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The antibody against MCCC2 was raised in rabbit using the Recombinant Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial protein (284-563AA) as the immunogen. This antibody exists as a non-conjugated isotype IgG, Antigen affinity purified. This antibody has been validated on ELISA, WB, IHC.
The antibody against MCCC2 was raised in rabbit using the Recombinant Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial protein (284-563AA) as the immunogen. This antibody exists as a non-conjugated isotype IgG, Antigen affinity purified. This antibody has been validated on ELISA, WB, IHC.
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Immunohistochemistry of paraffin-embedded human pancreatic cancer using MCCC2 Antibody at dilution of 1:1002
| Cat.No | ADC-16845A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | MCCC2 |
| Form | Liquid | Species Reactivity | Human, Mouse |
| Isotype | IgG | Storage Buffer | 50% Glycerol, PBS with 0.02% sodium azide, pH7.3. |
| Purification Method | Antigen affinity purified | Conjugate | Non-conjugated |
| Application | ELISA, IHC, WB | Storage | Upon receipt |
| Immunogen Description | Recombinant Human Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial protein (284-563AA) | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | Q9HCC0 |
Uniprot Id
Q9HCC0
Target Species
Human
Target Name
MCCC2
Target Full Name
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
Target Function
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.
Target Involvement
3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D)
Target Subcellular Location
Mitochondrion matrix.
Target Protein Families
AccD/PCCB family
Target Synonyms
MCCC2; MCCBMethylcrotonoyl-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
Target 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. Alternative splicing results in multiple transcript variants encoding distinct isoforms.
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