84-2336-85　［取扱停止］FXN (CyaY, d-FXN, FA, FARR, Frataxin mature form, Frataxin(81-210), FRDA, FRDA, Friedreich ataxia Protein, Fxn, i-FXN, m56-FXN, m78-FXN, m81-FXN, MGC57199, X25) 100ul　222806

特徴

• Friedreich ataxia is a progressive neurodegenerative disorder caused by loss of function mutations in the frataxin gene.
• The human frataxin gene maps to chromosome 9q13.The frataxin gene encodes a mitochondrial protein of the same name.
• Frataxin assembles into a stable homopolymer with iron-binding capabilities.
• When expressed in E.
• Coli, human frataxin binds iron atoms at a rate of 10 iron atoms per 1 molecule of the frataxin polymer.
• Thus, frataxin appears to function in some capacity for iron-storage for the mitochondria.
• Frataxin may also function as an activator of oxidative phosphorylation to increase mitochondrial membrane potential and elevate cellular ATP.
• Frataxin is expressed in tissues with high metabolic activity including heart, liver and brown fat.
• Applications:Suitable for use in Western Blot, Immunohistochemistry.
• Other applications not tested.
• Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
• Storage and Stability:May be stored at 4°C for short-term only.
• Aliquot to avoid repeated freezing and thawing.
• Store at -20°C.
• Aliquots are stable for 12 months after receipt.
• For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.