Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophy.

@article{Ling2012SevereND,
  title={Severe neuromuscular denervation of clinically relevant muscles in a mouse model of spinal muscular atrophy.},
  author={Karen K. Y. Ling and Rebecca M. Gibbs and Zhihua Feng and Chien-Ping Ko},
  journal={Human molecular genetics},
  year={2012},
  volume={21 1},
  pages={
          185-95
        }
}
Spinal muscular atrophy (SMA), a motoneuron disease caused by a deficiency of the survival of motor neuron (SMN) protein, is characterized by motoneuron loss and muscle weakness. It remains unclear whether widespread loss of neuromuscular junctions (NMJs) is involved in SMA pathogenesis. We undertook a systematic examination of NMJ innervation patterns in >20 muscles in the SMNΔ7 SMA mouse model. We found that severe denervation (<50% fully innervated endplates) occurs selectively in many… CONTINUE READING

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