[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 …

A critical review of white matter changes in Huntington's disease

C Casella, I Lipp, A Rosser, DK Jones… - Movement …, 2020 - Wiley Online Library
Huntington's disease is a genetic neurodegenerative disorder. White matter alterations have
recently been identified as a relevant pathophysiological feature of Huntington's disease, but …

[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 …

A TCF7L2-responsive suppression of both homeostatic and compensatory remyelination in Huntington disease mice

A Benraiss, JN Mariani, A Tate, PM Madsen, KM Clark… - Cell Reports, 2022 - cell.com
Huntington's disease (HD) is characterized by defective oligodendroglial differentiation and
white matter disease. Here, we investigate the role of oligodendrocyte progenitor cell (OPC) …

Structural signature of SCA3: from presymptomatic to late disease stages

TJR Rezende, JLR de Paiva, ARM Martinez… - Annals of …, 2018 - Wiley Online Library
Objective Machado–Joseph disease (SCA3/MJD) is the most frequent spinocerebellar
ataxia worldwide and characterized by brainstem, basal ganglia, and cerebellar damage …

[HTML][HTML] Brain regions showing white matter loss in Huntington's disease are enriched for synaptic and metabolic genes

P McColgan, S Gregory, KK Seunarine, A Razi… - Biological …, 2018 - Elsevier
Background The earliest white matter changes in Huntington's disease are seen before
disease onset in the premanifest stage around the striatum, within the corpus callosum, and …

Network spread determines severity of degeneration and disconnection in Huntington's disease

GR Poudel, IH Harding, GF Egan… - Human brain …, 2019 - Wiley Online Library
Trans‐neuronal propagation of mutant huntingtin protein contributes to the organised
spread of cortico‐striatal degeneration and disconnection in Huntington's disease (HD). We …

Vestibular function and cortical and sub-cortical alterations in an aging population

A Jacob, DJ Tward, S Resnick, PF Smith, C Lopez… - Heliyon, 2020 - cell.com
While it is well known that the vestibular system is responsible for maintaining balance,
posture and coordination, there is increasing evidence that it also plays an important role in …

The long and the short of Huntington's disease: how the sphingolipid profile is shifted in the caudate of advanced clinical cases

GR Phillips, JT Saville, SE Hancock… - Brain …, 2022 - academic.oup.com
Huntington's disease is a devastating neurodegenerative disorder that onsets in late
adulthood as progressive and terminal cognitive, psychiatric and motor deficits. The disease …

[HTML][HTML] Topological length of white matter connections predicts their rate of atrophy in premanifest Huntington's disease

P McColgan, KK Seunarine, S Gregory, A Razi… - JCI insight, 2017 - ncbi.nlm.nih.gov
We lack a mechanistic explanation for the stereotyped pattern of white matter loss seen in
Huntington's disease (HD). While the earliest white matter changes are seen around the …