[HTML][HTML] Synaptic pathology in Huntington's disease: Beyond the corticostriatal pathway

J Barry, MTN Bui, MS Levine, C Cepeda - Neurobiology of disease, 2022 - Elsevier
Huntington's disease (HD) is a heritable, fatal neurodegenerative disorder caused by a
mutation in the Huntingtin gene. It is characterized by chorea, as well as cognitive and …

Translation of microRNA-based huntingtin-lowering therapies from preclinical studies to the clinic

J Miniarikova, MM Evers, P Konstantinova - Molecular Therapy, 2018 - cell.com
The single mutation underlying the fatal neuropathology of Huntington's disease (HD) is a
CAG triplet expansion in exon 1 of the huntingtin (HTT) gene, given rise to a toxic mutant …

Distinct translatome changes in specific neural populations precede electroencephalographic changes in prion-infected mice

L Kaczmarczyk, M Schleif, L Dittrich, RH Williams… - PLoS …, 2022 - journals.plos.org
Selective vulnerability is an enigmatic feature of neurodegenerative diseases (NDs),
whereby a widely expressed protein causes lesions in specific cell types and brain regions …

[HTML][HTML] Abnormal patterns of sleep and EEG power distribution during non-rapid eye movement sleep in the sheep model of Huntington's disease

S Vas, AU Nicol, L Kalmar, J Miles, AJ Morton - Neurobiology of disease, 2021 - Elsevier
Sleep disruption is a common invisible symptom of neurological dysfunction in Huntington's
disease (HD) that takes an insidious toll on well-being of patients. Here we used …

Changes in brain activity with tominersen in early-manifest Huntington's disease

DJ Hawellek, P Garces, AH Meghdadi… - Brain …, 2022 - academic.oup.com
It is unknown whether alterations in EEG brain activity caused by Huntington's disease may
be responsive to huntingtin-lowering treatment. We analysed EEG recordings of 46 patients …

Neurophysiological hallmarks of Huntington's disease progression: an EEG and fMRI connectivity study

NV Ponomareva, SA Klyushnikov… - Frontiers in Aging …, 2023 - frontiersin.org
Electroencephalography (EEG) and functional magnetic resonance imaging (fMRI) can
provide corroborative data on neurophysiological alterations in Huntington's disease (HD) …

Sex differences in sleep architecture in a mouse model of Huntington's disease

E Chiem, K Zhao, G Stark, CA Ghiani… - Journal of …, 2024 - Wiley Online Library
Sleep and circadian rhythm disturbances are common features of Huntington's disease
(HD). HD is an autosomal dominant neurodegenerative disorder that affects men and …

Quantitative EEG analysis in Angelman syndrome: candidate method for assessing therapeutics

LA Martinez, HA Born, S Harris… - Clinical EEG and …, 2023 - journals.sagepub.com
The goal of these studies was to use quantitative (q) EEG techniques on data from children
with Angelman syndrome (AS) using spectral power analysis, and to evaluate this as a …

Circadian dysfunction in the Q175 model of Huntington's disease: Network analysis

B Smarr, T Cutler, DH Loh, T Kudo… - Journal of …, 2019 - Wiley Online Library
Disturbances in sleep/wake cycle are a common complaint of individuals with Huntington's
disease (HD) and are displayed by HD mouse models. The underlying mechanisms …

[HTML][HTML] Neurophysiological correlates of non-motor symptoms in late premanifest and early-stage manifest Huntington's disease

MC Davis, AT Hill, PB Fitzgerald, NW Bailey… - Clinical …, 2023 - Elsevier
Objective To find sensitive neurophysiological correlates of non-motor symptoms in
Huntington's disease (HD), which are essential for the development and assessment of …