NCBI Aggregator

  • Discovery of disrupted sustained attention and altered functional connectivity in far-from-onset Huntington's disease gene-expanded young adults
    Alzheimers Dement. 2026 Jan;22(1):e70944. doi: 10.1002/alz.70944.ABSTRACTBACKGROUND: Cognitive impairments are a hallmark of Huntington's disease (HD).METHODS: Seventy-one participants (43 HD gene-expanded [HDGE], 28 healthy controls) from the HD-Young Adult Study at two timepoints ≈ 4.7 years apart, completed the Cambridge Neuropsychological Test Automated Battery Rapid Visual Information Processing task and underwent...Read more
    Source: NCBI Date: 2026-01-12 By Christelle Langley
  • Fingolimod Effects on Motor Function and BDNF-TrkB Signaling in a Huntington's Mouse Model Are Disease-Stage-Dependent
    Int J Mol Sci. 2026 Jan 3;27(1):494. doi: 10.3390/ijms27010494.ABSTRACTHuntington's Disease (HD) is characterized by prominent degeneration of the principal neurons of the striatum and by progressive motor and cognitive deterioration. Striatal neurons degenerate in HD due to multiple cell-autonomous and non-autonomous factors. Impaired neurotrophin signaling by brain-derived neurotrophic factor (BDNF)...Read more
    Source: NCBI Date: 2026-01-09 By Khanh Q Nguyen
  • Eye manifestations in Huntington's disease: an update on the potential of ocular biomarkers
    J Neurol. 2026 Jan 8;273(1):68. doi: 10.1007/s00415-025-13600-4.ABSTRACTHuntington's disease (HD) remains a devastating neurodegenerative disorder caused by CAG repeat expansion in the HTT gene. Biomarkers are urgently needed to facilitate more accurate evaluation of disease onset, progression, and response to interventions. Characteristic clinical features of the disease are secondary to neuronal...Read more
    Source: NCBI Date: 2026-01-07 By William A Woods
  • Programmed cell death pathways in huntington's disease: spotlight on ferroptosis and pyroptosis
    Neurol Sci. 2026 Jan 2;47(1):83. doi: 10.1007/s10072-025-08689-4.ABSTRACTHuntington's disease (HD) is characterized by dementia, delayed psychomotor processes, abnormal choreatic movements, and cognitive impairments. This condition is brought on by an increase in cytosine-adenine-guanine repeats in the huntingtin (Htt) gene on chromosome-4, which produces the Htt protein's poly-glutamine (poly-Q). Despite significant progress...Read more
    Source: NCBI Date: 2026-01-02 By Veerta Sharma
  • Nursing in Huntington's Disease and Cell and Gene Therapy
    Adv Exp Med Biol. 2026;1490:291-298. doi: 10.1007/978-3-032-03402-1_31.ABSTRACTHuntington's Disease (HD) is an inherited, progressive, deteriorating, and non-curable disease affecting physical and cognitive states. Recent advancements in cell and gene therapy have brought in new treatment options that challenge Huntington's Disease, with the ultimate goal to modify its trajectory. This article emphasizes...Read more
    Source: NCBI Date: 2025-12-31 By Paraskevi Kyrana
  • Huntington's disease-associated ankyrin repeat palmitoyl transferases are rate-limiting factors in lysosome formation and fusion
    PLoS Genet. 2025 Dec 31;21(12):e1011607. doi: 10.1371/journal.pgen.1011607. eCollection 2025 Dec.ABSTRACTProtein palmitoylation in the Golgi apparatus is critical for the appropriate sorting of various proteins belonging to secretory and lysosomal systems, and defective palmitoylation can lead to the onset of severe pathologies. HIP14 and HIP14L ankyrin repeat-containing palmitoyl transferases were linked...Read more
    Source: NCBI Date: 2025-12-30 By Győző Szenci
  • Huntington's disease presenting with schizophreniform psychosis and narcolepsy in the premotor phase
    Orv Hetil. 2025 Dec 28;166(52):2064-2072. doi: 10.1556/650.2025.33430. Print 2025 Dec 28.ABSTRACTThe authors report the case of a patient diagnosed and treated with schizophrenia for nearly 20 years. A rapid change in the neurological status of the patient necessitated a diagnostic revision. The patient's additional medical history included narcolepsy and psoriasis....Read more
    Source: NCBI Date: 2025-12-27 By Gyöngyi Szabó
  • Clinical Manifestations
    Alzheimers Dement. 2025 Dec;21 Suppl 3:e105286. doi: 10.1002/alz70857_105286.ABSTRACTBACKGROUND: Apathy and impulsivity co-occur in Huntington's disease (HD), but these debilitating behavioural syndromes are multidimensional constructs, raising the question of which specific dimensions drive this relationship, and the stability of the co-occurring dimensions across time.METHODS: People with HD and controls completed multidimensional...Read more
    Source: NCBI Date: 2025-12-25 By Lee-Anne Morris
  • Basic Science and Pathogenesis
    Alzheimers Dement. 2025 Dec;21 Suppl 1:e106520. doi: 10.1002/alz70855_106520.ABSTRACTBACKGROUND: Huntington's disease (HD) differs from Alzheimer's disease (AD) in its clinical presentation, particularly regarding vigilance, with notable motor, functional, and cognitive impairments that may be mirrored in resting-state electroencephalographic (rsEEG) rhythms.METHOD: To evaluate this hypothesis, clinical and rsEEG data were gathered from...Read more
    Source: NCBI Date: 2025-12-24 By Giuseppe Noce
  • Clinical Manifestations
    Alzheimers Dement. 2025 Dec;21 Suppl 3:e098429. doi: 10.1002/alz70857_098429.ABSTRACTBACKGROUND: Huntington's disease (HD) is marked by progressive cognitive decline, with early deficits often presenting long before motor symptoms. These changes are subtle yet profoundly impactful, underscoring the need for sensitive tools like the Loewenstein-Acevedo Scales for Semantic Interference and Learning (LASSI-L). The...Read more
    Source: NCBI Date: 2025-12-23 By Luis A Sierra
  • Basic Science and Pathogenesis
    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
    Source: NCBI Date: 2025-12-22 By Ester Siantoputri
  • Small Gold Nanoparticles Alleviate Huntington's Disease via Modulating p38alpha Mitogen-Activated Protein Kinase and Pyruvate Dehydrogenase Kinase 1
    ACS Nano. 2026 Jan 13;20(1):683-699. doi: 10.1021/acsnano.5c14751. Epub 2025 Dec 22.ABSTRACTHuntington's disease (HD) is a severe neurodegenerative disorder that causes motor impairment and ultimately death. Yet, safe and effective disease-modifying treatments of HD are scarce, and delivery to the HD brain is challenging. Here, we present a small, ∼11 nm...Read more
    Source: NCBI Date: 2025-12-21 By Leo Kit Cheung Lee
  • Neuronal mitochondrial disaggregase CLPB ameliorates Huntington's disease pathology in mice
    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
    Source: NCBI Date: 2025-12-21 By Hyeonho Kim
  • Preclinical evaluation of [11C]CHDI-009R for quantification of mutant huntingtin aggregates
    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
    Source: NCBI Date: 2025-12-17 By Franziska Zajicek
  • A human CAGinSTEM platform for decoding HTT repeats' somatic instability links CAG interruption to HD pathology in neurons
    Cell Rep. 2025 Dec 23;44(12):116685. doi: 10.1016/j.celrep.2025.116685. Epub 2025 Dec 11.ABSTRACTSomatic CAG instability in the mutant Huntingtin (HTT) gene is increasingly recognized as a key hallmark of Huntington's disease (HD). Using our novel human CAGinSTEM platform, we manipulated cis genetic elements influencing instability in human HD neurons, monitoring repeat length....Read more
    Source: NCBI Date: 2025-12-12 By Martina Zobel
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