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FLASH GENE
Symbol SCO2 contributors: mct - updated : 06-06-2013
HGNC name SCO cytochrome oxidase deficient homolog 2 (yeast)
HGNC id 10604
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N terminal mitochondrial targeting presequence
  • a "core" region of greatest sequence identity and
  • a containing a copper binding CXXXC motif
  • a single transmembrane domain
  • conjugated MetalloP
    HOMOLOGY
    interspecies homolog to yeast SCO2
    homolog to C.elegans 01f1.2
    intraspecies paralog to SCO1
    Homologene
    FAMILY SCO1/2 family
    CATEGORY chaperone/stress , enzyme , transport carrier
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,cytoplasm,organelle,membrane
    basic FUNCTION
  • catalyzing the synthesis of cytochrome C oxidase 2
  • mitochondrial inner membrane protein that probably functions as a copper transporter, involved in mitochondria copper delivery
  • functioning as copper-binding protein
  • essential for the assembly of the catalytic core of cytochrome oxidase (COX)
  • involved in copper supply for the assembly of cytochrome c oxidase in eukaryotes (Banci 2007)
  • SCO2 acts upstream of SCO1, and that it is indispensable for CO II synthesis
  • cytochrome c oxidase assembly factor, is a metallochaperone which is involved in the biogenesis of cytochrome c oxidase subunit II
  • might possess a novel tumor suppressor function via the ROS-MAP3K5 kinase pathway and thus could be an important candidate for anticancer gene therapy
  • SCO1 and SCO2 are metallochaperones whose principal function is to add two copper ions to the catalytic core of cytochrome c oxidase (COX)
  • copper chaperone integral to oxygen reduction catalysis by cytochrome c oxidase of the mitochondrial respiratory chain
  • important for cytochrome C oxidase (COX) synthesis
  • copper homeostasis protein influential in mitochondrial cytochrome c oxidase activity
  • may play an important role in eye growth and development, particularly in those who become highly myopic
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS cellular trafficking transport
    text mitochondrial copper transport
    PATHWAY
    metabolism
    signaling
    a component COX assembly gene
    INTERACTION
    DNA
    RNA
    small molecule metal binding,
    copper Cu2+
    protein
  • interacting with SCO1 for copper delivery to cytochrome C oxidase
  • TP53 can modulate the metabolic pathways via the proteins SCO2 and TIGAR in human breast cancer
  • 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) CEML
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    in breast cancer (
    Susceptibility to high-grade myopia
    Variant & Polymorphism other
  • four heterozygous mutations—c.157C>T (p.Gln53*), c.341G>A (p.Arg114His), c.418G>A (p.Glu140Lys), and c.776C>T (p.Ala259Val)—associated with high-grade myopia
  • Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS