177 Phospholamban R14del Cardiomyopathy: a systematic summary of the pathophysiological mechanisms 7 CONCLUSION PLN-R14del mutation has been shown to affect various aspects of cardiomyocyte function, including calcium handling, contractility, metabolism, and protein homeostasis. The exact mechanisms underlying the development and progression of PLN-R14del cardiomyopathy are still not fully understood, and there is a need for further research to identify the key pathways involved and potential therapeutic targets. It is also important to investigate the relationship between the different abnormalities observed in PLN cardiomyopathy, as they may have complex interrelationships and contribute to the overall disease phenotype. Ultimately, a better understanding of the molecular mechanisms underlying PLN-R14del cardiomyopathy could lead to the development of more effective treatments for this devastating disease. ACKNOWLEDGEMENTS We thank the PLN Foundation for the support and knowledge as a structural basis for this review. We thank Prof. Marc Baldus and Anamika Gaur for their input and expertise on the PLN monomer-pentamer interaction information. FUNDING R.G.C.M. and L.K. are supported by the PLN Foundation. R.G.C.M. is also supported by the Dutch Research Council (NWO)-HARVEY (grant number 18747). F.S. and P.A.D. are supported by the Fondation Leducq CUREPlaN (grant number 18CVD01). J.P.G.S. is supported by Horizon2020 ERC-2016-COG-EVICARE (grant number 725229). P.A.D. and J.P.G.S. are supported by ZonMw Psider-Heart and NWO- HARVEY. CONFLICTS OF INTEREST The authors declare that there is no conflict of interest regarding the publication of this paper. DATA AVAILABILITY No new data were generated or analysed in support of this research.
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