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Huntington's disease LIG1 modifier variant increases ligase fidelity and suppresses somatic CAG repeat expansion
Proc Natl Acad Sci U S A. 2026 Mar 10;123(10):e2518854123. doi: 10.1073/pnas.2518854123. Epub 2026 Mar 2.ABSTRACTHuntington's disease (HD) is a fatal neurodegenerative disorder caused by inheriting an expanded CAG repeat tract in the huntingtin gene (HTT) that further expands in somatic cells over an individual's lifetime. Genome-wide association studies have...Read more
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Surface-Engineered Precision Nano-Systems for Targeted Treatment of Huntington's Disease: A Review of Recent Advancements
Int J Nanomedicine. 2026 Feb 20;21:548892. doi: 10.2147/IJN.S548892. eCollection 2026.ABSTRACTHuntington's disease is a progressive neurological disorder marked by motor, cognitive, and psychiatric symptoms. Currently, there are no definitive diagnostic tools or effective treatments to halt or reverse the disease. In recent years, surface-engineered nanosystems have emerged as innovative therapeutic platforms,...Read more
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Silmitasertib, an FDA-designated orphan CK2 inhibitor, ameliorates neuropathology and motor dysfunction in a Huntington's disease mouse model
Neurotherapeutics. 2026 Jan;23(1):e00859. doi: 10.1016/j.neurot.2026.e00859. Epub 2026 Feb 24.ABSTRACTHuntington's disease (HD) is a devastating autosomal dominant neurodegenerative disease that manifests with progressive motor, cognitive, and psychological impairments. HD is caused by a CAG (glutamine) repeat expansion in the huntingtin (HTT) gene, leading to the misfolding and aggregation of mutant HTT...Read more
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Electroconvulsive therapy in a patient with Huntington's disease and depression: a case-report
Tijdschr Psychiatr. 2026;68(2):82-85.ABSTRACTWe present the case of a 60-year-old woman with Huntington’s disease who was successfully treated with electroconvulsive therapy (ECT) for a recurrent depressive disorder presenting with acute suicidality. Depression is the most common neuropsychiatric disorder in Huntington’s disease and ranks among the most important risk factors for suicide...Read more
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Ubiquitin ligase Nedd4 regulates the abundance and toxicity of mutant huntingtin
JCI Insight. 2026 Feb 23;11(4):e181013. doi: 10.1172/jci.insight.181013. eCollection 2026 Feb 23.ABSTRACTHuntington's disease (HD) is a neurodegenerative disorder caused by the expansion of CAG repeats in the gene encoding huntingtin. Since accumulation of mutant huntingtin (mHtt) leads to dysfunction of numerous cellular pathways and toxicity, reducing levels of the mutant protein...Read more
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PROTAC-Mediated Degradation of mHTT Aggregates Attenuates Neurotoxicity in Cellular and R6/2 Mouse Models of Huntington's Disease
J Am Chem Soc. 2026 Mar 4;148(8):8107-8121. doi: 10.1021/jacs.5c14078. Epub 2026 Feb 19.ABSTRACTHuntington's disease (HD) is a fatal neurodegenerative disorder caused by an expanded CAG repeat in the HTT gene, producing mutant huntingtin (mHTT) that misfolds into β-sheet-rich aggregates and drives neuronal loss. Current HTT-lowering strategies face challenges, including invasive...Read more
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Differences in white matter detected by ex vivo 9.4 T MRI are associated with axonal changes in the R6/1 model of Huntington's disease
Neurobiol Dis. 2026 Mar;220:107318. doi: 10.1016/j.nbd.2026.107318. Epub 2026 Feb 11.ABSTRACTWhite matter volume loss has been reported as one of the first indicators in Huntington's disease (HD) patients, but the cellular basis of this deficit remains to be elucidated. To address this, we assessed white matter microstructure in the transgenic R6/1...Read more
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Decreased Physical Activity as an Early Digital Biomarker in Huntington's Disease: A One-Year Observational Study
Brain Behav. 2026 Feb;16(2):e71149. doi: 10.1002/brb3.71149.ABSTRACTINTRODUCTION: Huntington's disease (HD) is a neurodegenerative disorder characterised by motor dysfunction, cognitive impairment, and psychiatric disturbances. This study analyzed the relationship between clinical characteristics, sarcopenia, and physical activity (PA) levels in HD patients.METHODS: A 1-year observational study was conducted with symptomatic, ambulatory HD patients,...Read more
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Co-Design of a New Integrated Care Model With People Affected by Huntington's Disease: A Mixed Methods Study
Health Expect. 2026 Feb;29(1):e70584. doi: 10.1111/hex.70584.ABSTRACTBACKGROUND: People living with neurological conditions have needs that require an integrated care approach. Existing models of integrated care have often emphasized system structures but neglected the micro-level interactions that matter most to people.OBJECTIVES: To develop a micro-level model for integrated care that represents the...Read more
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Abnormal Resting-State EEG delta and alpha rhythms in Huntington's and Alzheimer's Diseases: An exploratory study
Clin Neurophysiol. 2026 Apr;184:2111518. doi: 10.1016/j.clinph.2026.2111518. Epub 2026 Jan 25.ABSTRACTOBJECTIVES: This exploratory study tested the hypothesis that Huntington's disease (HD) is characterized by distinct abnormalities in resting-state electroencephalographic (rsEEG) rhythms compared to Alzheimer's disease (AD).METHODS: Clinical and rsEEG data were collected from 35 patients with HD, 81 patients with AD,...Read more
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Executive Impairment in Huntington's Disease: Insights From a Systematic Review of the Literature
Brain Behav. 2026 Feb;16(2):e71238. doi: 10.1002/brb3.71238.ABSTRACTPURPOSE: This systematic review examines executive dysfunction in Huntington's disease (HD), an inherited neurodegenerative disorder characterized by cognitive alterations that may emerge years before the onset of motor symptoms. The objective of this review is to provide an updated and comprehensive synthesis of executive function...Read more
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Exploring huntington's disease from a neurodevelopmental perspective
Int J Biol Sci. 2026 Jan 1;22(3):1233-1246. doi: 10.7150/ijbs.124552. eCollection 2026.ABSTRACTHuntington's disease (HD) is a rare, inherited neurodegenerative disorder caused by mutations in the huntingtin (HTT) gene. The classic concept is that HD is a degenerative disease that primarily affects the striatum, caused by a gain-of-function mutant mHTT that kills...Read more
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Non-Directiveness and Authenticity in the Predictive Genetic Clinic
Sociol Health Illn. 2026 Feb;48(2):e70149. doi: 10.1111/1467-9566.70149.ABSTRACTThe predictive genetic clinic is a space where counsellors use non-directive counselling to facilitate asymptomatic patients at risk of carrying a dominantly inherited disease access a predictive genetic test. The social science literature has a history of examining practices within this clinic, but with...Read more
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From Cancer to Neuroprotection: Pazopanib Modulates the RIPK1/RIPK3/MLKL and PGAM5/DRP1 Pathways in 3- Nitropropionic Acid-Induced Huntington's Disease
Drug Dev Res. 2026 Apr;87(2):e70238. doi: 10.1002/ddr.70238.ABSTRACTHuntington's disease (HD) is a progressive neurodegenerative disorder with a poorly understood neurobiological basis. Among the various forms of programmed cell death implicated in neurodegeneration, necroptosis has recently garnered significant attention. Notably, dysregulated necroptosis provokes immune response that drives excessive pro-inflammatory cytokine production, promoting...Read more
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Shape matters: Predicting Huntington's disease using progression modelling
Comput Methods Programs Biomed. 2026 Apr;277:109250. doi: 10.1016/j.cmpb.2026.109250. Epub 2026 Jan 15.ABSTRACTBACKGROUND: Despite evidence of group-level differences in striatal morphometry among persons with Huntington's Disease (PwHD), current models of HD progression used for participant selection and assessment of treatment outcomes in clinical trials do not leverage shape information.METHODS: We first...Read more
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