2ygw
From Proteopedia
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{{STRUCTURE_2ygw| PDB=2ygw | SCENE= }} | {{STRUCTURE_2ygw| PDB=2ygw | SCENE= }} | ||
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===Crystal structure of human MCD=== | ===Crystal structure of human MCD=== | ||
| + | {{ABSTRACT_PUBMED_23791943}} | ||
| + | ==Disease== | ||
| + | [[http://www.uniprot.org/uniprot/DCMC_HUMAN DCMC_HUMAN]] Malonic aciduria. Defects in MLYCD are the cause of malonyl-CoA decarboxylase deficiency (MLYCD deficiency) [MIM:[http://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. | ||
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| + | ==Function== | ||
| + | [[http://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. | ||
==About this Structure== | ==About this Structure== | ||
[[2ygw]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2YGW OCA]. | [[2ygw]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2YGW OCA]. | ||
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| + | ==Reference== | ||
| + | <ref group="xtra">PMID:023791943</ref><references group="xtra"/><references/> | ||
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Malonyl-CoA decarboxylase]] | [[Category: Malonyl-CoA decarboxylase]] | ||
Revision as of 14:13, 10 July 2013
Contents |
Crystal structure of human MCD
Template:ABSTRACT PUBMED 23791943
Disease
[DCMC_HUMAN] Malonic aciduria. Defects in MLYCD are the cause of malonyl-CoA decarboxylase deficiency (MLYCD deficiency) [MIM:248360]. MLYCD deficiency is an autosomal recessive disease characterized by abdominal pain, chronic constipation, episodic vomiting, metabolic acidosis and malonic aciduria.
Function
[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.
About this Structure
2ygw is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Froese DS, Forouhar F, Tran TH, Vollmar M, Kim YS, Lew S, Neely H, Seetharaman J, Shen Y, Xiao R, Acton TB, Everett JK, Cannone G, Puranik S, Savitsky P, Krojer T, Pilka ES, Kiyani W, Lee WH, Marsden BD, von Delft F, Allerston CK, Spagnolo L, Gileadi O, Montelione GT, Oppermann U, Yue WW, Tong L. Crystal structures of malonyl-coenzyme a decarboxylase provide insights into its catalytic mechanism and disease-causing mutations. Structure. 2013 Jul 2;21(7):1182-92. doi: 10.1016/j.str.2013.05.001. Epub 2013, Jun 20. PMID:23791943 doi:10.1016/j.str.2013.05.001
