4ufm: Difference between revisions

No edit summary
No edit summary
Line 3: Line 3:
<StructureSection load='4ufm' size='340' side='right'caption='[[4ufm]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
<StructureSection load='4ufm' size='340' side='right'caption='[[4ufm]], [[Resolution|resolution]] 2.40&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[4ufm]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Lk3_transgenic_mice Lk3 transgenic mice]. 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>
<table><tr><td colspan='2'>[[4ufm]] 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=4UFM OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/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>
</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=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='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=4ufm FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4ufm OCA], [https://pdbe.org/4ufm PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4ufm RCSB], [https://www.ebi.ac.uk/pdbsum/4ufm PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4ufm ProSAT]</span></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>
<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://pdbe.org/4ufm PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=4ufm RCSB], [http://www.ebi.ac.uk/pdbsum/4ufm PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=4ufm ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
[[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.  
[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 ==
== 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>
[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;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
Line 29: Line 27:
__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: Galactosylceramidase]]
[[Category: Large Structures]]
[[Category: Large Structures]]
[[Category: Lk3 transgenic mice]]
[[Category: Mus musculus]]
[[Category: Christensen, S H]]
[[Category: Christensen SH]]
[[Category: Deane, J E]]
[[Category: Deane JE]]
[[Category: Hill, C H]]
[[Category: Hill CH]]
[[Category: Jensen, H H]]
[[Category: Jensen HH]]
[[Category: Moriarty, N W]]
[[Category: Moriarty NW]]
[[Category: Read, R J]]
[[Category: Read RJ]]
[[Category: Salamone, S]]
[[Category: Salamone S]]
[[Category: Spratley, S J]]
[[Category: Spratley SJ]]
[[Category: Viuff, A H]]
[[Category: Viuff AH]]
[[Category: Complex]]
[[Category: Hydrolase]]
[[Category: Lysosome]]

Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

OCA