Generation of an iPSC cell line (VANYHHi001-A) from a patient with cardiac arrythmias carrying CACNA1D, SCN5A, and DSP variants

Stem Cell Res. 2024 Dec:81:103608. doi: 10.1016/j.scr.2024.103608. Epub 2024 Nov 7.

Abstract

Progressive cardiac conduction defect often associated with variants in sodium voltage-gated channel SCN5A gene and variants in the L-type calcium voltage-gated channel CACNA1D gene are implicated in sinoatrial node dysfunction. We generated an induced pluripotent stem cell line (iPSC) from a 13-year-old patient with history of conduction system disease and ventricular tachycardia, carrying variants in SCN5A (c.2618C > G), CACNA1D (c.3786G > T), and DSP (c.1582C > G). The generated iPSC line exhibited pluripotency markers, differentiated into the three embryonic germ layers, and maintained a normal karyotype. This iPSC line offers insights into the pathophysiological mechanisms of cardiac arrhythmias and personalized therapies development.

Keywords: CACNA1D; DSP; Induced pluripotent stem cells; Progressive cardiac conduction defect; SCN5A; Sinoatrial node dysfunction.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Arrhythmias, Cardiac* / genetics
  • Calcium Channels, L-Type* / genetics
  • Calcium Channels, L-Type* / metabolism
  • Cell Differentiation
  • Cell Line
  • Humans
  • Induced Pluripotent Stem Cells* / metabolism
  • Male
  • NAV1.5 Voltage-Gated Sodium Channel* / genetics
  • NAV1.5 Voltage-Gated Sodium Channel* / metabolism

Substances

  • Calcium Channels, L-Type
  • NAV1.5 Voltage-Gated Sodium Channel
  • CACNA1D protein, human
  • SCN5A protein, human