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6g7o
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| - | '''Unreleased structure''' | ||
| - | + | ==Crystal structure of human alkaline ceramidase 3 (ACER3) at 2.7 Angstrom resolution== | |
| + | <StructureSection load='6g7o' size='340' side='right'caption='[[6g7o]], [[Resolution|resolution]] 2.70Å' scene=''> | ||
| + | == Structural highlights == | ||
| + | <table><tr><td colspan='2'>[[6g7o]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Escherichia_coli Escherichia coli] and [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6G7O OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6G7O 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]] 2.7Å</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=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=NA:SODIUM+ION'>NA</scene>, <scene name='pdbligand=OLB:(2S)-2,3-DIHYDROXYPROPYL+(9Z)-OCTADEC-9-ENOATE'>OLB</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</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=6g7o FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6g7o OCA], [https://pdbe.org/6g7o PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6g7o RCSB], [https://www.ebi.ac.uk/pdbsum/6g7o PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6g7o ProSAT]</span></td></tr> | ||
| + | </table> | ||
| + | == Disease == | ||
| + | [https://www.uniprot.org/uniprot/ACER3_HUMAN ACER3_HUMAN] Alkaline ceramidase 3 deficiency. | ||
| + | == Function == | ||
| + | [https://www.uniprot.org/uniprot/C562_ECOLX C562_ECOLX] Electron-transport protein of unknown function.[https://www.uniprot.org/uniprot/ACER3_HUMAN ACER3_HUMAN] Hydrolyzes only phytoceramide into phytosphingosine and free fatty acid. Does not have reverse activity. | ||
| + | <div style="background-color:#fffaf0;"> | ||
| + | == Publication Abstract from PubMed == | ||
| + | Alkaline ceramidases (ACERs) are a class of poorly understood transmembrane enzymes controlling the homeostasis of ceramides. They are implicated in human pathophysiology, including progressive leukodystrophy, colon cancer as well as acute myeloid leukemia. We report here the crystal structure of the human ACER type 3 (ACER3). Together with computational studies, the structure reveals that ACER3 is an intramembrane enzyme with a seven transmembrane domain architecture and a catalytic Zn(2+) binding site in its core, similar to adiponectin receptors. Interestingly, we uncover a Ca(2+) binding site physically and functionally connected to the Zn(2+) providing a structural explanation for the known regulatory role of Ca(2+) on ACER3 enzymatic activity and for the loss of function in E33G-ACER3 mutant found in leukodystrophic patients. | ||
| - | + | Structure of a human intramembrane ceramidase explains enzymatic dysfunction found in leukodystrophy.,Vasiliauskaite-Brooks I, Healey RD, Rochaix P, Saint-Paul J, Sounier R, Grison C, Waltrich-Augusto T, Fortier M, Hoh F, Saied EM, Arenz C, Basu S, Leyrat C, Granier S Nat Commun. 2018 Dec 21;9(1):5437. doi: 10.1038/s41467-018-07864-w. PMID:30575723<ref>PMID:30575723</ref> | |
| - | + | From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | |
| - | [[Category: | + | </div> |
| + | <div class="pdbe-citations 6g7o" style="background-color:#fffaf0;"></div> | ||
| + | |||
| + | ==See Also== | ||
| + | *[[Ceramidase 3D PDB structures|Ceramidase 3D PDB structures]] | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Escherichia coli]] | ||
| + | [[Category: Homo sapiens]] | ||
| + | [[Category: Large Structures]] | ||
| + | [[Category: Basu S]] | ||
| + | [[Category: Granier S]] | ||
| + | [[Category: Grison C]] | ||
| + | [[Category: Healey RD]] | ||
| + | [[Category: Hoh F]] | ||
| + | [[Category: Leyrat C]] | ||
| + | [[Category: Sounier R]] | ||
| + | [[Category: Vasiliauskaite-Brooks I]] | ||
Current revision
Crystal structure of human alkaline ceramidase 3 (ACER3) at 2.7 Angstrom resolution
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