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Anti-Human Frataxin Recombinant Nanobody (SAA2739) (HS777043)

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사양:
  • 50ug
  • 100ug
  • 1mg
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  • 개요

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  • 참고 문헌

  • 데이터시트

  • SDS

개요
카탈로그 번호HS777043
설명
Anti-Human Frataxin Nanobody (SAA2739) [SAA2739] (HS777043) is a alpaca monoclonal antibody detecting Frataxin in ELISA. Suitable for Human.
종 반응성Human
응용 분야ELISA
숙주 종Alpaca
클론성Monoclonal
이소타입VHH-His
클론 IDSAA2739
타겟 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
내독소 수준 Please contact with the lab for this information.
순도 >95% as determined by SDS-PAGE.
정제 Purified by Nickel column.
등록번호 Q16595
형태 Liquid
저장 완충액 0.01M PBS pH 7.4

데이터시트 인쇄본 또는 로트별 COA에 기재된 구체적인 완충액 정보를 참조하세요.

제품 사용 정보
응용 분야 희석
ELISA 1:2000-1:20000
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 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
이미지
참고 문헌
Formula
Mass (g) = Concentration (mol/L) × Volume (L) × MW (g/mol)
Enter any 2 of Mass, Concentration, Volume + Molecular Weight to solve for the unknown.
Mass
=
Concentration
×
Volume
Molecular Weight *
g/mol
Formula
C₁ × V₁ = C₂ × V₂
Enter any 3 of the 4 values to solve for the unknown.
Stock Solution
C₁ (Stock Conc.)
×
V₁ (Stock Vol.)
=
Working Solution
C₂ (Working Conc.)
×
V₂ (Working Vol.)
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