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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\u2005months), 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\u2005months. 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\u2005months, and that oxidative damage and electron transport chain impairment represent later states of the disease that are not optimal for assessing interventions.<\/jats:p>\n               <jats:p>This article has an associated First Person interview with the first author of the paper.<\/jats:p>","DOI":"10.1242\/dmm.035949","type":"journal-article","created":{"date-parts":[[2018,9,25]],"date-time":"2018-09-25T08:09:35Z","timestamp":1537862975000},"source":"Crossref","is-referenced-by-count":18,"title":["A transgenic minipig model of Huntington's disease shows early signs of behavioral and molecular pathologies"],"prefix":"10.1242","volume":"11","author":[{"given":"Georgina","family":"Askeland","sequence":"first","affiliation":[{"name":"Institute of Clinical Medicine, University of Oslo 1 Department of Medical Biochemistry , , 0372 Oslo , Norway"},{"name":"Oslo University Hospital 2 Department of 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, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5048-5857","authenticated-orcid":false,"given":"Monika","family":"Baxa","sequence":"additional","affiliation":[{"name":"Charles University in Prague and General University Hospital in Prague 3 Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine , , Prague 12808 , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra","family":"Smatlikova","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 4 Laboratory of Cell Regeneration and Plasticity , , Libechov 27721 , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bozena","family":"Bohuslavova","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 4 Laboratory of Cell Regeneration and Plasticity , , Libechov 27721 , Czech Republic"},{"name":"Charles University in Prague 5 Department of Cell Biology, Faculty of Science , , Prague 12843 , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0735-7155","authenticated-orcid":false,"given":"Jiri","family":"Klempir","sequence":"additional","affiliation":[{"name":"Charles University in Prague and General University Hospital in Prague 6 Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine , , Prague 12821 , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9799-5785","authenticated-orcid":false,"given":"The Duong","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 4 Laboratory of Cell Regeneration and Plasticity , , Libechov 27721 , Czech Republic"},{"name":"Charles University in Prague 5 Department of Cell Biology, Faculty of Science , , Prague 12843 , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Ku\u015bnierczyk","sequence":"additional","affiliation":[{"name":"Norwegian University of Science and Technology 7 Proteomics and Metabolomics Core Facility, PROMEC, Department of Clinical and Molecular Medicine , , 7491 Trondheim , Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8759-1170","authenticated-orcid":false,"given":"Magnar","family":"Bj\u00f8r\u00e5s","sequence":"additional","affiliation":[{"name":"Oslo University Hospital 2 Department of Microbiology , , 0372 Oslo , Norway"},{"name":"Norwegian University of Science and Technology 7 Proteomics and Metabolomics Core Facility, PROMEC, Department of Clinical and Molecular Medicine , , 7491 Trondheim , 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