免疫原
Synthesized peptide derived from human DCMC AA range: 243-293
特异性
This antibody detects endogenous levels of DCMC at Human/Mouse/Rat
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺（Immunogen）
The antibody was affinity-purified from mouse serum by affinity-chromatography using specific immunogen.
背景（Background）
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],
功能
catalytic activity:Malonyl-CoA = acetyl-CoA + CO(2).,disease: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: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.,pathway:Metabolic intermediate biosynthesis; acetyl-CoA biosynthesis; acetyl-CoA from malo
Fields
>>beta-Alanine metabolism;>>Propanoate metabolism;>>Metabolic pathways;>>Peroxisome;>>AMPK signaling pathway;>>Alcoholic liver disease
细胞定位
Cytoplasm . Mitochondrion matrix . Peroxisome . Peroxisome matrix . Enzymatically active in all three subcellular compartments. .
组织表达
Expressed in fibroblasts and hepatoblastoma cells (at protein level). Expressed strongly in heart, liver, skeletal muscle, kidney and pancreas. Expressed in myotubes. Expressed weakly in brain, placenta, spleen, thymus, testis, ovary and small intestine.
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