Fumarase Rabbit Polyclonal Antibody

CAT#: AP21256BT-N

Fumarase rabbit polyclonal antibody, Biotin

Specifications

Product Data

• Applications: ELISA, ID, IF, IP, R, WB
• Recommended Dilution: This product is intended for use in precipitating and non-precipitating antibody-binding assays (such as e.g., ELISA and Western blotting and Immunofluorescence or Histochemical techniques).
  Working dilutions in non-precipitating antibody-binding techniques: 1/1,000-1/5,000.
• Reactivities: Porcine
• Host: Rabbit
• Isotype: IgG
• Clonality: Polyclonal
• Immunogen: Fumarase is isolated and purified from Porcine heart
  Freund’s complete adjuvant is used in the first step of the immunization procedure.
• Specificity: The antibody recognizes Fumarase from Porcine heart.
  The reagents were evaluated for potency, purity and specificity using most or all of the following techniques: Immunoelectrophoresis, Cross-Immunoelectrophoresis, single Radial Immunodiffusion (Ouchterlony), block titration, ELISA, Immunoblotting and Enzyme Inhibition.
  Cross-reactivities against enzymes of other sources may occur but have not been determined.
• Formulation: PBS, pH 7.2 without preservatives and foreign proteins
  Label: Biotin
  State: Lyophilized hyperimmune IgG fraction
  Molar radio: Biotin/IgG ~7.6
• Reconstitution Method: Restore by adding 1.0 ml of sterile distilled water
• Concentration: 10.0 mg/ml
• Purification: Ammonium Sulphate Precipitation and Ion Exchange Chromatography
• Conjugation: Biotin
• Background: 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