Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

Complexity and Heterogeneity: What Drives the Ever-changing Brain in Huntington's Disease?

(2010) H. Diana Rosas et al.   Annals of the New York Academy of Sciences

Mitochondria and Huntington's Disease Pathogenesis

(2010) Susan E. Browne   Annals of the New York Academy of Sciences

An ovine transgenic Huntington's disease model

(2010) J. C. Jacobsen et al.   HUMAN MOLECULAR GENETICS

Erratum: Neural stem cell systems: physiological players or in vitro entities?

(2010) Luciano Conti et al.   NATURE REVIEWS NEUROSCIENCE

Sustained effects of nonallele-specificHuntingtinsilencing

(2009) Valérie Drouet et al.   ANNALS OF NEUROLOGY

Allele-Selective Inhibition of Mutant Huntingtin by Peptide Nucleic Acid-Peptide Conjugates, Locked Nucleic Acid, and Small Interfering RNA

(2009) Jiaxin Hu et al.   Annals of the New York Academy of Sciences

Modified low molecular weight cyclic peptides as mimetics of BDNF with improved potency, proteolytic stability and transmembrane passage in vitro

(2009) Jordan M. Fletcher et al.   BIOORGANIC & MEDICINAL CHEMISTRY

Acetylation Targets Mutant Huntingtin to Autophagosomes for Degradation

(2009) Hyunkyung Jeong et al.   CELL

4p16.3 haplotype modifying age at onset of Huntington disease

(2009) A Nørremølle et al.   CLINICAL GENETICS

Inactivation of Drosophila Huntingtin affects long-term adult functioning and the pathogenesis of a Huntington's disease model

(2009) S. Zhang et al.   Disease Models & Mechanisms

Riluzole protects Huntington disease patients from brain glucose hypometabolism and grey matter volume loss and increases production of neurotrophins

(2009) Ferdinando Squitieri et al.   EUROPEAN JOURNAL OF NUCLEAR MEDICINE AND MOLECULAR IMAGING

A majority of Huntington's disease patients may be treatable by individualized allele-specific RNA interference

(2009) Maria Stella Lombardi et al.   EXPERIMENTAL NEUROLOGY

Functional imaging in Huntington's disease

(2009) Jane S. Paulsen   EXPERIMENTAL NEUROLOGY

Longitudinal diffusion tensor imaging in Huntington's Disease

(2009) Kurt E. Weaver et al.   EXPERIMENTAL NEUROLOGY

Developing intrabodies for the therapeutic suppression of neurodegenerative pathology

(2009) Anne Messer et al.   EXPERT OPINION ON BIOLOGICAL THERAPY

Selective neuronal requirement for huntingtin in the developing zebrafish

(2009) T. L. Henshall et al.   HUMAN MOLECULAR GENETICS

IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome

(2009) Leslie Michels Thompson et al.   JOURNAL OF CELL BIOLOGY

Synthesis and in Vitro Evaluation of Sulfonamide Isatin Michael Acceptors as Small Molecule Inhibitors of Caspase-6

(2009) Wenhua Chu et al.   JOURNAL OF MEDICINAL CHEMISTRY

The Bifunctional microRNA miR-9/miR-9* Regulates REST and CoREST and Is Downregulated in Huntington's Disease

(2009) A. N. Packer et al.   JOURNAL OF NEUROSCIENCE

Decreased BDNF Levels Are a Major Contributor to the Embryonic Phenotype of Huntingtin Knockdown Zebrafish

(2009) H. Diekmann 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

Neural grafts in Huntington's disease: viability after 10 years

(2009) Anne-Catherine Bachoud-Lévi   LANCET NEUROLOGY

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

Brain-derived neurotrophic factor in neurodegenerative diseases

(2009) Chiara Zuccato et al.   Nature Reviews Neurology

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

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

Glutamate toxicity in the striatum of the R6/2 Huntington's disease transgenic mice is age-dependent and correlates with decreased levels of glutamate transporters

(2009) Ana María Estrada-Sánchez et al.   NEUROBIOLOGY OF DISEASE

Systematic behavioral evaluation of Huntington's disease transgenic and knock-in mouse models

(2009) Liliana Menalled et al.   NEUROBIOLOGY OF DISEASE

In vivo evidence for the selective subcortical degeneration in Huntington's disease

(2009) Gwenaëlle Douaud et al.   NEUROIMAGE

Normal and mutant HTT interact to affect clinical severity and progression in Huntington disease

(2009) N. A. Aziz et al.   NEUROLOGY

Gene Dysregulation in Huntington’s Disease: REST, MicroRNAs and Beyond

(2009) Rory Johnson et al.   NEUROMOLECULAR MEDICINE

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

RNAi Screening in Drosophila Cells Identifies New Modifiers of Mutant Huntingtin Aggregation

(2009) Joanna Doumanis et al.   PLoS One

Formation of Polyglutamine Inclusions in a Wide Range of Non-CNS Tissues in the HdhQ150 Knock-In Mouse Model of Huntington's Disease

(2009) Hilary Moffitt et al.   PLoS One

Impairment of developmental stem cell-mediated striatal neurogenesis and pluripotency genes in a knock-in model of Huntington's disease

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

Neural transplants in patients with Huntington's disease undergo disease-like neuronal degeneration

(2009) F. Cicchetti 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

Rhes, a Striatal Specific Protein, Mediates Mutant-Huntingtin Cytotoxicity

(2009) S. Subramaniam et al.   SCIENCE

Secondary Structure of Huntingtin Amino-Terminal Region

(2009) Mee Whi Kim et al.   STRUCTURE

Genetic and pharmacological inhibition of calcineurin corrects the BDNF transport defect in Huntington's disease

(2009) Jose R Pineda et al.   Molecular Brain

Detection of Alpha-Rod Protein Repeats Using a Neural Network and Application to Huntingtin

(2009) Gareth A. Palidwor et al.   PLoS Computational Biology

Huntington's disease: from pathology and genetics to potential therapies

(2008) Sara Imarisio et al.   BIOCHEMICAL JOURNAL

Measurements of mitochondrial calcium in vivo

(2008) Tullio Pozzan et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

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

(2008) Valerio Leoni et al.   BRAIN

Hypothalamic involvement in Huntington's disease: an in vivo PET study

(2008) M. Politis et al.   BRAIN

Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity

(2008) H. D. Rosas et al.   BRAIN

Magnetic resonance imaging as an approach towards identifying neuropathological biomarkers for Huntington's disease

(2008) India Bohanna et al.   BRAIN RESEARCH REVIEWS

The R6 lines of transgenic mice: A model for screening new therapies for Huntington's disease

(2008) Joana M. Gil et al.   BRAIN RESEARCH REVIEWS

Disease-Specific Induced Pluripotent Stem Cells

(2008) In-Hyun Park et al.   CELL

Capture of Authentic Embryonic Stem Cells from Rat Blastocysts

(2008) Mia Buehr et al.   CELL

A Study of Chorea After Tetrabenazine Withdrawal in Patients With Huntington Disease

(2008) Samuel Frank et al.   CLINICAL NEUROPHARMACOLOGY

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

(2008) Emilie Colin et al.   EMBO JOURNAL

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

Huntington’s disease: degradation of mutant huntingtin by autophagy

(2008) Sovan Sarkar et al.   FEBS Journal

AAV–BDNF mediated attenuation of quinolinic acid-induced neuropathology and motor function impairment

(2008) A P Kells et al.   GENE THERAPY

Genomewide linkage scan reveals novel loci modifying age of onset of Huntington's disease in the Venezuelan HD kindreds

(2008) Javier Gayán et al.   GENETIC EPIDEMIOLOGY

Cortical dysfunction in patients with Huntington's disease during working memory performance

(2008) Robert C. Wolf et al.   HUMAN BRAIN MAPPING

Identification and Allele-Specific Silencing of the Mutant Huntingtin Allele in Huntington's Disease Patient-Derived Fibroblasts

(2008) P.H.J. van Bilsen et al.   HUMAN GENE THERAPY

Neocortical expression of mutant huntingtin is not required for alterations in striatal gene expression or motor dysfunction in a transgenic mouse

(2008) T. B. Brown et al.   HUMAN MOLECULAR GENETICS

Congenital hydrocephalus associated with abnormal subcommissural organ in mice lacking huntingtin in Wnt1 cell lineages

(2008) P. Dietrich et al.   HUMAN MOLECULAR GENETICS

Dopamine determines the vulnerability of striatal neurons to the N-terminal fragment of mutant huntingtin through the regulation of mitochondrial complex II

(2008) Alexandra Benchoua et al.   HUMAN MOLECULAR GENETICS

Huntingtin Regulates RE1-silencing Transcription Factor/Neuron-restrictive Silencer Factor (REST/NRSF) Nuclear Trafficking Indirectly through a Complex with REST/NRSF-interacting LIM Domain Protein (RILP) and Dynactin p150Glued

(2008) Masahito Shimojo   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

Insertional oncogenesis in 4 patients after retrovirus-mediated gene therapy of SCID-X1

(2008) Salima Hacein-Bey-Abina et al.   JOURNAL OF CLINICAL INVESTIGATION

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

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

ASK1 and MAP2K6 as modifiers of age at onset in Huntington’s disease

(2008) Larissa Arning et al.   JOURNAL OF MOLECULAR MEDICINE-JMM

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

(2008) Kusumika Gharami et al.   JOURNAL OF NEUROCHEMISTRY

Allele-specific silencing of mutant Huntington’s disease gene

(2008) Yu Zhang et al.   JOURNAL OF NEUROCHEMISTRY

Dysregulated Information Processing by Medium Spiny Neurons in Striatum of Freely Behaving Mouse Models of Huntington's Disease

(2008) Benjamin R. Miller et al.   JOURNAL OF NEUROPHYSIOLOGY

Enhanced Sensitivity of Striatal Neurons to Axonal Transport Defects Induced by Mutant Huntingtin

(2008) L.-S. Her et al.   JOURNAL OF NEUROSCIENCE

Huntingtin Modulates Transcription, Occupies Gene Promoters In Vivo, and Binds Directly to DNA in a Polyglutamine-Dependent Manner

(2008) C. L. Benn et al.   JOURNAL OF NEUROSCIENCE

Dopaminergic and Glutamatergic Signaling Crosstalk in Huntington's Disease Neurodegeneration: The Role of p25/Cyclin-Dependent Kinase 5

(2008) P. Paoletti et al.   JOURNAL OF NEUROSCIENCE

Polyglutamine-Modulated Striatal Calpain Activity in YAC Transgenic Huntington Disease Mouse Model: Impact on NMDA Receptor Function and Toxicity

(2008) C. M. Cowan et al.   JOURNAL OF NEUROSCIENCE

Altered Information Processing in the Prefrontal Cortex of Huntington's Disease Mouse Models

(2008) A. G. Walker et al.   JOURNAL OF NEUROSCIENCE

Differential Activities of the Ubiquitin-Proteasome System in Neurons versus Glia May Account for the Preferential Accumulation of Misfolded Proteins in Neurons

(2008) S. Tydlacka 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

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

Blood level of brain-derived neurotrophic factor mRNA is progressively reduced in rodent models of Huntington's disease: Restoration by the neuroprotective compound CEP-1347

(2008) Paola Conforti et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Phosphorylation of huntingtin reduces the accumulation of its nuclear fragments

(2008) Simon C. Warby et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Towards a transgenic model of Huntington’s disease in a non-human primate

(2008) Shang-Hsun Yang et al.   NATURE

Induced pluripotent stem cells from a spinal muscular atrophy patient

(2008) Allison D. Ebert et al.   NATURE

Novel targets for Huntington's disease in an mTOR-independent autophagy pathway

(2008) Andrea Williams et al.   Nature Chemical Biology

Dopamine neurons implanted into people with Parkinson's disease survive without pathology for 14 years

(2008) Ivar Mendez et al.   NATURE MEDICINE

Lewy bodies in grafted neurons in subjects with Parkinson's disease suggest host-to-graft disease propagation

(2008) Jia-Yi Li et al.   NATURE MEDICINE

Lewy body–like pathology in long-term embryonic nigral transplants in Parkinson's disease

(2008) Jeffrey H Kordower et al.   NATURE MEDICINE

Therapeutic application of histone deacetylase inhibitors for central nervous system disorders

(2008) Aleksey G. Kazantsev et al.   NATURE REVIEWS DRUG DISCOVERY

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

(2008) Wenzhen Duan et al.   NEUROBIOLOGY OF DISEASE

Protective effect of BDNF against beta-amyloid induced neurotoxicity in vitro and in vivo in rats

(2008) S. Arancibia et al.   NEUROBIOLOGY OF DISEASE

Extensive early motor and non-motor behavioral deficits are followed by striatal neuronal loss in knock-in Huntington's disease mice

(2008) M.A. Hickey et al.   NEUROSCIENCE

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

Artificial miRNAs mitigate shRNA-mediated toxicity in the brain: Implications for the therapeutic development of RNAi

(2008) J. L. McBride et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Huntington's disease protein contributes to RNA-mediated gene silencing through association with Argonaute and P bodies

(2008) J. N. Savas et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Striatal progenitors derived from human ES cells mature into DARPP32 neurons in vitro and in quinolinic acid-lesioned rats

(2008) L. Aubry et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Induced Pluripotent Stem Cells Generated from Patients with ALS Can Be Differentiated into Motor Neurons

(2008) J. T. Dimos et al.   SCIENCE

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

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

BDNF mRNA splice variants display activity-dependent targeting to distinct hippocampal laminae

(2007) C. Chiaruttini et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Phylogenetic Comparison of Huntingtin Homologues Reveals the Appearance of a Primitive polyQ in Sea Urchin

(2007) M. Tartari et al.   MOLECULAR BIOLOGY AND EVOLUTION

A microRNA-based gene dysregulation pathway in Huntington's disease

(2007) Rory Johnson et al.   NEUROBIOLOGY OF DISEASE

Unexpected off-targeting effects of anti-huntingtin ribozymes and siRNA in vivo

(2007) Eileen M. Denovan-Wright et al.   NEUROBIOLOGY OF DISEASE