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3. Metabolic Maturation Media Improve Physiological Function of Human iPSC-Derived Cardiomyocytes.

Author

Feyen, Dries AM, McKeithan, Wesley L, Bruyneel, Arne AN, Spiering, Sean, Hörmann, Larissa, Ulmer, Bärbel, Zhang, Hui, Briganti, Francesca, Schweizer, Michaela, Hegyi, Bence, Liao, Zhandi, Pölönen, Risto-Pekka, Ginsburg, Kenneth S, Lam, Chi Keung, Serrano, Ricardo, Wahlquist, Christine, Kreymerman, Alexander, Vu, Michelle, Amatya, Prashila L, Behrens, Charlotta S, Ranjbarvaziri, Sara, Maas, Renee GC, Greenhaw, Matthew, Bernstein, Daniel, Wu, Joseph C, Bers, Donald M, Eschenhagen, Thomas, Metallo, Christian M, and Mercola, Mark

Subjects
cardiomyocyte, dilated cardiomyopathy, disease modeling, engineered heart tissues, induced pluripotent stem cells, long QT syndrome 3, maturation, physiology, Biochemistry and Cell Biology, Medical Physiology
Abstract

Induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs) have enormous potential for the study of human cardiac disorders. However, their physiological immaturity severely limits their utility as a model system and their adoption for drug discovery. Here, we describe maturation media designed to provide oxidative substrates adapted to the metabolic needs of human iPSC (hiPSC)-CMs. Compared with conventionally cultured hiPSC-CMs, metabolically matured hiPSC-CMs contract with greater force and show an increased reliance on cardiac sodium (Na+) channels and sarcoplasmic reticulum calcium (Ca2+) cycling. The media enhance the function, long-term survival, and sarcomere structures in engineered heart tissues. Use of the maturation media made it possible to reliably model two genetic cardiac diseases: long QT syndrome type 3 due to a mutation in the cardiac Na+ channel SCN5A and dilated cardiomyopathy due to a mutation in the RNA splicing factor RBM20. The maturation media should increase the fidelity of hiPSC-CMs as disease models.

Published
2020

9. Multi-omics analysis reveals a crucial role for Retinoic Acid in promoting epigenetic and transcriptional competence of anin vitromodel of human Pharyngeal Endoderm

Author

Cipriano, Andrea, primary, Colantoni, Alessio, additional, Gomes, Danielle, additional, Moqri, Mahdi, additional, Parker, Alexander, additional, Caldwell, Matthew, additional, Briganti, Francesca, additional, Fiorentino, Jonathan, additional, Roncarolo, Maria Grazia, additional, Baldini, Antonio, additional, Weinacht, Katja G, additional, Tartaglia, Gian Gaetano, additional, and Sebastiano, Vittorio, additional

Published
2022
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12. Metabolic Maturation Media Improve Physiological Function of Human iPSC-Derived Cardiomyocytes

Author

Cardiologie Arts-onderzoekers, Experimentele Afd. Cardiologie 2, Feyen, Dries A M, McKeithan, Wesley L, Bruyneel, Arne A N, Spiering, Sean, Hörmann, Larissa, Ulmer, Bärbel, Zhang, Hui, Briganti, Francesca, Schweizer, Michaela, Hegyi, Bence, Liao, Zhandi, Pölönen, Risto-Pekka, Ginsburg, Kenneth S, Lam, Chi Keung, Serrano, Ricardo, Wahlquist, Christine, Kreymerman, Alexander, Vu, Michelle, Amatya, Prashila L, Behrens, Charlotta S, Ranjbarvaziri, Sara, Maas, Renee G C, Greenhaw, Matthew, Bernstein, Daniel, Wu, Joseph C, Bers, Donald M, Eschenhagen, Thomas, Metallo, Christian M, Mercola, Mark, Cardiologie Arts-onderzoekers, Experimentele Afd. Cardiologie 2, Feyen, Dries A M, McKeithan, Wesley L, Bruyneel, Arne A N, Spiering, Sean, Hörmann, Larissa, Ulmer, Bärbel, Zhang, Hui, Briganti, Francesca, Schweizer, Michaela, Hegyi, Bence, Liao, Zhandi, Pölönen, Risto-Pekka, Ginsburg, Kenneth S, Lam, Chi Keung, Serrano, Ricardo, Wahlquist, Christine, Kreymerman, Alexander, Vu, Michelle, Amatya, Prashila L, Behrens, Charlotta S, Ranjbarvaziri, Sara, Maas, Renee G C, Greenhaw, Matthew, Bernstein, Daniel, Wu, Joseph C, Bers, Donald M, Eschenhagen, Thomas, Metallo, Christian M, and Mercola, Mark

Published
2020

14. iPSC Modeling of RBM20-Deficient DCM Identifies Upregulation of RBM20 as a Therapeutic Strategy

Author

Briganti, Francesca, primary, Sun, Han, additional, Wei, Wu, additional, Wu, Jingyan, additional, Zhu, Chenchen, additional, Liss, Martin, additional, Karakikes, Ioannis, additional, Rego, Shannon, additional, Cipriano, Andrea, additional, Snyder, Michael, additional, Meder, Benjamin, additional, Xu, Zhenyu, additional, Millat, Gilles, additional, Gotthardt, Michael, additional, Mercola, Mark, additional, and Steinmetz, Lars M., additional

Published
2020
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15. Circ-ZNF609 Is a Circular RNA that Can Be Translated and Functions in Myogenesis

Author

Legnini, Ivano, primary, Di Timoteo, Gaia, additional, Rossi, Francesca, additional, Morlando, Mariangela, additional, Briganti, Francesca, additional, Sthandier, Olga, additional, Fatica, Alessandro, additional, Santini, Tiziana, additional, Andronache, Adrian, additional, Wade, Mark, additional, Laneve, Pietro, additional, Rajewsky, Nikolaus, additional, and Bozzoni, Irene, additional

Published
2017
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16. Dysregulated ribonucleoprotein granules promote cardiomyopathy in RBM20gene-edited pigs

Author

Schneider, Jay W., Oommen, Saji, Qureshi, Muhammad Y., Goetsch, Sean C., Pease, David R., Sundsbak, Rhianna S., Guo, Wei, Sun, Mingming, Sun, Han, Kuroyanagi, Hidehito, Webster, Dennis A., Coutts, Alexander W., Holst, Kimberly A., Edwards, Brooks S., Newville, Nikolas, Hathcock, Matthew A., Melkamu, Tamene, Briganti, Francesca, Wei, Wu, Romanelli, Maria G., Fahrenkrug, Scott C., Frantz, Doug E., Olson, Timothy M., Steinmetz, Lars M., Carlson, Daniel F., and Nelson, Timothy J.

Abstract

Ribonucleoprotein (RNP) granules are biomolecular condensates—liquid–liquid phase-separated droplets that organize and manage messenger RNA metabolism, cell signaling, biopolymer assembly, biochemical reactions and stress granule responses to cellular adversity. Dysregulated RNP granules drive neuromuscular degenerative disease but have not previously been linked to heart failure. By exploring the molecular basis of congenital dilated cardiomyopathy (DCM) in genome-edited pigs homozygous for an RBM20allele encoding the pathogenic R636S variant of human RNA-binding motif protein-20 (RBM20), we discovered that RNP granules accumulated abnormally in the sarcoplasm, and we confirmed this finding in myocardium and reprogrammed cardiomyocytes from patients with DCM carrying the R636Sallele. Dysregulated sarcoplasmic RBM20 RNP granules displayed liquid-like material properties, docked at precisely spaced intervals along cytoskeletal elements, promoted phase partitioning of cardiac biomolecules and fused with stress granules. Our results link dysregulated RNP granules to myocardial cellular pathobiology and heart failure in gene-edited pigs and patients with DCM caused by RBM20mutation.

Published
2020
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17. HSP90 and TLR4 Interplay in Keloids

Author

Segreto, Francesco, primary, Marangi, Giovanni Francesco, additional, Gigliofiorito, Pierluigi, additional, Briganti, Francesca, additional, and Persichetti, Paolo, additional

Published
2016
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18. Author Correction: Dysregulated ribonucleoprotein granules promote cardiomyopathy in RBM20gene-edited pigs

Author

Schneider, Jay W., Oommen, Saji, Qureshi, Muhammad Y., Goetsch, Sean C., Pease, David R., Sundsbak, Rhianna S., Guo, Wei, Sun, Mingming, Sun, Han, Kuroyanagi, Hidehito, Webster, Dennis A., Coutts, Alexander W., Holst, Kimberly A., Edwards, Brooks S., Newville, Nikolas, Hathcock, Matthew A., Melkamu, Tamene, Briganti, Francesca, Wei, Wu, Romanelli, Maria G., Fahrenkrug, Scott C., Frantz, Doug E., Olson, Timothy M., Steinmetz, Lars M., Carlson, Daniel F., and Nelson, Timothy J.

Published
2021
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20. Dysregulated ribonucleoprotein granules promote cardiomyopathy in RBM20 gene-edited pigs.

Author

Schneider JW, Oommen S, Qureshi MY, Goetsch SC, Pease DR, Sundsbak RS, Guo W, Sun M, Sun H, Kuroyanagi H, Webster DA, Coutts AW, Holst KA, Edwards BS, Newville N, Hathcock MA, Melkamu T, Briganti F, Wei W, Romanelli MG, Fahrenkrug SC, Frantz DE, Olson TM, Steinmetz LM, Carlson DF, and Nelson TJ

Subjects
Alleles, Animals, Cardiomyopathy, Dilated physiopathology, Cellular Reprogramming, Disease Models, Animal, Female, Gene Editing, Humans, Male, Mutation genetics, Myocardium pathology, Myocytes, Cardiac metabolism, Myocytes, Cardiac pathology, RNA, Messenger genetics, Sarcoplasmic Reticulum genetics, Sarcoplasmic Reticulum metabolism, Secretory Vesicles genetics, Secretory Vesicles metabolism, Swine, Cardiomyopathy, Dilated genetics, Myocardium metabolism, RNA-Binding Proteins genetics, Ribonucleoproteins genetics
Abstract

Ribonucleoprotein (RNP) granules are biomolecular condensates-liquid-liquid phase-separated droplets that organize and manage messenger RNA metabolism, cell signaling, biopolymer assembly, biochemical reactions and stress granule responses to cellular adversity. Dysregulated RNP granules drive neuromuscular degenerative disease but have not previously been linked to heart failure. By exploring the molecular basis of congenital dilated cardiomyopathy (DCM) in genome-edited pigs homozygous for an RBM20 allele encoding the pathogenic R636S variant of human RNA-binding motif protein-20 (RBM20), we discovered that RNP granules accumulated abnormally in the sarcoplasm, and we confirmed this finding in myocardium and reprogrammed cardiomyocytes from patients with DCM carrying the R636S allele. Dysregulated sarcoplasmic RBM20 RNP granules displayed liquid-like material properties, docked at precisely spaced intervals along cytoskeletal elements, promoted phase partitioning of cardiac biomolecules and fused with stress granules. Our results link dysregulated RNP granules to myocardial cellular pathobiology and heart failure in gene-edited pigs and patients with DCM caused by RBM20 mutation.

Published
2020
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