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Generation of two human iPSC lines from dermal fibroblasts of adult- and juvenile-onset Huntington's disease patients and two healthy donors

M. Piechota, E. Latoszek, E. Liszewska, H. Hansíková, J. Klempíř, A. Mühlbäck, GB. Landwehrmeyer, J. Kuźnicki, M. Czeredys

. 2023 ; 71 (-) : 103194. [pub] 20230825

Language English Country England, Great Britain

Document type Journal Article, Research Support, Non-U.S. Gov't

Persistent link   https://www.medvik.cz/link/bmc23016304

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease caused by a mutation in the HTT gene. To generate human-induced pluripotent stem cells (hiPSCs), we used dermal fibroblasts from 1 healthy adult control (K-Pic2), 1 HD manifest patient (M-T2), 1 healthy juvenile control (jK-N1), and 1 juvenile HD patient (jHD-V1). HD stage of patients was assessed by neurological tests and donors were without comorbidities and were non-smokers. Characterization showed that the obtained hiPSCs have the same number of CAG repeats as the parental fibroblast lines, express pluripotency markers and have the ability to differentiate into all 3 germ layers.

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$a Latoszek, Ewelina $u Laboratory of Neurodegeneration, International Institute of Molecular and Cell Biology in Warsaw, Poland
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$a Liszewska, Ewa $u Laboratory of Molecular and Cellular Neurobiology, International Institute of Molecular and Cell Biology in Warsaw, Poland
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$a Hansíková, Hana $u Laboratory for Study of Mitochondrial Disorders, Department of Pediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic
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$a Klempíř, Jiří $u Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic
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$a Mühlbäck, Alžbeta $u Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic; Department of Neurology, Ulm University, Germany
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$a Landwehrmeyer, Georg Bernhard $u Department of Neurology, Ulm University, Germany
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$a Kuźnicki, Jacek $u Laboratory of Neurodegeneration, International Institute of Molecular and Cell Biology in Warsaw, Poland
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$a Czeredys, Magdalena $u Laboratory of Neurodegeneration, International Institute of Molecular and Cell Biology in Warsaw, Poland. Electronic address: mczeredys@iimcb.gov.pl
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