Huntington’s disease: novel therapeutic perspectives hanging in the balance

STEP inhibition reverses behavioral, electrophysiologic, and synaptic abnormalities in Fmr1 KO mice

(2018) Manavi Chatterjee et al.   NEUROPHARMACOLOGY

PDC-SGB: Prediction of effective drug combinations using a stochastic gradient boosting algorithm

(2017) Qian Xu et al.   JOURNAL OF THEORETICAL BIOLOGY

Therapies targeting DNA and RNA in Huntington's disease

(2017) Edward J Wild et al.   LANCET NEUROLOGY

The CREST-E study of creatine for Huntington disease

(2017) Steven M. Hersch et al.   NEUROLOGY

Synaptopathic mechanisms of neurodegeneration and dementia: Insights from Huntington’s disease

(2017) Shiraz Tyebji et al.   PROGRESS IN NEUROBIOLOGY

Unravelling and Exploiting Astrocyte Dysfunction in Huntington’s Disease

(2017) Baljit S. Khakh et al.   TRENDS IN NEUROSCIENCES

A selective inhibitor of histone deacetylase 3 prevents cognitive deficits and suppresses striatal CAG repeat expansions in Huntington’s disease mice

(2017) Nuria Suelves et al.   Scientific Reports

What is polypharmacy? A systematic review of definitions

(2017) Nashwa Masnoon et al.   BMC Geriatrics

ARL3 regulates trafficking of prenylated phototransduction proteins to the rod outer segment

(2016) Zachary C. Wright et al.   HUMAN MOLECULAR GENETICS

Mitochondria-targeted molecules MitoQ and SS31 reduce mutant huntingtin-induced mitochondrial toxicity and synaptic damage in Huntington's disease

(2016) Xiangling Yin et al.   HUMAN MOLECULAR GENETICS

Phosphodiesterase Inhibition and Regulation of Dopaminergic Frontal and Striatal Functioning: Clinical Implications

(2016) Pim R. A. Heckman et al.   INTERNATIONAL JOURNAL OF NEUROPSYCHOPHARMACOLOGY

Prostaglandin E2 EP2 activation reduces memory decline in R6/1 mouse model of Huntington's disease by the induction of BDNF-dependent synaptic plasticity

(2016) Marta Anglada-Huguet et al.   NEUROBIOLOGY OF DISEASE

Mitochondrial dynamics and quality control in Huntington's disease

(2016) Pedro Guedes-Dias et al.   NEUROBIOLOGY OF DISEASE

GluN3A promotes NMDA spiking by enhancing synaptic transmission in Huntington's disease models

(2016) Kashif Mahfooz et al.   NEUROBIOLOGY OF DISEASE

Specific promoter deacetylation of histone H3 is conserved across mouse models of Huntington's disease in the absence of bulk changes

(2016) Deisy Guiretti et al.   NEUROBIOLOGY OF DISEASE

Phosphodiesterase 10A Inhibition Improves Cortico-Basal Ganglia Function in Huntington’s Disease Models

(2016) Vahri Beaumont et al.   NEURON

The p75 neurotrophin receptor augments survival signaling in the striatum of pre-symptomatic Q175WT/HD mice

(2016) A.B. Wehner et al.   NEUROSCIENCE

GSK-3β-induced Tau pathology drives hippocampal neuronal cell death in Huntington’s disease: involvement of astrocyte–neuron interactions

(2016) F L'Episcopo et al.   Cell Death & Disease

Real-time imaging of glutamate clearance reveals normal striatal uptake in Huntington disease mouse models

(2016) Matthew P. Parsons et al.   Nature Communications

Human glia can both induce and rescue aspects of disease phenotype in Huntington disease

(2016) Abdellatif Benraiss et al.   Nature Communications

The Effects of Pharmacological Inhibition of Histone Deacetylase 3 (HDAC3) in Huntington’s Disease Mice

(2016) Haiqun Jia et al.   PLoS One

Cdk5-mediated mitochondrial fission: A key player in dopaminergic toxicity in Huntington's disease

(2015) Marta Cherubini et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

HDAC6 inhibition induces mitochondrial fusion, autophagic flux and reduces diffuse mutant huntingtin in striatal neurons

(2015) Pedro Guedes-Dias et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Altered PDE10A expression detectable early before symptomatic onset in Huntington’s disease

(2015) Flavia Niccolini et al.   BRAIN

Inhibition of the tyrosine phosphatase STEP61 restores BDNF expression and reverses motor and cognitive deficits in phencyclidine-treated mice

(2015) Jian Xu et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Appropriate Polypharmacy and Medicine Safety: When Many is not Too Many

(2015) Cathal A. Cadogan et al.   DRUG SAFETY

Advancing pharmacotherapy for treating Huntington’s disease: a review of the existing literature

(2015) Sarah L Mason et al.   EXPERT OPINION ON PHARMACOTHERAPY

Decreased glycogen synthase kinase-3 levels and activity contribute to Huntington's disease

(2015) Marta Fernández-Nogales et al.   HUMAN MOLECULAR GENETICS

Mitochondrial division inhibitor 1 protects against mutant huntingtin-induced abnormal mitochondrial dynamics and neuronal damage in Huntington's disease

(2015) Maria Manczak et al.   HUMAN MOLECULAR GENETICS

Fingolimod (FTY720) enhances hippocampal synaptic plasticity and memory in Huntington's disease by preventing p75NTRup-regulation and astrocyte-mediated inflammation

(2015) Andrés Miguez et al.   HUMAN MOLECULAR GENETICS

BDNF Induces Striatal-Enriched Protein Tyrosine Phosphatase 61 Degradation Through the Proteasome

(2015) Ana Saavedra et al.   MOLECULAR NEUROBIOLOGY

Exercise training normalizes mitochondrial respiratory capacity within the striatum of the R6/1 model of Huntington’s disease

(2015) E.A.F. Herbst et al.   NEUROSCIENCE

Neuroprotective effect of hemeoxygenase-1/glycogen synthase kinase-3β modulators in 3-nitropropionic acid-induced neurotoxicity in rats

(2015) A. Khan et al.   NEUROSCIENCE

Memantine exerts functional recovery by improving BDNF and GDNF expression in 3-nitropropionic acid intoxicated mice

(2015) Vijayan Ranju et al.   NEUROSCIENCE LETTERS

Transcriptional dysregulation in Huntington’s disease: The role of histone deacetylases

(2015) Sorabh Sharma et al.   PHARMACOLOGICAL RESEARCH

A Network-Based Target Overlap Score for Characterizing Drug Combinations: High Correlation with Cancer Clinical Trial Results

(2015) Balázs Ligeti et al.   PLoS One

BDNF Reduces Toxic Extrasynaptic NMDA Receptor Signaling via Synaptic NMDA Receptors and Nuclear-Calcium-Induced Transcription of inhba/Activin A

(2015) David Lau et al.   Cell Reports

N-Acetylcysteine improves mitochondrial function and ameliorates behavioral deficits in the R6/1 mouse model of Huntington's disease

(2015) D J Wright et al.   Translational Psychiatry

Localized changes to glycogen synthase kinase-3 and collapsin response mediator protein-2 in the Huntington's disease affected brain

(2014) N. K. H. Lim et al.   HUMAN MOLECULAR GENETICS

Neurotrophin receptor p75NTR mediates Huntington’s disease–associated synaptic and memory dysfunction

(2014) Verónica Brito et al.   JOURNAL OF CLINICAL INVESTIGATION

Antidepressant-like effects of the phosphodiesterase-4 inhibitor etazolate and phosphodiesterase-5 inhibitor sildenafil via cyclic AMP or cyclic GMP signaling in mice

(2014) Chuang Wang et al.   METABOLIC BRAIN DISEASE

Transcription, Epigenetics and Ameliorative Strategies in Huntington’s Disease: a Genome-Wide Perspective

(2014) Luis M. Valor   MOLECULAR NEUROBIOLOGY

Activation of IGF-1 and Insulin Signaling Pathways Ameliorate Mitochondrial Function and Energy Metabolism in Huntington’s Disease Human Lymphoblasts

(2014) Luana Naia et al.   MOLECULAR NEUROBIOLOGY

Cystathionine γ-lyase deficiency mediates neurodegeneration in Huntington’s disease

(2014) Bindu D. Paul et al.   NATURE

Extrasynaptic NMDA Receptor Involvement in Central Nervous System Disorders

(2014) Matthew P. Parsons et al.   NEURON

Impaired TrkB Receptor Signaling Underlies Corticostriatal Dysfunction in Huntington’s Disease

(2014) Joshua L. Plotkin et al.   NEURON

AAV-Dominant Negative Tumor Necrosis Factor (DN-TNF) Gene Transfer to the Striatum Does Not Rescue Medium Spiny Neurons in the YAC128 Mouse Model of Huntington's Disease

(2014) Laura Taylor Alto et al.   PLoS One

The NMDA receptor complex: a multifunctional machine at the glutamatergic synapse

(2014) Xuelai Fan et al.   Frontiers in Cellular Neuroscience

BDNF signaling and survival of striatal neurons

(2014) Maryna Baydyuk et al.   Frontiers in Cellular Neuroscience

Inhibitor of the Tyrosine Phosphatase STEP Reverses Cognitive Deficits in a Mouse Model of Alzheimer's Disease

(2014) Jian Xu et al.   PLOS BIOLOGY

Reversal of Cellular Phenotypes in Neural Cells Derived from Huntington’s Disease Monkey-Induced Pluripotent Stem Cells

(2014) Richard L. Carter et al.   Stem Cell Reports

Lysine deacetylases and mitochondrial dynamics in neurodegeneration

(2013) Pedro Guedes-Dias et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Neuroprotective role of PDE4 and PDE5 inhibitors in 3-nitropropionic acid induced behavioral and biochemical toxicities in rats

(2013) Tarun Thakur et al.   EUROPEAN JOURNAL OF PHARMACOLOGY

PDE10 inhibition increases GluA1 and CREB phosphorylation and improves spatial and recognition memories in a Huntington's disease mouse model

(2013) Albert Giralt et al.   HIPPOCAMPUS

FTY720 (fingolimod) is a neuroprotective and disease-modifying agent in cellular and mouse models of Huntington disease

(2013) Alba Di Pardo et al.   HUMAN MOLECULAR GENETICS

Small-molecule TrkB receptor agonists improve motor function and extend survival in a mouse model of Huntington's disease

(2013) M. Jiang et al.   HUMAN MOLECULAR GENETICS

Inhibition of mitochondrial fragmentation diminishes Huntington’s disease–associated neurodegeneration

(2013) Xing Guo et al.   JOURNAL OF CLINICAL INVESTIGATION

Genomic Landscape of Transcriptional and Epigenetic Dysregulation in Early Onset Polyglutamine Disease

(2013) L. M. Valor et al.   JOURNAL OF NEUROSCIENCE

A Small Molecule TrkB Ligand Reduces Motor Impairment and Neuropathology in R6/2 and BACHD Mouse Models of Huntington's Disease

(2013) D. A. Simmons et al.   JOURNAL OF NEUROSCIENCE

Suppressing aberrant GluN3A expression rescues synaptic and behavioral impairments in Huntington's disease models

(2013) Sonia Marco et al.   NATURE MEDICINE

Nuclear calcium signalling in the regulation of brain function

(2013) Hilmar Bading   NATURE REVIEWS NEUROSCIENCE

NMDA receptor subunit diversity: impact on receptor properties, synaptic plasticity and disease

(2013) Pierre Paoletti et al.   NATURE REVIEWS NEUROSCIENCE

Chronic blockade of extrasynaptic NMDA receptors ameliorates synaptic dysfunction and pro-death signaling in Huntington disease transgenic mice

(2013) Alejandro Dau et al.   NEUROBIOLOGY OF DISEASE

What's wrong with epigenetics in Huntington's disease?

(2013) Luis M. Valor et al.   NEUROPHARMACOLOGY

Regulation of Hippocampal cGMP Levels as a Candidate to Treat Cognitive Deficits in Huntington’s Disease

(2013) Ana Saavedra et al.   PLoS One

Huntington's disease: underlying molecular mechanisms and emerging concepts

(2013) John Labbadia et al.   TRENDS IN BIOCHEMICAL SCIENCES

Imbalance of p75NTR/TrkB protein expression in Huntington’s disease: implication for neuroprotective therapies

(2013) V Brito et al.   Cell Death & Disease

HDAC4 Reduction: A Novel Therapeutic Strategy to Target Cytoplasmic Huntingtin and Ameliorate Neurodegeneration

(2013) Michal Mielcarek et al.   PLOS BIOLOGY

The drug cocktail network

(2012) Ke-Jia Xu et al.   BMC Systems Biology

Activation of extrasynaptic NMDA receptors induces LTD in rat hippocampal CA1 neurons

(2012) Dan-dan Liu et al.   BRAIN RESEARCH BULLETIN

Discovery of fingolimod, the sphingosine 1-phosphate receptor modulator and its application for the therapy of multiple sclerosis

(2012) Kenji Chiba et al.   Future Medicinal Chemistry

Calpain and STriatal-Enriched protein tyrosine Phosphatase (STEP) activation contribute to extrasynaptic NMDA receptor localization in a Huntington's disease mouse model

(2012) C. M. Gladding et al.   HUMAN MOLECULAR GENETICS

PGC-1  Negatively Regulates Extrasynaptic NMDAR Activity and Excitotoxicity

(2012) C. Puddifoot et al.   JOURNAL OF NEUROSCIENCE

Mitochondria targeted therapeutic approaches in Parkinson's and Huntington's diseases

(2012) Rajnish K. Chaturvedi et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Mitigation of augmented extrasynaptic NMDAR signaling and apoptosis in cortico-striatal co-cultures from Huntington's disease mice

(2012) Austen J. Milnerwood et al.   NEUROBIOLOGY OF DISEASE

N-Acetylcysteine Reverses Mitochondrial Dysfunctions and Behavioral Abnormalities in 3-Nitropropionic Acid-Induced Huntington’s Disease

(2012) Rajat Sandhir et al.   Neurodegenerative Diseases

Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington's disease

(2011) P. Hemachandra Reddy et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Mutant huntingtin's interaction with mitochondrial protein Drp1 impairs mitochondrial biogenesis and causes defective axonal transport and synaptic degeneration in Huntington's disease

(2011) U. P. Shirendeb et al.   HUMAN MOLECULAR GENETICS

Mechanisms underlying NMDA receptor synaptic/extrasynaptic distribution and function

(2011) Clare M. Gladding et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

(2011) Wenjun Song et al.   NATURE MEDICINE

Combined Treatment with the Mood Stabilizers Lithium and Valproate Produces Multiple Beneficial Effects in Transgenic Mouse Models of Huntington's Disease

(2011) Chi-Tso Chiu et al.   NEUROPSYCHOPHARMACOLOGY

Antidepressant-like effect of sildenafil through oxytocin-dependent cyclic AMP response element-binding protein phosphorylation

(2011) H. Matsushita et al.   NEUROSCIENCE

In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects

(2010) M. Faideau et al.   HUMAN MOLECULAR GENETICS

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

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

Memantine Preferentially Blocks Extrasynaptic over Synaptic NMDA Receptor Currents in Hippocampal Autapses

(2010) P. Xia et al.   JOURNAL OF NEUROSCIENCE

Global Deprivation of Brain-Derived Neurotrophic Factor in the CNS Reveals an Area-Specific Requirement for Dendritic Growth

(2010) S. Rauskolb et al.   JOURNAL OF NEUROSCIENCE

Chronic Suppression of Phosphodiesterase 10A Alters Striatal Expression of Genes Responsible for Neurotransmitter Synthesis, Neurotransmission, and Signaling Pathways Implicated in Huntington's Disease

(2010) R. J. Kleiman et al.   JOURNAL OF PHARMACOLOGY AND EXPERIMENTAL THERAPEUTICS

Low anaerobic threshold and increased skeletal muscle lactate production in subjects with Huntington's disease

(2010) Andrea Ciammola et al.   MOVEMENT DISORDERS

Synaptic versus extrasynaptic NMDA receptor signalling: implications for neurodegenerative disorders

(2010) Giles E. Hardingham et al.   NATURE REVIEWS NEUROSCIENCE

Palmitoylation and function of glial glutamate transporter-1 is reduced in the YAC128 mouse model of Huntington disease

(2010) Kun Huang et al.   NEUROBIOLOGY OF DISEASE

Sildenafil protects against 3-nitropropionic acid neurotoxicity through the modulation of calpain, CREB, and BDNF

(2010) Elena Puerta et al.   NEUROBIOLOGY OF DISEASE

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

Molecular Mechanisms and Potential Therapeutical Targets in Huntington's Disease

(2010) Chiara Zuccato et al.   PHYSIOLOGICAL REVIEWS

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

SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis

(2010) Ruth Luthi-Carter et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A selective TrkB agonist with potent neurotrophic activities by 7,8-dihydroxyflavone

(2010) S.-W. Jang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Influence of the NR3A subunit on NMDA receptor functions

(2010) Maile A. Henson et al.   PROGRESS IN NEUROBIOLOGY

Earliest functional declines in Huntington disease

(2010) Leigh J. Beglinger et al.   PSYCHIATRY RESEARCH

Early synaptic pathophysiology in neurodegeneration: insights from Huntington's disease

(2010) Austen J. Milnerwood et al.   TRENDS IN NEUROSCIENCES

Impaired mitochondrial trafficking in Huntington's disease

(2009) Xiao-Jiang Li et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Acetylation Targets Mutant Huntingtin to Autophagosomes for Degradation

(2009) Hyunkyung Jeong et al.   CELL

PH domain leucine-rich repeat protein phosphatase 1 contributes to maintain the activation of the PI3K/Akt pro-survival pathway in Huntington's disease striatum

(2009) A Saavedra et al.   CELL DEATH AND DIFFERENTIATION

Extrasynaptic NMDA Receptors Couple Preferentially to Excitotoxicity via Calpain-Mediated Cleavage of STEP

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

Mutant huntingtin and glycogen synthase kinase 3-β accumulate in neuronal lipid rafts of a presymptomatic knock-in mouse model of Huntington's disease

(2009) Antonio Valencia et al.   JOURNAL OF NEUROSCIENCE RESEARCH

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

Phosphodiesterase 10 inhibition reduces striatal excitotoxicity in the quinolinic acid model of Huntington's disease

(2009) Carmela Giampà et al.   NEUROBIOLOGY OF DISEASE

CREB is a key regulator of striatal vulnerability in chemical and genetic models of Huntington's disease

(2009) Yun-Sik Choi et al.   NEUROBIOLOGY OF DISEASE

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

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

(2009) S. Subramaniam et al.   SCIENCE

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

Impaired ubiquitin–proteasome system activity in the synapses of Huntington's disease mice

(2008) Jianjun 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

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

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

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

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

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