MCCA rabbit pAb
- Catalog No.:YT7132
- Applications:WB;IHC
- Reactivity:Human;Mouse;Rat
- Target:
- MCCA
- Fields:
- >>Valine, leucine and isoleucine degradation;>>Metabolic pathways
- Gene Name:
- MCCC1 MCCA
- Protein Name:
- MCCA
- Human Gene Id:
- 56922
- Human Swiss Prot No:
- Q96RQ3
- Mouse Gene Id:
- 72039
- Mouse Swiss Prot No:
- Q99MR8
- Rat Gene Id:
- 294972
- Rat Swiss Prot No:
- Q5I0C3
- Immunogen:
- Synthesized peptide derived from human MCCA AA range: 620-670
- Specificity:
- This antibody detects endogenous levels of MCCA at Human/Mouse/Rat
- Formulation:
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- Source:
- Polyclonal, Rabbit,IgG
- Dilution:
- WB 1:500-2000;IHC 1:50-300
- Purification:
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
- Concentration:
- 1 mg/ml
- Storage Stability:
- -15°C to -25°C/1 year(Do not lower than -25°C)
- Molecular Weight(Da):
- 80kD
- 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
- Subcellular Location:
- Mitochondrion matrix .
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- Antibody-FAQs
- Products Images
- Western blot analysis of lysates from SH-SY5Y cells, primary antibody was diluted at 1:1000, 4°over night
- Immunohistochemical analysis of paraffin-embedded human Squamous cell carcinoma of lung. 1, Antibody was diluted at 1:200(4° overnight). 2, Tris-EDTA,pH9.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 45min).