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  4. Recombinant Mouse Frataxin,mitochondrial (Fxn)

Recombinant Mouse Frataxin,mitochondrial (Fxn)

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  • 中文名称：
    小鼠Fxn重组蛋白
  • 货号：
    CSB-EP009086MOa0
  • 规格：
    ￥1836
  • 图片：
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他：

产品详情

  • 纯度：
    Greater than 90% as determined by SDS-PAGE.
  • 基因名：
  • Uniprot No.：
  • 别名：
    Fxn; FrdaFrataxin; mitochondrial; Fxn; EC 1.16.3.1) [Cleaved into: Frataxin intermediate form; Frataxin mature form]
  • 种属：
    Mus musculus (Mouse)
  • 蛋白长度：
    Full Length of Mature Protein
  • 来源：
    E.coli
  • 分子量：
    19.9 kDa
  • 表达区域：
    78-207aa
  • 氨基酸序列
    LGTLDNPSSLDETAYERLAEETLDSLAEFFEDLADKPYTLEDYDVSFGDGVLTIKLGGDLGTYVINKQTPNKQIWLSSPSSGPKRYDWTGKNWVYSHDGVSLHELLARELTKALNTKLDLSSLAYSGKGT
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal 6xHis-tagged
  • 产品提供形式：
    Liquid or Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 缓冲液：
    Tris-based buffer,50% glycerol
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    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°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 货期：
    3-7 business days
  • 注意事项：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet & COA：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    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.
  • 基因功能参考文献：
    1. This study demonstrated that mouse model of Friedreich's ataxia showed that decreased grip strength endurance time , threshold of peripheral sensitivity using Von Frey monofilaments and gait parameters. PMID: 27575947
    2. Here, the s show that loss of Fxn in the nervous system in mice also activates an iron/sphingolipid/PDK1/Mef2 pathway, indicating that the mechanism is evolutionarily conserved. PMID: 27901468
    3. 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. PMID: 27516386
    4. Retinal FXN levels are increased in response to ischemia. Furthermore, elevated FXN levels had a clear neuroprotective effect as shown by increased ganglion cell survival after acute retinal ischemia/reperfusion. Frataxin's neuroprotective effect was associated with an upregulation of antioxidative enzymes. PMID: 27537261
    5. Frataxin Deficiency Promotes Excess Microglial DNA Damage and Inflammation that Is Rescued by PJ34 PMID: 26954031
    6. Frataxin-deficient mice, which had higher mitochondrial iron loading, showed impaired airway mucociliary clearance and higher pulmonary inflammation at baseline. PMID: 26752519
    7. Using a mouse model of hepatic FXN deficiency in combination with mice deficient for IRP1, a key regulator of cellular iron metabolism, we show that IRP1 activation in conditions of Fe-S deficiency increases the available cytosolic labile iron pool PMID: 25651183
    8. The Fxn KO/Mck mice tested from one to two months of age showed abnormal gait patterns accompanied by a loss in motor skills PMID: 25765157
    9. Reduced expression of frataxin in Friedreich's ataxia leads to elevation of COX2-mediated oxylipin synthesis stimulated by increases in transcription factors that respond to increased reactive oxygen species. PMID: 25104852
    10. Frataxin-deficient cells showed a specific inhibition of mitochondrial Complex I activity already at 70% residual frataxin levels, whereas the glutathione imbalance progressively increased after silencing. PMID: 24714088
    11. The results support a mechanistic hypothesis in which frataxin deficiency decreases Nrf2 expression in vivo, causing the sensitivity to oxidative stress in target tissues the DRG and the cerebella, which contributes to the process of neurodegeneration. PMID: 23350650
    12. rescue of the Friedreich ataxia knockout mutation in transgenic mice containing an FXN-EGFP genomic reporter PMID: 24667739
    13. these results indicate that IGF-I exerts cell-context neuroprotection in frataxin deficiency that maybe therapeutically effective. PMID: 23039828
    14. Data show that the respiratory chain defects accompanying frataxin deficiency cause progressive hyperacetylation of cardiac mitochondrial proteins due to the inhibition of SIRT3 deacetylase. PMID: 22394676
    15. dual, pro-proliferative but chemosensitizing role in astrocytic tumors PMID: 21863062
    16. Distribution of frataxin in eye retina of normal mice and of transgenic R7E mice with retinal degeneration PMID: 20799613
    17. As an attempt to generate a mouse model of Friedreich ataxia, we introduced a (GAA)(230) repeat within the mouse frataxin gene by homologous recombination. PMID: 11852098
    18. studies indicate an association between frataxin deficiency, iron deposits and cardiac fibrosis, but no obvious association between iron accumulation and neurodegeneration similar to Friedreich ataxia could be detected PMID: 12880182
    19. frataxin has a role in cellular growth arrest and apoptosis, and its absence can cause experimental diabetes PMID: 12925693
    20. We have generated two mouse models for FRDA that specifically develop progressive mixed cerebellar and sensory ataxia, the most prominent neurological features of Friedreich ataxia with a slowly progressive neurological degeneration PMID: 14985441
    21. frataxin may act as a mitochondrial tumor suppressor protein PMID: 16278235
    22. The identification of a core set of genes changing early in the FRDA pathogenesis can be a useful tool in both clarifying the disease process and in evaluating new therapeutic strategies. PMID: 16442805
    23. Reduced expression of mitochondrial frataxin in mice exacerbates diet-induced obesity. PMID: 17404227
    24. The fxn GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart. PMID: 18045775
    25. loss of Fxn markedly alters cellular Fe trafficking and Fe chelation limits myocardial hypertrophy in the mutant PMID: 18621680
    26. Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins. PMID: 19154341
    27. The PPARG pathway is dysregulated in frataxin deficient mice and Friedreich's ataxia patients. PMID: 19376812
    28. We showed that complete absence of murine frataxin in fibroblasts inhibits cell division and leads to cell death. This lethal phenotype was rescued through transgenic expression of human wild type as well as mutant (hFXN(G130V) and hFXN(I154F)) frataxin PMID: 19629184
    29. Results enable the construction of a model explaining the cytosolic iron deficiency and mitochondrial iron loading in the absence of frataxin, which is important for understanding the pathogenesis of Friedreich's ataxia. PMID: 19805308

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  • 亚细胞定位：
    Cytoplasm, cytosol. Mitochondrion. Note=PubMed:17597094 describes localization exclusively in mitochondria.
  • 蛋白家族：
    Frataxin family
  • 组织特异性：
    Heart, liver, skeletal muscle, kidney, spleen and thymus. Weakly expressed in the brain and lung.
  • 数据库链接：

    KEGG: mmu:14297

    STRING: 10090.ENSMUSP00000080081

    UniGene: Mm.7319