HIBADH (3-hydroxyisobutyrate dehydrogenase) is a 336aa 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.
Applications:Suitable for use in Western Blot.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:1000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.3, 0.05% sodium azide.
Specificity：Recognizes human HIBADH. Species Crossreactivity: mouse and rat
Isotype：IgG
Calc Applications Abbrev：WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to human HIBADH.
