HIBADH Polyclonal Antibody, AbBy Fluor™ 750 Conjugated
Product Specifications
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.
Synonyms
3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase; 3 hydroxyisobutyrate dehydrogenase; 3 hydroxyisobutyrate dehydrogenase mitochondrial; EC 1.1.1.31; MGC40361; NS5ATP1; 3HIDH_HUMAN.
Gene ID
11112
Cellular Locus
Cytoplasm
Host
Rabbit
Cross Reactivity
Human, Mouse
Immunogen
251-336/336
Target
HIBADH
Clonality
Polyclonal
Isotype
IgG
Conjugation
AbBy Fluor™ 750
Source
KLH conjugated synthetic peptide derived from human HIBADH
Applications
WB, IF (IHC-P), IF (IHC-F), IF (ICC)
Purification
Purified by Protein A.
Excitation Emission
749nm/775nm
Concentration
1µg/µl
Dilution
WB (1:300-5000), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Gene ID URL
11112
Predicted Cross Reactivity
Rat, Cow, Sheep, Horse
Curated Selection
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