HIBADH Polyclonal Antibody
Applications
Reactivity
Predicted Reactivity
| Overview | |
| Catalog # | bs-15483R-100uL |
| Product Name | HIBADH Polyclonal Antibody |
| Applications |
WB
ELISA
IHC-P
IHC-F
IF(IHC-P)
IF(IHC-F)
IF(ICC)
|
| Reactivity | Human, Mouse |
| Predicted Reactivity | Rat, Cow, Sheep, Horse |
| Specifications | |
| Conjugation | Unconjugated |
| Host | Rabbit |
| Source | KLH conjugated synthetic peptide derived from human HIBADH |
| Immunogen Range | 251-336/336 |
| Clonality | Polyclonal |
| Isotype | IgG |
| Concentration | 1ug/ul |
| Purification | Purified by Protein A. |
| Storage Buffer | 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Storage Condition | Shipped at 4C. Store at -20C for one year. 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 |
| ELISA | 1:500-1000 |
| IHC-P | 1:200-400 |
| IHC-F | 1:100-500 |
| IF(IHC-P) | 1:50-200 |
| IF(IHC-F) | 1:50-200 |
| IF(ICC) | 1:50-200 |
Frequently asked questions
Which applications is HIBADH Polyclonal Antibody (bs-15483R) validated for, and at what dilution?
bs-15483R is validated for WB (1:300-5000); ELISA (1:500-1000); IHC-P (1:200-400); IHC-F (1:100-500); IF(IHC-P) (1:50-200); IF(IHC-F) (1:50-200); IF(ICC) (1:50-200); ICC (1:100-500). These are recommended starting dilutions; optimal dilutions should be determined by the end user.
Which species does HIBADH Polyclonal Antibody (bs-15483R) react with?
Tested reactivity: Human, Mouse. Predicted reactivity (expected from sequence homology but not yet tested): Rat, Cow, Sheep, Horse.
How should bs-15483R be stored?
Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles. Storage buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
What immunogen was used to raise HIBADH Polyclonal Antibody (bs-15483R)?
KLH conjugated synthetic peptide derived from human HIBADH Immunogen range: 251-336/336.