• MCCB rabbit pAb
1 1

Upingbio

YP-Ab-07994-100UL

MCCB rabbit pAb

MCCB rabbit pAb

$0.00 USD
$0.00 USD
Sale Sold out
Shipping calculated at checkout.
Size
  • Gene Name: MCCC2 MCCB
  • Immunogen: Synthesized peptide derived from human MCCB AA range: 204-254
  • Specificity: This antibody detects endogenous levels of MCCB at Human/Mouse/Rat
  • Composition: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.108% sodium azide.
  • Source: Polyclonal, Rabbit,IgG
  • Dilution ratio: WB 1:500-2000
  • Purification process: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
  • Concentration: 1 mg/ml
  • Storage: -20°C/1 year
  • Other Names: 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)
  • 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.,
  • Species: Human; Mouse;Rat
  • Range: WB
  • Protein: MCCB
  • DA: 60kD
View full details