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Recombinant Human SCO2 protein (His tag)
SKU: PDEH100335-100
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Recombinant Human SCO2 protein (His tag)
| SKU # | PDEH100335 |
| Expression Host | E. coli |
Description
| Synonyms | Protein SCO2 homolog;mitochondrial;SCO2; |
| Species | Human |
| Expression_host | E.coli |
| Sequence | Gly 42-Ser 266 |
| Accession | O43819 |
| Mol_Mass | 24.6 kDa |
| AP_Mol_Mass | 30 kDa |
| Tag | N-His & C-His |
| Bio_Activity | Not validated for activity |
Propertie
| Purity | > 95 % as determined by reducing SDS-PAGE. |
| Endotoxin level | Please contact us for more information. |
| Storage | 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. |
| Shipping | This product is provided as lyophilized powder which is shipped with ice packs. |
| Formulation | Lyophilized from sterile PBS, pH 7.4. Normally 5 % - 8 % trehalose, mannitol and 0.01% Tween80 are added as protectants before lyophilization. Please refer to the specific buffer information in the printed manual. |
| Reconstitution | It is recommended that sterile water be added to the vial to prepare a stock solution of 0.5 mg/mL. Concentration is measured by UV-Vis |
Background
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.