Study of the molecular biology of the calcium regulation of muscle contraction was initiated by Professor Ebashi's discovery of a protein factor that sensitized actomyosin to calcium ions. This protein factor was separated into two proteins: tropomyosin and a novel protein named troponin. Troponin is a Ca2+-receptive protein for the Ca2+-regulation of muscle contraction and, in association with tropomyosin, sensitizes actomyosin to Ca2+. Troponin forms an ordered regulatory complex with tropomyosin in the thin filament. Several regulatory properties of troponin, which is composed of three different components, troponins C, I, and T, are discussed in this article. Genetic studies have revealed that many mutations of genes for troponin components, especially troponins T and 1, are involved in the three types of inherited cardiomyopathy. Results of functional analyses indicate that changes in the Ca2+-sensitivity caused by troponin mutations are the critical functional consequences leading to these disorders. Recent results of this pathophysiological aspect of troponin are also discussed. (c) 2007 Elsevier Inc. All rights reserved.