The Sarcomeric Spring Protein Titin: Biophysical Properties, Molecular Mechanisms, and Genetic Mutations Associated with Heart Failure and Cardiomyopathy.
The Sarcomeric Spring Protein Titin: Biophysical Properties, Molecular Mechanisms, and Genetic Mutations Associated with Heart Failure and Cardiomyopathy.
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DOI：
10.1007/s11886-021-01550-y
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发表时间：
2021-07-16
影响因子：
3.7
通讯作者：
Mestroni L
中科院分区：
文献类型：
--
作者：
Eldemire R;Tharp CA;Taylor MRG;Sbaizero O;Mestroni L
The giant protein titin forms the “elastic” filament of the sarcomere, essential for the mechanical compliance of the heart muscle. Titin serves a biological spring, and therefore structural modifications of titin affect function of the myocardium and are associated with heart failure and cardiomyopathy. In this review, we discuss the current understanding of titin’s biophysical properties and how modifications contribute to cardiac function and heart failure. In addition, we review the most recent data on the clinical impact and phenotype heterogeneity of TTN truncating variants, including diseases involving striated muscles, and prospects for future therapies. Because of the giant structure of the titin protein and the complexity of its function, titin’s role in health and disease is not yet completely understood. Future research efforts need to focus on novel therapeutic approaches able to modulate titin transcriptional and post-translational modification.
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影响因子：
37.8
作者：
LeWinter MM;Granzier HL
通讯作者：
Granzier HL
DOI：
10.1073/pnas.191189098
发表时间：
2001-09-11
影响因子：
11.1
作者：
Li, HB;Oberhauser, AF;Fernandez, JM
通讯作者：
Fernandez, JM
影响因子：
3.6
作者：
Gigli M;Begay RL;Morea G;Graw SL;Sinagra G;Taylor MR;Granzier H;Mestroni L
通讯作者：
Mestroni L
影响因子：
4
作者：
Lanzicher, Thomas;Zhou, Tiankun;Granzier, Henk
通讯作者：
Granzier, Henk
影响因子：
5.6
作者：
Li, HB;Fernandez, JM
通讯作者：
Fernandez, JM