Fumarase Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	ELISA, ID, IF, IP, R, WB
Recommend 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.
Reactivity	Porcine
Host	Rabbit
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	lot specific
Purification	Ammonium Sulphate Precipitation and Ion Exchange Chromatography
Conjugation	Biotin
Storage Condition	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.
Database Link	UniProt ID P10173
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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