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Infantile restrictive cardiomyopathy: cTnI-R170G/W impair the interplay of sarcomeric proteins and the integrity of thin filaments

TNNI3 encoding cTnI, the inhibitory subunit of the troponin complex, is the main target for mutations leading to restrictive cardiomyopathy (RCM). Here we investigate two cTnI-R170G/W amino acid replacements, identified in infantile RCM patients, which are located in the regulatory C-terminus of cTn...

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Detalles Bibliográficos
Autores principales: Cimiotti, Diana, Fujita-Becker, Setsuko, Möhner, Desirée, Smolina, Natalia, Budde, Heidi, Wies, Aline, Morgenstern, Lisa, Gudkova, Alexandra, Sejersen, Thomas, Sjöberg, Gunnar, Mügge, Andreas, Nowaczyk, Marc M., Reusch, Peter, Pfitzer, Gabriele, Stehle, Robert, Schröder, Rasmus R., Mannherz, Hans G., Kostareva, Anna, Jaquet, Kornelia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7077804/
https://www.ncbi.nlm.nih.gov/pubmed/32182250
http://dx.doi.org/10.1371/journal.pone.0229227