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- W2017342191 abstract "Familial hypertrophic cardiomyopathy (FHC) is a disease of the heart caused by autosomal dominant mutations in genes coding for all major sarcomeric proteins, including the myosin regulatory light chain (RLC). In this report, we have explored the rescue strategies to ameliorate the malignant cardiomyopathy phenotype induced by an aspartic acid to valine substitution (D166V) in the RLC. Previous studies on porcine reconstituted preparations showed that the phosphorylation mimic (S15D) in the background of the D166V mutation (S15D-D166V) restored the calcium sensitivity of force and improved Vmax of myosin ATPase that were largely compromised by the D166V mutation. Transgenic carrying the S15D-D166V mutation have been generated and subjected to structural and functional measurements. Small angle X-ray studies on freshly skinned papillary muscle fibers revealed that a D166V-induced reorganization in cross-bridge mass distribution was partially reversed in Rescue Mice (abnormally increased I1,1/I1,0 ratio observed in Tg-D166V fibers returned to the value near Tg-WT). Noteworthy, pseudo-phosphorylation of D166V significantly restored fiber elasticity allowing for changes in the cross-bride mass distribution on stretch. In vivo cardiac morphology and function were assessed by non-invasive echocardiography followed by invasive left ventricular pressure-volume measurements (P-V loops). Echocardiography assessment confirmed hypertrophy in Tg-D166V mice showing an increased posterior wall thickness in systole. Invasive hemodynamics showed diastolic and systolic dysfunction in Tg-D166V mice. The end-systolic P-V relationship, a measure of heart contractility, which was largely reduced in Tg-D166V mice was completely ameliorated in Rescue Mice. In addition, the maximum dP/dt - End-Diastolic Volume relation, which was compromised in Tg-D166V was fully reversed in Rescue Mice. In conclusion, our results suggest that pseudo-phosphorylation of myosin RLC can mitigate both the structural and functional abnormalities of the FHC heart. Supported by NIH-HL108343 and HL071778 (DSC)." @default.
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- W2017342191 date "2014-01-01" @default.
- W2017342191 modified "2023-09-29" @default.
- W2017342191 title "The Structure-Function Analysis of Myosin Pseudo-Phosphorylation in Mouse Model of FHC" @default.
- W2017342191 doi "https://doi.org/10.1016/j.bpj.2013.11.4235" @default.
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