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The antibody against MCCC2 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 284-563 of human MCCC2 (NP_071415.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, IF/ICC, ELISA.
The antibody against MCCC2 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 284-563 of human MCCC2 (NP_071415.1) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, IF/ICC, ELISA.
| Cat.No | ADA-00005A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | MCCC2 |
| Target Synonyms | MCCB; MCCCbeta; MCCC2 | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.02% sodium azide, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | HeLa, Mouse kidney, 22Rv1, HepG2, Mouse liver, U-251MG | Application | ELISA, WB, IF/ICC, IHC-P |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 284-563 of human MCCC2 (NP_071415.1). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q9HCC0 | Immunogen Sequence |
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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