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<p>Abstract</p> <p>Background</p> <p>Spinal Muscular Atrophy (SMA) is the leading genetic cause of infantile death. It is caused by the loss of functional <it>Survival Motor Neuron 1 </it>(<it>SMN1</it>). There is a nearly identical copy gene, <it>SMN2</it>, but it is unable to rescue from disease due to an alternative splicing event that excises a necessary exon (exon 7) from the majority of <it>SMN2</it>-derived transcripts. While SMNΔ7 protein has severely reduced functionality, the exon 7 sequences may not be specifically required for all activities. Therefore, aminoglycoside antibiotics previously shown to suppress stop codon recognition and promote translation read-through have been examined to increase the length of the SMNΔ7 C-terminus.</p> <p>Results</p> <p>Here we demonstrate that subcutaneous-administration of a read-through inducing compound (TC007) to an intermediate SMA model (<it>Smn</it>-/-; <it>SMN2</it>+/+; SMNΔ7) had beneficial effects on muscle fiber size and gross motor function.</p> <p>Conclusion</p> <p>Delivery of the read-through inducing compound TC007 reduces the disease-associated phenotype in SMA mice, however, does not significantly extend survival.</p>