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The antibody against HIBCH was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 33-330 of human HIBCH (NP_055177.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, ELISA.
The antibody against HIBCH was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 33-330 of human HIBCH (NP_055177.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, ELISA.
| Cat.No | ADA-05989A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | HIBCH |
| Target Synonyms | HIBYLCOAH; HIBCH | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Mouse heart, Rat heart | Application | ELISA, WB, IF/ICC |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 33-330 of human HIBCH (NP_055177.2). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q6NVY1 | Immunogen Sequence |
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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