Stem Cell Research (Oct 2022)

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

Journal volume & issue
Vol. 64
p. 102931

Abstract

Read online

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.