Frataxin, 42-210aa, Human

Catalog
01-1757
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Proteins, Recombinant
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Product Name Frataxin, 42-210aa, Human
Background Frataxin, also known as FXN, is an iron binding protein which belongs to FRATAXIN family. It is an anti-apoptotic protein which prevents mitochondrial damage, reactive oxygen species production and act as either an iron chaperone or an iron storage protein. Frataxin is localized to the mitochondrion and regulating mitochondrial iron transport and respiration. Defects in this protein cause Friedreich ataxia. Recombinant Frataxin protein was expressed in E.coli and purified by using conventional chromatography techniques.
Synonyms CyaY, FA, FARR, FRDA, X25, FXN, Friedreich ataxia protein.
Species Human
Amino Acid Sequence MGSSHHHHHH SSGLVPRGSH MLRTDIDATC TPRRASSNQR GLNQIWNVKK QSVYLMNLRK SGTLGHPGSL DETTYERLAE ETLDSLAEFF EDLADKPYTF EDYDVSFGSG VLTVKLGGDL GTYVINKQTP NKQIWLSSPS SGPKRYDWTG KNWVYSHDGV SLHELLAAEL TKALKTKLDL SSLAYSGKDA
Molecular Weight 25.6 kDa (222aa), confirmed by MALDI-TOF.
Concentration 1 mg/ml (determined by Bradford assay)
Form Liquid, in 20 mM Tris-HCl buffer (pH8.0) containing 1mM DTT 10% glycerol
Purity > 95% by SDS-PAGE
Applications SDS-PAGE
NCBI Accession # NP_000135
Storage Can be stored at +4C short term (1-2 weeks). For long term storage, aliquot and store at -20C or -70C. Avoid repeated freezing and thawing cycles.
Intended Use For Research Use Only
References Campuzano V., et al. (1996) Science. 271(5254):1423-7.
Shan Y., et al. (2007) Hum Mol Genet. 16(8):929-41.
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