Mouse Models of Polyglutamine Diseases in Therapeutic Approaches: Review and Data Table. Part II

Suppression of protein aggregation by chaperone modification of high molecular weight complexes

(2012) John Labbadia et al.   BRAIN

Targeting Kynurenine 3-Monooxygenase (KMO): Implications for Therapy in Huntingtons Disease

(2012) Mathuravani A. Thevandavakkam et al.   CNS & Neurological Disorders-Drug Targets

Hypothalamic and Neuroendocrine Changes in Huntingtons Disease

(2012) Sofia Hult et al.   CURRENT DRUG TARGETS

Transgenic Mouse Model Expressing the Caspase 6 Fragment of Mutant Huntingtin

(2012) E. Waldron-Roby et al.   JOURNAL OF NEUROSCIENCE

Bone Marrow Transplantation Confers Modest Benefits in Mouse Models of Huntington's Disease

(2012) W. Kwan et al.   JOURNAL OF NEUROSCIENCE

The group 2 metabotropic glutamate receptor agonist LY379268 rescues neuronal, neurochemical and motor abnormalities in R6/2 Huntington's disease mice

(2012) A. Reiner et al.   NEUROBIOLOGY OF DISEASE

NP03, a novel low-dose lithium formulation, is neuroprotective in the YAC128 mouse model of Huntington disease

(2012) Mahmoud A. Pouladi et al.   NEUROBIOLOGY OF DISEASE

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

(2012) Holly B. Kordasiewicz et al.   NEURON

Overexpression of HGF attenuates the degeneration of Purkinje cells and Bergmann glia in a knockin mouse model of spinocerebellar ataxia type 7

(2012) Satsuki Noma et al.   NEUROSCIENCE RESEARCH

Genetic Knock-Down of Hdac3 Does Not Modify Disease-Related Phenotypes in a Mouse Model of Huntington's Disease

(2012) Lara Moumné et al.   PLoS One

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

Kynurenine 3-Monooxygenase Inhibition in Blood Ameliorates Neurodegeneration

(2011) Daniel Zwilling et al.   CELL

Glial S100B Protein Modulates Mutant Ataxin-1 Aggregation and Toxicity: TRTK12 Peptide, a Potential Candidate for SCA1 Therapy

(2011) Parminder J. S. Vig et al.   CEREBELLUM

Sleep Disorders in Machado–Joseph Disease: Frequency, Discriminative Thresholds, Predictive Values, and Correlation with Ataxia-Related Motor and Non-Motor Features

(2011) José Luiz Pedroso et al.   CEREBELLUM

Expanded Polyglutamine-Binding Peptoid as a Novel Therapeutic Agent for Treatment of Huntington's Disease

(2011) Xuesong Chen et al.   CHEMISTRY & BIOLOGY

The Kynurenine Pathway Modulates Neurodegeneration in a Drosophila Model of Huntington's Disease

(2011) Susanna Campesan et al.   CURRENT BIOLOGY

Ataxin-1 and Brother of ataxin-1 are components of the Notch signalling pathway

(2011) Xin Tong et al.   EMBO REPORTS

IGF-1 protects against diabetic features in an in vivo model of Huntington's disease

(2011) A.I. Duarte et al.   EXPERIMENTAL NEUROLOGY

Transgenic mice expressing caspase-6-derived N-terminal fragments of mutant huntingtin develop neurologic abnormalities with predominant cytoplasmic inclusion pathology composed largely of a smaller proteolytic derivative

(2011) Andrew T.N. Tebbenkamp et al.   HUMAN MOLECULAR GENETICS

Cellular toxicity of expanded RNA repeats: focus on RNA foci

(2011) Marzena Wojciechowska et al.   HUMAN MOLECULAR GENETICS

IRS2 increases mitochondrial dysfunction and oxidative stress in a mouse model of Huntington disease

(2011) Marianna Sadagurski et al.   JOURNAL OF CLINICAL INVESTIGATION

Dysregulated brain creatine kinase is associated with hearing impairment in mouse models of Huntington disease

(2011) Yow-Sien Lin et al.   JOURNAL OF CLINICAL INVESTIGATION

Neuroprotective effects of granulocyte-colony stimulating factor in a novel transgenic mouse model of SCA17

(2011) Ya-Chin Chang et al.   JOURNAL OF NEUROCHEMISTRY

Aminopyridines Correct Early Dysfunction and Delay Neurodegeneration in a Mouse Model of Spinocerebellar Ataxia Type 1

(2011) R. Hourez et al.   JOURNAL OF NEUROSCIENCE

The Melatonin MT1 Receptor Axis Modulates Mutant Huntingtin-Mediated Toxicity

(2011) X. Wang et al.   JOURNAL OF NEUROSCIENCE

Cellular fusion for gene delivery to SCA1 affected Purkinje neurons

(2011) K. Amy Chen et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Dantrolene is neuroprotective in Huntington's disease transgenic mouse model

(2011) Xi Chen et al.   Molecular Neurodegeneration

Vascular endothelial growth factor ameliorates the ataxic phenotype in a mouse model of spinocerebellar ataxia type 1

(2011) Marija Cvetanovic et al.   NATURE MEDICINE

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

Potential therapeutic uses of BDNF in neurological and psychiatric disorders

(2011) Alan H. Nagahara et al.   NATURE REVIEWS DRUG DISCOVERY

“Brain training” improves cognitive performance and survival in a transgenic mouse model of Huntington's disease

(2011) Nigel I. Wood et al.   NEUROBIOLOGY OF DISEASE

Structural MRI detects progressive regional brain atrophy and neuroprotective effects in N171-82Q Huntington's disease mouse model

(2011) Yong Cheng et al.   NEUROIMAGE

Inhibition of mutant huntingtin expression by RNA duplex targeting expanded CAG repeats

(2011) Agnieszka Fiszer et al.   NUCLEIC ACIDS RESEARCH

Triplet repeat RNA structure and its role as pathogenic agent and therapeutic target

(2011) W. J. Krzyzosiak et al.   NUCLEIC ACIDS RESEARCH

A ketogenic diet delays weight loss and does not impair working memory or motor function in the R6/2 1J mouse model of Huntington's disease

(2011) David N. Ruskin et al.   PHYSIOLOGY & BEHAVIOR

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

(2011) Gisa Ellrichmann et al.   PLoS One

Hdac6 Knock-Out Increases Tubulin Acetylation but Does Not Modify Disease Progression in the R6/2 Mouse Model of Huntington's Disease

(2011) Anna Bobrowska et al.   PLoS One

A New Drug Design Targeting the Adenosinergic System for Huntington's Disease

(2011) Nai-Kuei Huang et al.   PLoS One

D-β-Hydroxybutyrate Is Protective in Mouse Models of Huntington's Disease

(2011) Soyeon Lim et al.   PLoS One

Human Mesenchymal Stem Cells Prolong Survival and Ameliorate Motor Deficit through Trophic Support in Huntington's Disease Mouse Models

(2011) Yuan-Ta Lin et al.   PLoS One

SAHA Decreases HDAC 2 and 4 Levels In Vivo and Improves Molecular Phenotypes in the R6/2 Mouse Model of Huntington's Disease

(2011) Michal Mielcarek et al.   PLoS One

Regional rescue of spinocerebellar ataxia type 1 phenotypes by14-3-3ε haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration

(2011) Paymaan Jafar-Nejad et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Necrostatin-1 ameliorates symptoms in R6/2 transgenic mouse model of Huntington's disease

(2011) S Zhu et al.   Cell Death & Disease

Modulation of lipid peroxidation and mitochondrial function improves neuropathology in Huntington’s disease mice

(2010) Junghee Lee et al.   ACTA NEUROPATHOLOGICA

Prospects for Minocycline Neuroprotection

(2010) Jennifer M. Plane et al.   ARCHIVES OF NEUROLOGY

Alterations in Striatal Synaptic Transmission are Consistent across Genetic Mouse Models of Huntington's Disease

(2010) Damian M Cummings et al.   ASN Neuro

Genetically engineered mesenchymal stem cells reduce behavioral deficits in the YAC 128 mouse model of Huntington's disease

(2010) Nicholas D. Dey et al.   BEHAVIOURAL BRAIN RESEARCH

Longitudinal analysis of the behavioural phenotype in YAC128 (C57BL/6J) Huntington's disease transgenic mice

(2010) Simon Brooks et al.   BRAIN RESEARCH BULLETIN

Allele-Selective Inhibition of Huntingtin Expression by Switching to an miRNA-like RNAi Mechanism

(2010) Jiaxin Hu et al.   CHEMISTRY & BIOLOGY

Inhibition of transglutaminase 2 mitigates transcriptional dysregulation in models of Huntington disease

(2010) Stephen J. McConoughey et al.   EMBO Molecular Medicine

Lack of efficacy of NMDA receptor-NR2B selective antagonists in the R6/2 model of Huntington disease

(2010) Sara J. Tallaksen-Greene et al.   EXPERIMENTAL NEUROLOGY

Neuropeptide Y modifies the disease course in the R6/2 transgenic model of Huntington's disease

(2010) M. Decressac et al.   EXPERIMENTAL NEUROLOGY

Ex vivo delivery of GDNF maintains motor function and prevents neuronal loss in a transgenic mouse model of Huntington's disease

(2010) Allison D. Ebert et al.   EXPERIMENTAL NEUROLOGY

Resveratrol protects against peripheral deficits in a mouse model of Huntington's disease

(2010) Daniel J. Ho et al.   EXPERIMENTAL NEUROLOGY

Therapeutic use of coenzyme Q10and coenzyme Q10-related compounds and formulations

(2010) Jose M Villalba et al.   EXPERT OPINION ON INVESTIGATIONAL DRUGS

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

Electroconvulsive shock ameliorates disease processes and extends survival in huntingtin mutant mice

(2010) Mohamed R. Mughal et al.   HUMAN MOLECULAR GENETICS

Rilmenidine attenuates toxicity of polyglutamine expansions in a mouse model of Huntington's disease

(2010) C. Rose et al.   HUMAN MOLECULAR GENETICS

Mitochondrial calcium uptake capacity as a therapeutic target in the R6/2 mouse model of Huntington's disease

(2010) Giselle M. Perry et al.   HUMAN MOLECULAR GENETICS

Early autophagic response in a novel knock-in model of Huntington disease

(2010) Mary Y. Heng et al.   HUMAN MOLECULAR GENETICS

ERK activation by the polyphenols fisetin and resveratrol provides neuroprotection in multiple models of Huntington's disease

(2010) Pamela Maher et al.   HUMAN MOLECULAR GENETICS

Modulation of energy deficiency in Huntington's disease via activation of the peroxisome proliferator-activated receptor gamma

(2010) Ming-Chang Chiang et al.   HUMAN MOLECULAR GENETICS

Proteolysis of Mutant Huntingtin Produces an Exon 1 Fragment That Accumulates as an Aggregated Protein in Neuronal Nuclei in Huntington Disease

(2010) Christian Landles et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Environmental Enrichment Reduces Neuronal Intranuclear Inclusion Load But Has No Effect on Messenger RNA Expression in a Mouse Model of Huntington Disease

(2010) Caroline L. Benn et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Early or Late-Stage Anti-N-Terminal Huntingtin Intrabody Gene Therapy Reduces Pathological Features in B6.HDR6/1 Mice

(2010) Abigail Snyder-Keller et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

BDNF Overexpression in the Forebrain Rescues Huntington's Disease Phenotypes in YAC128 Mice

(2010) Y. Xie et al.   JOURNAL OF NEUROSCIENCE

Acute Polyglutamine Expression in Inducible Mouse Model Unravels Ubiquitin/Proteasome System Impairment and Permanent Recovery Attributable to Aggregate Formation

(2010) Z. Ortega et al.   JOURNAL OF NEUROSCIENCE

Autosomal dominant cerebellar ataxias: polyglutamine expansions and beyond

(2010) Alexandra Durr   LANCET NEUROLOGY

Huntington's disease: from molecular pathogenesis to clinical treatment

(2010) Christopher A Ross et al.   LANCET NEUROLOGY

Tetrabenazine is neuroprotective in Huntington's disease mice

(2010) Hongyu Wang et al.   Molecular Neurodegeneration

The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease

(2010) Jonathan H Fox et al.   Molecular Neurodegeneration

A futility study of minocycline in Huntington's disease

(2010) Merit Cudkowicz   MOVEMENT DISORDERS

Harnessing chaperone-mediated autophagy for the selective degradation of mutant huntingtin protein

(2010) Peter O Bauer et al.   NATURE BIOTECHNOLOGY

Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease

(2010) Marta Martinez-Vicente et al.   NATURE NEUROSCIENCE

Brief ampakine treatments slow the progression of Huntington's disease phenotypes in R6/2 mice

(2010) Danielle A. Simmons et al.   NEUROBIOLOGY OF DISEASE

HDAC inhibitor sodium butyrate reverses transcriptional downregulation and ameliorates ataxic symptoms in a transgenic mouse model of SCA3

(2010) An-Hsun Chou et al.   NEUROBIOLOGY OF DISEASE

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

p53 activation mediates polyglutamine-expanded ataxin-3 upregulation of Bax expression in cerebellar and pontine nuclei neurons

(2010) An-Hsun Chou et al.   NEUROCHEMISTRY INTERNATIONAL

A Systematic Review of the Incidence and Prevalence of Long-Term Neurological Conditions in the UK

(2010) Thomas Hoppitt et al.   NEUROEPIDEMIOLOGY

Mouse Ataxin-3 Functional Knock-Out Model

(2010) Pawel M. Switonski et al.   NEUROMOLECULAR MEDICINE

Early Increase in Extrasynaptic NMDA Receptor Signaling and Expression Contributes to Phenotype Onset in Huntington's Disease Mice

(2010) Austen J. Milnerwood et al.   NEURON

Behavioural and molecular consequences of chronic cannabinoid treatment in Huntington's disease transgenic mice

(2010) M.J. Dowie et al.   NEUROSCIENCE

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

(2010) Chiara Zuccato et al.   PHYSIOLOGICAL REVIEWS

Responses to Environmental Enrichment Differ with Sex and Genotype in a Transgenic Mouse Model of Huntington's Disease

(2010) Nigel I. Wood et al.   PLoS One

Comprehensive Behavioral Testing in the R6/2 Mouse Model of Huntington's Disease Shows No Benefit from CoQ10 or Minocycline

(2010) Liliana B. Menalled et al.   PLoS One

Human Multipotent Stromal Cells (MSCs) Increase Neurogenesis and Decrease Atrophy of the Striatum in a Transgenic Mouse Model for Huntington's Disease

(2010) Brooke R. Snyder et al.   PLoS One

Inhibition of the Striatal Specific Phosphodiesterase PDE10A Ameliorates Striatal and Cortical Pathology in R6/2 Mouse Model of Huntington's Disease

(2010) Carmela Giampà et al.   PLoS One

Polyglutamine Diseases: Where does Toxicity Come from? What is Toxicity? Where are We Going?

(2010) T. Takahashi et al.   Journal of Molecular Cell Biology

Integration of Clearance Mechanisms: The Proteasome and Autophagy

(2010) E. Wong et al.   Cold Spring Harbor Perspectives in Biology

Slowed progression in models of huntington disease by adipose stem cell transplantation

(2009) Soon-Tae Lee et al.   ANNALS OF NEUROLOGY

Sustained effects of nonallele-specificHuntingtinsilencing

(2009) Valérie Drouet et al.   ANNALS OF NEUROLOGY

Mitochondrial calcium and the permeability transition in cell death

(2009) John J. Lemasters et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mouse models of Huntington's disease and methodological considerations for therapeutic trials

(2009) Robert J. Ferrante   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Multifaceted deaths orchestrated by mitochondria in neurones

(2009) Phillip Nagley et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Neuroprotective effects of L-carnitine in a transgenic animal model of Huntington's disease

(2009) Eniko Vamos et al.   BIOMEDICINE & PHARMACOTHERAPY

Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3

(2009) Fiona M. Menzies et al.   BRAIN

Management of sleep/wake cycles improves cognitive function in a transgenic mouse model of Huntington's disease

(2009) Patrick N. Pallier et al.   BRAIN RESEARCH

Five siRNAs Targeting Three SNPs May Provide Therapy for Three-Quarters of Huntington's Disease Patients

(2009) Edith L. Pfister et al.   CURRENT BIOLOGY

Phosphodiesterase type IV inhibition prevents sequestration of CREB binding protein, protects striatal parvalbumin interneurons and rescues motor deficits in the R6/2 mouse model of Huntington’s disease

(2009) Carmela Giampà et al.   EUROPEAN JOURNAL OF NEUROSCIENCE

Altered P2X7-receptor level and function in mouse models of Huntington’s disease and therapeutic efficacy of antagonist administration

(2009) Miguel Díaz-Hernández et al.   FASEB JOURNAL

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

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

The A2A adenosine receptor rescues the urea cycle deficiency of Huntington's disease by enhancing the activity of the ubiquitin–proteasome system

(2009) Ming-Chang Chiang et al.   HUMAN MOLECULAR GENETICS

Neuroprotective effects of probenecid in a transgenic animal model of Huntington’s disease

(2009) Eniko Vamos et al.   JOURNAL OF NEURAL TRANSMISSION

The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

(2009) Peter O. Bauer et al.   JOURNAL OF NEUROCHEMISTRY

Combination therapy with Coenzyme Q10and creatine produces additive neuroprotective effects in models of Parkinson’s and Huntington’s Diseases

(2009) Lichuan Yang et al.   JOURNAL OF NEUROCHEMISTRY

Polyglutamine-Expanded Androgen Receptor Truncation Fragments Activate a Bax-Dependent Apoptotic Cascade Mediated by DP5/Hrk

(2009) J. E. Young et al.   JOURNAL OF NEUROSCIENCE

Differential Susceptibility to Excitotoxic Stress in YAC128 Mouse Models of Huntington Disease between Initiation and Progression of Disease

(2009) R. K. Graham et al.   JOURNAL OF NEUROSCIENCE

Deranged Calcium Signaling and Neurodegeneration in Spinocerebellar Ataxia Type 2

(2009) J. Liu et al.   JOURNAL OF NEUROSCIENCE

Neuroprotective Effects of Inositol 1,4,5-Trisphosphate Receptor C-Terminal Fragment in a Huntington's Disease Mouse Model

(2009) T.-S. Tang et al.   JOURNAL OF NEUROSCIENCE

Striatal Expression of a Calmodulin Fragment Improved Motor Function, Weight Loss, and Neuropathology in the R6/2 Mouse Model of Huntington's Disease

(2009) Y. Dai et al.   JOURNAL OF NEUROSCIENCE

Alterations in Cortical Excitation and Inhibition in Genetic Mouse Models of Huntington's Disease

(2009) D. M. Cummings et al.   JOURNAL OF NEUROSCIENCE

Intrabody Gene Therapy Ameliorates Motor, Cognitive, and Neuropathological Symptoms in Multiple Mouse Models of Huntington's Disease

(2009) A. L. Southwell et al.   JOURNAL OF NEUROSCIENCE

Grafting Neural Precursor Cells Promotes Functional Recovery in an SCA1 Mouse Model

(2009) S. Chintawar et al.   JOURNAL OF NEUROSCIENCE

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

Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs

(2009) Jiaxin Hu et al.   NATURE BIOTECHNOLOGY

Balance between synaptic versus extrasynaptic NMDA receptor activity influences inclusions and neurotoxicity of mutant huntingtin

(2009) Shu-ichi Okamoto et al.   NATURE MEDICINE

Brain-derived neurotrophic factor in neurodegenerative diseases

(2009) Chiara Zuccato et al.   Nature Reviews Neurology

Y-27632 improves rotarod performance and reduces huntingtin levels in R6/2 mice

(2009) Mei Li et al.   NEUROBIOLOGY OF DISEASE

Overexpression of IGF-1 in Muscle Attenuates Disease in a Mouse Model of Spinal and Bulbar Muscular Atrophy

(2009) Isabella Palazzolo et al.   NEURON

Inhibition of apoptosis signal-regulating kinase 1 reduces endoplasmic reticulum stress and nuclear huntingtin fragments in a mouse model of Huntington disease

(2009) K.J. Cho et al.   NEUROSCIENCE

Evaluation of tetrathiomolybdate in the R6/2 model of Huntington disease

(2009) Sara J. Tallaksen-Greene et al.   NEUROSCIENCE LETTERS

Valproate ameliorates the survival and the motor performance in a transgenic mouse model of Huntington's disease

(2009) Dénes Zádori et al.   PHARMACOLOGY BIOCHEMISTRY AND BEHAVIOR

Genetic Knock-Down of HDAC7 Does Not Ameliorate Disease Pathogenesis in the R6/2 Mouse Model of Huntington's Disease

(2009) Caroline L. Benn et al.   PLoS One

Coupling diverse routes of calcium entry to mitochondrial dysfunction and glutamate excitotoxicity

(2009) R. I. Stanika et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Up-regulating BDNF with an ampakine rescues synaptic plasticity and memory in Huntington's disease knockin mice

(2009) D. A. Simmons et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Of mice, rats and men: Revisiting the quinolinic acid hypothesis of Huntington's disease

(2009) Robert Schwarcz et al.   PROGRESS IN NEUROBIOLOGY

Autophagy and the ubiquitin-proteasome system: Collaborators in neuroprotection

(2008) Natalia B. Nedelsky et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Wheel running from a juvenile age delays onset of specific motor deficits but does not alter protein aggregate density in a mouse model of Huntington's disease

(2008) Anton van Dellen et al.   BMC NEUROSCIENCE

The clinical diagnosis of autosomal dominant spinocerebellar ataxias

(2008) Thomas Klockgether   CEREBELLUM

Conformational Changes and Aggregation of Expanded Polyglutamine Proteins as Therapeutic Targets of the Polyglutamine Diseases: Exposed β-Sheet Hypothesis

(2008) Yoshitaka Nagai et al.   CURRENT PHARMACEUTICAL DESIGN

Exendin-4 Improves Glycemic Control, Ameliorates Brain and Pancreatic Pathologies, and Extends Survival in a Mouse Model of Huntington's Disease

(2008) B. Martin et al.   DIABETES

Lentivector-mediated rescue from cerebellar ataxia in a mouse model of spinocerebellar ataxia

(2008) Takashi Torashima et al.   EMBO REPORTS

Adenosine A2A receptor antagonism increases nNOS-immunoreactive neurons in the striatum of Huntington transgenic mice

(2008) S. Cipriani et al.   EXPERIMENTAL NEUROLOGY

Combinational approach of intrabody with enhanced Hsp70 expression addresses multiple pathologies in a fly model of Huntington’s disease

(2008) J. A. McLear et al.   FASEB JOURNAL

Neuronal viability is controlled by a functional relation between synaptic and extrasynaptic NMDA receptors

(2008) F. Léveillé et al.   FASEB JOURNAL

Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice

(2008) Toshiya Sato et al.   HUMAN MOLECULAR GENETICS

Blocking acid-sensing ion channel 1 alleviates Huntington's disease pathology via an ubiquitin-proteasome system-dependent mechanism

(2008) Hon Kit Wong et al.   HUMAN MOLECULAR GENETICS

17-DMAG ameliorates polyglutamine-mediated motor neuron degeneration through well-preserved proteasome function in an SBMA model mouse

(2008) Keisuke Tokui et al.   HUMAN MOLECULAR GENETICS

Sex-dependent Effect of BAG1 in Ameliorating Motor Deficits of Huntington Disease Transgenic Mice

(2008) Adam L. Orr et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin

(2008) Chuan-En Wang et al.   JOURNAL OF CELL BIOLOGY

Brain-derived neurotrophic factor over-expression in the forebrain ameliorates Huntington’s disease phenotypes in mice

(2008) Kusumika Gharami et al.   JOURNAL OF NEUROCHEMISTRY

Deranged Calcium Signaling and Neurodegeneration in Spinocerebellar Ataxia Type 3

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

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

N-Terminal Mutant Huntingtin Associates with Mitochondria and Impairs Mitochondrial Trafficking

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

Inhibitors of Cytochrome c Release with Therapeutic Potential for Huntington's Disease

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

Sustained striatal ciliary neurotrophic factor expression negatively affects behavior and gene expression in normal and R6/1 mice

(2008) Eileen M. Denovan-Wright et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Intracerebral transplantation of neural stem cells combined with trehalose ingestion alleviates pathology in a mouse model of Huntington's disease

(2008) Chia-Ron Yang et al.   JOURNAL OF NEUROSCIENCE RESEARCH

CEP-1347 reduces mutant huntingtin-associated neurotoxicity and restores BDNF levels in R6/2 mice

(2008) Barbara L. Apostol et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Absolute requirement of GDNF for adult catecholaminergic neuron survival

(2008) Alberto Pascual et al.   NATURE NEUROSCIENCE

Beneficial effects of rolipram in the R6/2 mouse model of Huntington's disease

(2008) Zena DeMarch et al.   NEUROBIOLOGY OF DISEASE

Full length mutant huntingtin is required for altered Ca2+ signaling and apoptosis of striatal neurons in the YAC mouse model of Huntington's disease

(2008) Hua Zhang et al.   NEUROBIOLOGY OF DISEASE

Rodent genetic models of Huntington disease

(2008) Mary Y. Heng et al.   NEUROBIOLOGY OF DISEASE

Functional roles for the striatal-enriched transcription factor, Bcl11b, in the control of striatal gene expression and transcriptional dysregulation in Huntington's disease

(2008) Paula A. Desplats et al.   NEUROBIOLOGY OF DISEASE

Tiagabine is neuroprotective in the N171-82Q and R6/2 mouse models of Huntington's disease

(2008) Naoki Masuda et al.   NEUROBIOLOGY OF DISEASE

Sertraline slows disease progression and increases neurogenesis in N171-82Q mouse model of Huntington's disease

(2008) Wenzhen Duan et al.   NEUROBIOLOGY OF DISEASE

Intrastriatal CERE-120 (AAV-Neurturin) protects striatal and cortical neurons and delays motor deficits in a transgenic mouse model of Huntington's disease

(2008) Shilpa Ramaswamy et al.   NEUROBIOLOGY OF DISEASE

From Synapse to Nucleus: Calcium-Dependent Gene Transcription in the Control of Synapse Development and Function

(2008) Paul L. Greer et al.   NEURON

Up-regulation of GLT1 expression increases glutamate uptake and attenuates the Huntington's disease phenotype in the R6/2 mouse

(2008) B.R. Miller et al.   NEUROSCIENCE

Brain-derived neurotrophic factor modulates the severity of cognitive alterations induced by mutant huntingtin: Involvement of phospholipaseCγ activity and glutamate receptor expression

(2008) A. Giralt et al.   NEUROSCIENCE

Delivery of the aggregate inhibitor peptide QBP1 into the mouse brain using PTDs and its therapeutic effect on polyglutamine disease mice

(2008) H. Akiko Popiel et al.   NEUROSCIENCE LETTERS

Allele-Specific RNA Silencing of Mutant Ataxin-3 Mediates Neuroprotection in a Rat Model of Machado-Joseph Disease

(2008) Sandro Alves et al.   PLoS One

The HDAC inhibitor 4b ameliorates the disease phenotype and transcriptional abnormalities in Huntington's disease transgenic mice

(2008) E. A. Thomas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The antidepressant sertraline improves the phenotype, promotes neurogenesis and increases BDNF levels in the R6/2 Huntington's disease mouse model

(2007) Qi Peng et al.   EXPERIMENTAL NEUROLOGY

Gene–environment interactions modulating cognitive function and molecular correlates of synaptic plasticity in Huntington’s disease transgenic mice

(2007) Jess Nithianantharajah et al.   NEUROBIOLOGY OF DISEASE