4ufm: Difference between revisions
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''' | ==Mouse Galactocerebrosidase complexed with 1-deoxy-galacto-nojirimycin DGJ== | ||
<StructureSection load='4ufm' size='340' side='right' caption='[[4ufm]], [[Resolution|resolution]] 2.40Å' scene=''> | |||
== Structural highlights == | |||
<table><tr><td colspan='2'>[[4ufm]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4UFM OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4UFM FirstGlance]. <br> | |||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=CA:CALCIUM+ION'>CA</scene>, <scene name='pdbligand=DGJ:(2R,3S,4R,5S)-2-(HYDROXYMETHYL)PIPERIDINE-3,4,5-TRIOL'>DGJ</scene>, <scene name='pdbligand=NAG:N-ACETYL-D-GLUCOSAMINE'>NAG</scene></td></tr> | |||
<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[4ufh|4ufh]], [[4ufi|4ufi]], [[4ufj|4ufj]], [[4ufk|4ufk]], [[4ufl|4ufl]]</td></tr> | |||
<tr id='activity'><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Galactosylceramidase Galactosylceramidase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.2.1.46 3.2.1.46] </span></td></tr> | |||
[[Category: | <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=4ufm FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ufm OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4ufm RCSB], [http://www.ebi.ac.uk/pdbsum/4ufm PDBsum]</span></td></tr> | ||
[[Category: | </table> | ||
[[Category: | == Disease == | ||
[[Category: | [[http://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 == | |||
[[http://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> | |||
== References == | |||
<references/> | |||
__TOC__ | |||
</StructureSection> | |||
[[Category: Galactosylceramidase]] | |||
[[Category: Christensen, S H]] | |||
[[Category: Deane, J E]] | |||
[[Category: Hill, C H]] | |||
[[Category: Jensen, H H]] | |||
[[Category: Moriarty, N W]] | |||
[[Category: Read, R J]] | |||
[[Category: Salamone, S]] | [[Category: Salamone, S]] | ||
[[Category: | [[Category: Spratley, S J]] | ||
[[Category: | [[Category: Viuff, A H]] | ||
[[Category: | [[Category: Complex]] | ||
[[Category: | [[Category: Hydrolase]] | ||
[[Category: | [[Category: Lysosome]] | ||
Revision as of 13:20, 1 April 2015
Mouse Galactocerebrosidase complexed with 1-deoxy-galacto-nojirimycin DGJ
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