Analytical Data
-
Gene name
Frataxin/FXN
-
简介
Frataxin (FXN) protein activates persulfide transfer, which is critical for [2Fe-2S] cluster assembly. It accelerates sulfur transfer from NFS1 persulfide to ISCU and small thiols, resulting in oversulfide and sulfide release. Frataxin/FXN Protein, Cynomolgus (His) is the recombinant cynomolgus-derived Frataxin/FXN protein, expressed by E. coli , with N-6*His labeled tag.
- Application
-
Alternative Names
FXN; FRDA1; QnpA-13971; Frataxin; mitochondrial; Fxn; EC 1.16.3.1) [Cleaved into: Frataxin intermediate form; Frataxin mature form]
-
Species
Cynomolgus
-
Source
E. coli
-
Tag
N-6*His
-
Purity
Greater than 90% as determined by SDS-PAGE.
-
Uniprot
Q8HXX9
-
Expression Region
S81-A210
-
Protein Length
Partial
-
Molecular Weight
22 kDa.The reducing (R) protein migrat es as 22 kDa in SDS-PAGE may be due to relative charge.
-
Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
-
Form
Freeze-dried powder
-
Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
-
Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
-
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.
-
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.
-
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
Related Products
Protein Description
Frataxin (FXN) is a mitochondrial protein essential for iron-sulfur cluster biosynthesis and iron metabolism, and its deficiency is linked to Friedreich's ataxia, a hereditary neurodegenerative disorder characterized by progressive ataxia, muscle weakness, and sensory loss. The FXN gene is located on chromosome 9 and is subject to a GAA trinucleotide repeat expansion, leading to reduced expression of the frataxin protein. This deficiency results in mitochondrial dysfunction, oxidative stress, and degeneration of spinal cord and cerebellar neurons. Research into recombinant frataxin protein focuses on understanding its structure and function, developing potential therapies to restore frataxin levels or improve mitochondrial function. Recombinant FXN has been explored as a therapeutic agent in preclinical and clinical studies, aiming to alleviate symptoms and slow disease progression in patients with Friedreich's ataxia. Scientists utilize various expression systems, such as E. coli and yeast, to produce large quantities of functional recombinant frataxin for biochemical analysis and potential therapeutic use. These studies are crucial for unraveling the molecular mechanisms of frataxin's role in cellular metabolism and for identifying novel treatment strategies that could enhance mitochondrial health and resilience in affected individuals.
