免疫原
Synthesized peptide derived from human MCCA AA range: 620-670
特异性
This antibody detects endogenous levels of MCCA at Human/Mouse/Rat
来源
Polyclonal, Rabbit,IgG
组成（Formulation）
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
WB 1:500-2000;IHC-p 1:50-300
纯化工艺（Immunogen）
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
背景（Background）
This gene encodes the large subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq, Jul 2008],
功能
catalytic activity:ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.,cofactor:Biotin.,disease:Defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 1 (MCC1 deficiency) [MIM:210200]. MCC1 deficiency is an autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency.,pathway:Amino-acid degradation; L-leucine degradation; HMG-CoA from 3-isovaleryl-CoA: step 2/3.,similarity:Contains 1 ATP-grasp domain.,similarity:Contains 1 biotin carboxylation domain.,similarity:Contains 1 biotinyl-binding domain.,subunit:Probably a dodecamer composed of
基因名称（Gene Name）
MCCC1 MCCA
Fields
>>Valine, leucine and isoleucine degradation;>>Metabolic pathways
细胞定位
Mitochondrion matrix .
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