MCCC2 Antibody

Code CSB-PA013573GA01HU
Size $600
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Product Details

Uniprot No.
Target Names
Alternative Names
MCCC2 antibody; MCCBMethylcrotonoyl-CoA carboxylase beta chain antibody; mitochondrial antibody; MCCase subunit beta antibody; EC antibody; 3-methylcrotonyl-CoA carboxylase 2 antibody; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit antibody; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta antibody
Raised in
Species Reactivity
Human MCCC2
Immunogen Species
Homo sapiens (Human)
Purification Method
Antigen Affinity Purified
It differs from different batches. Please contact us to confirm it.
PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
Tested Applications
Troubleshooting and FAQs
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

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Target Background

Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.
Gene References into Functions
  1. Novel mutation in MCCC2 gene was identified in Chinese population. PMID: 25382614
  2. Mutation in 3-methylcrotonyl CoA carboxylase 2 gene is associated with 3-methylcrotonyl-CoA carboxylase deficiency. PMID: 22264772
  3. The amino-termini containing 20 amino acids (MCCbeta) were both necessary and sufficient for targeting. Structural requirements for mitochondrial import were defined by site-directed mutagenesis. PMID: 16023992
  4. The Kd value of soraphen A for the BC domains of human ACC1 and ACC2 is 1 nM. This high binding affinity is mainly due to the extensive interactions between soraphen A and the human biotin carboxylase domain PMID: 17876819
Involvement in disease
3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D)
Subcellular Location
Mitochondrion matrix.
Protein Families
AccD/PCCB family
Database Links

HGNC: 6937

OMIM: 210210

KEGG: hsa:64087

STRING: 9606.ENSP00000343657

UniGene: Hs.604789

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