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TELO2

Telomere length regulation protein TEL2 homolog · UniProt Q9Y4R8

Length
837 aa
Mass
91.7 kDa
Annotated
2026-04-28
58 papers in source corpus 22 papers cited in narrative 22 extracted findings

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

TELO2 is a conserved HEAT-repeat co-chaperone that, together with TTI1 and TTI2 in the TTT complex, governs the folding, stability, and assembly of all six phosphatidylinositol 3-kinase-related kinases (PIKKs: ATM, ATR, DNA-PKcs, mTOR, SMG1, TRRAP) (PMID:18160036, PMID:20801936). TELO2 preferentially engages newly synthesized PIKKs via their HEAT/FAT domains and delivers them to the HSP90 chaperone through a CK2-phosphorylation-dependent interaction with the R2TP (RUVBL1–RUVBL2–RPAP3–PIH1D1) complex, as revealed by crystallographic and cryo-EM structures of the R2TP–TTT assembly (PMID:20864032, PMID:24794838, PMID:34233195, PMID:34838521). Beyond PIKK maturation, TELO2 participates in mTORC1/mTORC2 complex assembly and substrate engagement, nonsense-mediated mRNA decay via SMG1-UPF1, and the ATR–CHK1 S-phase/G2 DNA-damage checkpoint, where it stabilizes CHK1 and facilitates ATR–TopBP1 association (PMID:20427287, PMID:23831331, PMID:17384638, PMID:19097996). Biallelic loss-of-function variants in TELO2 destabilize the TTT complex in patient fibroblasts, linking TELO2 deficiency to a neurodevelopmental disorder (PMID:27132593).

Mechanistic history

Synthesis pass · year-by-year structured walk · 14 steps
  1. 2007 High

    Establishing TELO2 as a pan-PIKK stability factor resolved how a single gene could influence multiple DNA-damage and growth-signaling pathways simultaneously.

    Evidence Conditional MEF knockout, pulse-labeling, and in vitro binding showing Tel2 controls protein-level stability of all six PIKKs through direct HEAT-repeat interaction

    PMID:18160036

    Open questions at the time
    • Mechanism by which Tel2 stabilizes PIKKs (chaperone vs. degradation shield) was unresolved
    • Whether Tel2 acts alone or in a complex was unknown
    • Structural basis of HEAT-repeat recognition not determined
  2. 2007 High

    Discovery that HCLK2/TELO2 associates with ATR–ATRIP–Claspin–CHK1 and prevents unscheduled CHK1 degradation placed TELO2 directly in the replication-stress checkpoint, independent of its general PIKK-stabilizing role.

    Evidence siRNA depletion in human cells with Co-IP, DNA fiber assays, epistasis with ATR/CHK1 mutants

    PMID:17384638

    Open questions at the time
    • Whether TELO2 checkpoint function is separable from its PIKK-maturation role was unclear
    • Direct vs. indirect mechanism for CHK1 stabilization not resolved
  3. 2008 Medium

    Yeast Tel2–Tel1 interaction and DSB recruitment showed that TELO2 function in PIKK regulation is evolutionarily conserved and extends to directing PIKK localization to damage sites.

    Evidence Co-IP and ChIP at DSBs with tel2-1 mutant in S. cerevisiae, computational structural modeling

    PMID:18334620

    Open questions at the time
    • Yeast Tel2 interaction appears Tel1-specific, unclear how this maps to mammalian pan-PIKK function
    • Structural homology to ATRIP is computational, not experimentally validated
  4. 2008 High

    Demonstrating that TELO2 facilitates ATR–TopBP1 association and is required for full ATR kinase activity defined TELO2 as an activating cofactor, not merely a stability factor, for ATR signaling.

    Evidence In vitro ATR kinase reconstitution, Co-IP of ATR-ATRIP-TopBP1-HCLK2 complex, G2 checkpoint assays

    PMID:19097996

    Open questions at the time
    • Whether TELO2 directly bridges ATR and TopBP1 or acts indirectly through complex assembly was not distinguished
  5. 2010 High

    Identification of the TTT complex (TELO2–TTI1–TTI2) and its role in mTORC1/mTORC2 assembly, combined with structural determination of yeast Tel2 HEAT repeats and demonstration that HSP90 is the downstream chaperone, established the complete co-chaperone framework for PIKK biogenesis.

    Evidence Crystal structure of yeast Tel2, nascent-protein labeling, HSP90 inhibitor studies, reciprocal Co-IP and size-exclusion chromatography of TTT with mTOR complexes

    PMID:20427287 PMID:20801936

    Open questions at the time
    • How TTT hands off PIKKs to HSP90 mechanistically was unresolved
    • Whether TTI1 and TTI2 have separable functions beyond scaffolding was unclear
  6. 2010 High

    Discovering that CK2 phosphorylation of TELO2 Ser487/491 creates a docking site for PIH1D1, thereby linking the TTT complex to R2TP/prefoldin, revealed the molecular switch connecting PIKK clients to the HSP90 chaperone machinery.

    Evidence CK2 kinase assay, phosphosite mutagenesis, in vitro PIH1D1 binding, PIKK stability upon phosphomutant expression

    PMID:20864032

    Open questions at the time
    • Whether CK2 phosphorylation is regulated dynamically or constitutive in vivo was uncertain
    • Structural basis of PIH1D1–phospho-Tel2 interaction not yet solved at atomic resolution
  7. 2012 High

    PHD3-mediated prolyl hydroxylation of TELO2 as a prerequisite for ATR binding revealed an oxygen-sensing input into DNA damage signaling, explaining how hypoxia blunts the ATR–CHK1–p53 axis.

    Evidence In vitro hydroxylation assay, PHD3 KO mice, DMOG/hypoxia treatment, ATR signaling and apoptosis readouts

    PMID:22797300

    Open questions at the time
    • Identity of hydroxylated proline residue(s) on TELO2 not mapped
    • Whether hydroxylation affects TELO2 interactions with other PIKKs beyond ATR was not tested
  8. 2013 High

    SCF-Fbxo9-mediated ubiquitination and degradation of Tel2/Tti1 selectively within mTORC1 upon growth factor withdrawal revealed a regulated proteolytic mechanism that differentially controls mTORC1 vs. mTORC2.

    Evidence Co-IP of Fbxo9–Tel2/Tti1, ubiquitination assays, CK2 fractionation, signaling epistasis upon serum deprivation

    PMID:23263282

    Open questions at the time
    • Whether Fbxo9 targets TTT in non-mTOR PIKK contexts was not addressed
    • The structural basis for mTORC1-selective degradation was unclear
  9. 2013 Medium

    Linking CK2-phosphorylated TELO2 to SMG1 stability and NMD activity via UPF1 phosphorylation extended the functional consequences of TELO2's co-chaperone role to RNA quality control.

    Evidence CK2 inhibition, phospho-deficient TELO2 mutants, NMD reporter assays, SMG1-UPF1 Co-IP

    PMID:23831331

    Open questions at the time
    • Magnitude of NMD effect relative to other SMG1 regulators not quantified
    • Single-lab finding without independent replication
  10. 2014 High

    Crystal structures of PIH1D1 bound to phospho-Tel2 peptide and of R2TP–HSP90 subcomplexes provided the atomic-level framework for how the TTT–R2TP–HSP90 chaperone relay is physically organized.

    Evidence X-ray crystallography of PIH1D1-phospho-Tel2, Hsp90-Tah1-Pih1, and Hsp90-RPAP3 complexes with biochemical validation

    PMID:24794838

    Open questions at the time
    • Full reconstituted structure of the entire R2TP-TTT-PIKK assembly was still lacking
  11. 2014 High

    IP7/IP6K2 regulation of CK2 activity toward the TTT complex connected inositol signaling to PIKK-dependent apoptosis, revealing a metabolic input into TELO2 function.

    Evidence In vitro IP7-CK2 kinase assay, genetic and pharmacological epistasis in human cancer and murine B cells

    PMID:24657168

    Open questions at the time
    • Physiological conditions under which IP7 levels regulate TTT phosphorylation in vivo remain undefined
    • Whether IP7 affects all PIKK branches equally was not tested
  12. 2016 Medium

    Biallelic TELO2 loss-of-function variants in patients causing TTT complex instability established TELO2 deficiency as a Mendelian disease gene, though individual PIKK activities appeared preserved in patient cells.

    Evidence Patient fibroblast analysis, immunoblotting for TTT components, PIKK functional assays

    PMID:27132593

    Open questions at the time
    • Why individual PIKK function appeared normal despite reduced TTT levels was unexplained
    • Full clinical phenotypic spectrum and genotype-phenotype correlation not established in this study
  13. 2017 Medium

    MNK kinase promotion of TELO2–mTORC1 association and DEPTOR opposition of this interaction defined TELO2 as a regulated determinant of mTORC1 substrate engagement, beyond simple mTOR stability.

    Evidence Co-IP, rapamycin treatment, MNK inhibitor and siRNA epistasis

    PMID:28178522

    Open questions at the time
    • Direct phosphorylation targets of MNK on TELO2 or mTORC1 not identified
    • Structural basis for DEPTOR-TELO2 competition unresolved
  14. 2021 High

    Cryo-EM structures of the human TTT complex and the R2TP–TTT–TOR assembly at near-atomic resolution revealed the full architecture of the PIKK maturation machinery, showing TTT binds the TOR kinase domain without blocking activity and inhibits RUVBL1/2 ATPase.

    Evidence Cryo-EM at 4.2 Å (TTT) and of R2TP-TTT-TOR, mutagenesis, ATPase assays, clonogenic survival

    PMID:34233195 PMID:34838521

    Open questions at the time
    • Structures of TTT bound to PIKKs other than TOR/ATM are lacking
    • Conformational dynamics of PIKK handoff from TTT to HSP90 not captured
    • Whether TTT remains associated after PIKK folding is complete is unknown

Open questions

Synthesis pass · forward-looking unresolved questions
  • The complete dynamic mechanism by which the TTT–R2TP–HSP90 relay folds and releases mature PIKKs, and how post-translational modifications (hydroxylation, phosphorylation, ubiquitination) are integrated in vivo to tune individual PIKK outputs, remains to be resolved.
  • No time-resolved structural snapshots of the folding cycle exist
  • Relative contribution of TELO2 to each individual PIKK pathway in physiological tissues is unquantified
  • Therapeutic targeting of the TTT complex for PIKK modulation is unexplored

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0044183 protein folding chaperone 4 GO:0098772 molecular function regulator activity 3 GO:0060090 molecular adaptor activity 2
Localization
GO:0005829 cytosol 1
Pathway
R-HSA-392499 Metabolism of proteins 5 R-HSA-73894 DNA Repair 5 R-HSA-162582 Signal Transduction 4 R-HSA-5357801 Programmed Cell Death 2 R-HSA-8953854 Metabolism of RNA 1
Partners
ATMATRHSP90MTORPIH1D1RICTORTTI1TTI2
Complex memberships
R2TP-TTT co-chaperone assemblyTTT complex (TELO2-TTI1-TTI2)

Evidence

Reading pass · 22 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
2007 Mammalian TELO2 (Tel2) controls the stability of all six PIKK family members (ATM, ATR, DNA-PKcs, mTOR, SMG1, TRRAP) at the protein level without affecting their mRNA levels; in vivo pulse-labeling showed Tel2 controls ATM and mTOR stability, and Tel2 binds directly to HEAT repeat segments of ATM and mTOR in vitro and in vivo. Conditional gene deletion in MEFs, in vivo pulse-labeling, co-immunoprecipitation, in vitro binding assays Cell High 18160036
2010 Mammalian Tti1 constitutively interacts with mTOR in both mTORC1 and mTORC2, binds Tel2, and together Tti1 and Tel2 are required not only for PIKK stability but for proper assembly of mTORC1 and mTORC2 complexes; knockdown of either causes disassembly of both complexes. Co-immunoprecipitation, size-exclusion chromatography, siRNA knockdown with phosphorylation assays (S6K1, 4E-BP1, Akt) The Journal of biological chemistry High 20427287
2010 TEL2 is constitutively phosphorylated on conserved serines 487 and 491 by CK2; this CK2 phosphosite enables direct binding to PIH1D1 subunit of the R2TP/prefoldin-like complex in vitro, and failure to interact with R2TP/prefoldin-like complex (via phosphosite mutation) results in instability of PIKKs, principally mTOR and SMG1, establishing TEL2 as a scaffold coordinating R2TP and HSP90 chaperone activities. Proteomic analysis, in vitro binding with phosphosite mutants, co-immunoprecipitation, CK2 kinase assay Molecular cell High 20864032
2010 Tel2 acts with Hsp90 in maturation of PIKK complexes; Tel2 is substoichiometric relative to PIKKs and preferentially binds newly synthesized ATM, ATR, mTOR, and DNA-PKcs; Tel2 complexes contain Tti1-Tti2 and Hsp90, and Hsp90 inhibition disrupts Tel2-PIKK interaction; Tel2 and Hsp90 mediate formation of mTORC1/mTORC2 and ATR-ATRIP. Crystal structure of yeast Tel2 reveals HEAT-like helical repeats forming two α-solenoids; a conserved surface patch mediates Tti1-Tti2 binding. Quantitative immunoblotting, in vivo nascent-protein labeling, Hsp90 inhibitor treatment, X-ray crystallography of yeast Tel2, mutagenesis of conserved surface residues Genes & development High 20801936
2014 PIH1D1 contains a domain specifically for binding CK2 phosphorylation sites on Tel2; crystal/solution structures of Hsp90-Tah1-Pih1, Hsp90-RPAP3, and PIH1D1-Tel2 complexes define the structural basis for R2TP connecting to the TTT complex and thereby to Hsp90. X-ray crystallography, biochemical binding assays, structural analysis of R2TP-TTT interactions Structure High 24794838
2014 Inositol pyrophosphate IP7, generated by IP6K2, binds CK2 to enhance CK2 phosphorylation of the TTT complex (Tel2/Tti1/Tti2), thereby stabilizing DNA-PKcs and ATM, which stimulates p53 phosphorylation at Ser15 to activate cell death; this establishes an apoptotic cascade: IP6K2 → IP7 → CK2 → TTT → PIKKs → p53. In vitro CK2 kinase assay with IP7, co-immunoprecipitation, siRNA knockdown, phosphorylation assays in human cancer and murine B cells Molecular cell High 24657168
2013 SCF-Fbxo9 ubiquitin ligase targets Tel2 and Tti1 for degradation specifically within mTORC1 (not mTORC2) in response to growth factor withdrawal; this degradation is primed by CK2-mediated cytoplasmic phosphorylation of Tel2/Tti1, leading to mTORC1 inactivation while activating PI3K/TORC2/Akt signaling. Co-immunoprecipitation, ubiquitination assay, CK2 fractionation, siRNA/shRNA knockdown, cell-based signaling assays Nature cell biology High 23263282
2013 CK2-mediated phosphorylation of TEL2 increases SMG1 protein stability, which in turn increases UPF1 phosphorylation and augments nonsense-mediated mRNA decay (NMD); loss of TEL2 phosphorylation reduces SMG1-UPF1 complex formation and UPF1-bound PTC-containing mRNA. CK2 inhibition, TEL2 siRNA knockdown, phosphorylation-deficient TEL2 mutants, Co-IP, NMD reporter assays Biochimica et biophysica acta Medium 23831331
2007 HCLK2 (human TELO2/CLK-2) associates with the S-phase checkpoint components ATR, ATRIP, Claspin, and Chk1; its depletion causes accumulation of spontaneous DNA damage in S-phase, radio-resistant DNA synthesis, impaired FANCD2 monoubiquitination, and accelerated Chk1 degradation; ATR-mediated phosphorylation of Chk1-Ser345 targets Chk1 for proteasomal degradation, and HCLK2 prevents this unscheduled degradation. siRNA knockdown, co-immunoprecipitation, flow cytometry, DNA fiber assays, epistasis with ATR/Chk1 mutants Nature cell biology High 17384638
2008 HCLK2 forms a complex with ATR-ATRIP and the ATR activator TopBP1; HCLK2 facilitates efficient ATR-TopBP1 association and is required for ATR autophosphorylation and full-scale ATR kinase activity toward substrates in vitro and in vivo; HCLK2 depletion abrogates the G2 checkpoint. Co-immunoprecipitation, in vitro ATR kinase assay, siRNA knockdown with checkpoint and survival assays The Journal of biological chemistry High 19097996
2012 HCLK2 is hydroxylated by prolyl hydroxylase domain protein PHD3; this hydroxylation is required for HCLK2 interaction with ATR and subsequent activation of ATR/CHK1/p53 signaling; inhibition of PHD3 (by DMOG or hypoxia) prevents ATR/CHK1/p53 activation and decreases DNA damage-induced apoptosis. Co-immunoprecipitation, in vitro hydroxylation assay, PHD3 inhibitor/hypoxia treatment, PHD3 KO mice, apoptosis assays The Journal of clinical investigation High 22797300
2017 MNK kinase engages with mTORC1 and promotes mTORC1 association with TELO2, facilitating mTORC1-substrate binding and sustaining mTORC1 activity following rapamycin treatment; DEPTOR opposes mTORC1-substrate association by preventing TELO2-mTORC1 binding. Co-immunoprecipitation, rapamycin treatment, siRNA/MNK inhibitor assays, mTORC1 substrate phosphorylation readouts Cell reports Medium 28178522
2021 Cryo-EM structure of the human R2TP-TTT complex reveals that the HEAT-repeat TTT complex binds the kinase domain of TOR without blocking its activity, delivers TOR to the R2TP chaperone, inhibits RUVBL1-RUVBL2 ATPase activity, and modulates the conformation and interactions of PIH1D1 and RPAP3 components of R2TP. Cryo-EM structure determination, biochemical ATPase assays, binding experiments Cell reports High 34233195
2021 Cryo-EM structure of the human TELO2-TTI1-TTI2 (TTT) complex at 4.2 Å resolution shows all three proteins form elongated helical repeat structures; TTI1 provides a central platform; the TELO2 C-terminal domain (CTD) binds TTI1 and is required for ATM recruitment; TTI1 N- and C-terminal segments bind the FAT domain and N-terminal HEAT repeats of ATM, respectively; TELO2 CTD and TTI1 segments are required for cell survival after ionizing radiation. Cryo-EM structure, mutagenesis, co-immunoprecipitation, clonogenic survival assay Journal of molecular biology High 34838521
2011 Hsp90 forms complexes with the RUVBL1/2 complex and Tel2 complex and functions as a general regulator of all PIKK family proteins; Hsp90 inhibition causes reduction of all PIKKs and suppresses PIKK-mediated signaling. Co-immunoprecipitation of Hsp90-RUVBL1/2-Tel2 complexes, Hsp90 inhibitor treatment with PIKK immunoblotting Cancer science Medium 21951644
2022 Ivermectin B1a binds directly to TELO2 through its C-terminal α-helix; TELO2 knockdown reduces cytoplasmic β-catenin and β-catenin/TCF transcriptional activation; IVM reduces TELO2 and PIKK protein levels and AKT/S6K phosphorylation; mTOR kinase inhibition reduces cytoplasmic β-catenin, establishing that IVM binds TELO2 to inhibit PIKKs and reduce Wnt/β-catenin signaling. Affinity purification with immobilized ivermectin, TELO2 knockdown, mutagenesis of IVM-binding domain, signaling assays iScience Medium 35530256
2017 Cdc7-Dbf4 phosphorylates HSP90 at Ser164, stabilizing the HSP90-HCLK2-MRN complex and supporting ATM/ATR signaling and homologous recombination DNA repair under replication stress. Phosphoproteomics, in vitro kinase assay, Co-IP of HSP90-HCLK2-MRN complex, ATM/ATR signaling assays Scientific reports Medium 29209046
2020 TELO2 binds RICTOR (mTORC2 component) and promotes CRC cell growth, cell cycle progression, and metastasis in a serum-dependent manner; RICTOR induces degradation of TELO2 upon serum deprivation in an mTOR-independent manner. Co-immunoprecipitation, siRNA knockdown, cell proliferation/invasion/cell cycle assays, serum deprivation experiments Oncology reports Medium 33416177
2019 In cardiomyocytes, Tel2 overexpression increases mTOR expression with concomitant increases in mTORC1 and mTORC2 activity, and protects against ischemic cell death in an mTOR-dependent manner; Tel2 silencing decreases mTOR expression and mTORC1/mTORC2 activity, increasing vulnerability to hypoxia-induced cell death. Adenoviral overexpression, siRNA knockdown, mTOR inhibitor epistasis, cell viability assays in HL-1 cells and adult mouse cardiomyocytes Life sciences Medium 31323273
2016 Compound heterozygous loss-of-function variants in TELO2 cause reduced steady-state levels of TELO2 and other TTT complex components (TTI1, TTI2) in patient fibroblasts, perturbing TTT complex stability; individual PIKK function was normal in cellular assays, indicating TTT complex instability is the primary consequence. Patient fibroblast analysis, immunoblotting for TELO2/TTI1/TTI2 levels, PIKK functional assays American journal of human genetics Medium 27132593
2008 S. cerevisiae Tel2 interacts with Tel1 (ATM ortholog) and this interaction is specifically required for Tel1 localization to a DNA double-strand break and its activation of downstream targets; computational analysis revealed structural homology between Tel2 and Ddc2/ATRIP, suggesting a common structural principle for PIKK partners. Co-immunoprecipitation, chromatin immunoprecipitation at DSBs, genetic analysis with tel2-1 mutant, computational structural modeling Genes & development Medium 18334620
2026 PIWIL1 interacts with the R2TP chaperone complex and promotes its association with TELO2, facilitating mTOR-RAPTOR assembly and mTORC1 activation, leading to selective enhanced translation of TOP mRNAs. Co-immunoprecipitation of PIWIL1-R2TP-TELO2, mTOR-RAPTOR assembly assay, transcriptomic/translatomic/proteomic profiling of PIWIL1-KO cells, mTOR inhibitor epistasis Oncogene Medium 42020726

Source papers

Stage 0 corpus · 58 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2010 Tti1 and Tel2 are critical factors in mammalian target of rapamycin complex assembly. The Journal of biological chemistry 204 20427287
2007 Tel2 regulates the stability of PI3K-related protein kinases. Cell 183 18160036
2010 CK2 phospho-dependent binding of R2TP complex to TEL2 is essential for mTOR and SMG1 stability. Molecular cell 159 20864032
2010 Tel2 structure and function in the Hsp90-dependent maturation of mTOR and ATR complexes. Genes & development 151 20801936
2014 Inositol pyrophosphates mediate the DNA-PK/ATM-p53 cell death pathway by regulating CK2 phosphorylation of Tti1/Tel2. Molecular cell 105 24657168
2007 HCLK2 is essential for the mammalian S-phase checkpoint and impacts on Chk1 stability. Nature cell biology 100 17384638
1996 TEL2, an essential gene required for telomere length regulation and telomere position effect in Saccharomyces cerevisiae. Molecular and cellular biology 86 8649421
2013 SCFFbxo9 and CK2 direct the cellular response to growth factor withdrawal via Tel2/Tti1 degradation and promote survival in multiple myeloma. Nature cell biology 82 23263282
2020 TELO2 induced progression of colorectal cancer by binding with RICTOR through mTORC2. Oncology reports 81 33416177
2014 Structural basis for phosphorylation-dependent recruitment of Tel2 to Hsp90 by Pih1. Structure (London, England : 1993) 76 24794838
2000 Identification and characterization of a new human ETS-family transcription factor, TEL2, that is expressed in hematopoietic tissues and can associate with TEL1/ETV6. Blood 69 10828014
2012 PHD3-dependent hydroxylation of HCLK2 promotes the DNA damage response. The Journal of clinical investigation 64 22797300
2017 MNK Controls mTORC1:Substrate Association through Regulation of TELO2 Binding with mTORC1. Cell reports 57 28178522
2000 Tel-2 is a novel transcriptional repressor related to the Ets factor Tel/ETV-6. The Journal of biological chemistry 45 11108721
2008 Tel2 mediates activation and localization of ATM/Tel1 kinase to a double-strand break. Genes & development 44 18334620
2009 Functional dissection of Caenorhabditis elegans CLK-2/TEL2 cell cycle defects during embryogenesis and germline development. PLoS genetics 40 19360121
2006 Tel2 is required for activation of the Mrc1-mediated replication checkpoint. The Journal of biological chemistry 39 17189249
2011 Heat shock protein 90 regulates phosphatidylinositol 3-kinase-related protein kinase family proteins together with the RUVBL1/2 and Tel2-containing co-factor complex. Cancer science 38 21951644
2008 HCLK2 is required for activity of the DNA damage response kinase ATR. The Journal of biological chemistry 37 19097996
2005 The novel ETS factor TEL2 cooperates with Myc in B lymphomagenesis. Molecular and cellular biology 36 15743832
2005 The ETS factor TEL2 is a hematopoietic oncoprotein. Blood 36 16234363
2015 TEL2 suppresses metastasis by down-regulating SERPINE1 in nasopharyngeal carcinoma. Oncotarget 35 26335051
2016 A Syndromic Intellectual Disability Disorder Caused by Variants in TELO2, a Gene Encoding a Component of the TTT Complex. American journal of human genetics 33 27132593
1998 The yeast telomere length regulator TEL2 encodes a protein that binds to telomeric DNA. Nucleic acids research 28 9490802
2021 Structure of the TELO2-TTI1-TTI2 complex and its function in TOR recruitment to the R2TP chaperone. Cell reports 26 34233195
2018 Branching the Tel2 pathway for exact fit on phosphatidylinositol 3-kinase-related kinases. Current genetics 26 29470645
2017 Cdc7-Dbf4-mediated phosphorylation of HSP90-S164 stabilizes HSP90-HCLK2-MRN complex to enhance ATR/ATM signaling that overcomes replication stress in cancer. Scientific reports 22 29209046
2018 Snail promotes metastasis of nasopharyngeal carcinoma partly by down-regulating TEL2. Cancer communications (London, England) 21 30253797
2022 Tel2 regulates redifferentiation of bipotential progenitor cells via Hhex during zebrafish liver regeneration. Cell reports 18 35385752
2007 Tel2: a common partner of PIK-related kinases and a link between DNA checkpoint and nutritional response? Genes to cells : devoted to molecular & cellular mechanisms 18 18076567
2004 TEL2, an ETS factor expressed in human leukemia, regulates monocytic differentiation of U937 Cells and blocks the inhibitory effect of TEL1 on ras-induced cellular transformation. Cancer research 18 15342392
2017 Novel compound heterozygous mutations in TELO2 in a patient with severe expression of You-Hoover-Fong syndrome. Molecular genetics & genomic medicine 16 28944240
2013 CK2-mediated TEL2 phosphorylation augments nonsense-mediated mRNA decay (NMD) by increase of SMG1 stability. Biochimica et biophysica acta 15 23831331
2017 MNK inversely regulates TELO2 vs. DEPTOR to control mTORC1 signaling. Molecular & cellular oncology 14 28616583
2014 Hsp90 picks PIKKs via R2TP and Tel2. Structure (London, England : 1993) 13 24918336
2009 FANCM-FAAP24 and HCLK2: roles in ATR signalling and the Fanconi anemia pathway. Cell cycle (Georgetown, Tex.) 13 19282663
2021 Structure of the Human TELO2-TTI1-TTI2 Complex. Journal of molecular biology 10 34838521
2019 A tel2 Mutation That Destabilizes the Tel2-Tti1-Tti2 Complex Eliminates Rad3ATR Kinase Signaling in the DNA Replication Checkpoint and Leads to Telomere Shortening in Fission Yeast. Molecular and cellular biology 10 31332096
2019 NOTCH1 signaling in oral squamous cell carcinoma via a TEL2/SERPINE1 axis. Oncotarget 10 31827722
2016 CK2 phospho-independent assembly of the Tel2-associated stress-signaling complexes in Schizosaccharomyces pombe. Genes to cells : devoted to molecular & cellular mechanisms 10 27935167
2012 The SAM domain of human TEL2 can abrogate transcriptional output from TEL1 (ETV-6) and ETS1/ETS2. PloS one 10 22615925
2020 Cataract in You-Hoover-Fong syndrome: TELO2 deficiency. Ophthalmic genetics 9 32940098
2023 TELO2-related syndrome (You-Hoover-Fong syndrome): Description of 14 new affected individuals and review of the literature. American journal of medical genetics. Part A 8 36797513
2022 Ivermectin represses Wnt/β-catenin signaling by binding to TELO2, a regulator of phosphatidylinositol 3-kinase-related kinases. iScience 8 35530256
2021 CLK-2/TEL2 is a conserved component of the nonsense-mediated mRNA decay pathway. PloS one 8 33444416
2016 Overexpression of TELO2 decreases survival in human high-grade gliomas. Oncotarget 8 27329594
2020 Milder presentation of TELO2-related syndrome in two sisters homozygous for the p.Arg609His pathogenic variant. European journal of medical genetics 7 33307281
2008 Mec1 function in the DNA damage response does not require its interaction with Tel2. Cell cycle (Georgetown, Tex.) 7 19029808
2023 TTT (Tel2-Tti1-Tti2) Complex, the Co-Chaperone of PIKKs and a Potential Target for Cancer Chemotherapy. International journal of molecular sciences 6 37175973
2012 Drosophila Tel2 is expressed as a translational fusion with EpsinR and is a regulator of wingless signaling. PloS one 5 23029494
1991 Morphological analysis of the cellular interaction between thymocytes and a thymic stromal cell line (TEL-2). The Anatomical record 5 1928758
2012 Genetic and physical interactions between Tel2 and the Med15 Mediator subunit in Saccharomyces cerevisiae. PloS one 3 22291956
2011 Saccharomyces cerevisiae Tel2 plays roles in TORC signaling and telomere maintenance that can be mutationally separated. Biochemical and biophysical research communications 3 22227188
1996 Morphological study of thymus stromal cells (TEL-2 cell) which play a role in the elimination of double positive immature thymocytes by phagocytosis. The Anatomical record 3 8742694
2023 Novel compound heterozygous mutations in TELO2 in an infant with You-Hoover-Fong syndrome: A case report and literature review. Open life sciences 2 37215500
2019 The effects of Tel2 on cardiomyocyte survival. Life sciences 1 31323273
2026 PIWIL1 activates the R2TP-TELO2-mTORC1 axis independently of piRNA to promote TOP mRNA translation in gastric cancer. Oncogene 0 42020726
2025 [Analysis of a child with You-Hoover-Fong syndrome due to compound heterozygous variants of the TELO2 gene and a literature review]. Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics 0 41645377