Authors: Zhexin Wang, Michael Grange, Sabrina Pospich, Thorsten Wagner, Ay Lin Kho, Mathias Gautel, Stefan Raunser
Summary: Thin and thick actomyosin filaments are the key components of muscle. In skeletal muscle, the protein nebulin is essential for the length and strength of the thin filaments, with mutations of nebulin often leading to muscle diseases called nemaline myopathies. Wang et al. used cryo–electron tomography to identify nebulin integrated within the thin filament of native skeletal muscles. The authors determined a near-atomic in situ structure of nebulin and showed how it stabilizes thin filaments and functions as a “molecular ruler. ” The structure of nebulin along thin filaments is key to understanding the pathogenicity of nemaline myopathies.
Source: Science, 2022; 375 (6582)