HIBADH Antikörper (AA 251-336) (AbBy Fluor® 488)

Produktnummer ABIN1694580

Dieser Anti-HIBADH Antikörper ist ein Kaninchen Polyklonal Antikörper zur Detektion von HIBADH in WB, IF (cc) und IF (p). Geeignet für Human und Maus.

Kurzübersicht für HIBADH Antikörper (AA 251-336) (AbBy Fluor® 488) (ABIN1694580)

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

Reaktivität: Human, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser HIBADH Antikörper ist konjugiert mit AbBy Fluor® 488

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

Produktdetails anti-HIBADH Antikörper (AbBy Fluor® 488)

Bindungsspezifität: AA 251-336

Kreuzreaktivität: Human, Maus

Homologie: Rat,Cow,Sheep,Horse

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human HIBADH

Isotyp: IgG

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu HIBADH

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

Andere Bezeichnung: Hibadh

Hintergrund:

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.

Gen-ID: 11112