ArticleDisease models & mechanisms2018
A transgenic minipig model of Huntington's disease shows early signs of behavioral and molecular pathologies.
Article in Disease models & mechanisms, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- From farm to lab: gene-edited sheep transforming bone research.JBMR plus · 2026Review
- Molecular features of a Huntington's disease knock-in minipig.Disease models & mechanisms · 2026Article
- Oxidative Stress in Huntington's Disease.Biomolecules · 2025Review
- Huntingtin Co-Isolates with Small Extracellular Vesicles from Blood Plasma of TgHD and KI-HD Pig Models of Huntington's Disease and Human Blood Plasma.International journal of molecular sciences · 2022Article
- Longitudinal phenotype development in a minipig model of neurofibromatosis type 1.Scientific reports · 2020Article
- Generation of Transgenic Cloned Buffalo Embryos Harboring the EGFP Gene in the Y Chromosome Using CRISPR/Cas9-Mediated Targeted Integration.Frontiers in veterinary science · 2020Article
- Large Animal Models of Huntington's Disease: What We Have Learned and Where We Need to Go Next.Journal of Huntington's disease · 2020Review
- Longitudinal study revealing motor, cognitive and behavioral decline in a transgenic minipig model of Huntington's disease.Disease models & mechanisms · 2019Article
- Transgenic minipig model of Huntington's disease exhibiting gradually progressing neurodegeneration.Disease models & mechanisms · 2019Article
- PGC-1α, Sirtuins and PARPs in Huntington's Disease and Other Neurodegenerative Conditions: NAD+ to Rule Them All.Neurochemical research · 2019Review
- Induced pluripotent stem cells throughout the animal kingdom: Availability and applications.World journal of stem cells · 2019Review
- Deterioration of mitochondrial bioenergetics and ultrastructure impairment in skeletal muscle of a transgenic minipig model in the early stages of Huntington's disease.Disease models & mechanisms · 2019Article
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15 authors.
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Abstract
Huntington's disease (HD) is a monogenic, progressive, neurodegenerative disorder with currently no available treatment. The Libechov transgenic minipig model for HD (TgHD) displays neuroanatomical similarities to humans and exhibits slow disease progression, and is therefore more powerful than available mouse models for the development of therapy. The phenotypic characterization of this model is still ongoing, and it is essential to validate biomarkers to monitor disease progression and intervention. In this study, the behavioral phenotype (cognitive, motor and behavior) of the TgHD model was assessed, along with biomarkers for mitochondrial capacity, oxidative stress, DNA integrity and DNA repair at different ages (24, 36 and 48 months), and compared with age-matched controls. The TgHD minipigs showed progressive accumulation of the mutant huntingtin (mHTT) fragment in brain tissue and exhibited locomotor functional decline at 48 months. Interestingly, this neuropathology progressed without any significant age-dependent changes in any of the other biomarkers assessed. Rather, we observed genotype-specific effects on mitochondrial DNA (mtDNA) damage, mtDNA copy number, 8-oxoguanine DNA glycosylase activity and global level of the epigenetic marker 5-methylcytosine that we believe is indicative of a metabolic alteration that manifests in progressive neuropathology. Peripheral blood mononuclear cells (PBMCs) were relatively spared in the TgHD minipig, probably due to the lack of detectable mHTT. Our data demonstrate that neuropathology in the TgHD model has an age of onset of 48 months, and that oxidative damage and electron transport chain impairment represent later states of the disease that are not optimal for assessing interventions.This article has an associated First Person interview with the first author of the paper.
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