4f0x
From Proteopedia
Contents |
Crystal structure of human Malonyl-CoA Decarboxylase (Peroxisomal Isoform)
Template:ABSTRACT PUBMED 23482565
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
4f0x is a 8 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
