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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
DNA
TYPE functioning gene
STRUCTURE 17.12 kb     21 Exon(s)
MAPPING cloned Y linked N status provisional
RNA
TRANSCRIPTS type messenger
identificationnb exonstypebpproduct
ProteinkDaAAspecific expressionYearPubmed
21 - 3338 - 837 - 2007 18160036
EXPRESSION
Type
   expressed in (based on citations)
organ(s)
SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
Digestiveliver   highly
Nervousbrain   highly
Reproductivefemale systemovary  highly
 male systemtestis  highly
Respiratorylung   highly
Urinarykidney   highly
cell lineage
cell lines
fluid/secretion
at STAGE
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)