Defective neuromuscular junction organization and postnatal myogenesis in mice with severe spinal muscular atrophy.

@article{Dachs2011DefectiveNJ,
  title={Defective neuromuscular junction organization and postnatal myogenesis in mice with severe spinal muscular atrophy.},
  author={Elisabet Dachs and Marta Hereu and L{\'i}dia Piedrafita and A. Fern{\'a}ndez Casanovas and Jordi Calder{\'o} and Josep E. Esquerda},
  journal={Journal of neuropathology and experimental neurology},
  year={2011},
  volume={70 6},
  pages={
          444-61
        }
}
A detailed pathologic analysis was performed on Smn(-/-);SMN2 mice as a mouse model for human type I spinal muscular atrophy (SMA). We provide new data concerning changes in the spinal cord, neuromuscular junctions and muscle cells, and in the organs of the immune system. The expression of 10 synaptic proteins was analyzed in 3-dimensionally reconstructed neuromuscular junctions by confocal microscopy. In addition to defects in postsynaptic occupancy, there was a marked reduction in calcitonin… CONTINUE READING

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