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

Energy metabolism in ALS: an underappreciated opportunity?

(2018) Tijs Vandoorne et al.   ACTA NEUROPATHOLOGICA

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

(2018) Marcel Naumann et al.   Nature Communications

Oxidative Genome Damage and its Repair in Neurodegenerative Diseases: Function of Transition Metals as a Double-Edged Sword

(2018) Muralidhar L. Hegde et al.   JOURNAL OF ALZHEIMERS DISEASE

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

Kinetics of poly(ADP-ribosyl)ation, but not PARP1 itself, determines the cell fate in response to DNA damage in vitro and in vivo

(2017) Harald Schuhwerk et al.   NUCLEIC ACIDS RESEARCH

Human DNA Ligase I Interacts with and Is Targeted for Degradation by the DCAF7 Specificity Factor of the Cul4-DDB1 Ubiquitin Ligase Complex

(2016) Zhimin Peng et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

R521C and R521H mutations in FUS result in weak binding with Karyopherinβ2 leading to Amyotrophic lateral sclerosis: a molecular docking and dynamics study

(2016) Rayapadi G. Swetha et al.   JOURNAL OF BIOMOLECULAR STRUCTURE & DYNAMICS

Deficient RNA-editing enzyme ADAR2 in an amyotrophic lateral sclerosis patient with a FUSP525L mutation

(2016) Hitoshi Aizawa et al.   JOURNAL OF CLINICAL NEUROSCIENCE

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

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

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

(2016) Aarti Sharma et al.   Nature Communications

Pathogenesis of FUS-associated ALS and FTD: insights from rodent models

(2016) Matthew Nolan et al.   Acta Neuropathologica Communications

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

(2015) Avinash Patel et al.   CELL

The C-terminal Domain (CTD) of Human DNA Glycosylase NEIL1 Is Required for Forming BERosome Repair Complex with DNA Replication Proteins at the Replicating Genome

(2015) Pavana M. Hegde et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Interaction of amyotrophic lateral sclerosis/frontotemporal lobar degeneration–associated fused-in-sarcoma with proteins involved in metabolic and protein degradation pathways

(2015) Tao Wang et al.   NEUROBIOLOGY OF AGING

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

(2015) Matthias Altmeyer et al.   Nature Communications

FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis

(2015) Yoshihiro Kino et al.   Acta Neuropathologica Communications

Speckle-type POZ protein, SPOP, is involved in the DNA damage response

(2014) D. Zhang et al.   CARCINOGENESIS

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

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

DNA Damage and Its Links to Neurodegeneration

(2014) Ram Madabhushi et al.   NEURON

NEIL2 Protects against Oxidative DNA Damage Induced by Sidestream Smoke in Human Cells

(2014) Altaf H. Sarker et al.   PLoS One

MOF Phosphorylation by ATM Regulates 53BP1-Mediated Double-Strand Break Repair Pathway Choice

(2014) Arun Gupta et al.   Cell Reports

DNA repair mechanisms in dividing and non-dividing cells

(2013) Teruaki Iyama et al.   DNA REPAIR

Activation of cellular signaling by 8-oxoguanine DNA glycosylase-1-initiated DNA base excision repair

(2013) Peter German et al.   DNA REPAIR

ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules

(2013) Caroline Vance 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

Fused in sarcoma (FUS): An oncogene goes awry in neurodegeneration

(2013) Dorothee Dormann et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Site-specific characterization of the Asp- and Glu-ADP-ribosylated proteome

(2013) Yajie Zhang et al.   NATURE METHODS

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

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

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

Prereplicative repair of oxidized bases in the human genome is mediated by NEIL1 DNA glycosylase together with replication proteins

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

Genetic Screens in Human Cells Using the CRISPR-Cas9 System

(2013) Tim Wang et al.   SCIENCE

The Interaction between Polynucleotide Kinase Phosphatase and the DNA Repair Protein XRCC1 Is Critical for Repair of DNA Alkylation Damage and Stable Association at DNA Damage Sites

(2012) Julie Della-Maria et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Oxidative genome damage and its repair: Implications in aging and neurodegenerative diseases

(2012) Muralidhar L. Hegde et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Human Mre11/Human Rad50/Nbs1 and DNA Ligase IIIα/XRCC1 Protein Complexes Act Together in an Alternative Nonhomologous End Joining Pathway

(2011) Julie Della-Maria et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Oxidatively Generated Damage to DNA by UVA Radiation in Cells and Human Skin

(2011) Jean Cadet et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

P525L FUS mutation is consistently associated with a severe form of juvenile Amyotrophic Lateral Sclerosis

(2011) Amelia Conte et al.   NEUROMUSCULAR DISORDERS

TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

(2010) Ian RA Mackenzie et al.   LANCET NEUROLOGY

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

(2009) H. Wang et al.   CARCINOGENESIS

Oxidative Stress and Neurodegenerative Diseases: A Review of Upstream and Downstream Antioxidant Therapeutic Options

(2009) Bayani Uttara et al.   Current Neuropharmacology

Sensitized formation of oxidatively generated damage to cellular DNA by UVA radiation

(2009) Jean Cadet et al.   PHOTOCHEMICAL & PHOTOBIOLOGICAL SCIENCES

Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6

(2009) C. Vance et al.   SCIENCE

Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis

(2009) T. J. Kwiatkowski et al.   SCIENCE

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

Increased mitochondrial oxidative damage and oxidative DNA damage contributes to the neurodegenerative process in sporadic amyotrophic lateral sclerosis

(2008) Takahiko Murata et al.   FREE RADICAL RESEARCH

Physical and Functional Interaction between Human Oxidized Base-specific DNA Glycosylase NEIL1 and Flap Endonuclease 1

(2008) Muralidhar L. Hegde et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Single-strand break repair and genetic disease

(2008) Keith W. Caldecott   NATURE REVIEWS GENETICS