HIBADH antibody (AA 251-336) (AbBy Fluor® 647)

Catalog No. ABIN1698221

The Rabbit Polyclonal anti-HIBADH antibody has been validated for WB, IF (cc) and IF (p). It is suitable to detect HIBADH in samples from Human and Mouse.

Quick Overview for HIBADH antibody (AA 251-336) (AbBy Fluor® 647) (ABIN1698221)

Target: HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))

Reactivity: Human, Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This HIBADH antibody is conjugated to AbBy Fluor® 647

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Product Details anti-HIBADH Antibody (AbBy Fluor® 647)

Binding Specificity: AA 251-336

Cross-Reactivity: Human, Mouse

Predicted Reactivity: Rat,Cow,Sheep,Horse

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human HIBADH

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for HIBADH

Target: HIBADH (3-hydroxyisobutyrate Dehydrogenase (HIBADH))

Alternative Name: Hibadh

Background:

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.

Gene ID: 11112