Recombinant Mouse Frataxin, mitochondrial (Fxn) | CSB-EP009086MO

Recombinant Mouse Frataxin, mitochondrial (Fxn) | CSB-EP009086MO | Cusabio

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

Gene Names: Fxn

Research Areas: Others

Organism: Mus musculus (Mouse)

AA Sequence: LGTLDNPSSLDETAYERLAEETLDSLAEFFEDLADKPYTLEDYDVSFGDGVLTIKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLARELTKALNTKLDLSSLAYSGKGT

Source: E.coli

Tag Info: N-terminal 6xHis-SUMO-tagged

Expression Region: 78-207aa

Sequence Info: Full Length of Mature Protein

MW: 30.4 kDa

Purity: Greater than 90% as determined by SDS-PAGE.

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 .

Reference: Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant.Huang M.L., Becker E.M., Whitnall M., Rahmanto Y.S., Ponka P., Richardson D.R.Proc. Natl. Acad. Sci. U.S.A. 106:16381-16386(2009)

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.

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:

Subcellular Location: Cytoplasm, cytosol, Mitochondrion

Protein Families: Frataxin family

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

Paythway:

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.

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.

Uniprot ID: O35943

HGNC Database Link: N/A

UniGene Database Link: UniGene

KEGG Database Link: KEGG

STRING Database Link: STRING

OMIM Database Link: N/A