Analytical Data
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Gene name
Frataxin/FXN
- Application
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Alternative Names
Frataxin mitochondrial
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Species
Human
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Source
E. coli
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Tag
N- His-SUMO & C- Myc
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Purity
Greater than 90% as determined by SDS-PAGE.
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Uniprot
Q16595
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Expression Region
1-210aa
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Molecular Weight
43.1 kDa
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Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
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Form
Freeze-dried powder
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Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
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Stability Test
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
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Storage & Shelf Life
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
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Shipping
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
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Protein Description
Frataxin (FXN) is a mitochondrial protein critical for iron-sulfur cluster synthesis and overall mitochondrial function. Its deficiency is primarily associated with Friedreich's ataxia, a genetic disorder characterized by progressive neurological and muscular degeneration. The FXN gene, located on chromosome 9, encodes a 210-amino acid protein that plays a crucial role in cellular iron metabolism and protection against oxidative stress. Research has shown that mutations in the FXN gene lead to reduced frataxin levels, resulting in iron accumulation, mitochondrial dysfunction, and apoptosis in neuronal cells. The need for therapeutic interventions has spurred interest in the recombinant production of frataxin. Recombinant FXN can be utilized to study its biological functions, understand the molecular mechanisms underlying Friedreich's ataxia, and explore potential treatments. Such studies often involve evaluating the effects of frataxin on mitochondrial dynamics, cellular iron homeostasis, and overall cellular health. Additionally, the development of recombinant FXN as a therapeutic candidate could pave the way for innovative treatment strategies, including gene therapy, small molecules that enhance FXN expression, or protein replacement therapies. By deepening our understanding of the role of FXN in the cell and its implications in disease, researchers aim to uncover novel approaches to mitigate the devastating effects of Friedreich's ataxia and improve the quality of life for affected individuals.
