Huntington’s Disease and the Striatal Medium Spiny Neuron: Cell-Autonomous and Non-Cell-Autonomous Mechanisms of Disease

Huntington's disease, calcium, and mitochondria

(2011) Marta Giacomello et al.   BIOFACTORS

Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation

(2011) L. S. Havel et al.   HUMAN MOLECULAR GENETICS

Differential effects of the Huntington's disease CAG mutation in striatum and cerebellum are quantitative not qualitative

(2011) Elisa Fossale et al.   HUMAN MOLECULAR GENETICS

Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage

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

Peroxisome-Proliferator-Activated Receptor Gamma Coactivator 1   Contributes to Dysmyelination in Experimental Models of Huntington's Disease

(2011) Z. Xiang et al.   JOURNAL OF NEUROSCIENCE

Mechanisms underlying NMDA receptor synaptic/extrasynaptic distribution and function

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

Huntington's Disease: Can Mice Lead the Way to Treatment?

(2011) Zachary R. Crook et al.   NEURON

Pathophysiology of Huntington's disease: time-dependent alterations in synaptic and receptor function

(2011) L.A. Raymond et al.   NEUROSCIENCE

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

Genetic Mouse Models of Huntington's Disease: Focus on Electrophysiological Mechanisms

(2010) Carlos Cepeda et al.   ASN Neuro

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

Microglial activation in regions related to cognitive function predicts disease onset in Huntington's disease: A multimodal imaging study

(2010) Marios Politis et al.   HUMAN BRAIN MAPPING

A point mutation in the dynein heavy chain gene leads to striatal atrophy and compromises neurite outgrowth of striatal neurons

(2010) Kerstin E. Braunstein et al.   HUMAN MOLECULAR GENETICS

In vivo cell-autonomous transcriptional abnormalities revealed in mice expressing mutant huntingtin in striatal but not cortical neurons

(2010) Elizabeth A. Thomas et al.   HUMAN MOLECULAR GENETICS

Mutant Huntingtin in Glial Cells Exacerbates Neurological Symptoms of Huntington Disease Mice

(2010) Jennifer Bradford et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Decreased Lin7b Expression in Layer 5 Pyramidal Neurons May Contribute to Impaired Corticostriatal Connectivity in Huntington Disease

(2010) Birgit Zucker 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

Parvalbumin Deficiency and GABAergic Dysfunction in Mice Lacking PGC-1 

(2010) E. K. Lucas et al.   JOURNAL OF NEUROSCIENCE

Phosphorylation of Huntingtin at Ser421 in YAC128 Neurons Is Associated with Protection of YAC128 Neurons from NMDA-Mediated Excitotoxicity and Is Modulated by PP1 and PP2A

(2010) M. Metzler 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

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

Neurotransmitter roles in synaptic modulation, plasticity and learning in the dorsal striatum

(2010) David M. Lovinger   NEUROPHARMACOLOGY

Huntington’s Disease is a disorder of the corpus striatum: Focus on Rhes (Ras homologue enriched in the striatum)

(2010) Srinivasa Subramaniam et al.   NEUROPHARMACOLOGY

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

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

Sustained effects of nonallele-specificHuntingtinsilencing

(2009) Valérie Drouet et al.   ANNALS OF NEUROLOGY

Coupling of the NMDA receptor to neuroprotective and neurodestructive events

(2009) Giles E. Hardingham   BIOCHEMICAL SOCIETY TRANSACTIONS

Mitochondrial calcium function and dysfunction in the central nervous system

(2009) David G. Nicholls   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Microglial CB2 cannabinoid receptors are neuroprotective in Huntington's disease excitotoxicity

(2009) Javier Palazuelos et al.   BRAIN

The Nrf2–ARE cytoprotective pathway in astrocytes

(2009) Marcelo R. Vargas et al.   EXPERT REVIEWS IN MOLECULAR MEDICINE

Non–cell autonomous toxicity in neurodegenerative disorders: ALS and beyond

(2009) Hristelina Ilieva et al.   JOURNAL OF CELL BIOLOGY

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

In Vivo Evidence for NMDA Receptor-Mediated Excitotoxicity in a Murine Genetic Model of Huntington Disease

(2009) M. Y. Heng et al.   JOURNAL OF NEUROSCIENCE

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

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

Reduced calcineurin protein levels and activity in exon-1 mouse models of Huntington's disease: Role in excitotoxicity

(2009) Xavier Xifró et al.   NEUROBIOLOGY OF DISEASE

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

Dopamine D2 Receptor Stimulation Potentiates PolyQ-Huntingtin-Induced Mouse Striatal Neuron Dysfunctions via Rho/ROCK-II Activation

(2009) Carole Deyts et al.   PLoS One

Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms

(2009) Jennifer Bradford 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

Implication of the JNK pathway in a rat model of Huntington's disease

(2008) V. Perrin et al.   EXPERIMENTAL NEUROLOGY

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

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

Dopamine D1 Receptor-induced Signaling through TrkB Receptors in Striatal Neurons

(2008) Yuriko Iwakura et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

(2008) Kusumika Gharami et al.   JOURNAL OF NEUROCHEMISTRY

Dysregulation of Gene Expression in Primary Neuron Models of Huntington's Disease Shows That Polyglutamine-Related Effects on the Striatal Transcriptome May Not Be Dependent on Brain Circuitry

(2008) H. Runne 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

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

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

Disruption of striatal glutamatergic transmission induced by mutant huntingtin involves remodeling of both postsynaptic density and NMDA receptor signaling

(2007) Jesús F. Torres-Peraza et al.   NEUROBIOLOGY OF DISEASE

Role of phosphatidylinositide 3-kinase in brain-derived neurotrophic factor-induced DARPP-32 expression in medium size spiny neurons in vitro

(2003) Antonella Stroppolo et al.   JOURNAL OF NEUROCHEMISTRY