PulseExploreJournal ClubDebatesTrendingResearchersJournals
Instagram
HomeExploreJournal ClubTrending
Synapse
⌘+K
Synapse
May 13, 2026Stem Cell Research2 citationsOpen Access

Generation of iPSC lines from myotonic dystrophy type 1 patients with varying CTG repeat lengths

View Full Paper
THThomas D. HoekmanLRLisa RahmSASílvia Albert

Key Points

  • The study aims to generate induced pluripotent stem cell lines from myotonic dystrophy type 1 patients to investigate the effects of CTG repeat lengths.
  • Generated iPSC lines from fibroblasts of DM1 patients with varying CTG repeat lengths.
  • Utilized patient-derived fibroblasts to create disease-relevant cell types like muscle progenitor cells and neurons.
  • The iPSC lines varied in CTG repeat length and contributed to understanding the correlation with DM1 cellular phenotypes.
  • Differentiated cells displayed distinct characteristics based on the CTG repeat length.

Abstract

An expanded CTG trinucleotide repeat in the Dystrophia Myotonica Protein Kinase ( DMPK ) gene underpins myotonic dystrophy type 1 (DM1), an autosomal dominant neuromuscular disorder that affects almost every organ system, especially the skeletal muscle, central nervous system and the heart. In this study, we describe the generation of induced pluripotent stem cell (iPSC) lines from patient-derived fibroblasts carrying varying expanded (CTG) n repeats in DMPK . These lines provide a valuable resource of investigating how CTG repeat length correlates with DM1-related cellular phenotypes in differentiated, disease-relevant cell types, including muscle progenitor cells and neurons.

Ask AI
Helpful
Bookmark
Share
View Full Paper

Cite This Study

Hoekman et al. (2026) studied this question.

synapsesocial.com/papers/6a04141c79e20c90b44444b6https://doi.org/10.1016/j.scr.2026.104013
Ask AI
Helpful
Bookmark
Share
View Full Paper