Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons.

@article{Lee2011MusclesIA,
  title={Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons.},
  author={Young Il Lee and Michelle A Mikesh and Ian Nimmo Smith and Mendell Rimer and Wesley J. Thompson},
  journal={Developmental biology},
  year={2011},
  volume={356 2},
  pages={
          432-44
        }
}
A mouse model of the devastating human disease "spinal muscular atrophy" (SMA) was used to investigate the severe muscle weakness and spasticity that precede the death of these animals near the end of the 2nd postnatal week. Counts of motor units to the soleus muscle as well as of axons in the soleus muscle nerve showed no loss of motor neurons. Similarly, neither immunostaining of neuromuscular junctions nor the measurement of the tension generated by nerve stimulation gave evidence of any… CONTINUE READING
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