HIBADH anticorps (AA 251-336) (HRP)

N° du produit ABIN1711051

Cet anticorps Lapin Polyclonal détecte spécifiquement HIBADH dans WB, ELISA, IHC (p) et IHC (fro). Il présente une réactivité envers Humain et Souris.

Aperçu rapide pour HIBADH anticorps (AA 251-336) (HRP) (ABIN1711051)

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

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp HIBADH est conjugé à/à la HRP

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

Détail du produit anti-HIBADH anticorps (HRP)

Épitope: AA 251-336

Réactivité croisée: Humain, Souris

Homologie: Rat,Cow,Sheep,Horse

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human HIBADH

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

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.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Conseil sur la manipulation: Do NOT add Sodium Azide! Use of Sodium Azide will inhibit enzyme activity of horseradish peroxidase.

Stock: -20 °C

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

Date de péremption: 12 months

Détails sur HIBADH

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

Autre désignation: HIBADH

Sujet:

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.

ID gène: 11112