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The main feature of HD is the loss of striatal neurons, accompanied by metabolic and transcriptional alterations in both neural and peripheral tissues.<\/jats:p>\n                  <jats:p>Induced pluripotent stem cells (iPSCs) derived from a transgenic HD (TgHD) minipig model expressing a mutant HTT construct were generated to investigate early metabolic, antioxidant and DNA integrity changes associated with HD development. Gene expression analysis showed increased expression of vascular endothelial growth factor (VEGF), pyruvate dehydrogenase kinase 1 (PDK1) and glutamine-oxaloacetic transaminase 1 (GOT1), implying early metabolic alteration in TgHD iPSCs. Moreover, upregulated FANCD2\/FANCI-associated nuclease 1 (FAN1) expression indicated genotoxic stress linked to early HD development. These findings suggest metabolic shifts and putative genotoxic events in the pluripotent stem cell state of the TgHD model and point to early effect of the HD mutation. The model may be suitable for evaluating potential cell therapy and in vitro differentiation of iPSCs to neurons and other cells affected in HD.<\/jats:p>","DOI":"10.1242\/dmm.052585","type":"journal-article","created":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T08:44:02Z","timestamp":1778489042000},"source":"Crossref","is-referenced-by-count":2,"title":["Induced pluripotent stem cells from a transgenic minipig model of Huntington's disease reveal early metabolic changes"],"prefix":"10.1242","volume":"19","author":[{"given":"Irena","family":"Rysankova","sequence":"first","affiliation":[{"name":"Charles University in Prague and General University Hospital in Prague 1 Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine , , 120 00 Prague ,","place":["Czech Republic"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Sekac","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics of 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Academy of Sciences 8 Laboratory of Biotransformation , , 142 00 Prague ,","place":["Czech Republic"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiri","family":"Klempir","sequence":"additional","affiliation":[{"name":"Charles University in Prague and General University Hospital in Prague 1 Department of Neurology and Centre of Clinical Neuroscience, First Faculty of Medicine , , 120 00 Prague ,","place":["Czech Republic"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan","family":"Motlik","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics of the Czech Academy of Science 2 Laboratory of Cell Regeneration and Plasticity , , 277 21 Libechov ,","place":["Czech Republic"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jiri","family":"Klima","sequence":"additional","affiliation":[{"name":"Institute of Animal Physiology and Genetics of the Czech Academy of Science 2 Laboratory of Cell 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