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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

Published online by Cambridge University Press:  26 March 2019

Maria Giovanna Trivieri ,
Francesca Stillitano ,
Delaine Ceholski ,
Irene Turnbull ,
Kevin Costa ,
Thomas Weber ,
Kenneth Fish ,
Evangelia Kranias  and
Roger Hajjar
Maria Giovanna Trivieri
Affiliation:
Mount Sinai School of Medicine
Francesca Stillitano
Affiliation:
Mount Sinai School of Medicine
Delaine Ceholski
Affiliation:
Mount Sinai School of Medicine
Irene Turnbull
Affiliation:
Mount Sinai School of Medicine
Kevin Costa
Affiliation:
Mount Sinai School of Medicine
Thomas Weber
Affiliation:
Mount Sinai School of Medicine
Kenneth Fish
Affiliation:
Mount Sinai School of Medicine
Evangelia Kranias
Affiliation:
Mount Sinai School of Medicine
Roger Hajjar
Affiliation:
Mount Sinai School of Medicine
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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.

Type
Basic/Translational Science/Team Science
Information
Journal of Clinical and Translational Science , Volume 3 , Issue s1 , March 2019 , pp. 26
Creative Commons
Creative Common License - CCCreative Common License - BYCreative Common License - NCCreative Common License - ND
This is an Open Access article, distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives licence (http://creativecommons.org/licenses/by-ncnd/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is unaltered and is properly cited. The written permission of Cambridge University Press must be obtained for commercial re-use or in order to create a derivative work.
Copyright
© The Association for Clinical and Translational Science 2019