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Anti-Human Frataxin Recombinant Antibody (SAA2739) (HS777033)

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Aperçu
Numéro de catalogueHS777033
Reactivité des espècesHuman
ApplicationsELISA
Espèce hôteHuman
ClonalitéMonoclonal
IsotypeVHH-Fc
ID de cloneSAA2739
Cible 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
Niveau d'endotoxines Please contact with the lab for this information.
Pureté >95% as determined by SDS-PAGE.
Purification Protein A/G purified from cell culture supernatant.
Numéro d'accès Q16595
Forme Liquid
Tampon de stockage 0.01M PBS pH 7.4

Reférez-vous aux informations spécifiques sur le tampon dans la copie papier du datasheet ou dans le COA spécifique au lot.

Informations d'utilisation du produit
Application Dilution
ELISA 1:2000-1:20000
Stabilité et stockage 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.
Contexte

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
Note For research use only
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