Analytical Data
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基因名
Frataxin/FXN
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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
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别名
FXN; FRDA1; QnpA-13971; Frataxin; mitochondrial; Fxn; EC 1.16.3.1) [Cleaved into: Frataxin intermediate form; Frataxin mature form]
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种属
Cynomolgus
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表达系统
E. coli
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标签
N-6*His
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纯度
Greater than 90% as determined by SDS-PAGE.
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蛋白编号
Q8HXX9
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表达区间
S81-A210
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蛋白长度
Partial
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分子量
22 kDa.The reducing (R) protein migrat es as 22 kDa in SDS-PAGE may be due to relative charge.
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内毒素
< 1.0 EU per μg protein as determined by the LAL method.
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性状
Freeze-dried powder
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缓冲液
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
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复溶方法
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- 个性化定制
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稳定性测试
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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保存条件 & 期限
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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运输条件
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 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.
