HIBADH anticorps (AA 251-336) (Biotin)

N° du produit ABIN1700034

Cet anticorps anti-HIBADH est un anticorps Lapin Polyclonal détectant HIBADH dans WB, ELISA, IHC (p) et IHC (fro). Adapté pour Humain et Souris.

Aperçu rapide pour HIBADH anticorps (AA 251-336) (Biotin) (ABIN1700034)

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

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

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

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

Détail du produit anti-HIBADH anticorps (Biotin)

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.

Stock: -20 °C

Stockage commentaire: Store at -20°C for 12 months.

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