Product Name
Frataxin (FRDA), Recombinant Protein
Full Product Name
Recombinant mouse Frataxin, mitochondrial
Product Gene Name
FRDA recombinant protein
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Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
Sequence Positions
Frataxin mature form, 78-207aa
3D Structure
ModBase 3D Structure for O35943
Purity/Purification
Greater than 90% as determined by SDS-PAGE.
Form/Format
20mM Tris-HCl based buffer, pH8.0
Preparation and Storage
Store at -20 degree C, for extended storage, conserve at -20 degree C or -80 degree C. Repeated freezing and thawing is not recommended. Store working aliquots at 4 degree C for up to one week.
Other Notes
Small volumes of FRDA recombinant protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
FRDA recombinant protein
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.
NCBI/Uniprot data below describe general gene information for FRDA. It may not necessarily be applicable to this product.
NCBI Accession #
NP_032070.1
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NCBI GenBank Nucleotide #
NM_008044.2
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UniProt Primary Accession #
O35943
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UniProt Related Accession #
O35943[Other Products]
NCBI Official Full Name
frataxin, mitochondrial
NCBI Official Synonym Full Names
frataxin
NCBI Official Symbol
Fxn [Similar Products]
NCBI Official Synonym Symbols
FA; X25; FARR; Frda
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NCBI Protein Information
frataxin, mitochondrial
UniProt Protein Name
Frataxin, mitochondrial
UniProt Gene Name
Fxn [Similar Products]
UniProt Synonym Gene Names
Frda; Fxn [Similar Products]
UniProt Comments for FRDA
FXN: 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; 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. Defects in FXN are the cause of Friedreich ataxia (FRDA). 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. Belongs to the frataxin family. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 1.16.3.1; Mitochondrial; Motility/polarity/chemotaxis
Chromosomal Location of Human Ortholog: 19|19 B
Cellular Component: cytosol; mitochondrion
Molecular Function: 2 iron, 2 sulfur cluster binding; enzyme binding; ferric iron binding; ferrous iron binding; ferroxidase activity; iron-sulfur cluster binding
Biological Process: ***** walking behavior; aerobic respiration; cellular iron ion homeostasis; embryonic development ending in birth or egg hatching; iron incorporation into metallo-sulfur cluster; iron-sulfur cluster assembly; mitochondrion organization and biogenesis; negative regulation of apoptosis; negative regulation of multicellular organism growth; negative regulation of organ growth; oxidative phosphorylation; positive regulation of axon extension; positive regulation of catalytic activity; positive regulation of cell growth; positive regulation of cell proliferation; positive regulation of lyase activity; proprioception; protein autoprocessing; response to iron ion
Research Articles on FRDA
1. The in vitro antioxidant treatments trigger the axonal re-growth and the increase in stable MTs in shFxn, thus contributing to identify new neuronal targets of oxidation in this disease and providing a novel approach for antioxidant therapies.
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