Anti-Frataxin / FXN, clone FXN/2124

Artikelnummer Größe Datenblatt Manual SDB Lieferzeit Menge Preis
NSJ-V3992SAF-100UG 100 µg - -

3 - 10 Werktage*

752,00 €
 
1 mg/ml in 1X PBS, BSA free, sodium azide free. Frataxin is a nuclear-encoded mitochondrial... mehr
Produktinformationen "Anti-Frataxin / FXN, clone FXN/2124"
1 mg/ml in 1X PBS, BSA free, sodium azide free. Frataxin is a nuclear-encoded mitochondrial protein implicated in Friedreich's ataxia (FRDA), a human autosomal recessive neurodegenerative disease. Lack of Frataxin causes iron to accumulate in the mitochondrial matrix suggesting that Frataxin is involved in mitochondrial iron homeostasis and possibly in iron transport. Frataxin has an alpha-beta fold consisting of two helices flanking an antiparallel beta sheet. Protein function: Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe(2+) to Fe(3+), the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization, however, the physiological relevance is unsure as reports are conflicting and the function has only been shown using heterologous overexpression systems. Modulates the RNA-binding activity of ACO1. [The UniProt Consortium]
Schlagworte: Anti-FXN, Anti-FRDA, EC=1.16.3.1, Frataxin Antibody / FXN
Hersteller: NSJ Bioreagents
Hersteller-Nr: V3992SAF

Eigenschaften

Anwendung: IHC (paraffin)
Antikörper-Typ: Monoclonal
Klon: FXN/2124
Konjugat: No
Wirt: Mouse
Spezies-Reaktivität: human
Immunogen: Amino acids 57-210 from the human protein
Format: Purified

Handhabung & Sicherheit

Lagerung: +4°C
Versand: +4°C (International: +4°C)
Achtung
Nur für Forschungszwecke und Laboruntersuchungen: Nicht für die Anwendung im oder am Menschen!
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