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

LA Raymond, VM André, C Cepeda, CM Gladding… - Neuroscience, 2011 - Elsevier
Huntington's disease (HD) is a progressive, fatal neurological condition caused by an
expansion of CAG (glutamine) repeats in the coding region of the Huntington gene. To date …

Disrupted striatal neuron inputs and outputs in Huntington's disease

A Reiner, YP Deng - CNS neuroscience & therapeutics, 2018 - Wiley Online Library
Huntington's disease (HD) is a hereditary progressive neurodegenerative disorder caused
by a CAG repeat expansion in the gene coding for the protein huntingtin, resulting in a …

[HTML][HTML] Cell-autonomous and non-cell-autonomous pathogenic mechanisms in Huntington's disease: insights from in vitro and in vivo models

J Creus-Muncunill, ME Ehrlich - Neurotherapeutics, 2019 - Elsevier
Huntington's disease (HD) is an autosomal dominant disorder caused by an expansion in
the trinucleotide CAG repeat in exon-1 in the huntingtin gene, located on chromosome 4 …

Synaptic dysfunction in Huntington's disease: lessons from genetic animal models

C Cepeda, MS Levine - The Neuroscientist, 2022 - journals.sagepub.com
The understanding of the functional and structural changes occurring in the cerebral cortex
and basal ganglia in Huntington's disease (HD) has benefited considerably from the …

Differential susceptibility to excitotoxic stress in YAC128 mouse models of Huntington disease between initiation and progression of disease

RK Graham, MA Pouladi, P Joshi, G Lu… - Journal of …, 2009 - Soc Neuroscience
Huntington disease (HD) is a neurodegenerative disorder caused by an expanded CAG
tract in the HD gene. Polyglutamine expansion of huntingtin (htt) results in early, progressive …

Corticostriatal synaptic function in mouse models of Huntington's disease: early effects of huntingtin repeat length and protein load

AJ Milnerwood, LA Raymond - The Journal of physiology, 2007 - Wiley Online Library
Huntington's disease (HD) is an autosomal dominant, late onset, neurodegenerative
disease characterized by motor deficits and dementia that is caused by expansion of a CAG …

Changes in cortical and striatal neurons predict behavioral and electrophysiological abnormalities in a transgenic murine model of Huntington's disease

GA Laforet, E Sapp, K Chase, C McIntyre… - Journal of …, 2001 - Soc Neuroscience
Neurons in Huntington's disease exhibit selective morphological and subcellular alterations
in the striatum and cortex. The link between these neuronal changes and behavioral …

Replicating Huntington's disease phenotype in experimental animals

E Brouillet, F Conde, MF Beal, P Hantraye - Progress in neurobiology, 1999 - Elsevier
Huntington's disease (HD) is an inherited, autosomal dominant, neurodegenerative disorder
characterized by involuntary choreiform movements, cognitive decline and a progressive …

Striatal vulnerability in Huntington's disease: neuroprotection versus neurotoxicity

R Morigaki, S Goto - Brain sciences, 2017 - mdpi.com
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by
the expansion of a CAG trinucleotide repeat encoding an abnormally long polyglutamine …

Genetic mouse models of Huntington's disease: focus on electrophysiological mechanisms

C Cepeda, DM Cummings, VM André… - ASN …, 2010 - journals.sagepub.com
The discovery of the HD (Huntington's disease) gene in 1993 led to the creation of genetic
mouse models of the disease and opened the doors for mechanistic studies. In particular …