Schwinger Robert H G, Frank Konrad F
Laboratory of Muscle Research and Molecular Cardiology, Department of Internal Medicine III, Joseph-Stelzmannstrasse, 50924 Köln, Germany.
Sci STKE. 2003 Apr 29;2003(180):pe15. doi: 10.1126/stke.2003.180.pe15.
In cardiac cells, phospholamban is a potent inhibitor of sarcoplasmic reticulum calcium (Ca(2+)) transport. Overexpression of mutant forms of phospholamban may result in beneficial or detrimental effects on intracellular Ca(2+) handling and cardiac systolic and diastolic function. Mutations in phospholamban have also been linked to human cardiomyopathies, providing important insights into the underlying disease mechanisms and the key role of phospholamban in myocardial excitation-contraction coupling. This Perspective discusses new advances in our understanding of the role of phospholamban in intracellular Ca(2+) handling and the development of human and murine cardiomyopathies.
在心肌细胞中，受磷蛋白是肌浆网钙（Ca(2+)）转运的强效抑制剂。受磷蛋白突变形式的过表达可能对细胞内Ca(2+)处理以及心脏的收缩和舒张功能产生有益或有害的影响。受磷蛋白的突变也与人类心肌病有关，这为潜在的疾病机制以及受磷蛋白在心肌兴奋-收缩偶联中的关键作用提供了重要见解。本综述讨论了我们对受磷蛋白在细胞内Ca(2+)处理中的作用以及人类和小鼠心肌病发展的理解方面的新进展。
