Protein pathology of cardiomyopathy-related mutants of troponin

对引起心肌病的肌钙蛋白（肌肉收缩的调节蛋白）突变体进行了结构和动力学分析。小角X射线散射和X射线晶体结构分析结果表明，突变的引入导致突变区周围的局部氢键网络被破坏，使结构不稳定，从而改变了可能的构象分布。此外，通过中子非弹性散射的动力学分析的结果表明，肌钙蛋白突变体显示出比野生型肌钙蛋白更大的结构波动。

Structural and dynamics analysis has been carried out on the mutants of troponin, the regulatory protein of muscle contraction, that cause cardiomyopathy. The results of small-angle X-ray scattering and X-ray crystal structure analysis showed that introduction of the mutation causes disruption of the local hydrogen-bond network around the mutation region, which makes the structure unstable, and thereby changing the distribution of possible conformations. Furthermore, the results of the dynamics analysis by neutron inelastic scattering showed that the troponin mutant shows larger structural fluctuations than the wild-type troponin.