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

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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Main Authors: Diana Cimiotti, Setsuko Fujita-Becker, Desirée Möhner, Natalia Smolina, Heidi Budde, Aline Wies, Lisa Morgenstern, Alexandra Gudkova, Thomas Sejersen, Gunnar Sjöberg, Andreas Mügge, Marc M Nowaczyk, Peter Reusch, Gabriele Pfitzer, Robert Stehle, Rasmus R Schröder, Hans G Mannherz, Anna Kostareva, Kornelia Jaquet
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2020-01-01
Series:PLoS ONE
Online Access:https://doi.org/10.1371/journal.pone.0229227

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https://doi.org/10.1371/journal.pone.0229227

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