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| Cat.No | ACP04020 | Target Name | SCO2 |
|---|---|---|---|
| Form | Liquid or Lyophilized powder | Expression System | E.coli |
| Expression Range | 43-266aa | Mol Weight | 41.1kDa |
| Protein Length | Full Length of Mature Protein | Purity | Greater than 90% as determined by SDS-PAGE. |
| Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | Uniprot ID | O43819 |
|---|
Uniprot Id
O43819
Target Species
Human
Target Name
SCO2
Target Full Name
Protein SCO2 homolog, mitochondrial
Target Function
Copper metallochaperone essential for the synthesis and maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Involved in transporting copper to the Cu(A) site on MT-CO2/COX2. Also acts as a thiol-disulfide oxidoreductase to regulate the redox state of the cysteines in SCO1 during maturation of MT-CO2/COX2.
Target Involvement
Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency 1 (CEMCOX1); Myopia 6 (MYP6); Leigh syndrome (LS)
Target Subcellular Location
Mitochondrion inner membrane; Single-pass membrane protein.
Target Protein Families
SCO1/2 family
Target Tissue Specificity
Ubiquitous.
Target Research Area
Metabolism
Target Synonyms
Cytochrome oxidase deficient homolog 2; MGC125823; MGC125825; OTTHUMP00000196774; OTTHUMP00000196775; Protein SCO2 homolog; mitochondrial; SCO (cytochrome oxidase deficient; yeast) homolog 2; SCO 1L; SCO 2; SCO cytochrome oxidase deficient homolog 2 (yeast); SCO cytochrome oxidase deficient homolog 2; SCO1L; SCO2; SCO2_HUMAN; Synthesis of cytochrome c oxidase 2
Target Background
Cytochrome c oxidase (COX) catalyzes the transfer of electrons from cytochrome c to molecular oxygen, which helps to maintain the proton gradient across the inner mitochondrial membrane that is necessary for aerobic ATP production. Human COX is a multimeric protein complex that requires several assembly factors; this gene encodes one of the COX assembly factors. The encoded protein is a metallochaperone that is involved in the biogenesis of cytochrome c oxidase subunit II. Mutations in this gene are associated with fatal infantile encephalocardiomyopathy and myopia 6.
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