SCO2 Human
目录号 : GP24504SCO Cytochrome Oxidase Deficient Homolog 2 Human Recombinant
Sample solution is provided at 25 µL, 10mM.
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| Purity | Greater than 90% as determined by SDS-PAGE. | Source | Escherichia Coli. |
| Phycical Appearance | Sterile Filtered clear solution. | Shipping Condition | Shipped with Ice Packs. |
| Synonyms | SCO1L; SCO Cytochrome Oxidase Deficient Homolog 2 (yeast); Protein SCO2 Homolog-Mitochondrial; MGC125823; MGC125825. | ||
| Amino Acid Sequence | MGSSHHHHHH SSGLVPRGSH MGPAETGGQG QPQGPGLRTR LLITGLFGAG LGGAWLALRA EKERLQQQKR TEALRQAAVG QGDFHLLDHR GRARCKADFR GQWVLMYFGF THCPDICPDE LEKLVQVVRQ LEAEPGLPPV QPVFITVDPE RDDVEAMARY VQDFHPRLLG LTGSTKQVAQ ASHSYRVYYN AGPKDEDQDY IVDHSIAIYL LNPDGLFTDY YGRSRSAEQI SDSVRRHMAA FRSVLS | ||
| Stability | Store at 4°C if entire vial will be used within 2-4 weeks.Store, frozen at -20°C for longer periods of time.Please avoid freeze thaw cycles. | ||
| Formulation | The SCO2 protein solution (0.5mg/1ml) is formulated in 20mM Tris-HCl buffer (pH8.0) 2mM DTT, 200mM NaCl and 30% glycerol. | ||
SCO2 protein is a member of the SCO1/2 family. SCO1 and SCO2 proteins are found on the inner membrane of the mitochondria and takes a vital part copper insertion or transport to the active site of cytochrome c oxidase (COX). Flaws in SCO2 are the reason for deadly infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC) which is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle display declines in cytochrome c oxidase (COX) activity, while liver and fibroblasts show mild COX deficiencies.
Store at 4°C if entire vial will be used within 2-4 weeks.Store, frozen at -20°C for longer periods of time.Please avoid freeze thaw cycles.