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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
DNA
TYPE functioning gene
STRUCTURE 17.24 kb     6 Exon(s)
10 Kb 5' upstream gene genomic sequence study
MAPPING cloned Y linked N status provisional
Map pter - D17S804 - MYH8 - SCO1 - COX10 - D17S799 - cen
Authors GeneMap (98)
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
6 - 1768 - 301 - -
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularvessel   predominantly Homo sapiens
Digestiveesophagus   highly
 liver   highly Homo sapiens
Nervousnervecranial nerve  highly
Reproductivefemale systemuteruscervix highly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Connectivebone  highly
Muscularstriatumcardiac  
cell lineage
cell lines
fluid/secretion
at STAGE
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