Generation of catecholaminergic polymorphic ventricular tachycardia patient-specific induced pluripotent stem cell line

Stem Cell Res. 2022 Apr:60:102727. doi: 10.1016/j.scr.2022.102727. Epub 2022 Feb 25.

Abstract

Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a genetic disorder characterized by ventricular tachycardia, that can cause the heart to stop beating leading to death. The prevalence is 1/10.000 and in approximately 60% of cases, the syndrome can be due to a mutation of the cardiac ryanodine receptor gene (RyR2). We derived an induced pluripotent stem cell (iPSC) line from an 11-year-old patient blood-cells, carrying a heterozygous missense mutation on the 8th exon of the RyR2 N-terminal part. This reprogramed CPVT line displayed normal karyotype, expressed pluripotent markers and had a capacity to differentiate in trilineage embryonic layers.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Humans
  • Induced Pluripotent Stem Cells* / metabolism
  • Mutation / genetics
  • Ryanodine Receptor Calcium Release Channel / genetics
  • Ryanodine Receptor Calcium Release Channel / metabolism
  • Tachycardia, Ventricular* / genetics

Substances

  • Ryanodine Receptor Calcium Release Channel

Supplementary concepts

  • Polymorphic catecholergic ventricular tachycardia