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FLASH GENE
Symbol SCO1 contributors: mct - updated : 11-07-2014
HGNC name SCO cytochrome oxidase deficient homolog 1 (yeast)
HGNC id 10603
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N terminal mitochondrial leader peptide
  • a putuative transmembrane domain
  • a copper-binding domain common to yeast Sco1, Sco2
  • a single transmembrane domain
  • conjugated MetalloP
    HOMOLOGY
    interspecies homolog to yeast sco1
    intraspecies paralog to SCO2
    Homologene
    FAMILY SCO1/2 family
    CATEGORY chaperone/stress , transport carrier
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    text inner mitochondrial membrane in yeast
    basic FUNCTION
  • either involved in transport or in the insertion of copper into the active site of the enzyme
  • specifically required for a post-translational step of c oxidase (copper delivery to cytochrome oxidase)
  • can act both as a copper chaperone and a thioredoxin
  • function as copper-binding protein
  • implicated in the copper metallation of the Cu(A) site in MT-CO2 of cytochrome oxidase
  • although COX11 and SCO1 play distinct roles in COX assembly, they seem to play overlapping or related roles in peroxide metabolism
  • SCO1 and SCO2 are metallochaperones whose principal function is to add two copper ions to the catalytic core of cytochrome c oxidase (COX)
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS cellular trafficking transport
    text mitochondrial copper transport
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • interacting with SCO2 for copper delivery to cytochrome C oxidase
  • SCO2 acts upstream of SCO1, and that it is indispensable for CO II synthesis
  • COX19 is necessary for the transduction of a SCO1-dependent mitochondrial redox signal that regulates ATP7A-mediated cellular copper efflux
  • COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) HFND
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS