Journal of Clinical and Translational Science (Mar 2019)

3213 Unraveling the role of Phospholamban (PLN) in humans via the characterization of Induced Pluripotent Stem Cell (iPSC) Cardiomyocytes (CM) derived from carriers of a lethal PLN mutation

  • Maria Giovanna Trivieri,
  • Francesca Stillitano,
  • Delaine Ceholski,
  • Irene Turnbull,
  • Kevin Costa,
  • Thomas Weber,
  • Kenneth Fish,
  • Evangelia Kranias,
  • Roger Hajjar

DOI
https://doi.org/10.1017/cts.2019.62
Journal volume & issue
Vol. 3
pp. 26 – 26

Abstract

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OBJECTIVES/SPECIFIC AIMS: To study the biology of Phosholamban (PLN) in a human relevant model. METHODS/STUDY POPULATION: State of the art stem-cell technologies using iPSC-CMs derived from carriers of a lethal PLN mutation. RESULTS/ANTICIPATED RESULTS: Our preliminary data demonstrate that this particular PLN mutation (L39) results in reduced expression and mis-localization of PLN as well as increased incidence of early after depolarization in isolated iPSC-CMs. DISCUSSION/SIGNIFICANCE OF IMPACT: Phospholamban (PLN) is a critical regulator of Ca++ homeostasis yet many uncertainties still remain regarding its role in humans. Our study will provide unique insights into the pathophysiology of this protein in HF.