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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
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