NCBI-Aggregator

Unawareness of motor deficit in Huntington's disease

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

Source: NCBI Date: 2026-08-30 By Francesca Gargallo
L-theanine from Camellia sinensis (L.) attenuates 3-NP-induced Huntington's disease-like alterations by remedying behavioral patterns and modulating neurotransmitters across three distinct brain regions: evidence from in-vivo and in-silico approaches

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

Source: NCBI Date: 2026-08-29 By Susankar Kushari
Geometric properties of caudate and putamen mark the progression of Huntington's disease

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

Source: NCBI Date: 2026-08-26 By Dorian Pustina
Network-based stratification of allele-specific expression reveals patient subgroups in Huntington's disease

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

Source: NCBI Date: 2026-08-24 By Daan van Beek
Dysfunction of Primary Cilium in Huntington's Disease: How Mutant Huntingtin Disrupts This Cellular Signaling Hub

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

Source: NCBI Date: 2026-08-23 By Aleksandra S Churkina
The Role of MicroRNA in Diagnosis of Huntington's Disease: A Systematic Review

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

Source: NCBI Date: 2026-08-22 By Sariya Khan
The potential of vitamins in Huntington's disease: bridging mechanistic evidence with translational limitations and future strategies

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

Source: NCBI Date: 2026-08-19 By Vaishnavi Namdeo Padole
Cholesterol restores corticostriatal synaptic connectivity in Huntington's disease through BDNF/TrkB signalling, supporting a role for cholesterol pathways implicated by human genetic modifier studies

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

Source: NCBI Date: 2026-08-12 By Allegra Lenci
Blocking somatic repeat expansion and lowering huntingtin by RNAi synergize to attenuate Huntington's disease pathogenesis in mice

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

Source: NCBI Date: 2026-08-12 By Jillian Belgrad
In vivo mapping of striatal neurodegeneration in Huntington's disease with Soma and Neurite Density Imaging

Elife. 2026 Aug 11;14:RP107661. doi: 10.7554/eLife.107661.ABSTRACTHuntington's disease (HD) is an inherited neurodegenerative disorder characterised by progressive cognitive and motor decline driven by basal ganglia (BG) atrophy. Clinical trials of novel disease-modifying therapies are ongoing, creating a need for sensitive non-invasive imaging biomarkers. Soma and Neurite Density Imaging (SANDI) is a...Read more

Source: NCBI Date: 2026-08-11 By Vasileios Ioakeimidis
Gut microbiome composition in Huntington's disease: Stage-dependent differences between premanifest and manifest patients

Life Sci. 2026 Oct 15;403:124617. doi: 10.1016/j.lfs.2026.124617. Epub 2026 Aug 5.ABSTRACTBACKGROUND: Huntington's disease (HD) is a progressive neurodegenerative disorder with substantial clinical heterogeneity. The gut microbiome has been proposed as a potential modulator of neurodegeneration, but its role in HD and across disease stages remains unclear.METHODS: This cross-sectional case-control study...Read more

Source: NCBI Date: 2026-08-05 By Lucía Simón-Vicente
Cholesterol nose-to-brain delivery as a possible therapeutic strategy in Huntington's disease

Transl Neurodegener. 2026 Aug 4;15(1):37. doi: 10.1186/s40035-026-00569-x.ABSTRACTBACKGROUND: Huntington's disease (HD) is a genetically dominant neurodegenerative disorder characterized by several pathological mechanisms, including the disruption of brain cholesterol homeostasis. In several HD animal models, brain cholesterol biosynthesis and levels are reduced. Since circulating cholesterol cannot reach the brain, providing exogenous cholesterol...Read more

Source: NCBI Date: 2026-08-05 By Monica Favagrossa
Molecular insights of peroxisome proliferator-activated receptor-gamma signalling in amyotrophic lateral sclerosis and Huntington's disease

Int Rev Neurobiol. 2026;188:113-143. doi: 10.1016/bs.irn.2026.05.014. Epub 2026 May 28.ABSTRACTProgressive neuronal loss is a hallmark of neurodegenerative diseases like Huntingtons disease (HD) and Amyotrophic lateral sclerosis (ALS) which are caused by convergent mechanisms such as oxidative stress, mitochondrial dysfunction, neuroinflammation, impaired autophagy and dysregulated cell death pathways. Both conditions share...Read more

Source: NCBI Date: 2026-08-04 By Gopika Venu
A deep learning model for speech-based prediction of clinical scores in people with Huntington's disease: a longitudinal study with cross-sectional replication

Lancet Digit Health. 2026 Aug;8(8):101025. doi: 10.1016/j.landig.2026.101025. Epub 2026 Jul 23.ABSTRACTBACKGROUND: Sensitive monitoring tools are needed to track progression in neurodegenerative diseases and assess interventions before overt brain damage occurs. We propose speech as a non-invasive, easily collected biomarker to capture disease-related variation over time. We developed and validated Neurodegenerative...Read more

Source: NCBI Date: 2026-07-23 By Clément Le Moine Veillon
USP28 Deficiency is Linked to Impaired Ubiquitin-dependent Proteostasis in Huntington's Disease

Mol Neurobiol. 2026 Jul 20;63(1):780. doi: 10.1007/s12035-026-06073-7.ABSTRACTHuntington's disease (HD) is characterized by mutant huntingtin (mHTT) aggregation and impaired proteostasis; however, upstream regulators of ubiquitin system imbalance remain incompletely understood. This study identified the deubiquitinase USP28 as a potential modulator of ubiquitin-dependent proteostasis in HD. Bulk RNA sequencing of R6/2 mouse...Read more

Source: NCBI Date: 2026-07-20 By Kyoungjoo Cho

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