DNA Damage and Repair Deficiency in ALS/FTD-Associated Neurodegeneration: From Molecular Mechanisms to Therapeutic Implication

Defective Autophagy and Mitophagy in Aging and Alzheimer’s Disease

(2021) Michael Tran et al.   Frontiers in Neuroscience

FUS-dependent liquid–liquid phase separation is important for DNA repair initiation

(2021) Brunno R. Levone et al.   JOURNAL OF CELL BIOLOGY

TDP-43 maximizes nerve conduction velocity by repressing a cryptic exon for paranodal junction assembly in Schwann cells

(2021) Kae-Jiun Chang et al.   eLife

C9ORF72: What It Is, What It Does, and Why It Matters

(2021) Julie Smeyers et al.   Frontiers in Cellular Neuroscience

C9orf72-derived arginine-containing dipeptide repeats associate with axonal transport machinery and impede microtubule-based motility

(2021) Laura Fumagalli et al.   Science Advances

Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway

(2021) Yen-Chen Lin et al.   NATURE NEUROSCIENCE

Stress granules, RNA-binding proteins and polyglutamine diseases: too much aggregation?

(2021) Adriana Marcelo et al.   Cell Death & Disease

TDP-43 Mutation Affects Stress Granule Dynamics in Differentiated NSC-34 Motoneuron-Like Cells

(2021) Qiao Ding et al.   Frontiers in Cell and Developmental Biology

Dipeptide repeat proteins inhibit homology-directed DNA double strand break repair in C9ORF72 ALS/FTD

(2020) Nadja S. Andrade et al.   Molecular Neurodegeneration

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

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

Cellular Senescence in Neurodegenerative Diseases

(2020) Carmen Martínez-Cué et al.   Frontiers in Cellular Neuroscience

The Overlapping Genetics of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia

(2020) Yevgeniya A. Abramzon et al.   Frontiers in Neuroscience

In vivo stress granule misprocessing evidenced in a FUS knock-in ALS mouse model

(2020) Xue Zhang et al.   BRAIN

Mitochondrial and Nuclear DNA Oxidative Damage in Physiological and Pathological Aging

(2020) Marta Kowalska et al.   DNA AND CELL BIOLOGY

HDAC1 dysregulation induces aberrant cell cycle and DNA damage in progress of TDP‐43 proteinopathies

(2020) Cheng‐Chun Wu et al.   EMBO Molecular Medicine

ALS/FTD-associated protein FUS induces mitochondrial dysfunction by preferentially sequestering respiratory chain complex mRNAs

(2020) Yueh-Lin Tsai et al.   GENES & DEVELOPMENT

Fanconi anemia pathway as a prospective target for cancer intervention

(2020) Wenjun Liu et al.   Cell and Bioscience

Senataxin Ortholog Sen1 Limits DNA:RNA Hybrid Accumulation at DNA Double-Strand Breaks to Control End Resection and Repair Fidelity

(2020) Chetan C. Rawal et al.   Cell Reports

Altered Mitochondrial Dynamics in Motor Neuron Disease: An Emerging Perspective

(2020) Manohar Kodavati et al.   Cells

TDP-43 Triggers Mitochondrial DNA Release via mPTP to Activate cGAS/STING in ALS

(2020) Chien-Hsiung Yu et al.   CELL

The role of DNA damage response in amyotrophic lateral sclerosis

(2020) Yu Sun et al.   Essays in Biochemistry

Impaired NHEJ repair in amyotrophic lateral sclerosis is associated with TDP-43 mutations

(2020) Anna Konopka et al.   Molecular Neurodegeneration

Minimum Information for Reporting on the Comet Assay (MIRCA): recommendations for describing comet assay procedures and results

(2020) Peter Møller et al.   Nature Protocols

Amyotrophic Lateral Sclerosis: A Neurodegenerative Motor Neuron Disease With Ocular Involvement

(2020) Pilar Rojas et al.   Frontiers in Neuroscience

An Amyotrophic Lateral Sclerosis-Associated Mutant of C21ORF2 Is Stabilized by NEK1-Mediated Hyperphosphorylation and the Inability to Bind FBXO3

(2020) Yasuaki Watanabe et al.   iScience

Polymorphisms in the BER and NER pathways and their influence on survival and toxicity in never-smokers with lung cancer

(2020) Ana Casal-Mouriño et al.   Scientific Reports

TDP-43 mutations link Amyotrophic Lateral Sclerosis with R-loop homeostasis and R loop-mediated DNA damage

(2020) Marta Giannini et al.   PLoS Genetics

Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration

(2019) Ze’ev Melamed et al.   NATURE NEUROSCIENCE

RNA Dysregulation in Amyotrophic Lateral Sclerosis

(2019) Zoe Butti et al.   Frontiers in Genetics

Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis

(2019) Archana Prasad et al.   Frontiers in Molecular Neuroscience

Motor neuron disease-associated loss of nuclear TDP-43 is linked to DNA double-strand break repair defects

(2019) Joy Mitra et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Amyotrophic lateral sclerosis-associated TDP-43 mutation Q331K prevents nuclear translocation of XRCC4-DNA ligase 4 complex and is linked to genome damage-mediated neuronal apoptosis

(2019) Erika N Guerrero et al.   HUMAN MOLECULAR GENETICS

Misfolded SOD1 inclusions in patients with mutations in C9orf72 and other ALS/FTD-associated genes

(2019) Karin Forsberg et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Partial inhibition of the overactivated Ku80-dependent DNA repair pathway rescues neurodegeneration in C9ORF72-ALS/FTD

(2019) Rodrigo Lopez-Gonzalez et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response

(2019) Peng Wang et al.   PLoS Genetics

A Systematic Review of Suggested Molecular Strata, Biomarkers and Their Tissue Sources in ALS

(2019) Udaya Geetha Vijayakumar et al.   Frontiers in Neurology

Nuclear Phospho-SOD1 Protects DNA from Oxidative Stress Damage in Amyotrophic Lateral Sclerosis

(2019) Matteo Bordoni et al.   Journal of Clinical Medicine

Motor Neuron Susceptibility in ALS/FTD

(2019) Audrey M. G. Ragagnin et al.   Frontiers in Neuroscience

Poly-glycine–alanine exacerbates C9orf72 repeat expansion-mediated DNA damage via sequestration of phosphorylated ATM and loss of nuclear hnRNPA3

(2019) Yoshihiro Nihei et al.   ACTA NEUROPATHOLOGICA

Phenotypic Suppression of ALS/FTD-Associated Neurodegeneration Highlights Mechanisms of Dysfunction

(2019) Mathieu Bartoletti et al.   JOURNAL OF NEUROSCIENCE

Stress granules and neurodegeneration

(2019) Benjamin Wolozin et al.   NATURE REVIEWS NEUROSCIENCE

Cytoplasmic Restriction of Mutated SOD1 Impairs the DNA Repair Process in Spinal Cord Neurons

(2019) Li et al.   Cells

DNA Oxidation and Excision Repair Pathways

(2019) Tae-Hee Lee et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Changes to the TDP-43 and FUS Interactomes Induced by DNA Damage

(2019) Tetsuya Kawaguchi et al.   JOURNAL OF PROTEOME RESEARCH

Stress granule mediated protein aggregation and underlying gene defects in the FTD-ALS spectrum

(2019) Yalda Baradaran-Heravi et al.   NEUROBIOLOGY OF DISEASE

RT2 PCR array screening reveals distinct perturbations in DNA damage response signaling in FUS-associated motor neuron disease

(2019) Haibo Wang et al.   Molecular Brain

Harnessing DNA Double-Strand Break Repair for Cancer Treatment

(2019) Anika Trenner et al.   Frontiers in Oncology

Perturbed autophagy and DNA repair converge to promote neurodegeneration in amyotrophic lateral sclerosis and dementia

(2018) Callum Walker et al.   BRAIN

Dynamic duo – FMRP and TDP-43: Regulating common targets, causing different diseases

(2018) Diana Ferro et al.   BRAIN RESEARCH

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

TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD

(2018) Ching-Chieh Chou et al.   NATURE NEUROSCIENCE

Association between TBK1 mutations and risk of amyotrophic lateral sclerosis/frontotemporal dementia spectrum: a meta-analysis

(2018) Rongrong Cui et al.   NEUROLOGICAL SCIENCES

NEK1 loss-of-function mutation induces DNA damage accumulation in ALS patient-derived motoneurons

(2018) Julia Higelin et al.   Stem Cell Research

Senataxin resolves RNA:DNA hybrids forming at DNA double-strand breaks to prevent translocations

(2018) Sarah Cohen et al.   Nature Communications

C9orf72-mediated ALS and FTD: multiple pathways to disease

(2018) Rubika Balendra et al.   Nature Reviews Neurology

FUS interacts with ATP synthase beta subunit and induces mitochondrial unfolded protein response in cellular and animal models

(2018) Jianwen Deng et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Stress Granules and Processing Bodies in Translational Control

(2018) Pavel Ivanov et al.   Cold Spring Harbor Perspectives in Biology

Mutant FUS causes DNA ligation defects to inhibit oxidative damage repair in Amyotrophic Lateral Sclerosis

(2018) Haibo Wang et al.   Nature Communications

Analysis of eIF2B bodies and their relationships with stress granules and P-bodies

(2018) Stephanie L. Moon et al.   Scientific Reports

Amyotrophic Lateral Sclerosis associated FUS mutation shortens mitochondria and induces neurotoxicity

(2018) Tadashi Nakaya et al.   Scientific Reports

Nuclear poly(ADP-ribose) activity is a therapeutic target in amyotrophic lateral sclerosis

(2018) L. McGurk et al.   Acta Neuropathologica Communications

The Emerging Role of DNA Damage in the Pathogenesis of the C9orf72 Repeat Expansion in Amyotrophic Lateral Sclerosis

(2018) Anna Konopka et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mechanisms of DNA damage, repair, and mutagenesis

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

The DNA damage response (DDR) is induced by the C9orf72 repeat expansion in amyotrophic lateral sclerosis

(2017) Manal A. Farg et al.   HUMAN MOLECULAR GENETICS

Synaptic UNC13A protein variant causes increased neurotransmission and dyskinetic movement disorder

(2017) Noa Lipstein et al.   JOURNAL OF CLINICAL INVESTIGATION

Senataxin: Genome Guardian at the Interface of Transcription and Neurodegeneration

(2017) Matthias Groh et al.   JOURNAL OF MOLECULAR BIOLOGY

Chronic oxidative damage together with genome repair deficiency in the neurons is a double whammy for neurodegeneration: Is damage response signaling a potential therapeutic target?

(2017) Haibo Wang et al.   MECHANISMS OF AGEING AND DEVELOPMENT

C9orf72 expansion disrupts ATM-mediated chromosomal break repair

(2017) Callum Walker et al.   NATURE NEUROSCIENCE

TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics

(2017) Ian R. Mackenzie et al.   NEURON

Epigenetics and Oxidative Stress in Aging

(2017) Amy Guillaumet-Adkins et al.   Oxidative Medicine and Cellular Longevity

Toxic PRnpoly-dipeptides encoded by theC9orf72repeat expansion block nuclear import and export

(2017) Kevin Y. Shi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Evaluating In Vitro DNA Damage Using Comet Assay

(2017) Yanxin Lu et al.   Jove-Journal of Visualized Experiments

Effects of oxidative and thermal stresses on stress granule formation in human induced pluripotent stem cells

(2017) Freshteh Palangi et al.   PLoS One

ALS Associated Mutations in Matrin 3 Alter Protein-Protein Interactions and Impede mRNA Nuclear Export

(2017) Ashley Boehringer et al.   Scientific Reports

Stress granules at the intersection of autophagy and ALS

(2016) Zachary Monahan et al.   BRAIN RESEARCH

Keeping homologous recombination in check

(2016) Kasper Fugger et al.   CELL RESEARCH

Minor intron splicing is regulated by FUS and affected by ALS‐associated FUS mutants

(2016) Stefan Reber et al.   EMBO JOURNAL

ALS/FTD‐associated FUS activates GSK‐3β to disrupt the VAPB–PTPIP51 interaction and ER–mitochondria associations

(2016) Radu Stoica et al.   EMBO REPORTS

Malonate induces the assembly of cytoplasmic stress granules

(2016) Xue Fu et al.   FEBS LETTERS

Nek1 Regulates Rad54 to Orchestrate Homologous Recombination and Replication Fork Stability

(2016) Julian Spies et al.   MOLECULAR CELL

Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis

(2016) Wouter van Rheenen et al.   NATURE GENETICS

Poly(GR) in C9ORF72 -Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons

(2016) Rodrigo Lopez-Gonzalez et al.   NEURON

Microhomology-mediated end joining is activated in irradiated human cells due to phosphorylation-dependent formation of the XRCC1 repair complex

(2016) Arijit Dutta et al.   NUCLEIC ACIDS RESEARCH

Ataxia telangiectasia: a review

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

Two familial ALS proteins function in prevention/repair of transcription-associated DNA damage

(2016) Sarah J. Hill et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43/FUS in motor neuron disease: Complexity and challenges

(2016) Erika N. Guerrero et al.   PROGRESS IN NEUROBIOLOGY

Principles and Properties of Stress Granules

(2016) David S.W. Protter et al.   TRENDS IN CELL BIOLOGY

FUS Mislocalization and Vulnerability to DNA Damage in ALS Patients Derived hiPSCs and Aging Motoneurons

(2016) Julia Higelin et al.   Frontiers in Cellular Neuroscience

Increased neuronal death and disturbed axonal growth in the Polμ-deficient mouse embryonic retina

(2016) Jimena Baleriola et al.   Scientific Reports

Distinct stages in stress granule assembly and disassembly

(2016) Joshua R Wheeler et al.   eLife

TDP-43 or FUS-induced misfolded human wild-type SOD1 can propagate intercellularly in a prion-like fashion

(2016) Edward Pokrishevsky et al.   Scientific Reports

The NEK1 interactor, C21ORF2, is required for efficient DNA damage repair

(2015) Xiao Fang et al.   ACTA BIOCHIMICA ET BIOPHYSICA SINICA

Mutations in the ubiquitin-binding domain of OPTN/optineurin interfere with autophagy-mediated degradation of misfolded proteins by a dominant-negative mechanism

(2015) Wen-Chuan Shen et al.   Autophagy

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

(2015) Avinash Patel et al.   CELL

Activity-Induced DNA Breaks Govern the Expression of Neuronal Early-Response Genes

(2015) Ram Madabhushi et al.   CELL

DNA damage and repair regulate neuronal gene expression

(2015) Yijing Su et al.   CELL RESEARCH

ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cell-derived motoneurons

(2015) J. Lenzi et al.   Disease Models & Mechanisms

Rapamycin alleviates pathogenesis of a newDrosophilamodel of ALS-TDP

(2015) Ching-Wei Cheng et al.   JOURNAL OF NEUROGENETICS

The PINK1-PARKIN Mitochondrial Ubiquitylation Pathway Drives a Program of OPTN/NDP52 Recruitment and TBK1 Activation to Promote Mitophagy

(2015) Jin-Mi Heo et al.   MOLECULAR CELL

DNA-PKcs phosphorylates hnRNP-A1 to facilitate the RPA-to-POT1 switch and telomere capping after replication

(2015) Jiangdong Sui et al.   NUCLEIC ACIDS RESEARCH

TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD

(2015) J. P. Ling et al.   SCIENCE

Alterations in stress granule dynamics driven by TDP-43 and FUS: a link to pathological inclusions in ALS?

(2015) Anaïs Aulas et al.   Frontiers in Cellular Neuroscience

FUS Interacts with HSP60 to Promote Mitochondrial Damage

(2015) Jianwen Deng et al.   PLoS Genetics

Pathogenic role of BECN1/Beclin 1 in the development of amyotrophic lateral sclerosis

(2014) Melissa Nassif et al.   Autophagy

Sqstm1 knock-down causes a locomotor phenotype ameliorated by rapamycin in a zebrafish model of ALS/FTLD

(2014) Serena Lattante et al.   HUMAN MOLECULAR GENETICS

C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal trafficking

(2014) Manal A. Farg et al.   HUMAN MOLECULAR GENETICS

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

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

C9orf72 nucleotide repeat structures initiate molecular cascades of disease

(2014) Aaron R. Haeusler et al.   NATURE

Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis

(2014) Janel O Johnson et al.   NATURE NEUROSCIENCE

Autophagy regulates amyotrophic lateral sclerosis-linked fused in sarcoma-positive stress granules in neurons

(2014) Hyun-Hee Ryu et al.   NEUROBIOLOGY OF AGING

DNA Damage and Its Links to Neurodegeneration

(2014) Ram Madabhushi et al.   NEURON

Axonal Transport of TDP-43 mRNA Granules Is Impaired by ALS-Causing Mutations

(2014) Nael H. Alami et al.   NEURON

ER–mitochondria associations are regulated by the VAPB–PTPIP51 interaction and are disrupted by ALS/FTD-associated TDP-43

(2014) Radu Stoica et al.   Nature Communications

Superoxide dismutase 1 acts as a nuclear transcription factor to regulate oxidative stress resistance

(2014) Chi Kwan Tsang et al.   Nature Communications

Knock-In Reporter Mice Demonstrate that DNA Repair by Non-homologous End Joining Declines with Age

(2014) Amita Vaidya et al.   PLoS Genetics

DNA repair mechanisms in dividing and non-dividing cells

(2013) Teruaki Iyama et al.   DNA REPAIR

Characterization of the repeat expansion size in C9orf72 in amyotrophic lateral sclerosis and frontotemporal dementia

(2013) O. Dols-Icardo et al.   HUMAN MOLECULAR GENETICS

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

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

(2013) Hong Joo Kim et al.   NATURE

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

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

Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

(2013) Shuo-Chien Ling et al.   NEURON

PARP-1 dependent recruitment of the amyotrophic lateral sclerosis-associated protein FUS/TLS to sites of oxidative DNA damage

(2013) Stuart L. Rulten et al.   NUCLEIC ACIDS RESEARCH

Microhomology-mediated End Joining and Homologous Recombination share the initial end resection step to repair DNA double-strand breaks in mammalian cells

(2013) L. N. Truong et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

TDP-43-mediated neurodegeneration: towards a loss-of-function hypothesis?

(2013) Lies Vanden Broeck et al.   TRENDS IN MOLECULAR MEDICINE

Postreplicative Mismatch Repair

(2013) J. Jiricny   Cold Spring Harbor Perspectives in Biology

Regulation of DNA cross-link repair by the Fanconi anemia/BRCA pathway

(2012) H. Kim et al.   GENES & DEVELOPMENT

Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis

(2012) Julien Couthouis et al.   HUMAN MOLECULAR GENETICS

The ALS disease protein TDP-43 is actively transported in motor neuron axons and regulates axon outgrowth

(2012) Claudia Fallini et al.   HUMAN MOLECULAR GENETICS

TBK-1 Promotes Autophagy-Mediated Antimicrobial Defense by Controlling Autophagosome Maturation

(2012) Manohar Pilli et al.   IMMUNITY

Emerging functions of the VCP/p97 AAA-ATPase in the ubiquitin system

(2012) Hemmo Meyer et al.   NATURE CELL BIOLOGY

Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs

(2012) Clotilde Lagier-Tourenne et al.   NATURE NEUROSCIENCE

Aberrant Localization of FUS and TDP43 Is Associated with Misfolding of SOD1 in Amyotrophic Lateral Sclerosis

(2012) Edward Pokrishevsky et al.   PLoS One

Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43

(2012) I.-F. Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mutational analysis reveals the FUS homolog TAF15 as a candidate gene for familial amyotrophic lateral sclerosis

(2011) N. Ticozzi et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART B-NEUROPSYCHIATRIC GENETICS

Never-in-mitosis related Kinase 1 functions in DNA damage response and checkpoint control

(2011) Yumay Chen et al.   CELL CYCLE

Involvement of Matrin 3 and SFPQ/NONO in the DNA damage response

(2011) Maayan Salton et al.   CELL CYCLE

Functional characterization connects individual patient mutations in ataxia telangiectasia mutated (ATM) with dysfunction of specific DNA double-strand break-repair signaling pathways

(2011) Marlen Keimling et al.   FASEB JOURNAL

The ubiquitin-selective segregase VCP/p97 orchestrates the response to DNA double-strand breaks

(2011) Mayura Meerang et al.   NATURE CELL BIOLOGY

The AAA-ATPase VCP/p97 promotes 53BP1 recruitment by removing L3MBTL1 from DNA double-strand breaks

(2011) Klara Acs et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

Role of Cell Cycle Re-Entry in Neurons: A Common Apoptotic Mechanism of Neuronal Cell Death

(2011) Jaume Folch et al.   NEUROTOXICITY RESEARCH

Stable Formation of Compositionally Unique Stress Granules in Virus-Infected Cells

(2010) J. Piotrowska et al.   JOURNAL OF VIROLOGY

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden et al.   NATURE

Mutations of optineurin in amyotrophic lateral sclerosis

(2010) Hirofumi Maruyama et al.   NATURE

Exome Sequencing Reveals VCP Mutations as a Cause of Familial ALS

(2010) Janel O. Johnson et al.   NEURON

Preparation of heteroduplex enhanced green fluorescent protein plasmid for in vivo mismatch repair activity assay

(2009) Bisheng Zhou et al.   ANALYTICAL BIOCHEMISTRY

Human RIF1 encodes an anti-apoptotic factor required for DNA repair

(2009) H. Wang et al.   CARCINOGENESIS

Age-Dependent Deterioration of Nuclear Pore Complexes Causes a Loss of Nuclear Integrity in Postmitotic Cells

(2009) Maximiliano A. D'Angelo et al.   CELL

Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease

(2009) Jeong-Sun Ju et al.   JOURNAL OF CELL BIOLOGY

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

(2008) Mary Gardiner et al.   BIOCHEMICAL JOURNAL

Early steps in the DNA base excision/single-strand interruption repair pathway in mammalian cells

(2008) Muralidhar L Hegde et al.   CELL RESEARCH

The long amplicon quantitative PCR for DNA damage assay as a sensitive method of assessing DNA damage in the environmental model, Atlantic killifish (Fundulus heteroclitus)

(2008) Dawoon Jung et al.   COMPARATIVE BIOCHEMISTRY AND PHYSIOLOGY C-TOXICOLOGY & PHARMACOLOGY

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis

(2008) Edor Kabashi et al.   NATURE GENETICS