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FLASH GENE
Symbol OXA1L contributors: mct - updated : 13-07-2021
HGNC name oxidase (cytochrome c) assembly 1-like
HGNC id 8526
EXPRESSION
Type ubiquitous
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestiveliver   highly
Endocrineadrenal gland   highly
 thyroid   highly
Reproductivefemale systemuteruscervix highly
cell lineage
cell lines many cell lines
fluid/secretion
at STAGE
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • C-terminal tail is largely monomeric in solution but does tend to oligomerize at increasing salt concentrations
  • HOMOLOGY
    Homologene
    FAMILY OXA1/OXAA family
    CATEGORY enzyme
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,inner
    intracellular,cytoplasm,organelle,mitochondria,matrix
    intracellular,cytoplasm,organelle,membrane
    basic FUNCTION
  • required for the insertion of integral membrane proteins into the mitochondrial inner membrane
  • essential for the activity and assembly of cytochrome oxidase
  • represents a mitochondrial integral membrane protein required for the correct biogenesis of F(1)F(o)-ATP synthase and NADH:ubiquinone oxidoreductase
  • is required for the assembly of multiple respiratory chain complexes
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • OXA1L-C-terminal approximately 100-amino acid tail of OXA1L (Oxa1L-CTT) cross-links to mammalian mitochondrial homologs of the bacterial ribosomal proteins RPL13, RPL20, and RPL28
  • cardiolipin is required to stabilise the interaction of the mitochondrial ribosome with the membrane via its association with OXA1L during active translation
  • mitoribosomes interact with the OXA1L insertase to couple protein synthesis and membrane delivery
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    related resource MITOP database
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional germinal mutation      
    cause mitochondrial encephalopathy and a combined oxidative phosphorylation defect
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS