HIBADH antibody (AA 251-336) (AbBy Fluor® 750)

Catalog No. ABIN5004614

Product Details anti-HIBADH Antibody (AbBy Fluor® 750)

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

Binding Specificity: AA 251-336

Reactivity: Human, Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This HIBADH antibody is conjugated to AbBy Fluor® 750

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

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 (HIBADH Products)

Synonyms: NS5ATP1 antibody, 6430402H10Rik antibody, AI265272 antibody, hibadh antibody, zgc:66262 antibody, wu:fb07f02 antibody, zgc:100804 antibody, 3-hydroxyisobutyrate dehydrogenase antibody, 3-hydroxyisobutyrate dehydrogenase b antibody, 3-hydroxyisobutyrate dehydrogenase S homeolog antibody, 3-hydroxyisobutyrate dehydrogenase a antibody, HIBADH antibody, Hibadh antibody, hibadhb antibody, hibadh.S antibody, hibadha antibody

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