Product Description

MCCA Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES15013-50, ES15013-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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],

Reactivity: Human, Mouse, Rat

Host: Rabbit

Applications: WB, IHC

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from human MCCA AA range: 620-670

Isotype: IgG

Target: MCCA

Specificity: This antibody detects endogenous levels of MCCA at Human/Mouse/Rat

Dilution: WB 1:500-2000, IHC-p 1:50-300

Purification: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Molecular Weight (Da): 80kD

Fields: Valine, leucine and isoleucine degradation, Metabolic pathways

Function: 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

Subcellular Location: Mitochondrion matrix

Storage and Stability: -20°C/1 year

Gene Name: MCCC1 MCCA

Protein Name: MCCA

Mouse Gene ID: 72039

Mouse SwissProt Number: Q99MR8

Rat Gene ID: 294972

Rat SwissProt Number: Q5I0C3

Research Use Only