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Antibody Product Overview
| ELK.No | ES15012 |
| Product name | MCCB rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB |
| 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) |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 64087 |
| Human Swiss-Prot | Q9HCC0 |
| Source | Rabbit |
| Isotype | IgG |
| Target | MCCB |
| Fields | >>Valine, leucine and isoleucine degradation;>>Metabolic pathways |
| Gene name | MCCC2 MCCB |
| Protein name | MCCB |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 78038 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q3ULD5 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 361884 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | Q5XIT9 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human MCCB AA range: 204-254 |
| Specificity | This antibody detects endogenous levels of MCCB at Human/Mouse/Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.108% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | |
| Observed band (KD) | 60kD |
| 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., |
| Subcellular location | Mitochondrion matrix . |
| Expression | Testis,Uterus, |
Additional Images
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Western blot analysis of lysates from 3T3 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES15012-100
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