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| 카탈로그 번호 | HS777014 | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| 종 반응성 | Human | ||||||||
| 응용 분야 | ELISA, IHC, WB | ||||||||
| 숙주 종 | Rabbit | ||||||||
| 클론성 | Polyclonal | ||||||||
| 이소타입 | IgG | ||||||||
| 면역원 | E. coli - derived recombinant Human FXN (Ser57-Leu198). | ||||||||
| 타겟 | FXN, Frataxin(81-210), Friedreich ataxia protein, m56-FXN, Frataxin, mitochondrial, Fxn, d-FXN, m78-FXN, i-FXN, m81-FXN, X25, FRDA | ||||||||
| 정제 | Purified by antigen affinity column. | ||||||||
| 등록번호 | Q16595 | ||||||||
| 형태 | Liquid | ||||||||
| 저장 완충액 | 0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300. 데이터시트 인쇄본 또는 로트별 COA에 기재된 구체적인 완충액 정보를 참조하세요. |
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| 제품 사용 정보 |
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| 안정성 및 저장 | 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. | ||||||||
| 배경 | Frataxin, mitochondrial (FXN) 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 | ||||||||
| 참고사항 | For research use only. |
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