View larger

Recombinant Human Frataxin, mitochondrial(FxN)

CSB-EP613687HU(A4)b1

Recombinant Human Frataxin, mitochondrial(FxN)

See Below for Detailed Description

More details

CAD$709.00

More info

>Several Other Sizes Are Also Available. Please Inquire. Default Size: 200ug

Updated Date: Stock Protein updated on 20171018

Research areas: Tags & Cell Markers

Target / Protein: FxN

Biologically active: Not Tested

Expression system: E.coli

Species of origin: Homo sapiens (Human)

Delivery time: 3-7 business days

Uniprot ID: Q16595

AA Sequence: MWTLGRRAVAGLLASPSPAQAQTLTRVPRPAELAPLCGRRGLRTDIDATCTPRRASSNQRGLNQIWNVKKQSVYLMNLRKSGTLGHPGSLDETTYERLAEETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDLSSLAYSGKDA

Tag info: N-terminal 10xHis-tagged and C-terminal Myc-tagged

Expression Region: 1-210aa

Protein length: Full Length

MW: 28.1 kDa

Alternative Name(s): Friedreich ataxia protein

Relevance: Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe2+ 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 Fe2+ to Fe3+; 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.

Reference: "Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion." Campuzano V., Montermini L., Molto M.D., Pianese L., Cossee M., Cavalcanti F., Monros E., Rodius F., Duclos F., Monticelli A., Zara F., Canizares J., Koutnikova H., Bidichandani S., Gellera C., Brice A., Trouillas P., de Michele G.Pandolfo M. Science 271:1423-1427(1996)

Purity: Greater than 85% as determined by SDS-PAGE.

Storage: The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself. Generally, the shelf life of liquid form is 6 months at -20℃/-80℃. The shelf life of lyophilized form is 12 months at -20℃/-80℃.

Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4℃ for up to one week.

30 other products in the same category: