HIBADH Antikörper (AA 251-336)

Produktnummer ABIN1713536

Produktdetails anti-HIBADH Antikörper

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

Bindungsspezifität: AA 251-336

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)

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 (HIBADH Produkte)

Synonyme: NS5ATP1 antikoerper, 6430402H10Rik antikoerper, AI265272 antikoerper, hibadh antikoerper, zgc:66262 antikoerper, wu:fb07f02 antikoerper, zgc:100804 antikoerper, 3-hydroxyisobutyrate dehydrogenase antikoerper, 3-hydroxyisobutyrate dehydrogenase b antikoerper, 3-hydroxyisobutyrate dehydrogenase S homeolog antikoerper, 3-hydroxyisobutyrate dehydrogenase a antikoerper, HIBADH antikoerper, Hibadh antikoerper, hibadhb antikoerper, hibadh.S antikoerper, hibadha antikoerper

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