Key result
Deletion of two titin C-zone super-repeats in mice reduced thick filament length by 173 nm in cardiac muscle, leading to reduced force generation and a dilated cardiomyopathy phenotype.
Population
Male mice at 62-74 days of age.
Comparison
Genetic deletion of two of titin's C-zone… vs Wild-type (WT) mice.
Design
Preclinical
Authors
Loading...
Establishes a mechanistic basis for titin-truncating variants in dilated cardiomyopathy; leaves open.
Mean Difference: -173
Absolute Event Rate: 1435% vs 1608%
p-value: p=<0.0001
Deletion of titin's C-zone super-repeats in mice reduces thick filament length and causes a dilated cardiomyopathy phenotype, demonstrating titin's essential role as a molecular ruler for thick filaments.
Tonino et al. (2017) studied Dilated cardiomyopathy and muscle physiology. Deletion of titin C-zone super-repeats 1 and 2 (TtnΔC1-2) vs. Wild-type (WT) littermates was evaluated on Shrinkage-corrected A-band width (thick filament length) in cardiac muscle (MD -173 nm, p=<0.0001). Deletion of two titin C-zone super-repeats in mice reduced thick filament length by 173 nm in cardiac muscle, leading to reduced force generation and a dilated cardiomyopathy phenotype.
Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context: