Abstract：
:Spinal muscular atrophy (SMA), the leading genetic cause of infant death results from loss of spinal motor neurons causing atrophy of skeletal muscle. SMA is caused by loss of the Survival Motor Neuron 1 (SMN1) gene, however, an identically coding gene called SMN2 is retained, but is alternatively spliced to produce approximately 90% truncated protein. Most SMA translational and preclinical drug development has relied on the use of SMA mice to determine changes in SMN protein levels. However, the SMA mouse models are relatively severe and analysis of SMN-inducing compounds is confounded by the early mortality of these animals. An antibody that could detect SMN protein on a Smn background could circumvent this limitation and allow unaffected, heterozygous animals to be examined. Here we describe the generation and characterization of a monoclonal anti-SMN antibody, 4F11, which specifically recognizes human SMN protein. 4F11 detects SMN (human) but not native Smn (mouse) protein in SMN2 transgenic mice and in SMA cell lines. We demonstrate the feasibility of using 4F11 to detect changes in SMN2-derived SMN protein in SMA patient fibroblasts and in healthy SMN2 transgenic mice. This antibody is, therefore, an excellent tool for examining SMN2-inducing therapeutics in patient cells as well as in transgenic mice.
journal_name
J Neurosci Methodsjournal_title
Journal of neuroscience methodsauthors
Mattis VB,Butchbach ME,Lorson CLdoi
10.1016/j.jneumeth.2008.07.024subject
Has Abstractpub_date
2008-10-30 00:00:00pages
36-43issue
1eissn
0165-0270issn
1872-678Xpii
S0165-0270(08)00454-8journal_volume
175pub_type
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journal_title：Journal of neuroscience methods
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journal_title：Journal of neuroscience methods
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journal_title：Journal of neuroscience methods
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journal_title：Journal of neuroscience methods
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