NCBI-Aggregator

  • 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
  • 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
  • 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
  • Post-Translational Modifications of Huntingtin: Mechanistic Insights and Therapeutic Opportunities in Huntington's Disease
    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
    Source: NCBI Date: 2025-11-26 By Xiaoxia Zhang
  • Sigma-2 Receptor Antagonism Enhances the Neuroprotective Effects of Pridopidine, a Sigma-1 Receptor Agonist, in Huntington's Disease
    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
    Source: NCBI Date: 2025-11-20 By Jing Jin
  • Endothelial NLRP3-mediated pyroptosis induces blood-brain barrier and neuronal damage in Huntington's disease models
    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
    Source: NCBI Date: 2025-11-14 By Jing Cai
  • Suppression of Huntington's Disease Somatic Instability by Transcriptional Repression and Direct CAG Repeat Binding
    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
    Source: NCBI Date: 2025-11-13 By Ella W Mathews
  • Huntington's Disease and Psychiatric Comorbidities: A Retrospective Study in Portugal
    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
    Source: NCBI Date: 2025-11-07 By José Rocha
  • Bromelain Alleviates 3-Nitropropionic Acid-Induced Huntington's Disease-Like Symptoms in Rats Through the Interplay of SIRT1/PGC-1alpha and FOXO3a Pathways
    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
    Source: NCBI Date: 2025-11-03 By Omnia M Nady
  • CRISPR-based gene therapy for huntington's disease: current advances and future prospects
    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
    Source: NCBI Date: 2025-10-31 By Amna Zaheer
  • BDNF Mitigates Early Oxidative Stress and Promotes Release of Neuroprotective Mitochondria From Striatal Astrocytes of zQ175 Huntington's Disease Mice
    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
    Source: NCBI Date: 2025-10-28 By Federico López Couselo
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