Prospects for neuroprotective therapies in prodromal Huntington's disease

Vesicular Glycolysis Provides On-Board Energy for Fast Axonal Transport

(2013) Diana Zala et al.   CELL

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

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

PGC-1α, mitochondrial dysfunction, and Huntington's disease

(2013) Ashu Johri et al.   FREE RADICAL BIOLOGY AND MEDICINE

Metabolic network as a progression biomarker of premanifest Huntington’s disease

(2013) Chris C. Tang et al.   JOURNAL OF CLINICAL INVESTIGATION

Decreased Metabolism in the Cerebral Cortex in Early-Stage Huntington's Disease: A Possible Biomarker of Disease Progression?

(2013) Hyeeun Shin et al.   Journal of Clinical Neurology

Cognitive decline in prodromal Huntington Disease: implications for clinical trials

(2013) Jane S Paulsen et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data

(2013) Sarah J Tabrizi et al.   LANCET NEUROLOGY

The impact of single-nucleotide polymorphisms (SNPs) in OGG1 and XPC on the age at onset of Huntington disease

(2013) Frédérique Berger et al.   MUTATION RESEARCH-GENETIC TOXICOLOGY AND ENVIRONMENTAL MUTAGENESIS

Glutathione peroxidase activity is neuroprotective in models of Huntington's disease

(2013) Robert P Mason et al.   NATURE GENETICS

Huntington disease: How many repeats does it take?

(2013) P. Hogarth   NEUROLOGY

Redefining the genetic risk for Huntington disease

(2013) A. Feigin   NEUROLOGY

Characterization of the Huntington intermediate CAG repeat expansion phenotype in PHAROS

(2013) A. Killoran et al.   NEUROLOGY

Structural MRI in Huntington's disease and recommendations for its potential use in clinical trials

(2013) Nellie Georgiou-Karistianis et al.   NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS

A greatly extended PPARGC1A genomic locus encodes several new brain-specific isoforms and influences Huntington disease age of onset†

(2012) Selma M. Soyal et al.   HUMAN MOLECULAR GENETICS

Elevated NADPH oxidase activity contributes to oxidative stress and cell death in Huntington's disease

(2012) A. Valencia et al.   HUMAN MOLECULAR GENETICS

Mutant huntingtin fragmentation in immune cells tracks Huntington’s disease progression

(2012) Andreas Weiss et al.   JOURNAL OF CLINICAL INVESTIGATION

R6/2 Huntington's Disease Mice Develop Early and Progressive Abnormal Brain Metabolism and Seizures

(2012) E. Cepeda-Prado et al.   JOURNAL OF NEUROSCIENCE

Self-assembling Modified β-Cyclodextrin Nanoparticles as Neuronal siRNA Delivery Vectors: Focus on Huntington’s Disease

(2012) Bruno M. D. C. Godinho et al.   MOLECULAR PHARMACEUTICS

Huntington's disease: How intermediate are intermediate repeat lengths?

(2012) Ferdinando Squitieri et al.   MOVEMENT DISORDERS

Sustained Therapeutic Reversal of Huntington's Disease by Transient Repression of Huntingtin Synthesis

(2012) Holly B. Kordasiewicz et al.   NEURON

Metabolic State Determines Sensitivity to Cellular Stress in Huntington Disease: Normalization by Activation of PPARγ

(2012) Youngnam N. Jin et al.   PLoS One

Targeting of XJB-5-131 to Mitochondria Suppresses Oxidative DNA Damage and Motor Decline in a Mouse Model of Huntington’s Disease

(2012) Zhiyin Xun et al.   Cell Reports

Antioxidants in Huntington's disease

(2011) Ashu Johri et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Pharmacologic activation of mitochondrial biogenesis exerts widespread beneficial effects in a transgenic mouse model of Huntington's disease

(2011) Ashu Johri et al.   HUMAN MOLECULAR GENETICS

Energy deficit in Huntington disease: why it matters

(2011) Fanny Mochel et al.   JOURNAL OF CLINICAL INVESTIGATION

Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data

(2011) Sarah J Tabrizi et al.   LANCET NEUROLOGY

Neuroprotective role of Sirt1 in mammalian models of Huntington's disease through activation of multiple Sirt1 targets

(2011) Mali Jiang et al.   NATURE MEDICINE

Sirt1 mediates neuroprotection from mutant huntingtin by activation of the TORC1 and CREB transcriptional pathway

(2011) Hyunkyung Jeong et al.   NATURE MEDICINE

PPARgamma rescue of the mitochondrial dysfunction in Huntington's disease

(2011) Ming-Chang Chiang et al.   NEUROBIOLOGY OF DISEASE

Age-dependent neurovascular abnormalities and altered microglial morphology in the YAC128 mouse model of Huntington disease

(2011) Sonia Franciosi et al.   NEUROBIOLOGY OF DISEASE

Truncated Peroxisome Proliferator-Activated Receptor-γ Coactivator 1α Splice Variant Is Severely Altered in Huntington’s Disease

(2011) Ashu Johri et al.   Neurodegenerative Diseases

Efficacy of Fumaric Acid Esters in the R6/2 and YAC128 Models of Huntington's Disease

(2011) Gisa Ellrichmann et al.   PLoS One

Transcriptional modulator H2A histone family, member Y (H2AFY) marks Huntington disease activity in man and mouse

(2011) Y. Hu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Striatal and white matter predictors of estimated diagnosis for Huntington disease

(2010) Jane S. Paulsen et al.   BRAIN RESEARCH BULLETIN

Seeking Brain Biomarkers for Preventive Therapy in Huntington Disease

(2010) Mouna Esmaeilzadeh et al.   CNS Neuroscience & Therapeutics

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

Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease

(2010) J. Kim et al.   HUMAN MOLECULAR GENETICS

Impairment of PGC-1alpha expression, neuropathology and hepatic steatosis in a transgenic mouse model of Huntington's disease following chronic energy deprivation

(2010) R. K. Chaturvedi et al.   HUMAN MOLECULAR GENETICS

Longitudinal change in regional brain volumes in prodromal Huntington disease

(2010) E. H. Aylward et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis

(2010) Sarah J Tabrizi et al.   LANCET NEUROLOGY

Safety and tolerability of high-dosage coenzyme Q10 in Huntington's disease and healthy subjects

(2010)   MOVEMENT DISORDERS

Nicotinamide improves motor deficits and upregulates PGC-1α and BDNF gene expression in a mouse model of Huntington's disease

(2010) Tyisha Hathorn et al.   NEUROBIOLOGY OF DISEASE

Huntingtin Is Required for Mitotic Spindle Orientation and Mammalian Neurogenesis

(2010) Juliette D. Godin et al.   NEURON

Self-paced timing detects and tracks change in prodromal Huntington disease.

(2010) Kelly C. Rowe et al.   NEUROPSYCHOLOGY

Mitochondria Targeted Peptides Protect Against 1-Methyl-4-Phenyl-1,2,3,6-Tetrahydropyridine Neurotoxicity

(2009) Lichuan Yang et al.   ANTIOXIDANTS & REDOX SIGNALING

Huntington's disease: Silencing a brutal killer

(2009) Edith L. Pfister et al.   EXPERIMENTAL NEUROLOGY

Impaired PGC-1α function in muscle in Huntington's disease

(2009) Rajnish K. Chaturvedi et al.   HUMAN MOLECULAR GENETICS

Obsessive and Compulsive Symptoms in Prediagnosed Huntington's Disease

(2009) Leigh J. Beglinger et al.   JOURNAL OF CLINICAL PSYCHIATRY

The gene coding for PGC-1α modifies age at onset in Huntington's Disease

(2009) Patrick Weydt et al.   Molecular Neurodegeneration

PGC-1alpha as modifier of onset age in Huntington disease

(2009) Elahe Taherzadeh-Fard et al.   Molecular Neurodegeneration

Nonallele-specific Silencing of Mutant and Wild-type Huntingtin Demonstrates Therapeutic Efficacy in Huntington's Disease Mice

(2009) Ryan L Boudreau et al.   MOLECULAR THERAPY

Motor abnormalities in premanifest persons with Huntington's disease: The PREDICT-HD study

(2009) Kevin M. Biglan et al.   MOVEMENT DISORDERS

Automatic detection of preclinical neurodegeneration: Presymptomatic Huntington disease

(2009) S. Kloppel et al.   NEUROLOGY

Neuroprotective Effects of the Triterpenoid, CDDO Methyl Amide, a Potent Inducer of Nrf2-Mediated Transcription

(2009) Lichuan Yang et al.   PLoS One

Huntingtin as an essential integrator of intracellular vesicular trafficking

(2009) Juliane P. Caviston et al.   TRENDS IN CELL BIOLOGY

Plasma 24S-hydroxycholesterol and caudate MRI in pre-manifest and early Huntington's disease

(2008) Valerio Leoni et al.   BRAIN

Huntingtin phosphorylation acts as a molecular switch for anterograde/retrograde transport in neurons

(2008) Emilie Colin et al.   EMBO JOURNAL

A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease

(2008) Maria Björkqvist et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Detection of Huntington's disease decades before diagnosis: the Predict-HD study

(2007) J S Paulsen et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY