HIBADH Polyclonal Antibody, Cy3 Conjugated
Applications
Reactivity
Predicted Reactivity
| Overview | |
| Catalog # | bs-15483R-Cy3 |
| Product Name | HIBADH Polyclonal Antibody, Cy3 Conjugated |
| Applications |
WB
IF(IHC-P)
IF(IHC-F)
IF(ICC)
|
| Reactivity | Human, Mouse |
| Predicted Reactivity | Rat, Cow, Sheep, Horse |
| Specifications | |
| Conjugation | Cy3 |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human HIBADH |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Store at -20C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles. |
| Target | |
| Gene ID | 11112 |
| Subcellular location | Cytoplasm |
| Synonyms | 3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase; 3 hydroxyisobutyrate dehydrogenase; 3 hydroxyisobutyrate dehydrogenase mitochondrial; EC 1.1.1.31; MGC40361; NS5ATP1; 3HIDH_HUMAN. |
| Background | HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7.0 and 10.0, with optimal activity between 8.8 and 9.0. It was previously hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls. |
| Application Dilution | |
| WB | 1:300-5000 |
| IF(IHC-P) | 1:50-200 |
| IF(IHC-F) | 1:50-200 |
| IF(ICC) | 1:50-200 |