Is good housekeeping the key to motor neuron survival?
- PMID: 18485864
- DOI: 10.1016/j.cell.2008.05.002
Is good housekeeping the key to motor neuron survival?
Abstract
Spinal muscular atrophy (SMA) is caused by a drastic reduction in the ubiquitously expressed SMN protein, which is critical for the correct assembly of the snRNP complexes required for RNA splicing. However, it is unclear why loss of SMN and altered snRNP assembly only seem to affect motor neurons. Reporting in this issue, Zhang et al. (2008) challenge prior assumptions about the housekeeping function of SMN and demonstrate that loss of SMN leads to highly tissue-specific effects on splicing.
Comment on
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SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing.Cell. 2008 May 16;133(4):585-600. doi: 10.1016/j.cell.2008.03.031. Cell. 2008. PMID: 18485868 Free PMC article.
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