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Copper is essential in the aerobic respiration of all eukaryotes. In mitochondria, it is found in cytochrome c oxidase, which is the last protein in oxidative phosphorylation. Cytochrome c oxidase is the protein that binds the O 2 between a copper and an iron; the protein transfers 4 electrons to the O 2 molecule to reduce it to two molecules ...
SCO1 is a copper metallochaperone that is located in the inner mitochondrial membrane and is important for the maturation and stabilization of cytochrome c oxidase subunit II (MT-CO2/COX2). It plays a role in the regulation of copper homeostasis by controlling the localization and abundance of CTR1 and is responsible for the transportation of ...
The enzyme cytochrome c oxidase or Complex IV (was EC 1.9.3.1, now reclassified as a translocase EC 7.1.1.9) is a large transmembrane protein complex found in bacteria, archaea, and the mitochondria of eukaryotes.
SCO2 cytochrome c oxidase assembly (also known as SCO2 homolog, mitochondrial and SCO cytochrome oxidase deficient homolog 2) is a protein that in humans is encoded by the SCO2 gene. [ 5 ] [ 6 ] [ 7 ] The encoded protein is one of the cytochrome c oxidase (COX) (Complex IV) assembly factors.
It contains two adjacent transmembrane regions in its N-terminus and the major part of the protein is exposed to the periplasmic or to the mitochondrial intermembrane space, respectively. MT-CO2 provides the substrate-binding site and contains the binuclear copper A center, probably the primary acceptor in cytochrome c oxidase. [12] [13] [5]
CCS is imported to the mitochondria by Mia40 and Erv1 disulfide relay system. [7] The cysteine 64 of CCS Domain I generates a disulfide intermediate with Mia40. [7] This disulfide bond is transferred to link cysteine 64 and 27 of CCS, stabilizing the protein in the mitochondrial intermembrane space where it delivers Cu to the Cu-less apo-SOD1 ...
12856 Ensembl ENSG00000138495 ENSMUSG00000046516 UniProt Q14061 P56394 RefSeq (mRNA) NM_005694 NM_001017429 RefSeq (protein) NP_005685 NP_001368931 NP_001368932 NP_001017429 Location (UCSC) Chr 3: 119.65 – 119.68 Mb Chr 16: 38.17 – 38.17 Mb PubMed search Wikidata View/Edit Human View/Edit Mouse Cytochrome c oxidase copper chaperone is a protein that in humans is encoded by the COX17 gene ...
Location of the MT-CO1 gene in the human mitochondrial genome.MT-CO1 is one of the three cytochrome c oxidase subunit mitochondrial genes (orange boxes).. Cytochrome c oxidase I (COX1) also known as mitochondrially encoded cytochrome c oxidase I (MT-CO1) is a protein that is encoded by the MT-CO1 gene in eukaryotes. [6]