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FLASH GENE
Symbol TELO2 contributors: mct - updated : 25-05-2018
HGNC name TEL2, telomere maintenance 2, homolog (S. cerevisiae)
HGNC id 29099
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
secondary structure
  • HEAT-like helical repeats that assemble into two separate alpha-solenoids
    • HOMOLOGY
    interspecies ortholog to S. cerevisiae Tel2
    Homologene
    FAMILY
    CATEGORY regulatory
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,nucleus
    basic FUNCTION
  • TTI1 and TELO2 are important not only for MTOR stability but also for assembly of the MTOR complexes to maintain their activities
  • acts with HSP90AA1 in the maturation of PIKK complexes
  • acts as a scaffold to coordinate the activities of R2TP/prefoldin-like and HSP90 chaperone complexes during the assembly of the phosphatidylinositol 3-kinase-related kinases (PIKKs)
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
  • TTI1 and TTI2 exist in multiple complexes, including a 2-MDa complex with TELO2, called the Triple T complex, and phosphoinositide-3-kinase-related protein kinases (PIKKs) such as ataxia telangiectasia-mutated (ATM)
  • TELO2, TTI2, and TTI1 are the three components of the conserved Triple T (TTT) complex that modulates activity of phosphatidylinositol 3-kinase-related protein kinases
    • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • SMG1 associated with TELO2 and TELO2 is a highly conserved regulator of PIKK stability
  • TTI1 binds to TELO2 in mammalian cells, and interacts with and stabilizes all six members of the PIKK family of proteins (MTOR, ATM, ATR, DNA-PKcs, SMG1, and TRRAP)
  • TELO2-TTI1-TTI2 complex is a PIKK-specific cochaperone for HSP90AA1
  • DNA-PKcs and ATM are stabilized by the TTT (TELO2, TTI1, TTI2) cochaperone family, whose actions are mediated by CSNK2A1 phosphorylation
  • DLX2 expression reduces the protein components of the TTI1/TTI2/TELO2 complex, a key complex required for the proper folding and stabilization of ATM and other members of the PIKK (phosphatidylinositol 3-kinase-related kinase) family kinase
  • TTI2 interacts with TTI1 and TELO2 to form the Triple T complex, which is required for various cellular processes, including the double-strand DNA break response, nonsense-mediated mRNA decay, and telomerase assembly
  • TTT complex is essential for the expression, maturation, and stability of ATM and ATR in response to DNA damage
    • cell & other
    REGULATION
    Phosphorylated by constitutively phosphorylated on conserved serines 487 and 491 by casein kinase 2 (CSNK2A2)
    ASSOCIATED DISORDERS
    corresponding disease(s) MMRAV
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • homozygosity for a Telo2 knockout allele in mice produces embryonic lethality and S phase cell-cycle arrest in mouse embryonic fibroblasts (MEFs)