Generation of three human iPSC lines from patients with Huntington's disease with different CAG lengths and human control iPSC line from a healthy donor

Ewelina Latoszek; Marta Piechota; Ewa Liszewska; Hana Hansíková; Jiří Klempíř; Alžbeta Mühlbäck; Georg Bernhard Landwehrmeyer; Jacek Kuznicki; Magdalena Czeredys

doi:10.1016/j.scr.2022.102931

Generation of three human iPSC lines from patients with Huntington's disease with different CAG lengths and human control iPSC line from a healthy donor

Stem Cell Res. 2022 Oct:64:102931. doi: 10.1016/j.scr.2022.102931. Epub 2022 Oct 3.

Authors

Ewelina Latoszek¹, Marta Piechota¹, Ewa Liszewska², Hana Hansíková³, Jiří Klempíř⁴, Alžbeta Mühlbäck⁵, Georg Bernhard Landwehrmeyer⁶, Jacek Kuznicki¹, Magdalena Czeredys⁷

Affiliations

¹ Laboratory of Neurodegeneration, International Institute of Molecular and Cell Biology in Warsaw, Poland.
² Laboratory of Molecular and Cellular Neurobiology, International Institute of Molecular and Cell Biology in Warsaw, Poland.
³ 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.
⁴ Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic.
⁵ 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.
⁶ Department of Neurology, Ulm University, Germany.
⁷ Laboratory of Neurodegeneration, International Institute of Molecular and Cell Biology in Warsaw, Poland. Electronic address: mczeredys@iimcb.gov.pl.

PMID: 36228511
DOI: 10.1016/j.scr.2022.102931

Abstract

Huntington's disease (HD) is a progressive neurodegenerative disorder with autosomal-dominant heritability that affect the central nervous system and peripheral tissues. The human-induced pluripotent stem cells (hiPSC) lines were generated from dermal fibroblasts of patients without comorbidities, non-smokers, at the pre-manifest (IIMCBi004-A), early-manifest (IIMCBi005-A), and manifest (IIMCBi006-A) HD stage assessed by neurological tests, as well as from a healthy donor (IIMCBi003-A). Characterization showed that the obtained hiPSC lines contained different CAG repeats consistent with the number of CAG repeats in original fibroblasts. Moreover, hiPSCs expressed pluripotency markers and were able to differentiate into three-germ layers in vitro.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Humans
Huntington Disease* / genetics
Induced Pluripotent Stem Cells*