NCBI Aggregator

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

Metab Brain Dis. 2026 Aug 29;41(1):195. doi: 10.1007/s11011-026-01967-w.ABSTRACTHuntington's disease (HD) is a neurological condition with limited treatment. Its hallmarks are progressive loss of neurons, chorea, cognitive, motor, and metabolic impairments. The current study uses 3-nitropropionic acid (3-NP) to cause HD-like indications in rats. L-theanine (L-TH), an active component of Camellia...Read more

Exp Neurol. 2026 Dec;406:115995. doi: 10.1016/j.expneurol.2026.115995. Epub 2026 Aug 27.ABSTRACTHuntington's disease (HD) is marked by progressive neuronal loss and atrophy of grey matter structures, particularly the caudate and putamen. Brain imaging studies reveal that the white matter starts to decay in the brain of individuals bearing the HD mutation many...Read more

Neurosci Biobehav Rev. 2026 Nov;190:106933. doi: 10.1016/j.neubiorev.2026.106933. Epub 2026 Aug 26.ABSTRACTDepression affects Huntington's disease (HD) gene-expansion carriers at up to four times the general population rate, yet its neurobiological underpinnings remain inadequately understood, potentially contributing to persistent symptoms and suboptimal therapeutic outcomes, despite commonly used interventions. Understanding of the pathophysiological...Read more

Imaging Neurosci (Camb). 2026 Aug 24;4:IMAG.a.1351. doi: 10.1162/IMAG.a.1351. eCollection 2026.ABSTRACTVolumetric MRI of the caudate and putamen is a robust biomarker of striatal degeneration in Huntington's disease (HD). Striatal degeneration may be reflected beyond volume alone, in additional shape and intensity alterations. We evaluated five novel morphometric biomarkers of the caudate...Read more

Bioinformatics. 2026 Aug 1;42(Supplement_2):btag592. doi: 10.1093/bioinformatics/btag592.ABSTRACTMOTIVATION: Huntington's disease (HD) exhibits substantial variability in age of onset and disease progression that is not fully explained by CAG repeat length alone. Part of this residual variation is heritable, implicating additional genetic mechanisms. cis-regulatory variation, genetic variants that alter transcription and splicing of...Read more

Biochemistry (Mosc). 2026 Jul;91(7):1137-1156. doi: 10.1134/S0006297926601590.ABSTRACTPrimary cilia are solitary, antenna-like organelles that project from the surface of most vertebrate cells. They consist of a microtubule-based axoneme extending from a modified centriole (basal body) and enclosed by a lipid bilayer membrane. For several decades after their discovery, the functions of primary...Read more

Mol Neurobiol. 2026 Aug 22;63(1):850. doi: 10.1007/s12035-026-06143-w.ABSTRACTHuntington's disease (HD) is an autosomal dominant genetic neurodegenerative disorder with features of progressive motor, cognitive, and psychiatric dysfunction. Current diagnosis relies largely on clinical presentation and genetic testing but lacks sensitivity to early disease diagnosis or progression monitoring. MicroRNAs (miRNAs) are short non-coding...Read more

Metab Brain Dis. 2026 Aug 19;41(1):187. doi: 10.1007/s11011-026-01948-z.ABSTRACTNeurodegenerative diseases are characterized by impairment of neuronal functions and neuronal loss. Huntington's disease is one of the neurodegenerative diseases caused by the formation of mutant Huntingtin protein aggregates in the brain. The other pathological reasons behind this disease are oxidative stress, mitochondrial...Read more

Trends Neurosci. 2026 Sep;49(9):734-749. doi: 10.1016/j.tins.2026.07.006. Epub 2026 Aug 14.ABSTRACTAlternative splicing of RNA is a highly regulated process that increases the complexity of gene expression, with disruption of splicing leading to significant disruption of cellular function and, ultimately, disease. This spliceopathy is exemplified by myotonic dystrophy type 1, a CTG...Read more

Sci Transl Med. 2026 Aug 12;18(862):eaea3104. doi: 10.1126/scitranslmed.aea3104. Epub 2026 Aug 12.ABSTRACTHuntington's disease (HD) is a progressive neurodegenerative disorder with no approved therapies. Despite multiple clinical trials, huntingtin (HTT)-lowering strategies have yet to show meaningful clinical benefit. Both somatic expansion and toxic HTT species are key molecular drivers of HD,...Read more

Pharmacol Res. 2026 Sep;231:108375. doi: 10.1016/j.phrs.2026.108375. Epub 2026 Aug 12.ABSTRACTCorticostriatal synaptic dysfunction is an early hallmark of Huntington's disease (HD), yet the mechanisms underlying synapse loss and its reversibility remain poorly understood. Brain cholesterol is essential for neuronal and synaptic function, and dysregulated cholesterol metabolism has emerged as a key...Read more