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| カタログ番号 | HS777033 | ||||
|---|---|---|---|---|---|
| 種反応性 | Human | ||||
| アプリケーション | ELISA | ||||
| 宿主種 | Human | ||||
| クローン性 | Monoclonal | ||||
| アイソタイプ | VHH-Fc | ||||
| クローンID | SAA2739 | ||||
| ターゲット | EC:1.16.3.1, Extramitochondrial frataxin, FRDA, FXN, Frataxin intermediate form, Frataxin mature form, Frataxin(56-210), Frataxin(78-210), Frataxin(81-210), Frataxin, mitochondrial, Friedreich ataxia protein, Fxn, X25, d-FXN, i-FXN, m56-FXN, m78-FXN, m81-FXN | ||||
| エンドトキシンレベル | Please contact with the lab for this information. | ||||
| 純度 | >95% as determined by SDS-PAGE. | ||||
| 精製 | Protein A/G purified from cell culture supernatant. | ||||
| アクセッション番号 | Q16595 | ||||
| 形態 | Liquid | ||||
| 保存バッファー | 0.01M PBS pH 7.4 データシートのハードコピーまたはロット固有のCOAに記載された具体的なバッファー情報を参照してください。 |
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| 製品使用情報 |
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| 安定性と保存 | Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt. | ||||
| 背景 | Frataxin, mitochondrial is a ~23 kDa protein. Functions as an activator of persulfide transfer to the scaffoding protein ISCU as component of the core iron-sulfur cluster (ISC) assembly complex and participates to the [2Fe-2S] cluster assembly. Accelerates sulfur transfer from NFS1 persulfide intermediate to ISCU and to small thiols such as L-cysteine and glutathione leading to persulfuration of these thiols and ultimately sulfide release. Binds ferrous ion and is released from FXN upon the addition of both L-cysteine and reduced FDX2 during [2Fe-2S] cluster assembly. The core iron-sulfur cluster (ISC) assembly complex is involved in the de novo synthesis of a [2Fe-2S] cluster, the first step of the mitochondrial iron-sulfur protein biogenesis. This process is initiated by the cysteine desulfurase complex (NFS1:LYRM4:NDUFAB1) that produces persulfide which is delivered on the scaffold protein ISCU in a FXN-dependent manner. 1. Yoon, T. et al. (2003) Journal of the American Chemical Society 125, 6078-84. PMID: 12785837 2. Bridwell-Rabb, J. et al. (2014) Biochemistry 53, 4904-13. PMID: 24971490 3. Cai, K. et al. (2018) Journal of inorganic biochemistry 183, 107-116. PMID: 29576242 4. O'Neill, HA. et al. (2005) Biochemistry 44, 537-45. PMID: 15641778 5. Cavadini, P. et al. (2002) Human molecular genetics 11, 217-27. PMID: 11823441 6. Nichol, H. et al. (2003) Biochemistry 42, 5971-6. PMID: 12755598 7. Bulteau, AL. et al. (2004) Science (New York, N.Y.) 305, 242-5. PMID: 15247478 8. Yoon, T. et al. (2004) The Journal of biological chemistry 279, 25943-6. PMID: 15123683 9. Schoenfeld, RA. et al. (2005) Human molecular genetics 14, 3787-99. PMID: 16239244 | ||||
| 注意事項 | For research use only |
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