FH Antikörper (Biotin)

Produktnummer ABIN568201

Dieses Kaninchen Polyklonal-Antikörper erkennt spezifisch FH in WB, IF, IP, EIA, ID, RIA und DB. Er zeigt eine Reaktivität gegenüber Schwein.

Kurzübersicht für FH Antikörper (Biotin) (ABIN568201)

Target: FH (Fumarate Hydratase (FH))

Reaktivität: Schwein

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser FH Antikörper ist konjugiert mit Biotin

Applikation: Western Blotting (WB), Immunofluorescence (IF), Immunoprecipitation (IP), Enzyme Immunoassay (EIA), Immunodiffusion (ID), Radioimmunoassay (RIA), Dot Blot (DB)

Produktdetails anti-FH Antikörper (Biotin)

Produktmerkmale: Molar Ratio: Biotin/IgG ~7.6

Aufreinigung: Ammonium Sulphate Precipitation and Ion Exchange Chromatography

Immunogen: Fumarase is isolated and purified from Porcine heart Freund’s complete adjuvant is used in the first step of the immunization procedure.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Rekonstitution: Restore by adding 1.0 mL of sterile distilled water

Konzentration: 10.0 mg/mL

Buffer: PBS, pH 7.2 without preservatives and foreign proteins

Konservierungsmittel: Without preservative

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: Store the antibody lyophilized at 2-8 °C and reconstituted at 2-8 °C for one week or (in aliquots) at -20 °C for longer. If a slight precipitation occurs upon storage, this should be removed by centrifugation.

Details zu FH

Target: FH (Fumarate Hydratase (FH))

Andere Bezeichnung: Fumarase

Hintergrund: Defects in FH are the cause of fumarase deficiency (FHD) [MIM:606812], also known as fumaricaciduria. FHD is characterized by progressive encephalopathy, developmental delay, hypotonia, cerebral atrophy and lactic and pyruvic acidemia. Defects in FH are the cause of multiple cutaneous and uterine leiomyomata (MCUL1) [MIM:150800]. MCUL1 is an autosomal dominant condition in which affected individuals develop benign smooth muscle tumors (leiomyomata) of the skin. Affected females also usually develop leiomyomata of the uterus (fibroids). Defects in FH are the cause of hereditary leiomyomatosis and renal cell cancer (HLRCC) [MIM:605839].Synonyms: HLRCC, LRCC, MCL, MCUL1, mitochondrial Fumarate hydratase

Gen-ID: 9823

UniProt: P10173