Stem Cell Research (Sep 2023)

Modelling Duchenne muscular dystrophy in vitro with newly generated, blood cell-derived induced pluripotent stem cell line ORIONi003-A

  • Dominika Hajduchova,
  • Stanislava Suroviakova,
  • Sandra Mersakova,
  • Dusan Brany,
  • Romana Zahumenska,
  • Martin Rehak,
  • Henrieta Skovierova,
  • Slavomíra Nováková,
  • Vladimir Nosal,
  • Juraj Marcinek,
  • Michal Kalman,
  • Martin Jozef Pec,
  • Mariana Brozmanova,
  • Jana Melegova,
  • Stefan Juhas,
  • Jana Juhasova,
  • Hana Studenovska,
  • Barbora Mitruskova,
  • Michal Pokusa,
  • Marek Samec,
  • Matej Samos,
  • Andreas Nicodemou,
  • Lubos Danisovic,
  • Zuzana Dankova,
  • Egon Kurca,
  • Katarina Lexova Kolejakova,
  • Jan Chandoga,
  • Lukas Plank,
  • Erika Halasova,
  • Renata Pecova,
  • Jan Strnadel

Journal volume & issue
Vol. 71
p. 103187

Abstract

Read online

Here, we present newly derived in vitro model for modeling Duchenne muscular dystrophy. Our new cell line was derived by reprogramming of peripheral blood mononuclear cells (isolated from blood from pediatric patient) with Sendai virus encoding Yamanaka factors. Derived iPS cells are capable to differentiate in vitro into three germ layers as verified by immunocytochemistry. When differentiated in special medium, our iPSc formed spontaneously beating cardiomyocytes. As cardiomyopathy is the main clinical complication in patients with Duchenne muscular dystrophy, the cell line bearing the dystrophin gene mutation might be of interest to the research community.