Plakophilin-2 is required for transcription of genes that control calcium cycling and cardiac rhythm
2017; Nature Portfolio; Volume: 8; Issue: 1 Linguagem: Inglês
10.1038/s41467-017-00127-0
ISSN2041-1723
AutoresMarina Cerrone, Jérôme Montnach, Xianming Lin, Yan-Ting Zhao, Mingliang Zhang, Esperanza Agulló-Pascual, Alejandra Leo‐Macías, Francisco Alvarado, Igor Dolgalev, Thomas V. Karathanos, Kabir Malkani, Chantal J.M. van Opbergen, Joanne J.A. van Bavel, Hua-Qian Yang, Carolina Vásquez, David J. Tester, Steven J. Fowler, Feng‐Xia Liang, Eli Rothenberg, Adriana Heguy, Gregory E. Morley, William A. Coetzee, Natalia A. Trayanova, Michael J. Ackerman, Toon A.B. van Veen, Héctor H. Valdivia, Mario Delmar,
Tópico(s)Viral Infections and Immunology Research
ResumoPlakophilin-2 (PKP2) is a component of the desmosome and known for its role in cell-cell adhesion. Mutations in human PKP2 associate with a life-threatening arrhythmogenic cardiomyopathy, often of right ventricular predominance. Here, we use a range of state-of-the-art methods and a cardiomyocyte-specific, tamoxifen-activated, PKP2 knockout mouse to demonstrate that in addition to its role in cell adhesion, PKP2 is necessary to maintain transcription of genes that control intracellular calcium cycling. Lack of PKP2 reduces expression of Ryr2 (coding for Ryanodine Receptor 2), Ank2 (coding for Ankyrin-B), Cacna1c (coding for Ca
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