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

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 par...

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Autores principales: Trivieri, Maria Giovanna, Stillitano, Francesca, Ceholski, Delaine, Turnbull, Irene, Costa, Kevin, Weber, Thomas, Fish, Kenneth, Kranias, Evangelia, Hajjar, Roger
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cambridge University Press 2019
Materias:
Basic/Translational Science/Team Science
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6799595/
http://dx.doi.org/10.1017/cts.2019.62
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author Trivieri, Maria Giovanna
Stillitano, Francesca
Ceholski, Delaine
Turnbull, Irene
Costa, Kevin
Weber, Thomas
Fish, Kenneth
Kranias, Evangelia
Hajjar, Roger
author_facet Trivieri, Maria Giovanna
Stillitano, Francesca
Ceholski, Delaine
Turnbull, Irene
Costa, Kevin
Weber, Thomas
Fish, Kenneth
Kranias, Evangelia
Hajjar, Roger
author_sort Trivieri, Maria Giovanna
collection PubMed
description 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.
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spelling pubmed-67995952019-10-28 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 Trivieri, Maria Giovanna Stillitano, Francesca Ceholski, Delaine Turnbull, Irene Costa, Kevin Weber, Thomas Fish, Kenneth Kranias, Evangelia Hajjar, Roger J Clin Transl Sci Basic/Translational Science/Team Science 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. Cambridge University Press 2019-03-27 /pmc/articles/PMC6799595/ http://dx.doi.org/10.1017/cts.2019.62 Text en © The Association for Clinical and Translational Science 2019 http://creativecommons.org/licenses/by-nc-nd/4.0/ 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.
spellingShingle Basic/Translational Science/Team Science
Trivieri, Maria Giovanna
Stillitano, Francesca
Ceholski, Delaine
Turnbull, Irene
Costa, Kevin
Weber, Thomas
Fish, Kenneth
Kranias, Evangelia
Hajjar, Roger
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
title 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
title_full 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
title_fullStr 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
title_full_unstemmed 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
title_short 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
title_sort 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
topic Basic/Translational Science/Team Science
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6799595/
http://dx.doi.org/10.1017/cts.2019.62
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