Fumarase Rabbit Polyclonal Antibody
CAT#: AP21256AF-N
Fumarase rabbit polyclonal antibody, Azide Free
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CNY 3,402.00
货期*
5周
规格
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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), to prepare an insoluble immuno-affinity adsorbent, for labelling with a marker of the customer’s own choice. 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 State: Azide Free State: Lyophilized hyperimmune IgG fraction |
Reconstitution Method | Restore by adding 1.0 ml of sterile distilled water |
Concentration | lot specific |
Purification | Ammonium Sulphate Precipitation and Ion Exchange Chromatography |
Conjugation | Unconjugated |
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 | |
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 |
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