Molecular weight (DA)
60kD
Immunogen
Synthesized peptide derived from human MCCB AA range: 204-254
Specificity
This antibody detects endogenous levels of MCCB at Human/Mouse/Rat
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.108% sodium azide.
Dilution rate
WB 1:500-2000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
This gene encodes the small 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],
Function
catalytic activity:ATP + 3-methylcrotonoyl-CoA + HCO(3)(-) = ADP + phosphate + 3-methylglutaconyl-CoA.,disease:Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 2 (MCC2 deficiency) [MIM:210210]. MCC2 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:Belongs to the accD/PCCB family.,similarity:Contains 1 carboxyltransferase domain.,subunit:Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits.,
Other name
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta)
Fields
>>Valine, leucine and isoleucine degradation;>>Metabolic pathways
Human protein sequence Database
Q9HCC0
Mouse protein sequence database
Q3ULD5
Rat protein sequence database
Q5XIT9
Cellular localization
Mitochondrion matrix .
Tissue expression
Testis,Uterus,
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