Key features and details
- Expression system: Escherichia coli
- Purity: > 85% SDS-PAGE
- Tags: His tag N-Terminus
- Suitable for: SDS-PAGE
Product nameRecombinant Human Frataxin protein (Tagged)
See all Frataxin proteins and peptides
Purity> 85 % SDS-PAGE.
Expression systemEscherichia coli
Protein lengthFull length protein
SequenceMWTLGRRAVAGLLASPSPAQAQTLTRVPRPAELAPLCGRRGLRTDIDATC TPRRASSNQRGLNQIWNVKKQSVYLMNLRKSGTLGHPGSLDETTYERLAE ETLDSLAEFFEDLADKPYTFEDYDVSFGSGVLTVKLGGDLGTYVINKQTP NKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLAAELTKALKTKLDL SSLAYSGKDA
Predicted molecular weight28 kDa including tags
Amino acids1 to 210
TagsHis tag N-Terminus
Additional sequence informationN-terminal 10xHis-tagged and C-terminal Myc-tagged
- Anti-Frataxin antibody [17A11] (ab113691)
- Anti-Frataxin antibody (ab175402)
- Anti-6X His tag® antibody [HIS.H8] (ab18184)
- Anti-Myc tag antibody [Myc.A7] (ab18185)
- Anti-6X His tag® antibody [4D11] (ab5000)
- Anti-Myc tag antibody (ab9106)
- Anti-6X His tag® antibody (ab9108)
- Anti-Myc tag antibody - ChIP Grade (ab9132)
Our Abpromise guarantee covers the use of ab238218 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Concentration information loading...
Preparation and Storage
Stability and Storage
Shipped at 4°C. Store at -20°C or -80°C. Avoid freeze / thaw cycle.
Constituents: Tris buffer, 50% Glycerol (glycerin, glycerine)
FunctionPromotes 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.
Tissue specificityExpressed in the heart, peripheral blood lymphocytes and dermal fibroblasts.
Involvement in diseaseDefects in FXN are the cause of Friedreich ataxia (FRDA) [MIM:229300]. FRDA is an autosomal recessive, progressive degenerative disease characterized by neurodegeneration and cardiomyopathy it is the most common inherited ataxia. The disorder is usually manifest before adolescence and is generally characterized by incoordination of limb movements, dysarthria, nystagmus, diminished or absent tendon reflexes, Babinski sign, impairment of position and vibratory senses, scoliosis, pes cavus, and hammer toe. In most patients, FRDA is due to GAA triplet repeat expansions in the first intron of the frataxin gene. But in some cases the disease is due to mutations in the coding region.
Sequence similaritiesBelongs to the frataxin family.
modificationsProcessed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form (frataxin(81-210)) which is the predominant form. The additional forms, frataxin(56-210) and frataxin(78-210), seem to be produced when the normal maturation process is impaired; their physiological relevance is unsure.
Cellular localizationCytoplasm. Mitochondrion. PubMed:18725397 reports localization exclusively in mitochondria.
- Information by UniProt
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
ab238218 has not yet been referenced specifically in any publications.