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Anti-HIBADH Rabbit pAb (100 μl)
| Reactivity: | M,R |
| Applications: | WB |
| Host Species: | Rabbit |
| Clonality: | Polyclonal |
| Full Name: | HIBADH rabbit polyclonal antibody |
Gene Name: | 3-hydroxyisobutyrate dehydrogenase |
Synonyms: | HIBADH, mitochondrial, NS5ATP1, EC 1.1.1.31, MGC40361 |
Immunogen: | Recombinant protein corresponding to Mouse HIBADH |
Isotype: | IgG |
Purity: | Affinity purification |
Predicted MW. | 35 kDa |
Observed MW. | 30 kDa |
Uniprot ID: | Q99L13, P29266 |
Product Usage Information
Applications | Species | Dilution | Positive Sample |
WB | Mouse, Rat | 1: 500-1: 1000 | liver, kidney |
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.
Images
| Western blot analysis of HIBADH (GB111712) at dilution of 1: 500 |
Storage
| Storage | Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| Storage Buffer | PBS with 0.02%sodium azide,100 μg/ml BSA and 50% glycerol. |
