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The antibody against BCKDHA was raised in rabbit using the Recombinant Human 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial protein (268-407AA) 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, WB, IHC.
The antibody against BCKDHA was raised in rabbit using the Recombinant Human 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial protein (268-407AA) 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, WB, IHC.
$299.00
| Cat.No | ADC-06152A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | BCKDHA |
| Target Synonyms | alpha polypeptide (maple syrup urine disease) antibody; Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain antibody; MSU antibody; MSUD1 antibody; ODBA_HUMAN antibody, mitochondrial antibody; 2-oxoisovalerate dehydrogenase subunit alpha | 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, IHC, WB |
| Storage | Upon receipt |
| Immunogen Description | Recombinant Human 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial protein (268-407AA) | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | P12694 |
Uniprot Id
P12694
Target Species
Human
Target Name
BCKDHA
Target Full Name
2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Target Function
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Target Involvement
Maple syrup urine disease 1A (MSUD1A)
Target Subcellular Location
Mitochondrion matrix.
Target Protein Families
BCKDHA family
Target Synonyms
Branched chain alpha keto acid dehydrogenase E1 component alpha polypeptide; FLJ45695; OVD1A; 2 oxoisovalerate dehydrogenase (lipoamide); 2 oxoisovalerate dehydrogenase subunit alpha, mitochondrial ; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; BCKDE1A; BCKDH E1 alpha; BCKDH E1-alpha; BCKDHA; Branched chain alpha keto acid dehydrogenase E1 component alpha chain; Branched chain keto acid dehydrogenase E1 alpha polypeptide; Branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease); Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; MSU; MSUD1; ODBA_HUMAN
Target Background
The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.
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