Generation of human induced pluripotent stem cell lines HUJIi004 and HUJIi005 from two individuals carrying autosomal-dominant mutations in the CTNBB1 gene resulting in CTNNB1 neurodevelopmental disorder

Daniel J. Steinberg, Kian Maroun, Srinivasarao Repudi, Bruria Ben-Zeev, Rami I. Aqeilan

Research output: Contribution to journalArticlepeer-review

Abstract

Insufficient β-catenin gene activity leads to CTNNB1 neurodevelopmental disorder (CTNNB1-NDD), a prevalent condition causing diverse neurological effects. We derived induced pluripotent stem cell (iPSC) lines from two CTNNB1-NDD individuals. Comprehensive analysis, including karyotyping, sequencing, and functional assays, verified the stem cell properties, genomic stability, identity, and differentiation capabilities. Notably, variable protein expression levels were observed, hinting at potential functional irregularities. in this study we offer valuable groundwork for future research in the field.

Original languageEnglish
Article number103667
JournalStem Cell Research
Volume83
DOIs
StatePublished - Mar 2025

All Science Journal Classification (ASJC) codes

  • Developmental Biology
  • Cell Biology

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