NCBI Aggregator

  • 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
  • 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
  • From glycemic control to neuroprotection: alogliptin as a repurposed candidate for Huntington's disease
    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
    Source: NCBI Date: 2026-07-20 By Garima Choudhary
  • Emotion regulation across disease stages in Huntington's disease gene expansion carriers
    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
    Source: NCBI Date: 2026-07-17 By Emilie Poulsen
  • The Recommendation Paradox: Perspectives on Genetic Testing in Huntington's Disease Families
    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
    Source: NCBI Date: 2026-07-17 By Noit Inbar
  • Understanding the cellular architecture of Huntington's disease
    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
    Source: NCBI Date: 2026-07-14 By Dorian Pustina
  • CRISPR-Cas9-based therapies for Huntington's disease and Friedreich's ataxia: mechanisms, advances, and future perspectives
    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
    Source: NCBI Date: 2026-07-13 By Abha Ravikumar Mundada
  • Genistein (5,7-dihydroxy-3-(4-hydroxyphenyl)-4H-1-benzopyran-4-one) Is Effective in Reducing Symptoms of Huntington's Disease in Females of the R6/1 Mouse Model
    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
    Source: NCBI Date: 2026-07-07 By Lidia Gaffke
  • CDKN1A protects medium spiny neurons from Huntington's disease pathology
    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
    Source: NCBI Date: 2026-07-06 By Seong Won Lee
  • Neuroprotective potential of resveratrol in Parkinson, Huntington, amyotrophic lateral sclerosis, and multiple sclerosis: a comprehensive review
    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
    Source: NCBI Date: 2026-07-04 By Kasra Shahsavari
  • Development and validation of a machine learning model to detect psychiatric symptoms in Huntington's disease using speech analysis
    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
    Source: NCBI Date: 2026-07-01 By Quang Tuan Rémy Nguyen
  • Restoring cortical disinhibition improves Huntington's disease phenotypes
    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
    Source: NCBI Date: 2026-07-01 By Sonja Blumenstock
  • Topological modeling of gene expression in the brain with Huntington's disease reveals selective disruption of co-expression network
    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
    Source: NCBI Date: 2026-06-30 By Yuko Okamura-Oho
  • Proteomic Impact of Peripheral Expression of Mutant Huntingtin in C. elegans
    J Proteome Res. 2026 Aug 7;25(8):3845-3860. doi: 10.1021/acs.jproteome.5c00850.ABSTRACTHuntington's Disease (HD), a neurodegenerative disorder, is caused by the expansion of a polyglutamine (polyQ) tract near the N-terminus of the huntingtin protein (HTT), resulting in HTT aggregation. While associated with neurodegeneration, HTT is expressed ubiquitously throughout the body, leading to potential peripheral...Read more
    Source: NCBI Date: 2026-06-29 By Ephraim Ezeigbo
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