Fumarase Rabbit Polyclonal Antibody

CAT#: AP21256BT-N

Fumarase rabbit polyclonal antibody, Biotin


USD 410.00

2 Weeks*

Size
    • 1 ml

Product Images

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
Reference Data

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*Delivery time may vary from web posted schedule. Occasional delays may occur due to unforeseen complexities in the preparation of your product. International customers may expect an additional 1-2 weeks in shipping.