Protein SCO1 Homolog Mitochondrial SCO1/SCOD1 Protein(N-GST) (recombinant human)

Protein Construction: 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. Sequence: Gly132-Ser300. Fusion tag: N-GST Endotoxin: < 1.0 EU per µg as determined by the LAL method. Apparent Molecular Mass: 19 kDa. Protein function: 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. Protein function: Copper metallochaperone essential for the maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Not required for the synthesis of MT-CO2/COX2 but plays a crucial role in stabilizing MT-CO2/COX2 during its subsequent maturation. Involved in transporting copper to the Cu(A) site on MT-CO2/COX2 (PubMed:15659396, PubMed:16735468, PubMed:17189203, PubMed:19336478, PubMed:15229189). Plays an important role in the regulation of copper homeostasis by controlling the abundance and cell membrane localization of copper transporter CTR1. [The UniProt Consortium]