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The antibody against HIBCH was raised in rabbit using the Human HIBCH 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 HIBCH was raised in rabbit using the Human HIBCH as the immunogen. This antibody exists as a non-conjugated isotype IgG, Antigen affinity purified. This antibody has been validated on ELISA, WB, IHC.
$600.00
| Cat.No | ADC-48350A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | HIBCH |
| Form | Liquid | Species Reactivity | Human, Mouse, Rat |
| Isotype | IgG | Storage Buffer | 50% Glycerol, Avoid freeze / thaw cycles., PBS with 0.1% Sodium Azide |
| Purification Method | Antigen affinity purified | Conjugate | Non-conjugated |
| Application | ELISA, IHC, WB | Storage | Upon receipt |
| Immunogen Description | Human HIBCH | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | Q6NVY1 |
Uniprot Id
Q6NVY1
Target Species
Human
Target Name
HIBCH
Target Full Name
3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
Target Function
Hydrolyzes 3-hydroxyisobutyryl-CoA (HIBYL-CoA), a saline catabolite. Has high activity toward isobutyryl-CoA. Could be an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Also hydrolyzes 3-hydroxypropanoyl-CoA.
Target Involvement
3-hydroxyisobutryl-CoA hydrolase deficiency (HIBCHD)
Target Subcellular Location
Mitochondrion.
Target Protein Families
Enoyl-CoA hydratase/isomerase family
Target Tissue Specificity
Highly expressed in liver and kidney, also detected in heart, muscle and brain (at protein level). Not detected in lung.
Target Synonyms
3 hydroxyisobutyryl Coenzyme A hydrolase ; 3 hydroxyisobutyryl Coenzyme A hydrolase; mitochondrial ; 3-hydroxyisobutyryl-CoA hydrolase; 3-hydroxyisobutyryl-coenzyme A hydrolase; BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE; HIB CoA hydrolase; HIB-CoA hydrolase; HIBCH; HIBCH_HUMAN; HIBCoA hydrolase; HIBYL CoA H; HIBYL CoAH; HIBYL-CoA-H; HIBYLCoA H; HIBYLCoAH; mitochondrial
Target Background
This gene encodes the enzyme responsible for hydrolysis of both HIBYL-CoA and beta-hydroxypropionyl-CoA. Mutations in this gene have been associated with 3-hyroxyisobutyryl-CoA hydrolase deficiency. Alternative splicing results in multiple transcript variants.
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