HIBADH antibody (AA 251-336) (HRP)

Catalog No. ABIN1711051

This Rabbit Polyclonal antibody specifically detects HIBADH in WB, IHC (p) and IHC (fro). It exhibits reactivity toward Human.

Quick Overview for HIBADH antibody (AA 251-336) (HRP) (ABIN1711051)

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

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This HIBADH antibody is conjugated to HRP

Application: Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Product Details anti-HIBADH Antibody (HRP)

Binding Specificity: AA 251-336

Purpose: HIBADH Polyclonal Antibody, HRP Conjugated

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: WB(1:300-5000),

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, 0.02 % 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.

Handling Advice: Do NOT add Sodium Azide! Use of Sodium Azide will inhibit enzyme activity of horseradish peroxidase.

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