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The antibody against MCCC1 was raised in rabbit using the Recombinant Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial protein (1-200AA) as the immunogen. This antibody exists as a non-conjugated isotype IgG, purified by protein G with a purity greater than 95%. This antibody has been validated on ELISA, IF.
The antibody against MCCC1 was raised in rabbit using the Recombinant Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial protein (1-200AA) as the immunogen. This antibody exists as a non-conjugated isotype IgG, purified by protein G with a purity greater than 95%. This antibody has been validated on ELISA, IF.
$299.00
| Cat.No | ADC-04679A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | MCCC1 |
| Form | Liquid | Species Reactivity | Human |
| Isotype | IgG | Storage Buffer | 0.01M PBS, 0.03% Proclin 300; Constituents: 50% Glycerol, PH 7.4 |
| Purification Method | >95%, Protein G purified | Conjugate | Non-conjugated |
| Application | ELISA, IF | Storage | Upon receipt |
| Immunogen Description | Recombinant Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial protein (1-200AA) | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | Q96RQ3 |
Uniprot Id
Q96RQ3
Target Species
Human
Target Name
MCCC1
Target Full Name
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Target Function
Biotin-attachment 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.
Target Involvement
3-methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D)
Target Subcellular Location
Mitochondrion matrix.
Target Synonyms
MCCC1; MCCAMethylcrotonoyl-CoA carboxylase subunit alpha; mitochondrial; MCCase subunit alpha; EC 6.4.1.4; 3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
Target 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.
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