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March 6, 2026Stem Cell Research0 citationsOpen Access

Generation of hiPSCs lines from PRICKLE2-mutant individuals with epilepsy

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CZClara ZanninoAEAntonella EspositoMTMariagrazia Talarico

Key Points

  • The aim is to generate human induced pluripotent stem cell lines from individuals with mutations in the PRICKLE2 gene associated with epilepsy.
  • Reprogrammed peripheral blood mononuclear cells (PBMCs) from individuals carrying a PRICKLE2 mutation.
  • Generated human induced pluripotent stem cell (hiPSC) lines for further study.
  • Established hiPSC lines from two related individuals with a PRICKLE2 mutation and epilepsy.
  • Demonstrated the potential for using these hiPSCs in molecular and functional investigations.

Abstract

The PRICKLE2 gene encodes a protein implicated in the non-canonical Wnt signalling pathway and in the regulation of planar cell polarity, although its precise biological functions remain incompletely understood. To date, only few PRICKLE2 mutations have been reported, and these have been associated with diverse clinical phenotypes, including autism spectrum disorders, epilepsy, and neurodevelopmental delay 1 . Here we report the generation of human induced pluripotent stem cell (hiPSCs) lines from two related individuals carrying a PRICKLE2 mutation and affected by an epileptic syndrome, through reprogramming their peripheral blood mononuclear cells (PBMCs). These hiPSC lines will enable further molecular and functional investigations.

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Cite This Study

Zannino et al. (2026) studied this question.

synapsesocial.com/papers/69aa6f0d531e4c4a9ff59281https://doi.org/10.1016/j.scr.2026.103953
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