| Catalog# |
C259 |
| Source |
E.coli |
| Description |
Recombinant Human Protein SCO1 Homolog, Mitochondrial/SCO1 is produced by our E. coli expression system. The target protein is expressed with sequence (Gly132-Ser300) of Human SCO1. |
| Names |
Protein SCO1 Homolog Mitochondrial, SCO1, SCOD1 |
| Accession # |
O75880 |
| Formulation |
Lyophilized from a 0.2 μm filtered solution of 50mM PB, 1mM DTT, pH 7.2 |
| Shipping |
The product is shipped at ambient temperature. |
| Reconstitution |
Always centrifuge tubes before opening. Do not mix by vortex or pipetting.
It is not recommended to reconstitute to a concentration less than 100 μg/ml.
Dissolve the lyophilized protein in 1X PBS.
Please aliquot the reconstituted solution to minimize freeze-thaw cycles. |
| Storage |
Lyophilized protein should be stored at < -20°C, though stable at room temperature for 3 weeks.
Reconstituted protein solution can be stored at 4-7°C for 2-7 days.
Aliquots of reconstituted samples are stable at < -20°C for 3 months. |
| Purity |
Greater than 95% as determined by reducing SDS-PAGE. |
| Endotoxin |
Less than 0.1 ng/μg (1 IEU/μg). |
| Amino Acid Sequence |
GSPEFHMGKPLLGGPFSLTTHTGERKTDKDYLGQWLLIYFGFTHCPDVCPEELEKMIQVVDEIDS ITTLPDLTPLFISIDPERDTKEAIANYVKEFSPKLVGLTGTREEVDQVARAYRVYYSPGPKDEDE DYIVDHTIIMYLIGPDGEFLDYFGQNKRKGEIAASIATHMRPYRKKS
|
| Background |
Protein SCO1 Homolog, Mitochondrial (SCO1) is a member of the SCO1/2 family. SCO1 has a homodimer structure. SCO1 is located in mitochondrion and is highly expressed in muscle, heart, and brain. It is characterized by high rates of Oxidative Phosphorylation (OxPhos). SCO1 is thought to play a important role in cellular copper homeostasis, mitochondrial redox signaling and insertion of copper into the active site of COX. The defects of SCO1 can result in Mitochondrial Complex IV Deficiency (MT-C4D). A disorder of the mitochondrial respiratory chain has heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. |
