NCBI Aggregator

Alzheimers Dement. 2025 Dec;21 Suppl 1:e100462. doi: 10.1002/alz70855_100462.ABSTRACTBACKGROUND: The hippocampus, a critical region for learning, memory, and spatial cognition, is affected early in Alzheimer's Disease (AD), while it remains resilient in Huntington's Disease (HD).METHOD: We utilized Fluorescent Activated Nuclei Sorting (FANS) followed by bulk RNA and ATAC sequencing to characterize...Read more

Theranostics. 2026 Jan 1;16(5):2388-2404. doi: 10.7150/thno.122651. eCollection 2026.ABSTRACTBackground: Huntington's disease (HD) is a devastating neurodegenerative disorder caused by CAG repeat expansion in the HTT gene, resulting in a polyglutamine-expanded huntingtin (HTT) protein that forms toxic aggregates. Although heat-shock proteins are known to facilitate the refolding or clearance of misfolded proteins,...Read more

J Mol Med (Berl). 2025 Dec 18;104(1):7. doi: 10.1007/s00109-025-02621-1.ABSTRACTHuntington's disease (HD) is a neurodegenerative disorder caused by an expanded trinucleotide repeat in the huntingtin gene (HTT) that subsequently leads to aggregation of the mutant huntingtin (mHTT) protein. Thus, lowering mHTT is a key therapeutic approach used by several candidate therapeutics...Read more

Sci Rep. 2025 Dec 9;15(1):43609. doi: 10.1038/s41598-025-27147-x.ABSTRACTThe Huntington's Disease Cognitive Assessment Battery (HD-CAB) is frequently used in clinical trials; however, it is unclear whether the HD-CAB captures meaningful symptoms from patients' perspectives. We aimed to explore whether HD-CAB tests are considered meaningful to people with Huntington's disease (HD) by mapping...Read more

Commun Biol. 2025 Dec 3;8(1):1743. doi: 10.1038/s42003-025-09074-9.ABSTRACTSynaptic activity-regulated gene expression supports neuroprotection, plasticity, and memory. The transcription factor CREB is central to these processes. It is activated by synaptic NMDA receptors but inactivated by excitotoxic extrasynaptic NMDAR (esNMDAR) signaling. Using primary hippocampal neurons, we modeled neurodegeneration and found that esNMDAR...Read more

Int J Mol Sci. 2025 Nov 11;26(22):10907. doi: 10.3390/ijms262210907.ABSTRACTHuntingtin (HTT) is a large, ubiquitously expressed scaffold protein that participates in multiple cellular processes, including vesicular transport, transcriptional regulation, and energy metabolism. The mutant form of HTT (mHTT), characterized by an abnormal polyglutamine (polyQ) expansion in its N-terminal region, is the...Read more

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

Sleep Med. 2026 Jan;137:108675. doi: 10.1016/j.sleep.2025.108675. Epub 2025 Nov 14.ABSTRACTBACKGROUND: Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterized by motor dysfunction, cognitive impairment, and psychiatric disorders. Sleep alterations are common in HD and are a significant contributor to the disease burden, affecting the quality of life and possibly...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

J Neuroimmune Pharmacol. 2025 Nov 15;20(1):104. doi: 10.1007/s11481-025-10265-8.ABSTRACTHuntington's disease (HD) is an inherited neurodegenerative disorder caused by a CAG repeat expansion in the huntingtin (HTT) gene. It typically manifests as a triad of progressive psychiatric, cognitive, and motor symptoms. The resulting mutant HTT (mHTT) protein disrupts cellular homeostasis and promotes...Read more

J Neurol. 2025 Nov 14;272(12):760. doi: 10.1007/s00415-025-13409-1.ABSTRACTBACKGROUND: Huntington's disease (HD) is a neurodegenerative disorder caused by CAG repeat expansions. The Huntington's Disease Integrated Staging System (HD-ISS) classifies disease stages (0-3) using clinical, biological, and imaging assessments, with caudate and putamen volumes serving as key early biomarkers. Conventional automated volumetric methods...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

Int J Mol Sci. 2025 Nov 1;26(21):10672. doi: 10.3390/ijms262110672.ABSTRACTThere are no disease-modifying treatments available for Huntington's disease (HD), a neurodegenerative disease caused by a genetic mutation in the Huntingtin gene. Previous research suggests that disruptions in the bioenergetics of the mitochondria and increased oxidative stress are potential inducers of HD....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

Acta Neuropathol Commun. 2025 Nov 7;13(1):228. doi: 10.1186/s40478-025-02131-8.ABSTRACTBACKGROUND: Accumulated levels of mutant huntingtin protein (mHTT) and its fragments are considered contributors to the pathogenesis of Huntington's disease (HD). Stimulating autophagy may enhance clearance of mHTT and its aggregates which has been considered as a possible therapeutic strategy. However, the role...Read more