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SCO2 cytochrome c oxidase assembly protein
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols SCO2 ; CEMCOX1; MYP6; SCO1L
External IDs OMIM604272 HomoloGene68444 GeneCards: SCO2 Gene
RNA expression pattern
PBB GE ECGF1 205241 at tn.png
More reference expression data
Species Human Mouse
Entrez 9997 100126824
Ensembl ENSG00000130489 ENSMUSG00000091780
UniProt O43819 Q8VCL2
RefSeq (mRNA) NM_001169109 NM_001111288
RefSeq (protein) NP_001162580 NP_001104758
Location (UCSC) Chr 22:
50.96 – 50.96 Mb
Chr 15:
89.37 – 89.37 Mb
PubMed search [1] [2]

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.[1][2][3]

Mammalian cytochrome c oxidase (COX) catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. In yeast, 2 related COX assembly genes, SCO1 and SCO2 (synthesis of cytochrome c oxidase), enable subunits 1 and 2 to be incorporated into the holoprotein. This gene is the human homolog of the yeast SCO2 gene.[3]

Mutations in this gene that alter the regulation of copper and oxygen levels are associated with a severe COX deficiency in striated muscle, an early onset fatal cardiac encephalomyopathy,[4] and a severe form of nearsightedness.


  1. ^ Paret C, Ostermann K, Krause-Buchholz U, Rentzsch A, Rodel G (May 1999). "Human members of the SCO1 gene family: complementation analysis in yeast and intracellular localization". FEBS Lett 447 (1): 65–70. doi:10.1016/S0014-5793(99)00266-5. PMID 10218584. 
  2. ^ Horng YC, Leary SC, Cobine PA, Young FB, George GN, Shoubridge EA, Winge DR (Oct 2005). "Human Sco1 and Sco2 function as copper-binding proteins". J Biol Chem 280 (40): 34113–22. doi:10.1074/jbc.M506801200. PMID 16091356. 
  3. ^ a b "Entrez Gene: SCO2 SCO2 cytochrome c oxidase assembly protein [ Homo sapiens (human) ]". 
  4. ^ Jaksch, M.; Paret, C.; Stucka, R.; Horn, N.; Müller-Höcker, J.; Horvath, R.; Trepesch, N.; Stecker, G.; Freisinger, P.; Thirion, C.; Müller, J.; Lunkwitz, R.; Rödel, G.; Shoubridge, E. A.; Lochmüller, H. (2001). "Cytochrome c oxidase deficiency due to mutations in SCO2, encoding a mitochondrial copper-binding protein, is rescued by copper in human myoblasts". Human Molecular Genetics 10 (26): 3025–3035. doi:10.1093/hmg/10.26.3025. PMID 11751685.  edit

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