Cellular functions of the protein kinase ATM and their relevance to human disease

Proteome dynamics at broken replication forks reveal a distinct ATM-directed repair response suppressing DNA double-strand break ubiquitination

(2021) Kyosuke Nakamura et al.   MOLECULAR CELL

Crosstalk between nucleus and mitochondria in human disease: Mitochondrial iron and calcium homeostasis in Friedreich ataxia

(2021) Jordi Tamarit et al.   IUBMB LIFE

Poly-ADP-ribosylation drives loss of protein homeostasis in ATM and Mre11 deficiency

(2021) Ji-Hoon Lee et al.   MOLECULAR CELL

XRCC1 prevents toxic PARP1 trapping during DNA base excision repair

(2021) Annie A. Demin et al.   MOLECULAR CELL

53BP1: a DSB escort

(2020) Zachary Mirman et al.   GENES & DEVELOPMENT

PARPs and ADP-ribosylation in RNA biology: from RNA expression and processing to protein translation and proteostasis

(2020) Dae-Seok Kim et al.   GENES & DEVELOPMENT

Ataxia telangiectasia mutated interacts with Parkin and induces mitophagy independent of kinase activity. Evidence from mantle cell lymphoma

(2020) Aloke Sarkar et al.   HAEMATOLOGICA

HPF1 completes the PARP active site for DNA damage-induced ADP-ribosylation

(2020) Marcin J. Suskiewicz et al.   NATURE

Regulatory R-loops as facilitators of gene expression and genome stability

(2020) Christof Niehrs et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Atm deficiency in the DNA polymerase β null cerebellum results in cerebellar ataxia and Itpr1 reduction associated with alteration of cytosine methylation

(2020) Jusik Kim et al.   NUCLEIC ACIDS RESEARCH

Poly(ADP-ribose): A Dynamic Trigger for Biomolecular Condensate Formation

(2020) Anthony K.L. Leung   TRENDS IN CELL BIOLOGY

Endogenous topoisomerase II-mediated DNA breaks drive thymic cancer predisposition linked to ATM deficiency

(2020) Alejandro Álvarez-Quilón et al.   Nature Communications

DNA-dependent protein kinase promotes DNA end processing by MRN and CtIP

(2020) Rajashree A. Deshpande et al.   Science Advances

Induced Pluripotent Stem Cell (iPSC)-Based Neurodegenerative Disease Models for Phenotype Recapitulation and Drug Screening

(2020) Chia-Yu Chang et al.   MOLECULES

Mitochondria at the crossroads of ATM-mediated stress signaling and regulation of reactive oxygen species

(2020) Ji-Hoon Lee et al.   Redox Biology

Mitochondrial Electron Transport: Oxidative Phosphorylation, Mitochondrial Oxidant Production, and Methods of Measurement

(2020) Deirdre Nolfi-Donegan et al.   Redox Biology

Enzymatic degradation of RNA causes widespread protein aggregation in cell and tissue lysates

(2020) Johan Aarum et al.   EMBO REPORTS

Activation of ATM kinase by ROS generated during ionophore-induced mitophagy in human T and B cell malignancies

(2020) Aloke Sarkar et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

ATM antagonizes NHEJ proteins assembly and DNA-ends synapsis at single-ended DNA double strand breaks

(2020) Sébastien Britton et al.   NUCLEIC ACIDS RESEARCH

Oxidative stress modulates rearrangement of endoplasmic reticulum-mitochondria contacts and calcium dysregulation in a Friedreich's ataxia model

(2020) Laura R. Rodríguez et al.   Redox Biology

Phosphoproteomics reveals novel modes of function and inter‐relationships among PIKKs in response to genotoxic stress

(2020) Sapir Schlam‐Babayov et al.   EMBO JOURNAL

Redox activation of ATM enhances GSNOR translation to sustain mitophagy and tolerance to oxidative stress

(2020) Claudia Cirotti et al.   EMBO REPORTS

ATM inhibition enhances Auranofin-induced oxidative stress and cell death in lung cell lines

(2020) Vanessa Ehrenfeld et al.   PLoS One

Impaired endoplasmic reticulum-mitochondrial signaling in ataxia-telangiectasia

(2020) Abrey J. Yeo et al.   iScience

ATM is activated by ATP depletion and modulates mitochondrial function through NRF1

(2019) Hei-Man Chow et al.   JOURNAL OF CELL BIOLOGY

ATM pathway activation limits R-loop-associated genomic instability in Werner syndrome cells

(2019) Veronica Marabitti et al.   NUCLEIC ACIDS RESEARCH

ATM orchestrates the DNA-damage response to counter toxic non-homologous end-joining at broken replication forks

(2019) Gabriel Balmus et al.   Nature Communications

Opportunities and challenges for the use of induced pluripotent stem cells in modelling neurodegenerative disease

(2019) Yi-Ying Wu et al.   Open Biology

Intracellular citrate accumulation by oxidized ATM-mediated metabolism reprogramming via PFKP and CS enhances hypoxic breast cancer cell invasion and metastasis

(2019) Meixi Peng et al.   Cell Death & Disease

Protein misfolding and aggregation in neurodegenerative diseases: a review of pathogeneses, novel detection strategies, and potential therapeutics

(2019) Jason Gandhi et al.   REVIEWS IN THE NEUROSCIENCES

Histone H2AX promotes neuronal health by controlling mitochondrial homeostasis

(2019) Urbain Weyemi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Atrophy, oxidative switching and ultrastructural defects in skeletal muscle of the ataxia telangiectasia mouse model

(2019) Valentina Tassinari et al.   JOURNAL OF CELL SCIENCE

PARylation regulates stress granule dynamics, phase separation, and neurotoxicity of disease-related RNA-binding proteins

(2019) Yongjia Duan et al.   CELL RESEARCH

The new genetic landscape of Alzheimer’s disease: from amyloid cascade to genetically driven synaptic failure hypothesis?

(2019) Pierre Dourlen et al.   ACTA NEUROPATHOLOGICA

Structural basis of allosteric regulation of Tel1/ATM kinase

(2019) Jiyu Xin et al.   CELL RESEARCH

Oxidative Stress: A Key Modulator in Neurodegenerative Diseases

(2019) Anju Singh et al.   MOLECULES

Oxidative Stress in Neurodegenerative Diseases: From a Mitochondrial Point of View

(2019) Giovanna Cenini et al.   Oxidative Medicine and Cellular Longevity

Mechanistic modelling supports entwined rather than exclusively competitive DNA double-strand break repair pathway

(2019) S. P. Ingram et al.   Scientific Reports

PARP-1 Activation Directs FUS to DNA Damage Sites to Form PARG-Reversible Compartments Enriched in Damaged DNA

(2019) Anastasia S. Singatulina et al.   Cell Reports

Spinocerebellar ataxia

(2019) Thomas Klockgether et al.   Nature Reviews Disease Primers

Causative Links between Protein Aggregation and Oxidative Stress: A Review

(2019) Elise Lévy et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Parkinson’s disease is a type of amyloidosis featuring accumulation of amyloid fibrils of α-synuclein

(2019) Katsuya Araki et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Dual Processing of R-Loops and Topoisomerase I Induces Transcription-Dependent DNA Double-Strand Breaks

(2019) Agnese Cristini et al.   Cell Reports

Near-Complete Structure and Model of Tel1ATM from Chaetomium thermophilum Reveals a Robust Autoinhibited ATP State

(2019) Marijke Jansma et al.   STRUCTURE

Cryo-EM Structure of Nucleotide-Bound Tel1ATM Unravels the Molecular Basis of Inhibition and Structural Rationale for Disease-Associated Mutations

(2019) Luke A. Yates et al.   STRUCTURE

Denitrosylate and live longer: how ADH5/GSNOR links mitophagy to aging

(2018) Salvatore Rizza et al.   Autophagy

Calcium Signaling, PKC Gamma, IP3R1 and CAR8 Link Spinocerebellar Ataxias and Purkinje Cell Dendritic Development

(2018) Etsuko Shimobayashi et al.   Current Neuropharmacology

The prion-like domain of FUS is multiphosphorylated following DNA damage without altering nuclear localization

(2018) Shannon N. Rhoads et al.   MOLECULAR BIOLOGY OF THE CELL

Modulation of hypersensitivity to oxidative DNA damage in ATM defective cells induced by potassium bromate by inhibition of the Poly (ADP-ribose) polymerase (PARP)

(2018) P. Mosesso et al.   MUTATION RESEARCH-GENETIC TOXICOLOGY AND ENVIRONMENTAL MUTAGENESIS

Super-resolution imaging identifies PARP1 and the Ku complex acting as DNA double-strand break sensors

(2018) Guang Yang et al.   NUCLEIC ACIDS RESEARCH

RNA–DNA hybrids promote the expansion of Friedreich's ataxia (GAA)n repeats via break-induced replication

(2018) Alexander J Neil et al.   NUCLEIC ACIDS RESEARCH

A pharmacological screen for compounds that rescue the developmental lethality of a Drosophila ATM mutant

(2018) Stacey A. Rimkus et al.   PLoS One

PARP-1–dependent recruitment of cold-inducible RNA-binding protein promotes double-strand break repair and genome stability

(2018) Jung-Kuei Chen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Astrocytes restore connectivity and synchronization in dysfunctional cerebellar networks

(2018) Sivan Kanner et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

S-nitrosylation drives cell senescence and aging in mammals by controlling mitochondrial dynamics and mitophagy

(2018) Salvatore Rizza et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial redox sensing by the kinase ATM maintains cellular antioxidant capacity

(2018) Yichong Zhang et al.   Science Signaling

ATM directs DNA damage responses and proteostasis via genetically separable pathways

(2018) Ji-Hoon Lee et al.   Science Signaling

The role of endoplasmic reticulum-mitochondria contact sites in the control of glucose homeostasis: an update

(2018) Jennifer Rieusset   Cell Death & Disease

Histone H2AX deficiency causes neurobehavioral deficits and impaired redox homeostasis

(2018) Urbain Weyemi et al.   Nature Communications

Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation

(2018) Marcel Naumann et al.   Nature Communications

Mitochondrial redox sensing by the kinase ATM maintains cellular antioxidant capacity

(2018) Yichong Zhang et al.   Science Signaling

ATM directs DNA damage responses and proteostasis via genetically separable pathways

(2018) Ji-Hoon Lee et al.   Science Signaling

Brain Energy and Oxygen Metabolism: Emerging Role in Normal Function and Disease

(2018) Michelle E. Watts et al.   Frontiers in Molecular Neuroscience

An ATM/TRIM37/NEMO axis counteracts genotoxicity by activating nuclear-to-cytoplasmic NF-κB signaling

(2018) Geyan Wu et al.   CANCER RESEARCH

Human Rad52 Promotes XPG-Mediated R-loop Processing to Initiate Transcription-Associated Homologous Recombination Repair

(2018) Takaaki Yasuhara et al.   CELL

Essential Function of Mec1, the Budding Yeast ATM/ATR Checkpoint-Response Kinase, in Protein Homeostasis

(2018) Isaac Corcoles-Saez et al.   DEVELOPMENTAL CELL

Protein misfolding, aggregation, and conformational strains in neurodegenerative diseases

(2018) Claudio Soto et al.   NATURE NEUROSCIENCE

Systems-wide Analysis of Serine ADP-Ribosylation Reveals Widespread Occurrence and Site-Specific Overlap with Phosphorylation

(2018) Sara C. Larsen et al.   Cell Reports

Spinocerebellar ataxia: an update

(2018) Roisin Sullivan et al.   JOURNAL OF NEUROLOGY

Poly(ADP-ribose) drives pathologic α-synuclein neurodegeneration in Parkinson’s disease

(2018) Tae-In Kam et al.   SCIENCE

Calcium Deregulation: Novel Insights to Understand Friedreich’s Ataxia Pathophysiology

(2018) Rosella Abeti et al.   Frontiers in Cellular Neuroscience

Sae2/CtIP prevents R-loop accumulation in eukaryotic cells

(2018) Nodar Makharashvili et al.   eLife

Mechanisms of DNA damage, repair, and mutagenesis

(2017) Nimrat Chatterjee et al.   ENVIRONMENTAL AND MOLECULAR MUTAGENESIS

Genome integrity and disease prevention in the nervous system

(2017) Peter J. McKinnon   GENES & DEVELOPMENT

PARPs and ADP-ribosylation: recent advances linking molecular functions to biological outcomes

(2017) Rebecca Gupte et al.   GENES & DEVELOPMENT

Neurological disorders associated with DNA strand-break processing enzymes

(2017) Bingcheng Jiang et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Regulation of the DNA Damage Response by DNA-PKcs Inhibitory Phosphorylation of ATM

(2017) Yi Zhou et al.   MOLECULAR CELL

Serine ADP-Ribosylation Depends on HPF1

(2017) Juan José Bonfiglio 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

Mechanisms of mTORC1 activation by RHEB and inhibition by PRAS40

(2017) Haijuan Yang et al.   NATURE

C9orf72 expansion disrupts ATM-mediated chromosomal break repair

(2017) Callum Walker et al.   NATURE NEUROSCIENCE

Non-homologous DNA end joining and alternative pathways to double-strand break repair

(2017) Howard H. Y. Chang et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The multifaceted roles of PARP1 in DNA repair and chromatin remodelling

(2017) Arnab Ray Chaudhuri et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Biomolecular condensates: organizers of cellular biochemistry

(2017) Salman F. Banani et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

The role of mitochondria in amyotrophic lateral sclerosis

(2017) Emma F. Smith et al.   NEUROSCIENCE LETTERS

Modulation of proteostasis counteracts oxidative stress and affects DNA base excision repair capacity in ATM-deficient cells

(2017) Mattia Poletto et al.   NUCLEIC ACIDS RESEARCH

The role of poly ADP-ribosylation in the first wave of DNA damage response

(2017) Chao Liu et al.   NUCLEIC ACIDS RESEARCH

Comparison of Selected Parameters of Redox Homeostasis in Patients with Ataxia-Telangiectasia and Nijmegen Breakage Syndrome

(2017) Barbara Pietrucha et al.   Oxidative Medicine and Cellular Longevity

Pathology of Neurodegenerative Diseases

(2017) Brittany N. Dugger et al.   Cold Spring Harbor Perspectives in Biology

Proteomic analyses identify ARH3 as a serine mono-ADP-ribosylhydrolase

(2017) Jeannette Abplanalp et al.   Nature Communications

RUG3 and ATM synergistically regulate the alternative splicing of mitochondrial nad2 and the DNA damage response in Arabidopsis thaliana

(2017) Chao Su et al.   Scientific Reports

Structures of closed and open conformations of dimeric human ATM

(2017) Domagoj Baretić et al.   Science Advances

Protein misfolding in neurodegenerative diseases: implications and strategies

(2017) Patrick Sweeney et al.   Translational Neurodegeneration

Mechanisms of non-canonical activation of ataxia telangiectasia mutated

(2016) S. V. Khoronenkova   BIOCHEMISTRY-MOSCOW

Structure of the human dimeric ATM kinase

(2016) Wilson C. Y. Lau et al.   CELL CYCLE

NAD + Replenishment Improves Lifespan and Healthspan in Ataxia Telangiectasia Models via Mitophagy and DNA Repair

(2016) Evandro Fei Fang et al.   Cell Metabolism

Neural Differentiation in the Third Dimension: Generating a Human Midbrain

(2016) Rebecca M. Marton et al.   Cell Stem Cell

Mechanism and regulation of DNA end resection in eukaryotes

(2016) Lorraine S. Symington   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

ATM/ATR‐mediated phosphorylation of PALB2 promotes RAD51 function

(2016) Johanna K Ahlskog et al.   EMBO REPORTS

MRN, CtIP, and BRCA1 mediate repair of topoisomerase II–DNA adducts

(2016) Tomas Aparicio et al.   JOURNAL OF CELL BIOLOGY

Rats with a missense mutation in Atm display neuroinflammation and neurodegeneration subsequent to accumulation of cytosolic DNA following unrepaired DNA damage

(2016) Hazel Quek et al.   JOURNAL OF LEUKOCYTE BIOLOGY

ATM protein is located on presynaptic vesicles and its deficit leads to failures in synaptic plasticity

(2016) Graham Vail et al.   JOURNAL OF NEUROPHYSIOLOGY

Mre11 Is Essential for the Removal of Lethal Topoisomerase 2 Covalent Cleavage Complexes

(2016) Nguyen Ngoc Hoa et al.   MOLECULAR CELL

HPF1/C4orf27 Is a PARP-1-Interacting Protein that Regulates PARP-1 ADP-Ribosylation Activity

(2016) Ian Gibbs-Seymour et al.   MOLECULAR CELL

The impact of glutamine supplementation on the symptoms of ataxia-telangiectasia: a preclinical assessment

(2016) Jianmin Chen et al.   Molecular Neurodegeneration

XRCC1 mutation is associated with PARP1 hyperactivation and cerebellar ataxia

(2016) Nicolas C. Hoch et al.   NATURE

Roles of inositol 1,4,5-trisphosphate receptors in spinocerebellar ataxias

(2016) Masayoshi Tada et al.   NEUROCHEMISTRY INTERNATIONAL

Ataxia telangiectasia: a review

(2016) Cynthia Rothblum-Oviatt et al.   Orphanet Journal of Rare Diseases

Laying a trap to kill cancer cells: PARP inhibitors and their mechanisms of action

(2016) Y. Pommier et al.   Science Translational Medicine

Repair Pathway Choices and Consequences at the Double-Strand Break

(2016) Raphael Ceccaldi et al.   TRENDS IN CELL BIOLOGY

Structure of the intact ATM/Tel1 kinase

(2016) Xuejuan Wang et al.   Nature Communications

Coordinated nuclease activities counteract Ku at single-ended DNA double-strand breaks

(2016) Pauline Chanut et al.   Nature Communications

NADPH: new oxygen for the ROS theory of aging

(2016) Pablo J. Fernandez-Marcos et al.   Oncotarget

Stalled DNA Replication Forks at the Endogenous GAA Repeats Drive Repeat Expansion in Friedreich’s Ataxia Cells

(2016) Jeannine Gerhardt 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

Extension of chronological life span by reduced TOR signaling requires down-regulation of Sch9p and involves increased mitochondrial OXPHOS complex density

(2016) Yong Pan et al.   Aging-US

Biochemical Properties and Biological Functions of FET Proteins

(2015) Jacob C. Schwartz et al.   Annual Review of Biochemistry

Mechanisms of ATM Activation

(2015) Tanya T. Paull   Annual Review of Biochemistry

A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation

(2015) Avinash Patel et al.   CELL

A novel porcine model of ataxia telangiectasia reproduces neurological features and motor deficits of human disease

(2015) Rosanna Beraldi et al.   HUMAN MOLECULAR GENETICS

PARP-1 involvement in neurodegeneration: A focus on Alzheimer’s and Parkinson’s diseases

(2015) Sara Martire et al.   MECHANISMS OF AGEING AND DEVELOPMENT

The core spliceosome as target and effector of non-canonical ATM signalling

(2015) Maria Tresini et al.   NATURE

ATM functions at the peroxisome to induce pexophagy in response to ROS

(2015) Jiangwei Zhang et al.   NATURE CELL BIOLOGY

ATM-dependent phosphorylation of MRE11 controls extent of resection during homology directed repair by signalling through Exonuclease 1

(2015) Amanda W. Kijas et al.   NUCLEIC ACIDS RESEARCH

DNA-PK triggers histone ubiquitination and signaling in response to DNA double-strand breaks produced during the repair of transcription-blocking topoisomerase I lesions

(2015) Agnese Cristini et al.   NUCLEIC ACIDS RESEARCH

NADPH oxidase 4 is a critical mediator in Ataxia telangiectasia disease

(2015) Urbain Weyemi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ATM prevents DSB formation by coordinating SSB repair and cell cycle progression

(2015) Svetlana V. Khoronenkova et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The DNA-dependent protein kinase: A multifunctional protein kinase with roles in DNA double strand break repair and mitosis

(2015) Nicholas Jette et al.   PROGRESS IN BIOPHYSICS & MOLECULAR BIOLOGY

An Overview of the Molecular Mechanisms of Recombinational DNA Repair

(2015) Stephen C. Kowalczykowski   Cold Spring Harbor Perspectives in Biology

Liquid demixing of intrinsically disordered proteins is seeded by poly(ADP-ribose)

(2015) Matthias Altmeyer et al.   Nature Communications

Non-redundant Functions of ATM and DNA-PKcs in Response to DNA Double-Strand Breaks

(2015) Pierre Caron et al.   Cell Reports

Poly (ADP-ribose) in the pathogenesis of Parkinson's disease

(2014) Yunjong Lee et al.   BMB Reports

Cdk1-dependent regulation of the Mre11 complex couples DNA repair pathways to cell cycle progression

(2014) Antoine Simoneau et al.   CELL CYCLE

The Mechanistic Links Between Proteasome Activity, Aging and Agerelated Diseases

(2014) Isabel Saez et al.   CURRENT GENOMICS

Tyrosyl-DNA-phosphodiesterases (TDP1 and TDP2)

(2014) Yves Pommier et al.   DNA REPAIR

FUS is Phosphorylated by DNA-PK and Accumulates in the Cytoplasm after DNA Damage

(2014) Q. Deng et al.   JOURNAL OF NEUROSCIENCE

Transcription-Coupled Nucleotide Excision Repair Factors Promote R-Loop-Induced Genome Instability

(2014) Julie Sollier et al.   MOLECULAR CELL

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

(2014) Sachin Katyal et al.   NATURE NEUROSCIENCE

ATM regulates NF-κB-dependent immediate-early genes via RelA Ser 276 phosphorylation coupled to CDK9 promoter recruitment

(2014) Ling Fang et al.   NUCLEIC ACIDS RESEARCH

ATM Deficiency Results in Accumulation of DNA-Topoisomerase I Covalent Intermediates in Neural Cells

(2014) Meryem Alagoz et al.   PLoS One

R-Loops in Proliferating Cells but Not in the Brain: Implications for AOA2 and Other Autosomal Recessive Ataxias

(2014) Abrey J. Yeo et al.   PLoS One

Ionizing radiation-induced XRCC4 phosphorylation is mediated through ATM in addition to DNA-PK

(2014) Shoji IMAMICHI et al.   PROCEEDINGS OF THE JAPAN ACADEMY SERIES B-PHYSICAL AND BIOLOGICAL SCIENCES

DNA damage: RNA-binding proteins protect from near and far

(2014) Martin Dutertre et al.   TRENDS IN BIOCHEMICAL SCIENCES

ATM specifically mediates repair of double-strand breaks with blocked DNA ends

(2014) Alejandro Álvarez-Quilón et al.   Nature Communications

Phosphorylation of EXO1 by CDKs 1 and 2 regulates DNA end resection and repair pathway choice

(2014) Nozomi Tomimatsu et al.   Nature Communications

R-loops Associated with Triplet Repeat Expansions Promote Gene Silencing in Friedreich Ataxia and Fragile X Syndrome

(2014) Matthias Groh et al.   PLoS Genetics

The cellular response to DNA damage: A focus on MDC1 and its interacting proteins

(2014) Gideon Coster et al.   Nucleus

Ionizing Radiation-Induced DNA Damage, Response, and Repair

(2013) Wil L. Santivasi et al.   ANTIOXIDANTS & REDOX SIGNALING

Pathogenesis of ataxia-telangiectasia: the next generation of ATM functions

(2013) M. Ambrose et al.   BLOOD

DNA DSB repair pathway choice: an orchestrated handover mechanism

(2013) A Kakarougkas et al.   BRITISH JOURNAL OF RADIOLOGY

53BP1 Mediates Productive and Mutagenic DNA Repair through Distinct Phosphoprotein Interactions

(2013) Elsa Callen et al.   CELL

Epigenetic Silencing Mediates Mitochondria Stress-Induced Longevity

(2013) Elizabeth A. Schroeder et al.   Cell Metabolism

The appropriateness of the mouse model for ataxia-telangiectasia: Neurological defects but no neurodegeneration

(2013) Martin F. Lavin   DNA REPAIR

Intrinsic mitochondrial DNA repair defects in Ataxia Telangiectasia

(2013) Nilesh K. Sharma et al.   DNA REPAIR

The RNA-binding Protein Fused in Sarcoma (FUS) Functions Downstream of Poly(ADP-ribose) Polymerase (PARP) in Response to DNA Damage

(2013) Adam S. Mastrocola et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

RIF1 Counteracts BRCA1-mediated End Resection during DNA Repair

(2013) Lin Feng 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

RIF1 Is Essential for 53BP1-Dependent Nonhomologous End Joining and Suppression of DNA Double-Strand Break Resection

(2013) J. Ross Chapman et al.   MOLECULAR CELL

EZH2-mediated H3K27 trimethylation mediates neurodegeneration in ataxia-telangiectasia

(2013) Jiali Li et al.   NATURE NEUROSCIENCE

Interaction of FUS and HDAC1 regulates DNA damage response and repair in neurons

(2013) Wen-Yuan Wang et al.   NATURE NEUROSCIENCE

The ATM protein kinase: regulating the cellular response to genotoxic stress and more

(2013) Yosef Shiloh et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Protein misfolding in the late-onset neurodegenerative diseases: Common themes and the unique case of amyotrophic lateral sclerosis

(2013) Vikram Khipple Mulligan et al.   PROTEINS-STRUCTURE FUNCTION AND BIOINFORMATICS

53BP1 Regulates DSB Repair Using Rif1 to Control 5' End Resection

(2013) M. Zimmermann et al.   SCIENCE

Rif1 Prevents Resection of DNA Breaks and Promotes Immunoglobulin Class Switching

(2013) M. Di Virgilio et al.   SCIENCE

DNA Damage Sensing by the ATM and ATR Kinases

(2013) A. Marechal et al.   Cold Spring Harbor Perspectives in Biology

The Interaction of CtIP and Nbs1 Connects CDK and ATM to Regulate HR–Mediated Double-Strand Break Repair

(2013) Hailong Wang et al.   PLoS Genetics

Controlling DNA-end resection: a new task for CDKs

(2013) Lorenza P. Ferretti et al.   Frontiers in Genetics

Drugging Topoisomerases: Lessons and Challenges

(2012) Yves Pommier   ACS Chemical Biology

Ataxia telangiectasia mutated-dependent regulation of topoisomerase II alpha expression and sensitivity to topoisomerase II inhibitor

(2012) Hiroyuki Tamaichi et al.   CANCER SCIENCE

CDK targeting of NBS1 promotes DNA-end resection, replication restart and homologous recombination

(2012) Jacob Falck et al.   EMBO REPORTS

Oxidative stress, neurodegeneration, and the balance of protein degradation and protein synthesis

(2012) Kalavathi Dasuri et al.   FREE RADICAL BIOLOGY AND MEDICINE

Cell biology of spinocerebellar ataxia

(2012) Harry T. Orr   JOURNAL OF CELL BIOLOGY

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

Senataxin, Defective in the Neurodegenerative Disorder Ataxia with Oculomotor Apraxia 2, Lies at the Interface of Transcription and the DNA Damage Response

(2012) O. Yuce et al.   MOLECULAR AND CELLULAR BIOLOGY

Activation of DSB Processing Requires Phosphorylation of CtIP by ATR

(2012) Shaun E. Peterson et al.   MOLECULAR CELL

Reducing Mitochondrial ROS Improves Disease-related Pathology in a Mouse Model of Ataxia-telangiectasia

(2012) Anthony D D'Souza et al.   MOLECULAR THERAPY

Mre11 regulates CtIP-dependent double-strand break repair by interaction with CDK2

(2012) Jeffrey Buis et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Activation of PARP by Oxidative Stress Induced by β-Amyloid: Implications for Alzheimer’s Disease

(2012) Rosella Abeti et al.   NEUROCHEMICAL RESEARCH

Mechanism of Oxidative Stress in Neurodegeneration

(2012) Sonia Gandhi et al.   Oxidative Medicine and Cellular Longevity

Hyperactivation of PARP Triggers Nonhomologous End-Joining in Repair-Deficient Mouse Fibroblasts

(2012) Natalie R. Gassman et al.   PLoS One

TDP-43: gumming up neurons through protein–protein and protein–RNA interactions

(2012) Emanuele Buratti et al.   TRENDS IN BIOCHEMICAL SCIENCES

MRE11 facilitates the removal of human topoisomerase II complexes from genomic DNA

(2012) K. C. Lee et al.   Biology Open

The Role of the Neuro-Astro-Vascular Unit in the Etiology of Ataxia Telangiectasia

(2012) Leenoy Meshulam et al.   Frontiers in Pharmacology

Mitochondrial dysfunction in ataxia-telangiectasia

(2011) Y. A. Valentin-Vega et al.   BLOOD

ATM protects against oxidative stress induced by oxidized low-density lipoprotein

(2011) Michaela Semlitsch et al.   DNA REPAIR

Dynamics of DNA damage response proteins at DNA breaks: a focus on protein modifications

(2011) S. E. Polo et al.   GENES & DEVELOPMENT

Sublethal Doses of β-Amyloid Peptide Abrogate DNA-dependent Protein Kinase Activity

(2011) Alessio Cardinale et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Aurora-B Mediated ATM Serine 1403 Phosphorylation Is Required for Mitotic ATM Activation and the Spindle Checkpoint

(2011) Chunying Yang et al.   MOLECULAR CELL

Regulation of DNA End Joining, Resection, and Immunoglobulin Class Switch Recombination by 53BP1

(2011) Anne Bothmer et al.   MOLECULAR CELL

The structure and catalytic mechanism of a poly(ADP-ribose) glycohydrolase

(2011) Dea Slade et al.   NATURE

Constructing and Deconstructing Stem Cell Models of Neurological Disease

(2011) Steve S.W. Han et al.   NEURON

A Novel Role for ATM in Regulating Proteasome-Mediated Protein Degradation through Suppression of the ISG15 Conjugation Pathway

(2011) Laurence M. Wood et al.   PLoS One

RNA-binding proteins with prion-like domains in ALS and FTLD-U

(2011) Aaron D. Gitler et al.   Prion

Signals from the Nucleus: Activation of NF- B by Cytosolic ATM in the DNA Damage Response

(2011) K. Hadian et al.   Science Signaling

53BP1 Inhibits Homologous Recombination in Brca1-Deficient Cells by Blocking Resection of DNA Breaks

(2010) Samuel F. Bunting et al.   CELL

TDP1 serine 81 promotes interaction with DNA ligase IIIα and facilitates cell survival following DNA damage

(2010) Shih-Chieh Chiang et al.   CELL CYCLE

DNA double-strand breaks and ATM activation by transcription-blocking DNA lesions

(2010) Olivier Sordet et al.   CELL CYCLE

Nuclear initiated NF-κB signaling: NEMO and ATM take center stage

(2010) Shigeki Miyamoto   CELL RESEARCH

Deranged Calcium Signaling in Purkinje Cells and Pathogenesis in Spinocerebellar Ataxia 2 (SCA2) and Other Ataxias

(2010) Adebimpe Kasumu et al.   CEREBELLUM

DNA Topoisomerases and Their Poisoning by Anticancer and Antibacterial Drugs

(2010) Yves Pommier et al.   CHEMISTRY & BIOLOGY

ATM activates the pentose phosphate pathway promoting anti-oxidant defence and DNA repair

(2010) Claudia Cosentino et al.   EMBO JOURNAL

Autophosphorylation and ATM Activation

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

NAD+ Depletion Is Necessary and Sufficient forPoly(ADP-Ribose) Polymerase-1-Mediated Neuronal Death

(2010) C. C. Alano et al.   JOURNAL OF NEUROSCIENCE

A Cytoplasmic ATM-TRAF6-cIAP1 Module Links Nuclear DNA Damage Signaling to Ubiquitin-Mediated NF-κB Activation

(2010) Michael Hinz et al.   MOLECULAR CELL

ATM Activation by Oxidative Stress

(2010) Z. Guo et al.   SCIENCE

ATM-Dependent and -Independent Dynamics of the Nuclear Phosphoproteome After DNA Damage

(2010) A. Bensimon et al.   Science Signaling

Mitochondrial dysfunction in Parkinson's disease

(2009) Konstanze F. Winklhofer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

DNA Damage Regulates Alternative Splicing through Inhibition of RNA Polymerase II Elongation

(2009) Manuel J. Muñoz et al.   CELL

Cytoplasmic ATM in Neurons Modulates Synaptic Function

(2009) Jiali Li et al.   CURRENT BIOLOGY

PIKK-dependent phosphorylation of Mre11 induces MRN complex inactivation by disassembly from chromatin

(2009) Michela Di Virgilio et al.   DNA REPAIR

Common mechanisms of PIKK regulation

(2009) Courtney A. Lovejoy et al.   DNA REPAIR

Optimal function of the DNA repair enzyme TDP1 requires its phosphorylation by ATM and/or DNA-PK

(2009) Benu Brata Das et al.   EMBO JOURNAL

Ataxia telangiectasia mutated activation by transcription- and topoisomerase I-induced DNA double-strand breaks

(2009) Olivier Sordet et al.   EMBO REPORTS

Parthanatos, a messenger of death

(2009) Karen Kate David   Frontiers in Bioscience-Landmark

Oxidative Stress Is Linked to ERK1/2-p16 Signaling-mediated Growth Defect in ATM-deficient Astrocytes

(2009) Jeesun Kim et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Proteasome-dependent Processing of Topoisomerase I-DNA Adducts into DNA Double Strand Breaks at Arrested Replication Forks

(2009) Chao-Po Lin et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Human CtIP Mediates Cell Cycle Control of DNA End Resection and Double Strand Break Repair

(2009) Pablo Huertas et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

CtIP Links DNA Double-Strand Break Sensing to Resection

(2009) Zhongsheng You et al.   MOLECULAR CELL

The DNA-damage response in human biology and disease

(2009) Stephen P. Jackson et al.   NATURE

Phosphorylation of Exo1 modulates homologous recombination repair of DNA double-strand breaks

(2009) Emma Bolderson et al.   NUCLEIC ACIDS RESEARCH

Loss of ATM Impairs Proliferation of Neural Stem Cells Through Oxidative Stress-Mediated p38 MAPK Signaling

(2009) Jeesun Kim et al.   STEM CELLS

Identification and characterization of FUS/TLS as a new target of ATM

(2008) Mary Gardiner et al.   BIOCHEMICAL JOURNAL

DNA-PK and ATM phosphorylation sites in XLF/Cernunnos are not required for repair of DNA double strand breaks

(2008) Y YU et al.   DNA REPAIR

Chk2-dependent phosphorylation of XRCC1 in the DNA damage response promotes base excision repair

(2008) Wen-Cheng Chou et al.   EMBO JOURNAL

Phosphorylation of SDT repeats in the MDC1 N terminus triggers retention of NBS1 at the DNA damage–modified chromatin

(2008) Fredrik Melander et al.   JOURNAL OF CELL BIOLOGY

Constitutive phosphorylation of MDC1 physically links the MRE11–RAD50–NBS1 complex to damaged chromatin

(2008) Christoph Spycher et al.   JOURNAL OF CELL BIOLOGY

Deranged Calcium Signaling and Neurodegeneration in Spinocerebellar Ataxia Type 3

(2008) X. Chen et al.   JOURNAL OF NEUROSCIENCE

ATM Activation and Signaling under Hypoxic Conditions

(2008) Z. Bencokova et al.   MOLECULAR AND CELLULAR BIOLOGY

CDK targets Sae2 to control DNA-end resection and homologous recombination

(2008) Pablo Huertas et al.   NATURE

Ataxia-telangiectasia: from a rare disorder to a paradigm for cell signalling and cancer

(2008) Martin F. Lavin   NATURE REVIEWS MOLECULAR CELL BIOLOGY

MDC1 regulates intra-S-phase checkpoint by targeting NBS1 to DNA double-strand breaks

(2008) L. Wu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Complementary functions of ATM and H2AX in development and suppression of genomic instability

(2008) S. Zha et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA