NCBI-Aggregator

Mitochondrial Dysfunction as a Driver of Neurodegeneration in Parkinson's and Huntington's Disease: Molecular Insights and Emerging Interventions

Mol Neurobiol. 2026 Jun 10;63(1):685. doi: 10.1007/s12035-026-05991-w.ABSTRACTMitochondrial dysfunction has emerged as a central contributor to the pathogenesis of major neurodegenerative disorders, such as Parkinson's and Huntington's disease. In Parkinson's disease, mitochondrial abnormalities are often linked to mutations in genes like PINK1 and Parkin, which regulate mitochondrial quality control, while α-synuclein...Read more

Source: NCBI Date: 2026-06-10 By Omkar Kumar Kuwar
Counseling patients in the predictive genetics clinic for Huntington's disease: A qualitative analysis of ethnographic observations

J Genet Couns. 2026 Jun;35(3):e70240. doi: 10.1002/jgc4.70240.ABSTRACTLittle research has been conducted on patients' experiences inside the predictive genetics clinic. In this clinic, a person with a familial risk of inheriting a rare disease seeks a test that will provide them with information about their future health. Counselors combine gentle challenges...Read more

Source: NCBI Date: 2026-06-04 By Shane Doheny
3-Nitropropionic Acid-Induced Huntington's Disease in Preclinical Models: Mechanisms, Peripheral Toxicities, Model Gaps, and Future Directions

Mol Neurobiol. 2026 May 30;63(1):662. doi: 10.1007/s12035-026-05967-w.ABSTRACTHuntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by an expanded cytosine-adenine-guanine (CAG) repeat (> 36 repeats) in the huntingtin gene, leading to progressive motor dysfunction, cognitive decline, and psychiatric disturbances. Despite extensive research, the precise pathophysiological mechanisms underlying HD remain incompletely...Read more

Source: NCBI Date: 2026-05-30 By Harikrishna Reddy Dontiboina
Neuroprotective Potential of Pinostrobin in a Rat Model of Huntington's Disease: Behavioural, Biochemical, and Molecular Docking Evidence

Mol Neurobiol. 2026 May 29;63(1):660. doi: 10.1007/s12035-026-05946-1.ABSTRACTHuntington's disease (HD) is an enormously destructive autosomal hereditary neurodegenerative disease that results in malfunction of motor, psychological, and cognitive deficits. The neurotoxin 3-nitropropionic acid (3-NPA) is known to induce HD-like signs in the in vivo rat model. The current research is aimed at...Read more

Source: NCBI Date: 2026-05-29 By Sami I Alzarea
Transposable element small RNAs and large RNAs in aging brains and implications in Huntington's and Parkinson's disease

Genome Res. 2026 Jun 1;36(6):1125-1140. doi: 10.1101/gr.280565.125.ABSTRACTTransposable elements (TEs) are implicated in aging and neurodegenerative disorders, but the impact on brain TE RNA dynamics in these phenomena is not fully understood. Therefore, we quantify TE RNA changes in aging postmortem human and mouse brains and in the neurodegenerative disorders Huntington's...Read more

Source: NCBI Date: 2026-05-28 By Gargi Dayama
Molecular features of a Huntington's disease knock-in minipig

Dis Model Mech. 2026 May 1;19(5):dmm052803. doi: 10.1242/dmm.052803. Epub 2026 May 26.ABSTRACTHuntington's disease is caused by a CAG expansion in the HTT gene, leading to somatic repeat instability, alternative processing of HTT pre-mRNA, and mutant huntingtin protein production. To model these features, we generated a knock-in minipig (KI-85Q-HD) carrying a...Read more

Source: NCBI Date: 2026-05-26 By Anastasiia Kolesnikova
DNA methylation profiling in Huntington's disease reveals disease associated changes in the striatum

Clin Epigenetics. 2026 May 26;18(1):92. doi: 10.1186/s13148-026-02082-4.ABSTRACTBACKGROUND: Huntington's disease is caused by a trinucleotide CAG repeat expansion in the HTT gene. Despite displaying autosomal dominance, phenotypic variation exists amongst mutation carriers, in particular relating to the age that symptoms first occur. This variation is primarily driven by an inverse relationship...Read more

Source: NCBI Date: 2026-05-25 By Gregory Wheildon
The pathological Huntingtin CAG triplet expansion differentially affects the diagnosis of systemic and organ-specific autoimmune diseases

Front Immunol. 2026 May 8;17:1689962. doi: 10.3389/fimmu.2026.1689962. eCollection 2026.ABSTRACTBACKGROUND: In Huntington's disease (HD), signs of inflammatory activation are found in the brain, cerebrospinal fluid, and blood. HD monocytes are reported to be hyperreactive in vitro. Thus, HD mutation might affect the immune system.AIM: To explore the frequency of autoimmune diseases...Read more

Source: NCBI Date: 2026-05-25 By Moritz Heyd
A machine learning-derived speech index as a biomarker for Huntington's disease severity

J Neurol. 2026 May 22;273(6):333. doi: 10.1007/s00415-026-13873-3.ABSTRACTBACKGROUND: The development of disease-modifying therapies for Huntington's disease (HD) necessitates sensitive, scalable, and objective biomarkers for patient stratification and tracking. Current clinical scales are rater-dependent and time-consuming, while neuroimaging is costly and inaccessible. We aim to develop and validate a novel Speech Index,...Read more

Source: NCBI Date: 2026-05-22 By Lishan Lin
Exploring stigma in Huntington's disease: A scoping review of methods and conceptualizations for understanding experiences of gene expansion carriers and at-risk individuals

J Genet Couns. 2026 Jun;35(3):e70230. doi: 10.1002/jgc4.70230.ABSTRACTMany individuals affected by the hereditary neurological condition Huntington's disease (HD) have reported experiences of stigmatization, yet the extant literature is currently theoretically and methodologically underdeveloped. Therefore, this scoping review aimed to examine the methodological approaches and theoretical conceptualizations of stigma used and to...Read more

Source: NCBI Date: 2026-05-20 By Tierney Tindall
Beyond motor symptoms: efficacy of pharmacotherapies on motor, functional, and cognitive domains in Huntington's disease - a systematic review

Eur J Pharmacol. 2026 Jun 15;1027:178993. doi: 10.1016/j.ejphar.2026.178993. Epub 2026 May 19.ABSTRACTINTRODUCTION: Huntington's disease (HD) is a progressive neurodegenerative disorder characterized by motor, neuropsychiatric, cognitive, and peripheral alterations impairing functional capacity and quality of life (QoL). Pharmacotherapies, including dopaminergic and glutamatergic drugs, primarily target motor symptoms, while cholinergic modulators address...Read more

Source: NCBI Date: 2026-05-20 By Melissa Talita Wiprich
Changing care for patients at risk for Huntington's disease in the United States due to the 21st Century Cures Act

J Genet Couns. 2026 Jun;35(3):e70217. doi: 10.1002/jgc4.70217.ABSTRACTThe Huntington's Disease Society of America (HDSA) protocol requires in-person result disclosure for predictive testing due to the increased suicidality risk in the Huntington's disease (HD) population. The 21st Century Cures Act (Cures), a United States law requiring immediate release of all healthcare test...Read more

Source: NCBI Date: 2026-05-15 By Abigayle E Rose
Induced pluripotent stem cells from a transgenic minipig model of Huntington's disease reveal early metabolic changes

Dis Model Mech. 2026 Jun 1;19(6):dmm052585. doi: 10.1242/dmm.052585. Epub 2026 May 11.ABSTRACTHuntington's disease (HD) is a neurodegenerative autosomal dominant hereditary disease caused by a CAG triplet repeat expansion mutation in the gene encoding the huntingtin (HTT) protein. The main feature of HD is the loss of striatal neurons, accompanied by...Read more

Source: NCBI Date: 2026-05-11 By Irena Rysankova
Agmatine interaction with imidazoline receptor inhibits manifestation of depression-like behavior in 3-Nitropropionic acid-induced Huntington's disease-like phenotype in rats

Eur J Pharmacol. 2026 May 28;1025:178916. doi: 10.1016/j.ejphar.2026.178916. Epub 2026 May 1.ABSTRACTHuntington's disease (HD)-associated depression is a prevalent psychiatric symptom affecting between one-third and over two-thirds of patients, significantly impacting their quality of life. Agmatine, a biogenic amine produced by the decarboxylation of L-arginine, has recently garnered attention for its...Read more

Source: NCBI Date: 2026-05-03 By Raj Katariya
Huntington s disease - overview

Laeknabladid. 2026 May;112(5):216-222. doi: 10.17992/lbl.2026.05.891.ABSTRACTHuntington's disease (HD) is a rare, autosomal dominant neurodegenerative disorder characterized by progressive movement disturbances, psychiatric symptoms, and cognitive impairment. The disease is caused by an increased number of repeats of the cytosine-adenine-guanine (CAG) base sequence in the HTT gene, leading to the production of a...Read more

Source: NCBI Date: 2026-04-28 By Saga Briem

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