FH anticorps (Biotin)

N° du produit ABIN568201

Cet anticorps Lapin Polyclonal détecte spécifiquement FH dans WB, IF, IP, EIA, ID, RIA et DB. Il présente une réactivité envers Porc.

Aperçu rapide pour FH anticorps (Biotin) (ABIN568201)

Antigène: FH (Fumarate Hydratase (FH))

Reactivité: Porc

Hôte: Lapin

Clonalité: Polyclonal

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

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

Détail du produit anti-FH anticorps (Biotin)

Attributs du produit: Molar Ratio: Biotin/IgG ~7.6

Purification: Ammonium Sulphate Precipitation and Ion Exchange Chromatography

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

Isotype: IgG

Information d'application

Indications d'application: Optimal working dilution should be determined by the investigator.

Restrictions: For Research Use only

Stockage

Format: Liquid

Reconstitution: Restore by adding 1.0 mL of sterile distilled water

Concentration: 10.0 mg/mL

Buffer: PBS, pH 7.2 without preservatives and foreign proteins

Agent conservateur: Without preservative

Stock: 4 °C/-20 °C

Stockage commentaire: 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.

Détails sur FH

Antigène: FH (Fumarate Hydratase (FH))

Autre désignation: Fumarase

Sujet: 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

ID gène: 9823

UniProt: P10173