsum/2vcc PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2vcc ProSAT]</span></td></tr> |
| | </table> | | </table> |
| | + | == Function == |
| | + | [https://www.uniprot.org/uniprot/A0A0H2YU91_CLOP1 A0A0H2YU91_CLOP1] |
| | == Evolutionary Conservation == | | == Evolutionary Conservation == |
| | [[Image:Consurf_key_small.gif|200px|right]] | | [[Image:Consurf_key_small.gif|200px|right]] |
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| | __TOC__ | | __TOC__ |
| | </StructureSection> | | </StructureSection> |
| - | [[Category: Bacillus perfringens veillon and zuber 1898]] | + | [[Category: Clostridium perfringens]] |
| - | [[Category: Alpha-N-acetylglucosaminidase]]
| + | |
| | [[Category: Large Structures]] | | [[Category: Large Structures]] |
| - | [[Category: Berg, O]] | + | [[Category: Berg O]] |
| - | [[Category: Boraston, A B]] | + | [[Category: Boraston AB]] |
| - | [[Category: Ficko-Blean, E]] | + | [[Category: Ficko-Blean E]] |
| - | [[Category: Stubbs, K A]] | + | [[Category: Stubbs KA]] |
| - | [[Category: Vocadlo, D J]] | + | [[Category: Vocadlo DJ]] |
| - | [[Category: Alpha-n-acetylglucosaminidase]]
| + | |
| - | [[Category: Family 89 glycoside hydrolase]]
| + | |
| - | [[Category: Gh89]]
| + | |
| - | [[Category: Hydrolase]]
| + | |
| - | [[Category: Mucopolysaccharidosis]]
| + | |
| - | [[Category: Naglu]]
| + | |
| - | [[Category: Sanfilippo disease]]
| + | |
| Structural highlights
Function
A0A0H2YU91_CLOP1
Evolutionary Conservation
Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf.
Publication Abstract from PubMed
Mucopolysaccharidosis III (MPS III) has four forms (A-D) that result from buildup of an improperly degraded glycosaminoglycan in lysosomes. MPS IIIB is attributable to the decreased activity of a lysosomal alpha-N-acetylglucosaminidase (NAGLU). Here, we describe the structure, catalytic mechanism, and inhibition of CpGH89 from Clostridium perfringens, a close bacterial homolog of NAGLU. The structure enables the generation of a homology model of NAGLU, an enzyme that has resisted structural studies despite having been studied for >20 years. This model reveals which mutations giving rise to MPS IIIB map to the active site and which map to regions distant from the active site. The identification of potent inhibitors of CpGH89 and the structures of these inhibitors in complex with the enzyme suggest small-molecule candidates for use as chemical chaperones. These studies therefore illuminate the genetic basis of MPS IIIB, provide a clear biochemical rationale for the necessary sequential action of heparan-degrading enzymes, and open the door to the design and optimization of chemical chaperones for treating MPS IIIB.
Structural and mechanistic insight into the basis of mucopolysaccharidosis IIIB.,Ficko-Blean E, Stubbs KA, Nemirovsky O, Vocadlo DJ, Boraston AB Proc Natl Acad Sci U S A. 2008 May 6;105(18):6560-5. Epub 2008 Apr 28. PMID:18443291[1]
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
References
- ↑ Ficko-Blean E, Stubbs KA, Nemirovsky O, Vocadlo DJ, Boraston AB. Structural and mechanistic insight into the basis of mucopolysaccharidosis IIIB. Proc Natl Acad Sci U S A. 2008 May 6;105(18):6560-5. Epub 2008 Apr 28. PMID:18443291
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