Years ago the lack of the survival motor neuron (SMN) protein was linked to Spinal Muscular Atrophy, opening a path to a possible cure for this devastating disease. Many questions on how precisely the disease works still remain unanswered.
A study published in the April 11, 2012 issue of The Journal of Neuroscience showed, that missing SMN protein also causes decreased levels of plastin 3 (PLS3) protein.
Furthermore adding PLS3 back to motor neurons in zebrafish that were genetically altered so they couldn't produce SMN increased the spontaneous swimming and turning. The recovery of motor function was not complete though, so adding PLS3 alone is not a therapeutic option. But further defining this protein's role increases understanding of how spinal muscular atrophy develops.
A study published in the April 11, 2012 issue of The Journal of Neuroscience showed, that missing SMN protein also causes decreased levels of plastin 3 (PLS3) protein.
Furthermore adding PLS3 back to motor neurons in zebrafish that were genetically altered so they couldn't produce SMN increased the spontaneous swimming and turning. The recovery of motor function was not complete though, so adding PLS3 alone is not a therapeutic option. But further defining this protein's role increases understanding of how spinal muscular atrophy develops.
"What all is lost when SMN is lost? That's something we're still struggling with," said Christine Beattie, associate professor of neuroscience at Ohio State and lead author of the study.
"We think part of the motor neuron defects that are seen in spinal muscular atrophy are caused by this decrease in plastin 3 we get when SMN is lowered. And when we add plastin 3 back to motor neurons we can rescue defects that are seen when SMN is decreased, suggesting that a decrease in plastin 3 is contributing to some of the disease's characteristics."
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