NCBI-Aggregator

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

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

PLoS One. 2026 Jul 1;21(7):e0350118. doi: 10.1371/journal.pone.0350118. eCollection 2026.ABSTRACTHuntington's disease (HD) causes progressive disability through motor, psychiatric, and cognitive symptoms. Machine learning speech analysis can detect motor and cognitive symptoms of HD, but not yet psychiatric symptoms. This study investigated whether speech analyses can detect the presence of psychiatric symptoms...Read more

Sci Rep. 2026 Jun 30;16(1):18328. doi: 10.1038/s41598-026-56101-8.ABSTRACTWe applied transcriptome tomography to create a whole-brain model of early-stage Huntington's disease (HD) in R6/2 mice, which ubiquitously express truncated human mutant HTT containing approximately 150 CAG repeats. Medium spiny neuron (MSN)-related genes showed abnormal expression in the HD brain, in terms of...Read more

Neurobiol Dis. 2026 Sep;227:107502. doi: 10.1016/j.nbd.2026.107502. Epub 2026 Jun 26.ABSTRACTHuntington's disease (HD) is a monogenic neurodegenerative disorder characterized by extensive brain pathology. While its underlying cause has been attributed to intracellular mutant huntingtin (mHTT), growing evidence reveals that mHTT is also present, and biologically active, outside cells. Detected in cerebrospinal...Read more

EMBO Mol Med. 2026 Jul;18(7):2838-2866. doi: 10.1038/s44321-026-00459-9. Epub 2026 Jun 26.ABSTRACTHuntington's disease (HD) is a hereditary movement disorder caused by a CAG repeat expansion in the huntingtin gene. HD is characterized by deposition of mutant huntingtin (mHTT) aggregates, and by severe neurodegeneration of the basal ganglia and neocortex. No cure...Read more

PLoS One. 2026 Jun 25;21(6):e0336088. doi: 10.1371/journal.pone.0336088. eCollection 2026.ABSTRACTAlthough adult Huntington's disease (HD) studies have significantly advanced our understanding of the course of degeneration, they may underrepresent critical neurodevelopmental aspects of the disease. Significant gaps remain in understanding how mutant huntingtin affects early neurodevelopment, its long-term impact, as well as...Read more

BMJ Case Rep. 2026 Jun 23;19(6):e273348. doi: 10.1136/bcr-2026-273348.ABSTRACTHuntington's disease (HD) is characterised by progressive motor impairment and neuropsychiatric dysfunction; however, early presentations may be dominated by psychiatric symptoms. We describe a man in his 50s with a documented diagnosis of HD established several years prior and a strong family history,...Read more