Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2).Defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC) . This disease is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle show reductions in cytochrome c oxidase (COX) activity, whereas liver and fibroblasts show mild COX deficiencies.

Dettagli prodotto

• Codice: PDEH100989_1mg
• Marca: Elabscience
• Specie target: Human
• Host: E.coli
• Confezionamento: Lyophilized from sterile PBS, pH 7.4.<br/>Normally 5%-8% trehalose, mannitol and 0.01% Tween 80 are added as protectants before lyophilization.<br/>Please refer to the specific buffer information in the printed manual.
• Link: Apri link
• Stoccaggio: Generally, lyophilized proteins are stable for up to 12 months when stored at -20 to -80℃. Reconstituted protein solution can be stored at 4-8℃ for 2-7 days. Aliquots of reconstituted samples are stable at < -20℃ for 3 months.
• Simbolo target: SCO2
• Ig target: Protein SCO2 homolog;SCO2;mitochondrial
• Area di ricerca: Metabolism;Signal Transduction;Tags and Cell Markers