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The antibody against IVD was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 33-160 of human IVD(NP_002216.3) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
The antibody against IVD was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 33-160 of human IVD(NP_002216.3) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
| Cat.No | ADA-08100A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | IVD |
| Target Synonyms | IVDH; ACAD2; IVD | Form | Liquid |
| Species Reactivity | Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.05% proclin300, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Mouse heart | Application | ELISA, WB |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 33-160 of human IVD(NP_002216.3). | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | HSLLPVDDAINGLSEEQRQLRQTMAKFLQEHLAPKAQEIDRSNEFKNLREFWKQLGNLGVLGITAPVQYGGSGLGYLEHVLVMEEISRASGAVGLSYGAHSNLCINQLVRNGNEAQKEKYLPKLISGE | 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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