{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T13:48:10Z","timestamp":1780321690449,"version":"3.54.1"},"reference-count":55,"publisher":"The Company of Biologists","issue":"2","license":[{"start":{"date-parts":[[2021,2,9]],"date-time":"2021-02-09T00:00:00Z","timestamp":1612828800000},"content-version":"vor","delay-in-days":425,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100010449","name":"Czech Ministry of Education, Youth and Sports","doi-asserted-by":"publisher","award":["LO1609"],"award-info":[{"award-number":["LO1609"]}],"id":[{"id":"10.13039\/100010449","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005725","name":"CHDI foundation","doi-asserted-by":"publisher","award":["RA: A11609"],"award-info":[{"award-number":["RA: A11609"]}],"id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,2,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Recently developed therapeutic approaches for the treatment of Huntington's disease (HD) require preclinical testing in large animal models. The minipig is a suitable experimental animal because of its large gyrencephalic brain, body weight of 70-100\u2005kg, long lifespan, and anatomical, physiological and metabolic resemblance to humans. The Libechov transgenic minipig model for HD (TgHD) has proven useful for proof of concept of developing new therapies. However, to evaluate the efficacy of different therapies on disease progression, a broader phenotypic characterization of the TgHD minipig is needed. In this study, we analyzed the brain tissues of TgHD minipigs at the age of 48 and 60-70\u2005months, and compared them to wild-type animals. We were able to demonstrate not only an accumulation of different forms of mutant huntingtin (mHTT) in TgHD brain, but also pathological changes associated with cellular damage caused by mHTT. At 48\u2005months, we detected pathological changes that included the demyelination of brain white matter, loss of function of striatal neurons in the putamen and activation of microglia. At 60-70\u2005months, we found a clear marker of neurodegeneration: significant cell loss detected in the caudate nucleus, putamen and cortex. This was accompanied by clusters of structures accumulating in the neurites of some neurons, a sign of their degeneration that is also seen in Alzheimer's disease, and a significant activation of astrocytes. In summary, our data demonstrate age-dependent neuropathology with later onset of neurodegeneration in TgHD minipigs.<\/jats:p>","DOI":"10.1242\/dmm.041319","type":"journal-article","created":{"date-parts":[[2019,10,23]],"date-time":"2019-10-23T13:42:49Z","timestamp":1571838169000},"source":"Crossref","is-referenced-by-count":20,"title":["Transgenic minipig model of Huntington's disease exhibiting gradually progressing neurodegeneration"],"prefix":"10.1242","volume":"13","author":[{"given":"Taras","family":"Ardan","sequence":"first","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Monika","family":"Baxa","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bo\u017eena","family":"Levinsk\u00e1","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miroslava","family":"Sedl\u00e1\u010dkov\u00e1","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"},{"name":"Masaryk University in Brno 2 Department of Histology and Embryology , , Faculty of Medicine, 62500 Brno , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"The Duong","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji\u0159\u00ed","family":"Kl\u00edma","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"\u0160tefan","family":"Juh\u00e1s","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jana","family":"Juh\u00e1sov\u00e1","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra","family":"\u0160matl\u00edkov\u00e1","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petra","family":"Vochozkov\u00e1","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Motl\u00edk","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6695-6345","authenticated-orcid":false,"given":"Zdenka","family":"Ellederov\u00e1","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics, Czech Academy of Science 1 Laboratory of Cell Regeneration and Plasticity , , 27721 Libechov , Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2019,12,12]]},"reference":[{"key":"2024061302414043200_DMM041319C1","doi-asserted-by":"publisher","first-page":"dmm035949","DOI":"10.1242\/dmm.035949","article-title":"A transgenic minipig model of Huntington's disease shows early signs of behavioral and molecular pathologies","volume":"11","author":"Askeland","year":"2018","journal-title":"Dis. 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