HIBADH Antikörper (AA 251-336)

Produktnummer ABIN1713536

Der Kaninchen Polyklonal Anti-HIBADH-Antikörper wurde für WB, ELISA, IF (cc), IF (p), IHC (p), IHC (fro) und ICC validiert. Er ist geeignet, HIBADH in Proben von Human und Maus zu detektieren.

Kurzübersicht für HIBADH Antikörper (AA 251-336) (ABIN1713536)

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

Reaktivität: Human, Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser HIBADH Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA, Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro)), Immunocytochemistry (ICC)

Produktdetails anti-HIBADH Antikörper

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: WB 1:300-5000
ELISA 1:500-1000
IHC-P 1:200-400
IHC-F 1:100-500
IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200
ICC 1:100-500

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % 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: 4 °C,-20 °C

Informationen zur Lagerung: Shipped at 4°C. Store at -20°C for one year. 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