NCBI-Aggregator

Nat Commun. 2026 Sep 12;17(1):10775. doi: 10.1038/s41467-026-77474-4.ABSTRACTBrain vascular barriers are dysfunctional in many neurological disorders, including Huntington's disease (HD), but which vessels are affected and how remains unclear. Using in vivo two-photon microscopy in R6/2 HD-model mice, we reveal a striking divergence in barrier dysfunction. Surface vessels of the blood-cerebrospinal...Read more

Mol Neurobiol. 2026 Oct 7;63(1):951. doi: 10.1007/s12035-026-06205-z.ABSTRACTHuntington's disease (HD) is a neurodegenerative disorder caused by the expression of the mutant huntingtin protein (mHTT), characterized by a progressive decline in motor function, cognitive abilities, and psychiatric symptoms. Exosomes, a distinct class of small extracellular vesicles, have emerged as promising diagnostic biomarkers...Read more

Philos Trans R Soc Lond B Biol Sci. 2026 Oct 1;381(1960):20250269. doi: 10.1098/rstb.2025.0269.ABSTRACTHuntington's disease is caused by the trinucleotide repeat CAG expansion in the huntingtin gene, resulting in an N-terminal polyglutamine (polyQ) tract that renders the huntingtin protein (HTT) prone to misfolding and aggregation. Although polyQ expansion is an intrinsic...Read more

Dis Model Mech. 2026 Sep 1;19(9):dmm052922. doi: 10.1242/dmm.052922. Epub 2026 Sep 30.ABSTRACTHuntington's disease (HD) is a neurodegenerative disease with progressive decline in psychiatric, motor and cognitive symptoms. Hallmarks of HD include brain volume loss and abnormal metabolism. However, HD is a multi-system disorder, and the impact on other organ systems...Read more

J Integr Neurosci. 2026 Sep 22;25(9):49403. doi: 10.31083/JIN49403.ABSTRACTHuntington's disease (HD) is a lethal autosomal dominant neurodegenerative disorder characterized by progressing cognitive, motor, and other behavioral deficits. Animal models, especially rodent-based, are a valuable tool to study brain disorders, including HD. Given the existence of multiple valid and predictive models of...Read more

Methods Mol Biol. 2026;3065:65-81. doi: 10.1007/978-1-0716-5476-7_4.ABSTRACTThe field of neuromuscular and neurodegenerative diseases has been revolutionized by the advent of genetics and molecular biology to evaluate the pathogenicity, thereby providing considerable insight to develop suitable therapies. With the successful translation of antisense oligonucleotides (AOs) from in vitro into animal models and...Read more

Mol Biol Rep. 2026 Sep 27;53(1):1626. doi: 10.1007/s11033-026-12823-6.ABSTRACTHuntington's Disease is a neurodegenerative disorder that progresses over time and can be passed down from parent to child. In uncontrolled motor activities, behavioural problems come into play, as well as progressive mental decline. The CAG triplet in the HTT gene found on...Read more

CONCLUSIONS: These findings demonstrate that critical role of Rad23b in miR-196a-reduced pathological aggregates, and highly suggest downregulating Rad23b or disrupting its interaction with mHTT may offer novel strategies to mitigate mHTT aggregates to delay disease progression.Read more

PURPOSE OF REVIEW: Growing interest in earlier and mechanism-targeted interventions for Huntington's disease (HD) has brought focus on biomarkers capable of informing trial design and regulatory pathways. This review discusses recent advances in fluid and neuroimaging biomarkers since the introduction of the HD-Integrated Staging System (HD-ISS), focusing on candidates with...Read more

Int Rev Neurobiol. 2026;190:113-126. doi: 10.1016/bs.irn.2026.08.017. Epub 2026 Sep 4.ABSTRACTThe glymphatic system, the cerebrospinal fluid-based circulation and waste clearance system for the brain, is gaining increasing attention in Huntington's disease (HD) due to its potential role in neurodegeneration. Glymphatic dysfunction may contribute to progressive neuronal damage and key clinical features...Read more

J Neurosci. 2026 Oct 7;46(40):e1862242026. doi: 10.1523/JNEUROSCI.1862-24.2026.ABSTRACTDopamine neuromodulation is a critical process that facilitates learning, motivation, and motor control. Disruption of these processes has been implicated in several neurodegenerative disorders including Huntington's disease (HD). While dopaminergic signaling is a therapeutic target for treating physical and psychiatric HD symptoms, the mechanism...Read more

J Neurol. 2026 Sep 12;273(10):593. doi: 10.1007/s00415-026-14112-5.ABSTRACTBACKGROUND: In Huntington's disease (HD), clinical heterogeneity in age at onset and disease progression poses demands on precision medicine approaches. Biofluid biomarkers are needed to monitor disease progression, but may also provide insights into pathogenic mechanisms and support the development of disease-modifying therapies (DMTs).OBJECTIVE:...Read more

Mol Biol Rep. 2026 Sep 9;53(1):1557. doi: 10.1007/s11033-026-12739-1.ABSTRACTBACKGROUND: Huntington's disease (HD) is a monogenic neurodegenerative disorder caused by expansion of CAG trinucleotide repeats in the HTT gene, resulting in an aggregation-prone mutant huntingtin protein (mHTT) and progressive neuronal dysfunction. HD pathogenesis involves disturbances in protein homeostasis, mitochondrial function, oxidative stress,...Read more

Int J Pharm. 2026 Oct 5;703:127362. doi: 10.1016/j.ijpharm.2026.127362. Epub 2026 Sep 3.ABSTRACTReduced brain cholesterol synthesis is an early dysfunction that plays a major role in Huntington's disease (HD) pathogenesis. Because the blood-brain barrier (BBB) prevents cholesterol uptake from the circulation, surface-modified, biodegradable, and biocompatible nanoparticles (NPs) that enhance BBB crossing...Read more

Neurol Sci. 2026 Aug 31;47(9):746. doi: 10.1007/s10072-026-09311-x.ABSTRACTINTRODUCTION: Unawareness of chorea is well-known in Huntington's disease (HD). This study investigated unawareness for the whole gamut of motor impairments in daily life, which has not been explored previously.METHODS: Data from 71 consecutive patients with stage I or II HD were assessed retrospectively....Read more