- Antibody type:Primary
- Antigen name:HIBADH
- Clonality:Polyclonal
- Conjugation:Cy5®
- Host:Rabbit
- ImmunoChemistry:Yes
- ImmunoFluorescence:Yes
- Isotype:IgG
- Reactivity:Human,Mouse
- Western blot:Yes
- Environmentally Preferable:
- Epitope:251-336/336
- Form:Liquid
- Gene ID:11112
- Antigen synonyms:NS5ATP1|3HIDH_HUMAN|MGC40361|3 hydroxyisobutyrate dehydrogenase|EC 1,1,1,31|3 hydroxyisobutyrate dehydrogenase mitochondrial|3 hydroxy 2 methylpropanoate:NAD+ oxidoreductase
- Modification:Unmodified
- Storage buffer:Aqueous buffered solution containing 0,01 M TBS (pH 7,4) with 1% BSA, 0,03% Proclin300 and 50% Glycerol
- Molecular weight:32 kDa
- Storage temperature:Store at –20 °C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- Concentration:1 µg/µl
- Shipping temperature:4 °C
- Immunogen:KLH conjugated synthetic peptide derived from human HIBADH
- Tested applications:ICC
- Purification:Purified by Protein A
- Pack type:Vial
- Pk:100 µl
HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation 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 hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised 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.
Type: Primary
Antigen: HIBADH
Clonality: Polyclonal
Clone:
Conjugation: Cy5
Public Immunogen Range: 251-336/336
Host: Rabbit
Isotype: IgG
Reactivity: Human; Mouse
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