Nebulin-deficient mice exhibit shorter thin filament lengths and reduced contractile function in skeletal muscle

@article{Bang2006NebulindeficientME,
  title={Nebulin-deficient mice exhibit shorter thin filament lengths and reduced contractile function in skeletal muscle},
  author={M D Bang and Xiaodong Li and Ryan S. Littlefield and Shannon N. Bremner and Andrea K Thor and Kirk U. Knowlton and Richard L Lieber and Ju Chen},
  journal={The Journal of Cell Biology},
  year={2006},
  volume={173},
  pages={905 - 916}
}
Nebulin is a giant modular sarcomeric protein that has been proposed to play critical roles in myofibrillogenesis, thin filament length regulation, and muscle contraction. To investigate the functional role of nebulin in vivo, we generated nebulin-deficient mice by using a Cre knock-in strategy. Lineage studies utilizing this mouse model demonstrated that nebulin is expressed uniformly in all skeletal muscles. Nebulin-deficient mice die within 8-11 d after birth, with symptoms including… CONTINUE READING
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