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6y6t
From Proteopedia
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| - | '''Unreleased structure''' | ||
| - | + | ==Mouse Galactocerebrosidase complexed with galacto-noeurostegine GNS at pH 4.6== | |
| + | <StructureSection load='6y6t' size='340' side='right'caption='[[6y6t]], [[Resolution|resolution]] 2.25Å' scene=''> | ||
| + | == Structural highlights == | ||
| + | <table><tr><td colspan='2'>[[6y6t]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Mus_musculus Mus musculus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6Y6T OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6Y6T FirstGlance]. <br> | ||
| + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.25Å</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=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene>, <scene name='pdbligand=NI:NICKEL+(II)+ION'>NI</scene>, <scene name='pdbligand=ODW:(1~{R},2~{S},3~{S},4~{R},5~{R})-4-(hydroxymethyl)-8-azabicyclo[3.2.1]octane-1,2,3-triol'>ODW</scene></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=6y6t FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6y6t OCA], [https://pdbe.org/6y6t PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6y6t RCSB], [https://www.ebi.ac.uk/pdbsum/6y6t PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6y6t ProSAT]</span></td></tr> | ||
| + | </table> | ||
| + | == Disease == | ||
| + | [https://www.uniprot.org/uniprot/GALC_MOUSE GALC_MOUSE] Defects in Galc are the cause of the 'twitcher' phenotype; an autosomal recessive leukodystrophy similar to the human disease (Krabbe disease). This deficiency results in the insufficient catabolism of several galactolipids that are important in the production of normal myelin. | ||
| + | == Function == | ||
| + | [https://www.uniprot.org/uniprot/GALC_MOUSE GALC_MOUSE] Hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Enzyme with very low activity responsible for the lysosomal catabolism of galactosylceramide, a major lipid in myelin, kidney and epithelial cells of small intestine and colon.<ref>PMID:8769874</ref> | ||
| + | <div style="background-color:#fffaf0;"> | ||
| + | == Publication Abstract from PubMed == | ||
| + | Competitive inhibitors of galactocerebrosidase (GALC) could be candidates for pharmacological chaperone therapy of patients with Krabbe disease. The known and selective nortropane-type iminosugar galacto-noeurostegine has been found to competitively inhibit GALC with K (i) = 7 muM at pH 4.6, which is 330-fold more potent than the analogous deoxynoeurostegine. It was shown through X-ray protein crystallography that galacto-noeurostegine binds to the active site of GALC in its bicyclic form. | ||
| - | + | The Bicyclic Form of galacto-Noeurostegine Is a Potent Inhibitor of beta-Galactocerebrosidase.,Viuff A, Salamone S, McLoughlin J, Deane JE, Jensen HH ACS Med Chem Lett. 2020 Dec 18;12(1):56-59. doi: 10.1021/acsmedchemlett.0c00377. , eCollection 2021 Jan 14. PMID:33488964<ref>PMID:33488964</ref> | |
| - | + | From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | |
| - | [[Category: | + | </div> |
| - | [[Category: | + | <div class="pdbe-citations 6y6t" style="background-color:#fffaf0;"></div> |
| - | [[Category: Deane | + | |
| + | ==See Also== | ||
| + | *[[Galactosylceramidase|Galactosylceramidase]] | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Large Structures]] | ||
| + | [[Category: Mus musculus]] | ||
| + | [[Category: Deane JE]] | ||
| + | [[Category: McLoughlin J]] | ||
Current revision
Mouse Galactocerebrosidase complexed with galacto-noeurostegine GNS at pH 4.6
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