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Human Molecular Genetics Advance Access originally published online on July 3, 2008
Human Molecular Genetics 2008 17(18):2900-2909; doi:10.1093/hmg/ddn189
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© The Author 2008. Published by Oxford University Press. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Embryonic motor axon development in the severe SMA mouse

Vicki L. McGovern1, Tatiana O. Gavrilina1,2, Christine E. Beattie3 and Arthur H.M. Burghes1,*

1 Department of Molecular and Cellular Biochemistry 2 Department of Neurology 3 Department of Neuroscience and Center for Molecular Neurobiology, The Ohio State University, Columbus, OH 43210, USA

* To whom correspondence should be addressed at: Department of Molecular and Cellular Biochemistry, The Ohio State University, 363 Hamilton Hall, 1645 Neil Ave, Columbus, OH 43210, USA. Tel: +1 6146884759; Fax: +1 6142924118; Email: burghes.1{at}osu.edu

Received May 16, 2008; Accepted July 1, 2008

Spinal muscular atrophy (SMA) is caused by reduced levels of survival motor neuron (SMN) protein. Previously, cultured SMA motor neurons showed reduced growth cone size and axonal length. Furthermore, reduction of SMN in zebrafish resulted in truncation followed by branching of motor neuron axons. In this study, motor neurons labeled with green fluorescent protein (GFP) were examined in SMA mice from embryonic day 10.5 to postnatal day 2. SMA motor axons showed no defect in axonal formation or outgrowth at any stage of development. However, a significant increase in synapses lacking motor axon input was detected in embryonic SMA mice. Therefore, one of the earliest detectable morphological defects in the SMA mice is the loss of synapse occupation by motor axons. This indicates that in severe SMA mice there are no defects in motor axon formation however, we find evidence of denervation in embryogenesis.


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