Journal
JOURNAL OF MOLECULAR NEUROSCIENCE
Volume 32, Issue 2, Pages 120-131Publisher
HUMANA PRESS INC
DOI: 10.1007/s12031-007-0024-5
Keywords
spinal muscular atrophy; survival motor neuron; Smn knockdown; profilin IIa
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Spinal muscular atrophy (SMA) is the most common genetic disease resulting in infant mortality due to severe loss of a-motor neurons. SMA is caused by mutations or deletions of the ubiquitously expressed survival motor neuron (SMN) gene. However, why a-motor neurons of SMA patients are specifically affected is not clear. We demonstrate here that Smn knockdown in PC12 cells alters the expression pattern of profilin II, resulting in an increase in the neuronal-specific profilin IIa isoform. Moreover, the depletion of Smn, a known interacting partner of profilin IIa, further contributes to the increased profilin IIa availability. Altogether, this leads to an increased formation of ROCK/profilin IIa complex and an inappropriate activation of the RhoA/ROCK pathway,
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