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Mitochondria "Shackled" by Mutant Huntingtin: Analysis of Morphological Alterations and Disruptions of Intracellular Transport
Biochemistry (Mosc). 2026 Feb;91(2):253-273. doi: 10.1134/S0006297925602850.ABSTRACTMitochondria are semi-autonomous, multifunctional organelles that supply cells with energy. They are highly dynamic structures, capable of moving, fusing, dividing, and forming branched networks. The number, density, and complexity of mitochondrial network are unique to each cell type and reflect cellular demands for ATP and...Read more
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A Two-Track Model of Huntington's Disease Pathology: Striatal Atrophy Mediates Maladaptive Immune Dysregulation
Int J Mol Sci. 2026 Mar 4;27(5):2384. doi: 10.3390/ijms27052384.ABSTRACTHuntington's disease (HD) is characterized by progressive striatal atrophy and complex proteomic changes in the central nervous system. Using the ultrasensitive Next-Gen Ultra-Sensitive Immunoassay (NULISA) proteomic platform, we analyzed cerebrospinal fluid (CSF) from 88 persons with HD to dissect the biological correlates...Read more
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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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Electroencephalography biomarkers in Huntington's disease: A systematic review of resting-state and sleep EEG alterations
Clin Neurophysiol. 2026 May;185:2111701. doi: 10.1016/j.clinph.2026.2111701. Epub 2026 Feb 12.ABSTRACTOBJECTIVE: To systematically review EEG-based biomarkers in Huntington's disease across resting-state, sleep, medication response, and clinical correlation studies.METHODS: PubMed, Web of Science, and EMBASE databases were systematically searched for studies of sufficient methodological quality that examined EEG alterations in patients with...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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Glucose transporter 3 gene deficiency modifies Huntington's disease progression in zQ175 model mice
Exp Neurol. 2026 Jun;400:115700. doi: 10.1016/j.expneurol.2026.115700. Epub 2026 Feb 18.ABSTRACTIn Huntington's disease (HD), reduced neuronal glucose transport plays an important role. We investigated the effects of GLUT3 knock-down (KD) on the trajectory of the HD phenotype in zQ175 model mice. GLUT3 (G3) expression was reduced in heterozygous (HT) and homozygous...Read more
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Neuroinflammatory control of metabolism in huntington's disease: central role of P2 7 receptor mediated redox lipid/epigenetic crosstalk
Inflammopharmacology. 2026 Mar;34(3):2027-2038. doi: 10.1007/s10787-026-02146-0. Epub 2026 Feb 20.ABSTRACTHuntington's disease (HD) is a progressive neurodegenerative disorder in which neuroinflammation, oxidative stress, and mitochondrial dysfunction are increasingly recognized as important contributors to neuronal vulnerability. Recent evidence indicates that the ATP-gated P2X7 receptor (P2X7R) may participate in coordinating several of these pathological...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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Huntington's disease is the best investment in neuroscience today
Trends Mol Med. 2026 Mar;32(3):204-207. doi: 10.1016/j.molmed.2026.01.001. Epub 2026 Feb 14.ABSTRACTHuntington's disease (HD) is usually described as rare, tragic, and intractable. Yet, HD offers a strategically unique entry point for neuroscience. With its genetic clarity, relatively predictable course, and organized global community, HD provides the clearest path to advancing brain...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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