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The antibody against IVD was raised in rabbit using the Recombinant Human Isovaleryl-CoA dehydrogenase, mitochondrial protein (139-241AA) 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, IHC.
The antibody against IVD was raised in rabbit using the Recombinant Human Isovaleryl-CoA dehydrogenase, mitochondrial protein (139-241AA) 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, IHC.
$299.00
| Cat.No | ADC-03514A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | IVD |
| Target Synonyms | ACAD2 antibody; FLJ12715 antibody; FLJ34849 antibody; Isovaleryl CoA dehydrogenase antibody; Isovaleryl CoA dehydrogenase, mitochondrial antibody; Isovaleryl Coenzyme A dehydrogenase antibody; Isovaleryl-CoA dehydrogenase antibody; IVD antibody; IVD_HUMAN antibody; mitochondrial antibody | 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 |
| Storage | Upon receipt |
| Immunogen Description | Recombinant Human Isovaleryl-CoA dehydrogenase, mitochondrial protein (139-241AA) | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | P26440 |
Uniprot Id
P26440
Target Species
Human
Target Name
IVD
Target Full Name
Isovaleryl-CoA dehydrogenase, mitochondrial
Target Function
Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway. To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA.
Target Involvement
Isovaleric acidemia (IVA)
Target Subcellular Location
Mitochondrion matrix.
Target Protein Families
Acyl-CoA dehydrogenase family
Target Synonyms
ACAD2; FLJ12715; FLJ34849; Isovaleryl CoA dehydrogenase; Isovaleryl CoA dehydrogenase, mitochondrial ; Isovaleryl Coenzyme A dehydrogenase; Isovaleryl-CoA dehydrogenase; IVD; IVD_HUMAN; mitochondrial
Target Background
Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants have been found for this gene.
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