Co-regulation of survival of motor neuron and Bcl-xL expression: implications for neuroprotection in spinal muscular atrophy.

@article{Anderton2012CoregulationOS,
  title={Co-regulation of survival of motor neuron and Bcl-xL expression: implications for neuroprotection in spinal muscular atrophy.},
  author={Ryan S. Anderton and Lori Lyn Price and Bradley J. Turner and Bruno P Meloni and Chalermchai Mitrpant and Francis L Mastaglia and C Goh and Stephen D. Wilton and Sherif Boulos},
  journal={Neuroscience},
  year={2012},
  volume={220},
  pages={228-36}
}
Spinal muscular atrophy (SMA), a fatal genetic motor disorder of infants, is caused by diminished full-length survival of motor neuron (SMN) protein levels. Normally involved in small nuclear ribonucleoprotein (snRNP) assembly and pre-mRNA splicing, recent studies suggest that SMN plays a critical role in regulating apoptosis. Interestingly, the anti-apoptotic Bcl-x isoform, Bcl-xL, is reduced in SMA. In a related finding, Sam68, an RNA-binding protein, was found to modulate splicing of SMN and… CONTINUE READING