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Sarcomeric versus Non-Sarcomeric HCM
Hypertrophic cardiomyopathy (HCM) is the most common heritable cardiovascular disorder and is characterized by left ventricular hypertrophy (LVH), which is unexplained by abnormal loading conditions. HCM is inherited as an autosomal dominant trait and, in about 40% of patients, the causal mutation is identified in genes encoding sarcomere proteins ...
Borrelli, Felice +8 more
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Sarcomere Length Nonuniformity and Force Regulation in Myofibrils and Sarcomeres [PDF]
The smallest contractile unit in striated muscles is the sarcomere. Although some of the classic features of contraction assume a uniform behavior of sarcomeres within myofibrils, the occurrence of sarcomere length nonuniformities has been well recognized for years, but it is yet not well understood. In the past years, there has been a great advance in
Felipe de Souza Leite, Dilson E. Rassier
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Spontaneous sarcomere dynamics [PDF]
Sarcomeres are the basic force generating units of striated muscles and consist of an interdigitating arrangement of actin and myosin filaments. While muscle contraction is usually triggered by neural signals, which eventually set myosin motors into motion, isolated sarcomeres can oscillate spontaneously between a contracted and a relaxed state.
Gunther, Stefan, Kruse, Karsten
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Active Viscoelasticity of Sarcomeres [PDF]
The perturbation response of muscle is important for the versatile, stable and agile control capabilities of animals. Muscle resists being stretched by developing forces in the passive tissues and in the active crossbridges. This review focuses on the active perturbation response of the sarcomere. The active response exhibits typical stress relaxation,
Khoi D. Nguyen +2 more
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Modular proteins from the Drosophila sallimus (sls) gene and their expression in muscles with different extensibility [PDF]
The passive elasticity of the sarcomere in striated muscle is determined by large modular proteins, such as titin in vertebrates. In insects, the function of titin is divided between two shorter proteins, projectin and sallimus (Sls), which are the ...
Ayme-Southgate +53 more
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When a muscle is stretched during a contraction, the resulting steady-state force is higher than the isometric force produced at a comparable sarcomere length.
Ricarda M. Haeger, Dilson E. Rassier
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Summary: Actinins are strain-sensing actin cross-linkers that are ubiquitously expressed and harbor mutations in human diseases. We utilize CRISPR, pluripotent stem cells, and BioID to study actinin interactomes in human cardiomyocytes.
Feria A. Ladha +9 more
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Valsartan for attenuating disease evolution in early sarcomeric hypertrophic cardiomyopathy: the design of the Valsartan for Attenuating Disease Evolution in Early Sarcomeric Hypertrophic Cardiomyopathy (VANISH) trial [PDF]
Background: Hypertrophic cardiomyopathy (HCM) is often caused by sarcomere gene mutations, resulting in left ventricular hypertrophy (LVH), myocardial fibrosis, and increased risk of sudden cardiac death and heart failure.
Braunwald, Eugene +11 more
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Antisense-mediated exon skipping: a therapeutic strategy for titin-based dilated cardiomyopathy [PDF]
Frameshift mutations in the TTN gene encoding titin are a major cause for inherited forms of dilated cardiomyopathy (DCM), a heart disease characterized by ventricular dilatation, systolic dysfunction, and progressive heart failure. To date, there are no
Alessandra, Moretti +22 more
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Sarcomere and Inter-Sarcomere Dynamics within Skeletal Muscle Myofibrils [PDF]
Introduction: Inter-sarcomere dynamics within muscle myofibrils underlie basic mechanisms of contraction. In this study, we used microfluidic perfusions to control the environment of sarcomeres within isolated myofibrils to directly investigate inter-sarcomere dynamics.
Felipe de Souza Leite, Dilson E. Rassier
openaire +1 more source

