4f0x
From Proteopedia
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- | {{STRUCTURE_4f0x| PDB=4f0x | SCENE= }} | ||
- | ===Crystal structure of human Malonyl-CoA Decarboxylase (Peroxisomal Isoform)=== | ||
- | {{ABSTRACT_PUBMED_23482565}} | ||
- | == | + | ==Crystal structure of human Malonyl-CoA Decarboxylase (Peroxisomal Isoform)== |
- | [[http://www.uniprot.org/uniprot/DCMC_HUMAN DCMC_HUMAN | + | <StructureSection load='4f0x' size='340' side='right'caption='[[4f0x]], [[Resolution|resolution]] 3.29Å' scene=''> |
- | + | == Structural highlights == | |
- | ==Function== | + | <table><tr><td colspan='2'>[[4f0x]] is a 8 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4F0X OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4F0X 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]] 3.29Å</td></tr> |
- | + | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=0OR:N~3~-[(2R)-2-HYDROXY-4-{[(S)-HYDROXY(PHOSPHONOOXY)PHOSPHORYL]OXY}-3,3-DIMETHYLBUTANOYL]-BETA-ALANINAMIDE'>0OR</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=4f0x FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4f0x OCA], [https://pdbe.org/4f0x PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4f0x RCSB], [https://www.ebi.ac.uk/pdbsum/4f0x PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4f0x ProSAT]</span></td></tr> | |
- | + | </table> | |
+ | == Disease == | ||
+ | [https://www.uniprot.org/uniprot/DCMC_HUMAN DCMC_HUMAN] Malonic aciduria. Defects in MLYCD are the cause of malonyl-CoA decarboxylase deficiency (MLYCD deficiency) [MIM:[https://omim.org/entry/248360 248360]. MLYCD deficiency is an autosomal recessive disease characterized by abdominal pain, chronic constipation, episodic vomiting, metabolic acidosis and malonic aciduria. | ||
+ | == Function == | ||
+ | [https://www.uniprot.org/uniprot/DCMC_HUMAN DCMC_HUMAN] Catalyzes the conversion of malonyl-CoA to acetyl-CoA. In the fatty acid biosynthesis MCD selectively removes malonyl-CoA and thus assures that methyl-malonyl-CoA is the only chain elongating substrate for fatty acid synthase and that fatty acids with multiple methyl side chains are produced. In peroxisomes it may be involved in degrading intraperoxisomal malonyl-CoA, which is generated by the peroxisomal beta-oxidation of odd chain-length dicarboxylic fatty acids. | ||
+ | __TOC__ | ||
+ | </StructureSection> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
- | [[Category: | + | [[Category: Large Structures]] |
- | [[Category: Aparicio | + | [[Category: Aparicio D]] |
- | [[Category: Fita | + | [[Category: Fita I]] |
- | [[Category: Perez | + | [[Category: Perez R]] |
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Current revision
Crystal structure of human Malonyl-CoA Decarboxylase (Peroxisomal Isoform)
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