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FLASH GENE
Symbol COX1 contributors: mct/ - updated : 22-05-2019
HGNC name mitochondrially encoded cytochrome c oxidase I
HGNC id 7419
Corresponding disease
AINHL aminoglycoside-induced and nonsyndromic hearing loss
LHON Leber hereditary optic neuropathy
MTCO1 mitochondrial motor neuron disease
Location mt      Physical location : -
Synonym name
  • cytochrome c oxidase polypeptide I
  • cytochrome c oxidase subunit I
    • Synonym symbol(s) COI, MTCO1, COXI, MT-CO1
    EC.number 1.9.3.1
    DNA
    TYPE functioning gene
    STRUCTURE 1.00 kb     1 Exon(s)
    MAPPING cloned   linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    1 - - 57 513 - -
    EXPRESSION
    Type
    constitutive of
       expressed in (based on citations)
    organ(s)
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
    HOMOLOGY
    Homologene
    FAMILY
  • heme-copper respiratory oxidase family, complex IV subunit
    • CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    basic FUNCTION
  • catalyzing the reduction of oxygen to water
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS respiration , electron transport
    PATHWAY
    metabolism
    signaling
    a component
  • component of the respiratory chain
    • INTERACTION
    DNA
    RNA
    small molecule metal binding,
  • Cu2+
  • Fe2+
    • protein
  • COA1 links the MSS51 post-translational function to COX1 cofactor insertion in cytochrome c oxidase assembly
  • COA3 and COX14 form assembly intermediates with newly synthesized COX1 and are required for MSS51 association with these complexes
  • COA3, is a novel regulator of mitochondrial COX1 translation and cytochrome oxidase assembly
  • COA3 interacted with newly synthesized COX1, and specific role of COA3 in COX1 translation, indicating a tight coupling between complex assembly and the translational regulation of its core subunit
  • in addition to affecting MSS51-dependent heme sensing, oxidative stress compromises MSS51 roles in COX1 mRNA processing and translation
  • COA1 plays an important role in the assembly of COX1 within the respiratory chain complex
    • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) MTCO1 , AINHL , LHON
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS