Nebulin binding impedes mutant desmin filament assembly

@inproceedings{Baker2013NebulinBI,
  title={Nebulin binding impedes mutant desmin filament assembly},
  author={Laura K. Baker and David C. Gillis and Sarika Sharma and Andy Ambrus and Hartmut Herrmann and Gloria M. Conover},
  booktitle={Molecular biology of the cell},
  year={2013}
}
Desmin intermediate filaments (DIFs) form an intricate meshwork that organizes myofibers within striated muscle cells. The mechanisms that regulate the association of desmin to sarcomeres and their role in desminopathy are incompletely understood. Here we compare the effect nebulin binding has on the assembly kinetics of desmin and three desminopathy-causing mutant desmin variants carrying mutations in the head, rod, or tail domains of desmin (S46F, E245D, and T453I). These mutants were chosen… CONTINUE READING

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