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
EXPRESSION
Type
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheart    
Digestivesalivary gland    
Endocrineparathyroid   highly
Nervousnervecranial nerve   
cell lineage
cell lines
fluid/secretion lymph
at STAGE
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