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Biochem Biophys Res Commun. 2008 Apr 25;369(1):62-73. Epub 2007 Dec 26.

Troponin: regulatory function and disorders.

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1
Department of Cell Physiology, Jikei University School of Medicine, 3-25-8, Nishi-shimbashi, Minato-ku, Tokyo 105-8461, Japan.

Abstract

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 Ca(2+)-receptive protein for the Ca(2+)-regulation of muscle contraction and, in association with tropomyosin, sensitizes actomyosin to Ca(2+). 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 I, are involved in the three types of inherited cardiomyopathy. Results of functional analyses indicate that changes in the Ca(2+)-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.

PMID:
18154728
DOI:
10.1016/j.bbrc.2007.11.187
[Indexed for MEDLINE]
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