ATM, ATR and DNA-PKcs kinases—the lessons from the mouse models: inhibition ≠ deletion

Ataxia‐telangiectasia: A review of clinical features and molecular pathology

(2019) Parisa Amirifar et al.   PEDIATRIC ALLERGY AND IMMUNOLOGY

Quantitative phosphoproteomics reveals mitotic function of the ATR activator ETAA1

(2019) Thomas E. Bass et al.   JOURNAL OF CELL BIOLOGY

Common motifs in ETAA1 and TOPBP1 required for ATR kinase activation

(2019) Vaughn Thada et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Phosphorylation at S2053 in Murine (S2056 in Human) DNA-PKcs Is Dispensable for Lymphocyte Development and Class Switch Recombination

(2019) Wenxia Jiang et al.   JOURNAL OF IMMUNOLOGY

Understanding the structure and role of DNA-PK in NHEJ: How X-ray diffraction and cryo-EM contribute in complementary ways

(2019) Qian Wu et al.   PROGRESS IN BIOPHYSICS & MOLECULAR BIOLOGY

DNA double-strand break repair-pathway choice in somatic mammalian cells

(2019) Ralph Scully et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Structural Insights into Yeast Telomerase Recruitment to Telomeres

(2018) Hongwen Chen et al.   CELL

The ATR Inhibitor AZD6738 Synergizes with GemcitabineIn VitroandIn Vivoto Induce Pancreatic Ductal Adenocarcinoma Regression

(2018) Yann Wallez et al.   MOLECULAR CANCER THERAPEUTICS

MRI Is a DNA Damage Response Adaptor during Classical Non-homologous End Joining

(2018) Putzer J. Hung et al.   MOLECULAR CELL

NAD+ analog reveals PARP-1 substrate-blocking mechanism and allosteric communication from catalytic center to DNA-binding domains

(2018) Marie-France Langelier et al.   Nature Communications

Actionable perturbations of damage responses by TCL1/ATM and epigenetic lesions form the basis of T-PLL

(2018) A. Schrader et al.   Nature Communications

ATR is a multifunctional regulator of male mouse meiosis

(2018) Alexander Widger et al.   Nature Communications

ATR is required to complete meiotic recombination in mice

(2018) Sarai Pacheco et al.   Nature Communications

Intragenic origins due to short G1 phases underlie oncogene-induced DNA replication stress

(2018) Morgane Macheret et al.   NATURE

Kinase-dependent structural role of DNA-PKcs during immunoglobulin class switch recombination

(2018) Jennifer L. Crowe et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

An intrinsic S/G2 checkpoint enforced by ATR

(2018) Joshua C. Saldivar et al.   SCIENCE

Genome-wide Identification of Structure-Forming Repeats as Principal Sites of Fork Collapse upon ATR Inhibition

(2018) Nishita Shastri et al.   MOLECULAR CELL

Kinase-dead ATR differs from ATR loss by limiting the dynamic exchange of ATR and RPA

(2018) Demis Menolfi et al.   Nature Communications

ATR inhibition disrupts rewired homologous recombination and fork protection pathways in PARP inhibitor-resistant BRCA-deficient cancer cells

(2017) Stephanie A. Yazinski et al.   GENES & DEVELOPMENT

AZD6738, A Novel Oral Inhibitor of ATR, Induces Synthetic Lethality with ATM Deficiency in Gastric Cancer Cells

(2017) Ahrum Min et al.   MOLECULAR CANCER THERAPEUTICS

Single-Molecule Imaging Reveals How Mre11-Rad50-Nbs1 Initiates DNA Break Repair

(2017) Logan R. Myler et al.   MOLECULAR CELL

ATM, ATR, and DNA-PK: The Trinity at the Heart of the DNA Damage Response

(2017) Andrew N. Blackford et al.   MOLECULAR CELL

The essential kinase ATR: ensuring faithful duplication of a challenging genome

(2017) Joshua C. Saldivar et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Cryo-EM structure of the DNA-PK holoenzyme

(2017) Humayun Sharif et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ATM loss leads to synthetic lethality in BRCA1 BRCT mutant mice associated with exacerbated defects in homology-directed repair

(2017) Chun-Chin Chen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

3.9 Å structure of the yeast Mec1-Ddc2 complex, a homolog of human ATR-ATRIP

(2017) Xuejuan Wang et al.   SCIENCE

A mitosis-specific and R loop–driven ATR pathway promotes faithful chromosome segregation

(2017) Lilian Kabeche et al.   SCIENCE

DNA-PKcs structure suggests an allosteric mechanism modulating DNA double-strand break repair

(2017) Bancinyane L. Sibanda et al.   SCIENCE

PAXX promotes KU accumulation at DNA breaks and is essential for end-joining in XLF-deficient mice

(2017) Xiangyu Liu et al.   Nature Communications

RPA-Binding Protein ETAA1 Is an ATR Activator Involved in DNA Replication Stress Response

(2016) Yuan-Cho Lee et al.   CURRENT BIOLOGY

Ewing Tumor-associated Antigen 1 Interacts with Replication Protein A to Promote Restart of Stalled Replication Forks

(2016) Sumin Feng et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Small-molecule inhibitors of Ataxia Telangiectasia and Rad3 related kinase (ATR) sensitize lymphoma cells to UVA radiation

(2016) Edyta Biskup et al.   JOURNAL OF DERMATOLOGICAL SCIENCE

Two-Stage Synapsis of DNA Ends during Non-homologous End Joining

(2016) Thomas G.W. Graham et al.   MOLECULAR CELL

ETAA1 acts at stalled replication forks to maintain genome integrity

(2016) Thomas E. Bass et al.   NATURE CELL BIOLOGY

Activation of the ATR kinase by the RPA-binding protein ETAA1

(2016) Peter Haahr et al.   NATURE CELL BIOLOGY

PAXX and XLF DNA repair factors are functionally redundant in joining DNA breaks in a G1-arrested progenitor B-cell line

(2016) Vipul Kumar et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Specific Roles of XRCC4 Paralogs PAXX and XLF during V(D)J Recombination

(2016) Chloé Lescale et al.   Cell Reports

Kinase-dead ATM protein is highly oncogenic and can be preferentially targeted by Topo-isomerase I inhibitors

(2016) Kenta Yamamoto et al.   eLife

Mechanisms of ATM Activation

(2015) Tanya T. Paull   Annual Review of Biochemistry

ATR inhibition induces synthetic lethality and overcomes chemoresistance in TP53- or ATM-defective chronic lymphocytic leukemia cells

(2015) M. Kwok et al.   BLOOD

Molecular Pathways: Targeting ATR in Cancer Therapy

(2015) L. M. Karnitz et al.   CLINICAL CANCER RESEARCH

IncreasedRrm2gene dosage reduces fragile site breakage and prolongs survival of ATR mutant mice

(2015) Andres J. Lopez-Contreras et al.   GENES & DEVELOPMENT

A novel mouse model for ataxia-telangiectasia with a N-terminal mutation displays a behavioral defect and a low incidence of lymphoma but no increased oxidative burden

(2015) Andrew Campbell et al.   HUMAN MOLECULAR GENETICS

DNA-PKcs Is Involved in Ig Class Switch Recombination in Human B Cells

(2015) Andrea Björkman et al.   JOURNAL OF IMMUNOLOGY

Differential Phosphorylation of DNA-PKcs Regulates the Interplay between End-Processing and End-Ligation during Nonhomologous End-Joining

(2015) Wenxia Jiang et al.   MOLECULAR CELL

Distinct but Concerted Roles of ATR, DNA-PK, and Chk1 in Countering Replication Stress during S Phase

(2015) Rémi Buisson et al.   MOLECULAR CELL

PARP-1 Activation Requires Local Unfolding of an Autoinhibitory Domain

(2015) Jennine M. Dawicki-McKenna et al.   MOLECULAR CELL

Mutations driving CLL and their evolution in progression and relapse

(2015) Dan A. Landau et al.   NATURE

Organization and dynamics of the nonhomologous end-joining machinery during DNA double-strand break repair

(2015) Dylan A. Reid et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

PAXX, a paralog of XRCC4 and XLF, interacts with Ku to promote DNA double-strand break repair

(2015) T. Ochi et al.   SCIENCE

Loss of ATM accelerates pancreatic cancer formation and epithelial–mesenchymal transition

(2015) Ronan Russell et al.   Nature Communications

ATR Inhibitors VE-821 and VX-970 Sensitize Cancer Cells to Topoisomerase I Inhibitors by Disabling DNA Replication Initiation and Fork Elongation Responses

(2014) R. Josse et al.   CANCER RESEARCH

ATR Pathway Inhibition Is Synthetically Lethal in Cancer Cells with ERCC1 Deficiency

(2014) K. N. Mohni et al.   CANCER RESEARCH

Base excision repair: A critical player in many games

(2014) Susan S. Wallace   DNA REPAIR

The clinical impact of deficiency in DNA non-homologous end-joining

(2014) Lisa Woodbine et al.   DNA REPAIR

Pot1a Prevents Telomere Dysfunction and ATM-Dependent Neuronal Loss

(2014) Y. Lee et al.   JOURNAL OF NEUROSCIENCE

A Synthetic Lethal Screen Reveals Enhanced Sensitivity to ATR Inhibitor Treatment in Mantle Cell Lymphoma with ATM Loss-of-Function

(2014) Daniel L. Menezes et al.   MOLECULAR CANCER RESEARCH

Aberrant topoisomerase-1 DNA lesions are pathogenic in neurodegenerative genome instability syndromes

(2014) Sachin Katyal et al.   NATURE NEUROSCIENCE

A Functional Cancer Genomics Screen Identifies a Druggable Synthetic Lethal Interaction between MSH3 and PRKDC

(2014) F. Dietlein et al.   Cancer Discovery

ATR Inhibition Broadly Sensitizes Ovarian Cancer Cells to Chemotherapy Independent of BRCA Status

(2013) C. J. Huntoon et al.   CANCER RESEARCH

ATR acts stage specifically to regulate multiple aspects of mammalian meiotic silencing

(2013) H. Royo et al.   GENES & DEVELOPMENT

Extreme Growth Failure is a Common Presentation of Ligase IV Deficiency

(2013) Jennie E. Murray et al.   HUMAN MUTATION

Ataxia Telangiectasia Mutated (ATM) Is Dispensable for Endonuclease I-SceI-induced Homologous Recombination in Mouse Embryonic Stem Cells

(2013) Emilie Rass et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A new method for high-resolution imaging of Ku foci to decipher mechanisms of DNA double-strand break repair

(2013) Sébastien Britton et al.   JOURNAL OF CELL BIOLOGY

PRKDC mutations in a SCID patient with profound neurological abnormalities

(2013) Lisa Woodbine et al.   JOURNAL OF CLINICAL INVESTIGATION

Functional Intersection of ATM and DNA-Dependent Protein Kinase Catalytic Subunit in Coding End Joining during V(D)J Recombination

(2013) B.-S. Lee et al.   MOLECULAR AND CELLULAR BIOLOGY

mTOR kinase structure, mechanism and regulation

(2013) Haijuan Yang et al.   NATURE

Ataxia Telangiectasia Mutated and Rad3 Related (ATR) Protein Kinase Inhibition Is Synthetically Lethal in XRCC1 Deficient Ovarian Cancer Cells

(2013) Rebeka Sultana et al.   PLoS One

RNA recognition by the DNA end-binding Ku heterodimer

(2013) A. B. Dalby et al.   RNA

Therapeutic Targeting of a Robust Non-Oncogene Addiction to PRKDC in ATM-Defective Tumors

(2013) A. Riabinska et al.   Science Translational Medicine

Nucleotide Excision Repair in Eukaryotes

(2013) O. D. Scharer   Cold Spring Harbor Perspectives in Biology

An Essential Function for the ATR-Activation-Domain (AAD) of TopBP1 in Mouse Development and Cellular Senescence

(2013) Zhong-Wei Zhou et al.   PLoS Genetics

The novel ATR inhibitor VE-821 increases sensitivity of pancreatic cancer cells to radiation and chemotherapy

(2012) Remko Prevo et al.   CANCER BIOLOGY & THERAPY

Primary Microcephaly, Impaired DNA Replication, and Genomic Instability Caused by Compound HeterozygousATRMutations

(2012) Houda Mokrani-Benhelli et al.   HUMAN MUTATION

Kinase-dead ATM protein causes genomic instability and early embryonic lethality in mice

(2012) Kenta Yamamoto et al.   JOURNAL OF CELL BIOLOGY

Loss of ATM kinase activity leads to embryonic lethality in mice

(2012) Jeremy A. Daniel et al.   JOURNAL OF CELL BIOLOGY

The DNA damage response and cancer therapy

(2012) Christopher J. Lord et al.   NATURE

Congenital bone marrow failure in DNA-PKcs mutant mice associated with deficiencies in DNA repair

(2011) Shichuan Zhang et al.   JOURNAL OF CELL BIOLOGY

Oncogenic stress sensitizes murine cancers to hypomorphic suppression of ATR

(2011) David W. Schoppy et al.   JOURNAL OF CLINICAL INVESTIGATION

ATM controls meiotic double-strand-break formation

(2011) Julian Lange et al.   NATURE

Ataxia telangiectasia-mutated protein and DNA-dependent protein kinase have complementary V(D)J recombination functions

(2011) Shan Zha et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Ataxia telangiectasia mutated (Atm) and DNA-PKcs kinases have overlapping activities during chromosomal signal joint formation

(2011) Eric J. Gapud et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Mechanism of Double-Strand DNA Break Repair by the Nonhomologous DNA End-Joining Pathway

(2010) Michael R. Lieber   Annual Review of Biochemistry

Autophosphorylation and ATM Activation

(2010) Sergei V. Kozlov et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Alternative end-joining catalyzes class switch recombination in the absence of both Ku70 and DNA ligase 4

(2010) Cristian Boboila et al.   JOURNAL OF EXPERIMENTAL MEDICINE

ATM-deficient thymic lymphoma is associated with aberranttcrdrearrangement and gene amplification

(2010) Shan Zha et al.   JOURNAL OF EXPERIMENTAL MEDICINE

ATM Deficiency Sensitizes Mantle Cell Lymphoma Cells to Poly(ADP-Ribose) Polymerase-1 Inhibitors

(2010) C. T. Williamson et al.   MOLECULAR CANCER THERAPEUTICS

The DNA Damage Response: Making It Safe to Play with Knives

(2010) Alberto Ciccia et al.   MOLECULAR CELL

ATM damage response and XLF repair factor are functionally redundant in joining DNA breaks

(2010) Shan Zha et al.   NATURE

Maintaining genome stability at the replication fork

(2010) Dana Branzei et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

ATR contributes to telomere maintenance in human cells

(2010) Gaëlle Pennarun et al.   NUCLEIC ACIDS RESEARCH

ATM Activation by Oxidative Stress

(2010) Z. Guo et al.   SCIENCE

DNA-PKcs and ATM co-regulate DNA double-strand break repair

(2009) Meena Shrivastav et al.   DNA REPAIR

Essential Role for DNA-PKcs in DNA Double-Strand Break Repair and Apoptosis in ATM-Deficient Lymphocytes

(2009) Elsa Callén et al.   MOLECULAR CELL

Tissue regenerative delays and synthetic lethality in adult mice after combined deletion of Atr and Trp53

(2009) Yaroslava Ruzankina et al.   NATURE GENETICS

A mouse model of ATR-Seckel shows embryonic replicative stress and accelerated aging

(2009) Matilde Murga et al.   NATURE GENETICS

Mechanism and Regulation of Class Switch Recombination

(2008) Janet Stavnezer et al.   Annual Review of Immunology

Chk1 Suppresses a Caspase-2 Apoptotic Response to DNA Damage that Bypasses p53, Bcl-2, and Caspase-3

(2008) Samuel Sidi et al.   CELL

Mutations to Ku reveal differences in human somatic cell lines

(2008) Kazi R. Fattah et al.   DNA REPAIR

Multiple autophosphorylation sites are dispensable for murine ATM activation in vivo

(2008) Jeremy A. Daniel et al.   JOURNAL OF CELL BIOLOGY

DNA-PKcs and Artemis function in the end-joining phase of immunoglobulin heavy chain class switch recombination

(2008) Sonia Franco et al.   JOURNAL OF EXPERIMENTAL MEDICINE

DNA Double-Strand Break Formation upon UV-Induced Replication Stress Activates ATM and DNA-PKcs Kinases

(2008) Hirohiko Yajima et al.   JOURNAL OF MOLECULAR BIOLOGY