期刊
BIOCHEMISTRY-MOSCOW
卷 85, 期 SUPPL 1, 页码 20-33出版社
MAIK NAUKA/INTERPERIODICA/SPRINGER
DOI: 10.1134/S0006297920140023
关键词
tropomyosin; homodimers and heterodimers; regulation of muscle contraction; myopathies and cardiomyopathies; point mutations in tropomyosin genes
资金
- Russian Foundation for Basic Research [17-00-00065]
The review is devoted to tropomyosin (Tpm)-actin-binding protein, which plays a crucial role in the regulation of contraction of skeletal and cardiac muscles. Special attention is paid to myopathies and cardiomyopathies-severe hereditary diseases of skeletal and cardiac muscles associated with point mutations in Tpm genes. The current views on the molecular mechanisms of these diseases and the effects of such mutations on the Tpm structure and functions are considered in detail. Besides, some part of the review is devoted to analysis of the properties of Tpm homodimers and heterodimers with myopathic substitutions of amino acid residues in only one of the two chains of the Tpm dimeric molecule.
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