Abbreviation: Frataxin;FXN
Target Synonym: Frataxin;mitochondrial;Friedreich ataxia protein;FXN;FRDA;X25;Frataxin intermediate form
Target Species: Human
Expression Host: HEK293 Cells
Fusion Tag: C-His
UNIProt ID: Q16595
Background: Frataxin intermediate form can cleave by mitochondrial processing peptidase(MPP) to the mature frataxin. Frataxin is a nuclear-encoded mitochondrial protein highly conserved in prokaryotes and eukaryotes. Its deficiency was initially described as the phenotype of Friedreich's ataxia, an autosomal recessive disease in humans. Although several functions have been described for frataxin, that is, involvement in Fe-S cluster and heme synthesis, energy conversion and oxidative phosphorylation, iron handling and response to oxidative damage, its precise function remains unclear. Although there is a general consensus on the participation of frataxin in the maintenance of cellular iron homeostasis and in iron metabolism, this protein may have other specific functions in different tissues and organisms.
Sequence: Leu42-Ala210
Purity: > 95 % as determined by reducing SDS-PAGE.
Formulation: Lyophilized from sterile PBS, pH 7.4.
Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization.
Please refer to the specific buffer information in the printed manual.
Endotoxin: Please contact us for more information.
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