Huntingtin is a scaffolding protein in the ATM oxidative DNA damage response complex

Huntingtin N17 domain is a reactive oxygen species sensor regulating huntingtin phosphorylation and localization

(2016) Laura F. DiGiovanni et al.   HUMAN MOLECULAR GENETICS

Structure of the intact ATM/Tel1 kinase

(2016) Xuejuan Wang et al.   Nature Communications

Huntingtin’s spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function

(2016) Ravi Vijayvargia et al.   eLife

Identification of Genetic Factors that Modify Clinical Onset of Huntington’s Disease

(2015) Jong-Min Lee et al.   CELL

Huntington disease iPSCs show early molecular changes in intracellular signaling, the expression of oxidative stress proteins and the p53 pathway

(2015) Wojciech J. Szlachcic et al.   Disease Models & Mechanisms

Reactive Oxygen Species (ROS)-Activated ATM-Dependent Phosphorylation of Cytoplasmic Substrates Identified by Large-Scale Phosphoproteomics Screen

(2015) Sergei V. Kozlov et al.   MOLECULAR & CELLULAR PROTEOMICS

Quantitative characterization of protein–protein complexes involved in base excision DNA repair

(2015) Nina A. Moor et al.   NUCLEIC ACIDS RESEARCH

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

Suppression of Somatic Expansion Delays the Onset of Pathophysiology in a Mouse Model of Huntington’s Disease

(2015) Helen Budworth et al.   PLoS Genetics

A novel manganese-dependent ATM-p53 signaling pathway is selectively impaired in patient-based neuroprogenitor and murine striatal models of Huntington's disease

(2014) Andrew M. Tidball et al.   HUMAN MOLECULAR GENETICS

A huntingtin-mediated fast stress response halting endosomal trafficking is defective in Huntington's disease

(2014) Siddharth Nath et al.   HUMAN MOLECULAR GENETICS

Selection and characterization of llama single domain antibodies against N-terminal huntingtin

(2014) Menno H. Schut et al.   NEUROLOGICAL SCIENCES

Targeting ATM ameliorates mutant Huntingtin toxicity in cell and animal models of Huntington's disease

(2014) X.-H. Lu et al.   Science Translational Medicine

OpenComet: An automated tool for comet assay image analysis

(2014) Benjamin M. Gyori et al.   Redox Biology

Role of oxidative DNA damage in mitochondrial dysfunction and Huntington’s disease pathogenesis

(2013) Sylvette Ayala-Peña   FREE RADICAL BIOLOGY AND MEDICINE

The huntingtin N17 domain is a multifunctional CRM1 and Ran-dependent nuclear and cilial export signal

(2013) T. Maiuri et al.   HUMAN MOLECULAR GENETICS

Live cell imaging and biophotonic methods reveal two types of mutant huntingtin inclusions

(2013) Nicholas S. Caron et al.   HUMAN MOLECULAR GENETICS

Mutations of the Huntington’s Disease Protein Impact on the ATM-Dependent Signaling and Repair Pathways of the Radiation-Induced DNA Double-Strand Breaks: Corrective Effect of Statins and Bisphosphonates

(2013) Mélanie L. Ferlazzo et al.   MOLECULAR NEUROBIOLOGY

Distinct spatiotemporal patterns and PARP dependence of XRCC1 recruitment to single-strand break and base excision repair

(2013) Anna Campalans et al.   NUCLEIC ACIDS RESEARCH

Comparison of Modules of Wild Type and Mutant Huntingtin and TP53 Protein Interaction Networks: Implications in Biological Processes and Functions

(2013) Mahashweta Basu et al.   PLoS One

Polyglutamine domain flexibility mediates the proximity between flanking sequences in huntingtin

(2013) Nicholas Stephane Caron et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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

Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice

(2012) A. Di Pardo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mutant huntingtin causes defective actin remodeling during stress: defining a new role for transglutaminase 2 in neurodegenerative disease

(2011) Lise Munsie et al.   HUMAN MOLECULAR GENETICS

Kinase inhibitors modulate huntingtin cell localization and toxicity

(2011) Randy Singh Atwal et al.   Nature Chemical Biology

Triterpenoids CDDO-ethyl amide and CDDO-trifluoroethyl amide improve the behavioral phenotype and brain pathology in a transgenic mouse model of Huntington's disease

(2010) Cliona Stack et al.   FREE RADICAL BIOLOGY AND MEDICINE

Mutant huntingtin impairs Ku70-mediated DNA repair

(2010) Yasushi Enokido et al.   JOURNAL OF CELL BIOLOGY

Oxidative stress triggers the preferential assembly of base excision repair complexes on open chromatin regions

(2010) Rachel Amouroux et al.   NUCLEIC ACIDS RESEARCH

PR65, the HEAT-repeat scaffold of phosphatase PP2A, is an elastic connector that links force and catalysis

(2010) A. Grinthal et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

ATM Activation by Oxidative Stress

(2010) Z. Guo et al.   SCIENCE

Aurora B-Mediated Abscission Checkpoint Protects against Tetraploidization

(2009) Patrick Steigemann et al.   CELL

Polyglutamine disruption of the huntingtin exon 1 N terminus triggers a complex aggregation mechanism

(2009) Ashwani K Thakur et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Serines 13 and 16 Are Critical Determinants of Full-Length Human Mutant Huntingtin Induced Disease Pathogenesis in HD Mice

(2009) Xiaofeng Gu et al.   NEURON

Mitochondrial DNA damage is a hallmark of chemically induced and the R6/2 transgenic model of Huntington's disease

(2008) Karina Acevedo-Torres et al.   DNA REPAIR

Full-Length Human Mutant Huntingtin with a Stable Polyglutamine Repeat Can Elicit Progressive and Selective Neuropathogenesis in BACHD Mice

(2008) M. Gray et al.   JOURNAL OF NEUROSCIENCE

Intrabodies Binding the Proline-Rich Domains of Mutant Huntingtin Increase Its Turnover and Reduce Neurotoxicity

(2008) A. L. Southwell et al.   JOURNAL OF NEUROSCIENCE

DNA breakage and induction of DNA damage response proteins precede the appearance of visible mutant huntingtin aggregates

(2008) Jennifer Illuzzi et al.   JOURNAL OF NEUROSCIENCE RESEARCH