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    GENE:

    ATM (ATM serine/threonine kinase)

    i
    Other names: ATM, ATA, ATC, ATD, ATDC, TEL1, TELO1, ATM serine/threonine kinase
    2d
    Amplification and Overexpression of Cyclin D1 in Epstein-Barr Virus-Positive Inflammatory Follicular Dendritic Cell Sarcoma: A Targeted Next-Generation Sequencing Study. (PubMed, Mod Pathol)
    In addition, tumor cells exhibited a type II EBV latency pattern (LMP1+/EBNA2-) in 84.6% of cases, and LMP1 expression correlated with larger tumor size (p=0.021). Taken together, these findings support a role for EBV-driven activation of the cyclin D1 pathway and suggest that EBV-LMP1-CCND1 signaling may contribute to the pathogenesis of IFDCS.
    Journal • Next-generation sequencing
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    MYC (V-myc avian myelocytomatosis viral oncogene homolog) • ATM (ATM serine/threonine kinase) • CCND1 (Cyclin D1)
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    ATM mutation • EZH2 mutation
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    TruSight Oncology 500 Assay
    4d
    Impact of ataxia-telangiectasia mutated (ATM) loss on radiobiological and immune response to radium-223 in prostate cancer in vitro models. (PubMed, Clin Transl Radiat Oncol)
    These findings demonstrate that ATM-deficiency enhances radiobiological response and amplifies immune activation to 223Ra, supporting further pre-clinical evaluation of ATM loss as a determinant of response and therapeutic target to optimise 223Ra based strategies for mCRPC.
    Preclinical • Journal • BRCA Biomarker • PARP Biomarker
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    BRCA1 (Breast cancer 1, early onset) • BRCA2 (Breast cancer 2, early onset) • ATM (ATM serine/threonine kinase) • CXCL10 (Chemokine (C-X-C motif) ligand 10) • STING (stimulator of interferon response cGAMP interactor 1) • TP53BP1 (Tumor Protein P53 Binding Protein 1) • IFIT2 (Interferon Induced Protein With Tetratricopeptide Repeats 2)
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    BRCA2 mutation • BRCA1 mutation
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    Xofigo (radium Ra-223 dichloride)
    7d
    Targeted Early Detection Program in Men at High Genetic Risk for Prostate Cancer (clinicaltrials.gov)
    P=N/A, N=200, Recruiting, University of Michigan Rogel Cancer Center
    New trial
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    BRCA1 (Breast cancer 1, early onset) • ATM (ATM serine/threonine kinase) • BRCA (Breast cancer early onset) • CHEK2 (Checkpoint kinase 2) • BRIP1 (BRCA1 Interacting Protein C-terminal Helicase 1) • RAD51C (RAD51 paralog C) • RAD51D (RAD51 paralog D) • NBN (Nibrin Nijmegen Breakage Syndrome 1 (Nibrin)) • ABRAXAS1 (Abraxas 1 BRCA1 A Complex Subunit 2) • GEN1 (GEN1 Holliday junction 5' flap endonuclease) • HOXB13 (Homeobox B13)
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    TP53 mutation • BRCA1 mutation • ATM mutation • PALB2 mutation • CHEK2 mutation • BRIP1 mutation • RAD51C mutation • RAD51D mutation • BRCA mutation
    7d
    Avelumab and M6620 for the Treatment of DDR Deficient Metastatic or Unresectable Solid Tumors (clinicaltrials.gov)
    P1/2, N=23, Active, not recruiting, M.D. Anderson Cancer Center | Trial completion date: Dec 2025 --> Jan 2027
    Trial completion date
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    BRCA1 (Breast cancer 1, early onset) • BRCA2 (Breast cancer 2, early onset) • ATM (ATM serine/threonine kinase) • ARID1A (AT-rich interaction domain 1A) • BAP1 (BRCA1 Associated Protein 1) • MSH2 (MutS Homolog 2) • CDK12 (Cyclin dependent kinase 12) • ATRX (ATRX Chromatin Remodeler) • CHEK2 (Checkpoint kinase 2) • SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1) • RAD51 (RAD51 Homolog A) • FANCA (FA Complementation Group A) • BRIP1 (BRCA1 Interacting Protein C-terminal Helicase 1) • RAD51C (RAD51 paralog C) • CD4 (CD4 Molecule) • RAD51D (RAD51 paralog D) • BARD1 (BRCA1 Associated RING Domain 1) • NBN (Nibrin Nijmegen Breakage Syndrome 1 (Nibrin)) • FANCF (FA complementation group F) • FANCM (FA Complementation Group M) • FANCD2 (FA Complementation Group D2) • FANCE (FA Complementation Group E) • FANCC (FA Complementation Group C)
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    Bavencio (avelumab) • berzosertib (M6620)
    13d
    The Role of Hematopoietic Cell Transplantation in Ataxia-Telangiectasia. (PubMed, Pediatr Blood Cancer)
    The use of HCT for the treatment of immunodeficiency and relapsed/refractory hematologic malignancies in patients with A-T is not well described and understood. Current evidence is limited and heterogeneous, supporting its use only in select high-risk patients at specialized centers, with multicenter collaboration needed to define best practices.
    Review • Journal
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    ATM (ATM serine/threonine kinase)
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    fludarabine IV
    14d
    Paired ctDNA analysis reveals diverse resistance mechanisms to mobocertinib in EGFR exon 20 insertion NSCLC. (PubMed, Front Oncol)
    Mobocertinib demonstrated clinically meaningful activity, regardless of previous exposure to amivantamab, in EGFR exon 20 insertion-positive NSCLC subjects. Acquired resistance mechanisms to mobocertinib were diverse, which poses challenges to sustained efficacy, emphasizing the need for development of a tailored subsequent therapeutic strategy.
    Journal • Circulating tumor DNA
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    EGFR (Epidermal growth factor receptor) • ATM (ATM serine/threonine kinase)
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    EGFR mutation • EGFR amplification • EGFR exon 20 insertion • EGFR exon 20 mutation • KRAS amplification
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    Exkivity (mobocertinib)
    17d
    Ceralasertib Monotherapy in Patients with ATM-Altered Advanced Solid Tumors or Metastatic Castration-Resistant Prostate Cancer: Data from the Phase 2a PLANETTE Study. (PubMed, Cancer Res Commun)
    Ceralasertib monotherapy was tolerated; however, responses were limited. Alternative patient selection and combination treatments are being explored.
    P2a data • Journal
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    ATM (ATM serine/threonine kinase)
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    ceralasertib (AZD6738)
    20d
    ATM-kinase deficiency triggers early multi-compartment remodeling of the cerebellar microenvironment. (PubMed, Acta Neuropathol Commun)
    Consistently, the compromised cerebellar output is confirmed by the progressive motor impairment developed by AtmKDF/CNS-KO mice. Our findings indicate that Atm kinase deficiency disrupts cerebellar homeostasis through interconnected and bidirectional mechanisms involving ECM remodeling, astrocytosis, impaired oligodendrocyte functioning and altered intrinsic excitability, reflecting a network level destabilization of the cerebellar microenvironment.
    Journal
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    ATM (ATM serine/threonine kinase)
    21d
    Study of Tuvusertib (M1774) in Combination With DNA Damage Response Inhibitor or Immune Checkpoint Inhibitor (DDRiver Solid Tumors 320) (clinicaltrials.gov)
    P1, N=120, Active, not recruiting, EMD Serono Research & Development Institute, Inc. | Trial completion date: Apr 2026 --> Jan 2027 | Trial primary completion date: Apr 2026 --> Jan 2027
    Trial completion date • Trial primary completion date • Checkpoint inhibition • IO biomarker
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    ATM (ATM serine/threonine kinase) • ARID1A (AT-rich interaction domain 1A)
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    ARID1A mutation
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    Bavencio (avelumab) • tuvusertib (M1774) • lartesertib (M4076)
    21d
    A Presentation of Meckel's Diverticulum With Malignancy. (PubMed, Cureus)
    Undifferentiated carcinoma should be considered in the differential diagnosis of large mesenteric masses. This case emphasizes the importance of recognizing atypical presentations of MD and the critical role of molecular characterization in guiding personalized therapy when conventional management strategies are limited.
    Journal • Tumor mutational burden • MSi-H Biomarker • PD(L)-1 Biomarker • IO biomarker
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    PD-L1 (Programmed death ligand 1) • TMB (Tumor Mutational Burden) • MSI (Microsatellite instability) • ATM (ATM serine/threonine kinase)
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    TMB-H • MSI-H/dMMR • ATM mutation
    26d
    Discovery of a selective and effective CRBN-recruiting ATR degrader with strong antitumor activity. (PubMed, Eur J Med Chem)
    In addition, I-1 had acceptable pharmacokinetic properties and exhibited significant anti-tumor activity in the LoVo xenograft tumor mouse model. Collectively, these findings suggest that ATR degraders may represent a promising strategy for the treatment of cancer with deficient in ATM.
    Journal
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    ATM (ATM serine/threonine kinase) • CRBN (Cereblon) • ATR (Ataxia telangiectasia and Rad3-related protein)
    1m
    Germline Pathogenic Variants in Breast Cancer-Predisposing Genes Among Early-Onset Female and Male Breast Cancer in Ethiopia. (PubMed, JCO Glob Oncol)
    Genetic predisposition plays an important role among young women and men in the study setting, with nearly one in four patients carrying a PV. The results call for further research and interventions targeting individuals at high risk, with the long-term potential of reduced morbidity and mortality.
    Journal • BRCA Biomarker
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    TP53 (Tumor protein P53) • BRCA1 (Breast cancer 1, early onset) • BRCA2 (Breast cancer 2, early onset) • PTEN (Phosphatase and tensin homolog) • ATM (ATM serine/threonine kinase) • STK11 (Serine/threonine kinase 11) • PALB2 (Partner and localizer of BRCA2) • CDH1 (Cadherin 1) • CHEK2 (Checkpoint kinase 2) • RAD51C (RAD51 paralog C) • RAD51D (RAD51 paralog D) • BARD1 (BRCA1 Associated RING Domain 1)