NCBI-Aggregator

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

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

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

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

Metab Brain Dis. 2026 Jul 20;41(1):169. doi: 10.1007/s11011-026-01942-5.ABSTRACTHuntington's disease (HD) is a progressive, autosomal dominant neurodegenerative disorder characterized by motor dysfunction, cognitive decline, and psychiatric disturbances, for which no disease-modifying therapies are currently available. Emerging evidence implicates metabolic impairment, mitochondrial dysfunction, oxidative stress, and neuroinflammation as central contributors to HD...Read more

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

Arch Clin Neuropsychol. 2026 May 29;41(5):acag052. doi: 10.1093/arclin/acag052.ABSTRACTOBJECTIVE: Emotion regulation difficulties are increasingly recognized in Huntington's disease (HD) yet remain understudied with inconsistent findings. While motor symptoms are prominent, patients and caregivers often describe emotional and behavioral changes as distressing. This study assessed perceived emotion regulation abilities and strategies across...Read more

J Genet Couns. 2026 Aug;35(4):e70265. doi: 10.1002/jgc4.70265.ABSTRACTHuntington's disease (HD) families face complex decisions about predictive genetic testing and reproductive options, including preimplantation genetic testing (PGT), and particularly PGT for Monogenic Disorders (PGT-M). We examined attitudes toward genetic testing and reproductive options across affected groups within HD families (e.g., people with...Read more

Elife. 2026 Jul 14;15:e112225. doi: 10.7554/eLife.112225.ABSTRACTA new diffusion MRI approach offers a glimpse of the anomalies of cellular architecture underlying basal ganglia degeneration in Huntington's disease.PMID:42446515 | DOI:10.7554/eLife.112225Read more

Neurogenetics. 2026 Jul 13;27(1):49. doi: 10.1007/s10048-026-00921-3.ABSTRACTHuntington's disease (HD) and Friedreich's ataxia (FRDA) are progressive inherited neurodegenerative disorders caused by trinucleotide repeat expansions but characterized by distinct pathogenic mechanisms. HD arises from a coding-region CAG expansion in the HTT gene that produces toxic gain-of-function effects of mutant huntingtin (mHTT), whereas FRDA...Read more

Front Biosci (Landmark Ed). 2026 Jun 26;31(6):51780. doi: 10.31083/FBL51780.ABSTRACTBACKGROUND: Huntington's disease (HD) is an inherited (autosomal dominant) disorder caused by the occurrence of a pathogenic variant of the HTT gene. The genetic defect consists of an expansion of CAG repeats in exon 1, resulting in the production of a toxic...Read more

Neurobiol Dis. 2026 Sep;227:107522. doi: 10.1016/j.nbd.2026.107522. Epub 2026 Jul 6.ABSTRACTHuntington's disease (HD) arises from abnormal expansion of CAG trinucleotide repeats within the HTT gene, leading to mutant huntingtin (mHTT) aggregation, progressive loss of striatal medium spiny neurons (MSNs), and progressive neurodegeneration. While the genetic cause is established, the mechanisms that...Read more

Int Immunopharmacol. 2026 Oct 1;186:117092. doi: 10.1016/j.intimp.2026.117092. Epub 2026 Jul 6.ABSTRACTBACKGROUND: Investigate the effects of berbamine (BBM) on alleviating motor and cognitive impairment in animal models of Huntington's disease (HD).METHODS: Intraperitoneal injection of 3-nitropropionic acid (3-NP) mice and B6-hHTT130-N transgenic mice were used as the HD models. We evaluated the...Read more

Mol Biol Rep. 2026 Jul 4;53(1):1102. doi: 10.1007/s11033-026-12300-0.ABSTRACTResveratrol shows neuroprotective effects in preclinical studies across a number of neurodegenerative illnesses, including Parkinson's disease (PD), Amyotrophic Lateral Sclerosis (ALS), Multiple Sclerosis (MS), and Huntington's disease (HD), and it enhances mitochondrial function through stimulation of the AMPK/SIRT1/PGC-1α pathway, thereby improving mitochondrial oxidative...Read more

Nature. 2026 Jul;655(8125):1262-1270. doi: 10.1038/s41586-026-10671-9. Epub 2026 Jul 1.ABSTRACTHuntington's disease (HD) is a devastating movement disorder without a cure at present1. Although the monogenic basis of HD is well defined2, the complex downstream effects that underlie behavioural symptoms are poorly understood. These effects include cortical dysfunction3,4, yet the roles of...Read more