Recombinant Human Protein SCO1 Homolog Mitochondrial SCO1/SCOD1 (N-GST)

Source: E.coli. MW :20.14kD. Recombinant Human Protein SCO1 Homolog Mitochondrial is produced by our E.coli expression system and the target gene encoding Gly132-Ser300 is expressed with a GST tag at the N-terminus. 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.