Selected-GenAtlas references SOURCE GeneCards NCBI Gene Swiss-Prot Ensembl
HGNC UniGene Nucleotide OMIM UCSC
Home Page
FLASH GENE
Symbol COA3 contributors: mct - updated : 18-03-2015
HGNC name cytochrome c oxidase assembly factor 3
HGNC id 24990
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a single pass transmembrane domain (from AAs 58 to 78)
  • a protein-protein interaction coiled coil domain (from AAs 79 to 104)
  • C terminus exposed to the intermembrane space (IMS)
  • HOMOLOGY
    interspecies homolog to Drosophila CG42630
    ortholog to murine Ccdc56
    Homologene
    FAMILY coiled coil domain-containing family of protein
    CATEGORY unknown/unspecified
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    text
  • efficiently imported into mitochondria and became protected against protease treatment
  • basic FUNCTION
  • COA3 and COX14 are essential for negative feedback regulation of COX1 translation in mitochondria
  • important role for COA3 in the oxidative phosphorylation system and in particular in COX function
  • plays a critical role in the biogenesis and activity of complex IV and is therefore essential for the function of the OXPHOS system
  • inner-mitochondrial-membrane protein with its C terminus exposed to the intermembrane space (IMS)
  • is a new factor essential for COX biogenesis
  • stabilizes COX1 co-translationally and promotes its assembly with COX partner subunits
  • positively regulates mitochondrial fission via regulation of COX activity and the mitochondrial recruitment of DNM1L
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • COA3 and COX14 form assembly intermediates with newly synthesized COX1 and are required for MSS51 association with these complexes
  • COA3 interacted with newly synthesized COX1
  • specific role of COA3 in COX1 translation, indicating a tight coupling between complex assembly and the translational regulation of its core subunit
  • interacts with both the mitochondrial translation machinery and COX structural subunits
  • ATAD3A interacted with COA3, a subunit of the cytochrome oxidase (COX)-assembly complex
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional     --low  
    induces a complex IV assembly defect
    Susceptibility
    Variant & Polymorphism
    Candidate gene
  • new candidate when screening for genes responsible for mitochondrial diseases associated with COX deficiency
  • Marker
    Therapy target
    ANIMAL & CELL MODELS