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 …

Mechanisms of neurodegeneration in Huntington's disease

JM Gil, AC Rego - European Journal of Neuroscience, 2008 - Wiley Online Library
Huntington's disease (HD) is caused by an expansion of cytosine–adenine–guanine (CAG)
repeats in the huntingtin gene, which leads to neuronal loss in the striatum and cortex and to …

[HTML][HTML] Microglia and complement mediate early corticostriatal synapse loss and cognitive dysfunction in Huntington's disease

DK Wilton, K Mastro, MD Heller, FW Gergits… - Nature medicine, 2023 - nature.com
Huntington's disease (HD) is a devastating monogenic neurodegenerative disease
characterized by early, selective pathology in the basal ganglia despite the ubiquitous …

Early increase in extrasynaptic NMDA receptor signaling and expression contributes to phenotype onset in Huntington's disease mice

AJ Milnerwood, CM Gladding, MA Pouladi… - Neuron, 2010 - cell.com
N-methyl-D-aspartate receptor (NMDAR) excitotoxicity is implicated in the pathogenesis of
Huntington's disease (HD), a late-onset neurodegenerative disorder. However, NMDARs …

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

AJ Milnerwood, LA Raymond - Trends in neurosciences, 2010 - cell.com
Investigations of synaptic transmission and plasticity in mouse models of Huntington's
disease (HD) demonstrate neuronal dysfunction long before the onset of classical disease …

Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington's disease

T Heikkinen, K Lehtimäki, N Vartiainen, J Puoliväli… - PloS one, 2012 - journals.plos.org
Huntington's disease (HD) is an autosomal neurodegenerative disorder, characterized by
severe behavioral, cognitive, and motor deficits. Since the discovery of the huntingtin gene …

[HTML][HTML] The contribution of glial cells to Huntington's disease pathogenesis

DK Wilton, B Stevens - Neurobiology of disease, 2020 - Elsevier
Glial cells play critical roles in the normal development and function of neural circuits, but in
many neurodegenerative diseases, they become dysregulated and may contribute to the …

Mechanisms underlying NMDA receptor synaptic/extrasynaptic distribution and function

CM Gladding, LA Raymond - Molecular and Cellular Neuroscience, 2011 - Elsevier
Research over the last few decades has shaped our understanding of the crucial
involvement of the N-methyl-d-aspartate receptor (NMDAR) in mediating excitatory synaptic …

Altered m6A RNA methylation contributes to hippocampal memory deficits in Huntington's disease mice

A Pupak, A Singh, A Sancho-Balsells… - Cellular and Molecular …, 2022 - Springer
Abstract N6-methyladenosine (m6A) regulates many aspects of RNA metabolism and is
involved in learning and memory processes. Yet, the impact of a dysregulation of post …

The sigma-1 receptor mediates the beneficial effects of pridopidine in a mouse model of Huntington disease

D Ryskamp, J Wu, M Geva, R Kusko, I Grossman… - Neurobiology of …, 2017 - Elsevier
The tri-nucleotide repeat expansion underlying Huntington disease (HD) results in
corticostriatal synaptic dysfunction and subsequent neurodegeneration of striatal medium …