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FLASH GENE
Symbol COX10 contributors: shn - updated : 15-10-2010
HGNC name COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase (yeast)
HGNC id 2260
EXPRESSION
Type widely
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Cardiovascularheart   highly
Digestiveintestinesmall intestine  lowly
 intestinelarge intestinecolon moderately
 liver   lowly
Endocrinepancreas   lowly
Lymphoid/Immunespleen   lowly
 thymus   lowly
Nervousbrain   lowly
Reproductivefemale systemplacenta  lowly
 female systemovary  lowly
 male systemprostate  lowly
 male systemtestis  highly
Respiratorylung   lowly
Urinarykidney   lowly
tissue
SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
Muscularstriatumskeletal highly
cells
SystemCellPubmedSpeciesStageRna symbol
Blood/Hematopoieticleukocyte
cell lineage
cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • a long relatively hydrophilic N terminal
  • seve to nine potential transmembrane segments (TM9)
  • mono polymer homomer , complex
    HOMOLOGY
    interspecies ortholog to COX10, S.cerevisiae
    ortholog to COX10, Pan troglodytes
    ortholog to Cox10, Mus musculus
    ortholog to cox10, Danio rerio
    Homologene
    FAMILY
  • ub1a prenyltransferase family
  • CATEGORY enzyme , transport
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,membrane
    text localized in mitochondrial inner membrane
    basic FUNCTION
  • terminal component of the mitochondrial respiratory chain that catalyzes the electron transfer from reduced cytochrome c to oxygen
  • catalyzes the conversion of protoheme (heme B) to heme O via the farnesylation of a vinyl group at position C2 in the first step of the mitochondrial heme A biosynthetic
  • involved in heme A biosynthesis
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS mitochondrial transport , electron transport
    text cellular respiration
    PATHWAY
    metabolism energetic
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • ISCU (iron-sulfur cluster scaffold homolog) and COX10 (cytochrome c oxidase assembly protein), two important factors of the mitochondria electron transport chain and the tricarboxylic acid cycle have been identified as potential targets of MIR210
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) COX10D
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional   deletion    
    in HNPP (hereditary neuropathy with liability to pressure palsies)
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • COX10-null mice have only 20-40% of wild-type mitochondrial function and the transgenic overexpressionof PGC-1a in these mice restores full mitochondrial function
  • COX10-/- mice treated with bezafibrate have 85% of WT OxPhos activity and their muscle function is improved compared with untreated littermates