A YAC Mouse Model for Huntington’s Disease with Full-Length Mutant Huntingtin, Cytoplasmic Toxicity, and Selective Striatal Neurodegeneration

John Hodgson, N. Agopyan, Claire‐Anne Gutekunst, +16

Neuron

Neurodegenerative DiseasesFull-length Mutant HuntingtinSelective Striatal NeurodegenerationGeneticsYac Mouse Model +6

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

Victor Ona, Mingwei Li, Jean Paul Vonsattel, +12

Nature

Knockout MouseCell DeathDegenerative DiseaseNeurodegenerationSystems Biology +4

A huntingtin-associated protein enriched in brain with implications for pathology

Xiao‐Jiang Li, Shihua Li, Alan H. Sharp, +6

Nature

Huntingtin-associated ProteinMedicineDegenerative PathologyNeurochemical BiomarkersNeuroscience +6

Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity

Ji‐Yeon Shin, Zhihui Fang, Zhao-Xue Yu, +3

The Journal of Cell Biology

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

Adam L. Orr, Shihua Li, Chuan-En Wang, +7

Journal of Neuroscience

CRISPR/Cas9-mediated gene editing ameliorates neurotoxicity in mouse model of Huntington’s disease

Su Yang, Renbao Chang, Huiming Yang, +8

Journal of Clinical Investigation

GeneticsPolyglutamine RepeatMolecular BiologyHuntingtin GeneExperimental Neuropathology +15

Adenosine A2A receptor mediates microglial process retraction

Anna G. Orr, Adam L. Orr, Xiao‐Jiang Li, +2

Nature Neuroscience

MedicineBrain-immune InteractionNeuroscienceNeuroimmunologyCellular Physiology +2

Interaction of Huntington Disease Protein with Transcriptional Activator Sp1

Shihua Li, Ann‐Lii Cheng, Hui Zhou, +4

Molecular and Cellular Biology

Transcriptional RegulationNeurodegenerative DiseasesNatural SciencesGeneticsMolecular Biology +11

A Huntingtin Knockin Pig Model Recapitulates Features of Selective Neurodegeneration in Huntington’s Disease

Sen Yan, Zhuchi Tu, Zhaoming Liu, +18

Cell

Knockout MouseNeurodegenerative DiseasesNeurobiology Of DiseaseHuntington ’Genetics +11

Amino-terminal fragments of mutant huntingtin show selective accumulation in striatal neurons and synaptic toxicity

Li He, Shihua Li, Heather Johnston, +2

Nature Genetics

Molecular NeuroscienceDevelopmental BiologySynaptic SignalingSynaptic ToxicityNeurotransmitter +9