Alternative Name(s):

Fxn; FrdaFrataxin; mitochondrial; Fxn; EC 1.16.3.1) [Cleaved into: Frataxin intermediate form; Frataxin mature form]

Species: (Organism)

Mus musculus (Mouse)

Gene Names:

Fxn

Tag info:

N-terminal 6xHis-tagged

Target Protein AA Sequence:

LGTLDNPSSLDETAYERLAEETLDSLAEFFEDLADKPYTLEDYDVSFGDGVLTIKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLARELTKALNTKLDLSSLAYSGKGT

Expression Region:

78-207aa

Subcellular Location:

Cytoplasm, cytosol, Mitochondrion

Tissue Specificity:

Heart, liver, skeletal muscle, kidney, spleen and thymus. Weakly expressed in the brain and lung.

Protein Length:

Full Length of Mature Protein

Pathway:

Mol. Weight:

19.9 kDa

Purity:

Greater than 90% as determined by SDS-PAGE.

Form:

Liquid or Lyophilized powder

Buffer:

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.

Research Areas:

Others

Function:

Promotes 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. Modulates the RNA-binding activity of ACO1 (By similarity).

Involvement in disease:

Relevance:

Promotes the biosynthesis of he and assbly 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. Modulates the RNA-binding activity of ACO1 .

Reconstitution:

We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.

Protein Families:

Frataxin family

Reference:

Studies of human, mouse and yeast homologues indicate a mitochondrial function for frataxin.Koutnikova H., Campuzano V., Foury F., Dolle P., Cazzalini O., Koenig M.Nat. Genet. 16:345-351(1997)