1
/
of
1
Upingbio
SKU:YP-Ab-11767-100UL
MCCA rabbit pAb
MCCA rabbit pAb
Regular price
$0.00 USD
Regular price
Sale price
$0.00 USD
Unit price
/
per
Shipping calculated at checkout.
Couldn't load pickup availability
- Gene Name: MCCC1 MCCA
- Immunogen: Synthesized peptide derived from human MCCA AA range: 620-670
- Specificity: This antibody detects endogenous levels of MCCA at Human/Mouse/Rat
- Composition: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- Source: Polyclonal, Rabbit,IgG
- Dilution ratio: WB 1:500-2000;IHC-p 1:50-300
- 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
- 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],
- 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
- Species: Human; Mouse;Rat
- Range: WB;IHC
- Protein: MCCA
Share
