Recombinant Human Frataxin/FXN protein (His Tag) (PDMH100380)
For research use only.
| Synonyms | FRDA, FXN, Frataxin, Frataxin intermediate form, Friedreich ataxia protein, X25, mitochondrial |
| Species | Human |
| Expression Host | HEK293 Cells |
| Sequence | Leu42-Ala210 |
| Accession | Q16595 |
| Calculated Molecular Weight | 18.5 kDa |
| Observed Molecular Weight | 18 kDa |
| Tag | C-His |
| Bio-activity | Not validated for activity |
| Purity | > 90% as determined by reducing SDS-PAGE. |
| Endotoxin | < 1.0 EU/mg of the protein as determined by the LAL method |
| Storage | Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months. |
| Shipping | This product is provided as lyophilized powder which is shipped with ice packs. |
| Formulation | Lyophilized from a 0.2 μm filtered solution in PBS with 5% Trehalose and 5% Mannitol. |
| Reconstitution | It is recommended that sterile water be added to the vial to prepare a stock solution of 0.5 mg/mL. Concentration is measured by UV-Vis. |
| 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. |
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