Malonyl-Coa Decarboxylase (MLYCD) Antibody – Rabbit Polyclonal

The product of this gene catalyzes the breakdown of malonyl-CoA to acetyl-CoA and carbon dioxide. Malonyl-CoA is an intermediate in fatty acid biosynthesis, and also inhibits the transport of fatty acyl CoAs into mitochondria. Consequently, the encoded protein acts to increase the rate of fatty acid oxidation. It is found in mitochondria, peroxisomes, and the cytoplasm. Mutations in this gene result in malonyl-CoA decarboyxlase deficiency. [provided by RefSeq, Jul 2008]

MLYCD: 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. Defects in MLYCD are the cause of malonyl-CoA decarboxylase deficiency (MLYCD deficiency). MLYCD deficiency is an autosomal recessive disease characterized by abdominal pain, chronic constipation, episodic vomiting, metabolic acidosis and malonic aciduria.

Protein type: Other Amino Acids Metabolism - beta-alanine; EC 4.1.1.9; Carbohydrate Metabolism - propanoate; Lyase

Chromosomal Location of Human Ortholog: 16q24

Cellular Component: peroxisomal matrix; mitochondrion; mitochondrial matrix; cytoplasm; peroxisome

Molecular Function: malonyl-CoA decarboxylase activity; receptor binding

Biological Process: positive regulation of fatty acid oxidation; acetyl-CoA biosynthetic process; cellular lipid metabolic process; fatty acid biosynthetic process

Disease: Malonyl-coa Decarboxylase Deficiency