NCBI-Aggregator

Mol Neurobiol. 2025 Nov 21;63(1):121. doi: 10.1007/s12035-025-05393-4.ABSTRACTPridopidine is a selective sigma-1 receptor (S1R) agonist in clinical development for Huntington's Disease (HD) and Amyotrophic Lateral Sclerosis (ALS). Activation of the S1R by pridopidine is neuroprotective in multiple preclinical models of neurodegenerative disease. The sigma-2 receptor (S2R) is evolutionarily and structurally unique...Read more

J Pharmacol Sci. 2025 Dec;159(4):256-267. doi: 10.1016/j.jphs.2025.09.003. Epub 2025 Sep 14.ABSTRACTThe NLRP3 inflammasome is primarily expressed and activated in microglial and endothelial cells. Extensive research has been conducted on the activation of NLRP3 inflammasomes by microglial cells leading to pyroptosis. However, there have been no reports on the activation of...Read more

Nat Commun. 2025 Nov 14;16(1):10009. doi: 10.1038/s41467-025-64936-4.ABSTRACTHuntington's disease arises from a CAG expansion in the huntingtin gene beyond a critical threshold. Current therapeutics primarily aim to reduce toxicity by lowering levels of mutant HTT mRNA and protein. Genetic data support a role for somatic instability in HTT's CAG repeat as...Read more

Acta Med Port. 2025 Nov 3;38(11):689-698. doi: 10.20344/amp.23438. Epub 2025 Nov 3.ABSTRACTINTRODUCTION: Huntington's disease is a progressive neurodegenerative disorder characterized by motor, cognitive, and behavioral symptoms. While psychiatric comorbidity is common and may influence disease outcomes, there is a lack of data on its prevalence and impact in Portugal. This...Read more

Biofactors. 2025 Nov-Dec;51(6):e70054. doi: 10.1002/biof.70054.ABSTRACTBromelain, a combination of pineapple-derived enzymes, has demonstrated neuroprotective effects owing to its antioxidant and anti-inflammatory attributes. The current study intended to explore bromelain's neuroprotective impact against 3-nitropropionic acid (3-NP)-induced Huntington's disease (HD)-like symptoms in rats. Four groups of rats were randomly allocated: group 1 received...Read more

Neurogenetics. 2025 Oct 31;26(1):76. doi: 10.1007/s10048-025-00858-z.ABSTRACTCRISPR-Cas9 technology offers transformative potential in treating Huntington's Disease (HD) by directly addressing its genetic root causes. This manuscript explores the pathophysiological mechanisms of HD, characterized by toxic mutant huntingtin (mHTT) protein resulting from expanded CAG repeats in the HTT gene, and the challenges posed...Read more

J Neurochem. 2025 Oct;169(10):e70276. doi: 10.1111/jnc.70276.ABSTRACTHuntington's disease (HD) is a neurodegenerative disorder characterized by mutant huntingtin (mHTT) aggregation, striatal neuron degeneration, astrocyte dysfunction, and accompanied by oxidative stress and mitochondrial impairment. We previously demonstrated that the neurotrophin brain-derived neurotrophic factor (BDNF) exerts antioxidant and anti-inflammatory effects in cultured rat astrocytes....Read more

Dis Model Mech. 2025 Oct 1;18(10):dmm052510. doi: 10.1242/dmm.052510. Epub 2025 Oct 28.ABSTRACTHuntington's disease (HD) is traditionally viewed as an age-related disorder. Emerging evidence suggests that mutant huntingtin (mHTT) disrupts early neurodevelopment, although the contribution of developmental alterations to the late disease onset remains to be clarified. Leveraging human pluripotent stem...Read more

Int J Mol Sci. 2025 Oct 7;26(19):9755. doi: 10.3390/ijms26199755.ABSTRACTSeveral genetic diseases affecting the human nervous system are incurable and insufficiently understood. Among them, nine rare diseases form the polyglutamine (polyQ) family: Huntington's disease (HD), spinocerebellar ataxia types 1, 2, 3, 6, 7, and 17, dentatorubral pallidoluysian atrophy, and spinal and...Read more

Cells. 2025 Sep 28;14(19):1514. doi: 10.3390/cells14191514.ABSTRACTHuntington's Disease (HD) is an inherited neurodegenerative condition caused by an expansion of CAG repeats in the Huntingtin (HTT) gene, leading to a toxic form of the HTT protein. Despite advances in understanding the disease and developing symptomatic treatments, effective therapies for modifying its progression...Read more

J Clin Invest. 2025 Oct 15;135(20):e187941. doi: 10.1172/JCI187941. eCollection 2025 Oct 15.ABSTRACTDysfunction of striatal medium spiny neurons (MSNs) is implicated in several neurological disorders, including Huntington's disease (HD). Despite progress in characterizing MSN pathology in HD, mechanisms underlying MSN susceptibility remain unknown, driving the need for MSNs derived from human...Read more

J Neurol. 2025 Oct 14;272(10):693. doi: 10.1007/s00415-025-13453-x.ABSTRACTOBJECTIVE: To estimate prevalence and describe clinical and genetic features of Huntington's disease (HD) in the Middle East, North Africa, and Turkey (MENAT).METHODS: We conducted a PRISMA-guided systematic review and meta-analysis of EMBASE, PubMed, and Scopus from inception to October 2024. All study designs...Read more

Int J Mol Sci. 2025 Sep 13;26(18):8930. doi: 10.3390/ijms26188930.ABSTRACTiPSCs and their derivatives are used to investigate the molecular genetic mechanisms of human diseases, to identify therapeutic targets, and to screen for small molecules. Combining technologies for generating patient-specific iPSC lines and genome editing allows us to create...Read more

Int J Mol Sci. 2025 Sep 13;26(18):8934. doi: 10.3390/ijms26188934.ABSTRACTCortical transcriptional dysregulation is widespread in Huntington's disease (HD). We re-examined prefrontal Brodmann Area 9 (BA9) RNA-seq (GSE64810; 20 HD, 49 controls) using BH-FDR and GEO2R to obtain differential-expression statistics for downstream in silico integration. A compact, direction-aware 80-gene...Read more

Parkinsonism Relat Disord. 2025 Nov;140:108017. doi: 10.1016/j.parkreldis.2025.108017. Epub 2025 Sep 3.ABSTRACTINTRODUCTION: Chorea and behavioral symptoms in Huntington's disease (HD) have been treated with neuroleptics and related drugs for over 50 years, yet direct randomized comparisons are scarce. This study assessed the efficacy and safety of olanzapine, tetrabenazine, and tiapride in...Read more