Protein: Protein SCO1 homolog, mitochondrial (O75880)
Organism: Homo sapiens (Human)
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. Plays an important role in the regulation of copper homeostasis by controlling the abundance and cell membrane localization of copper transporter CTR1 (By similarity).
Subcellular location: Mitochondrion {ECO:0000269|PubMed:9878253}. Mitochondrion inner membrane {ECO:0000269|PubMed:15229189}; Single-pass membrane protein {ECO:0000255}.
Sequence: MAMLVLVPGRVMRPLGGQLWRFLPRGLEFWGPAEGTARVLLRQFCARQAEAWRASGRPGYCLGTRPLSTARPPPPWSQKGPGDSTRPSKPGPVSWKSLAITFAIGGALLAGMKHVKKEKAEKLEKERQRHIGKPLLGGPFSLTTHTGERKTDKDYLGQWLLIYFGFTHCPDVCPEELEKMIQVVDEIDSITTLPDLTPLFISIDPERDTKEAIANYVKEFSPKLVGLTGTREEVDQVARAYRVYYSPGPKDEDEDYIVDHTIIMYLIGPDGEFLDYFGQNKRKGEIAASIATHMRPYRKKS
InterPro domains: - IPR036249: Thioredoxin-like superfamily (homologous_superfamily) [134-295]
- IPR017276: Synthesis of cytochrome c oxidase, Sco1/Sco2 (family) [32-301]
- IPR003782: Copper chaperone SCO1/SenC (family) [79-299]
Interaction partners: - Cytochrome c oxidase subunit 2
- Cytochrome c oxidase copper chaperone
- Heme A synthase COX15
- Cytochrome c oxidase subunit 1
- Cytochrome c oxidase subunit 3
- Surfeit locus protein 1
- Protoheme IX farnesyltransferase, mitochondrial
- Cytochrome c oxidase assembly protein COX11
- Cytochrome c oxidase assembly factor 6 homolog
- Cytochrome c oxidase subunit 6B1
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0003824 (catalytic activity), GO:0140096 (catalytic activity, acting on a protein), GO:0005515 (protein binding), GO:0016491 (oxidoreductase activity), GO:0016667 (oxidoreductase activity, acting on a sulfur group of donors), GO:0015035 (protein-disulfide reductase activity), GO:0015036 (disulfide oxidoreductase activity)
Biological Process (BP): GO:0008150 (biological process), GO:0032502 (developmental process), GO:0009987 (cellular process), GO:0032501 (multicellular organismal process), GO:0071840 (cellular component organization or biogenesis), GO:0048856 (anatomical structure development), GO:0007275 (multicellular organism development), GO:0016043 (cellular component organization), GO:0044085 (cellular component biogenesis), GO:0048731 (system development), GO:0048513 (animal organ development), GO:0043933 (protein-containing complex organization), GO:0006996 (organelle organization), GO:0048880 (sensory system development), GO:0007423 (sensory organ development), GO:0022607 (cellular component assembly), GO:0065003 (protein-containing complex assembly), GO:0007005 (mitochondrion organization), GO:0001654 (eye development), GO:0150063 (visual system development), GO:0033108 (mitochondrial respiratory chain complex assembly), GO:0017004 (cytochrome complex assembly), GO:0008535 (respiratory chain complex IV assembly), GO:0033617 (mitochondrial cytochrome c oxidase assembly)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0031975 (envelope), GO:0016020 (membrane), GO:0005737 (cytoplasm), GO:0031974 (membrane-enclosed lumen), GO:0099080 (supramolecular complex), GO:0099081 (supramolecular polymer), GO:0031090 (organelle membrane), GO:0043292 (contractile fiber), GO:0031967 (organelle envelope), GO:0005739 (mitochondrion), GO:0043233 (organelle lumen), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0043228 (non-membrane-bounded organelle), GO:0005740 (mitochondrial envelope), GO:0019866 (organelle inner membrane), GO:0043231 (intracellular membrane-bounded organelle), GO:0099512 (supramolecular fiber), GO:0005759 (mitochondrial matrix), GO:0030016 (myofibril), GO:0070013 (intracellular organelle lumen), GO:0043232 (intracellular non-membrane-bounded organelle), GO:0031966 (mitochondrial membrane), GO:0005743 (mitochondrial inner membrane)