Transcriptome patterns show normalization of muscle gene expression after restoration of dystrophin in MDX mice by exon skipping
Adams, A.M., Fletcher, S., Ly, T., Johnsen, R. and Wilton, S. (2009) Transcriptome patterns show normalization of muscle gene expression after restoration of dystrophin in MDX mice by exon skipping. In: 33rd HGSA Annual Scientific Meeting, 3 - 6 May 2009, Fremantle, Western Australia.
Duchenne muscular dystrophy (DMD) is a recessive X-linked form of muscular dystrophy caused by protein-truncating mutations in the dystrophin gene. It is characterized by rapid progression of muscle degeneration, eventually leading to loss of ambulation and death from respiratory or cardiac complications. The mdx mouse model of muscular dystrophy has a nonsense mutation in exon 23 of the dystrophin gene. We have previously shown that a morpholino antisense oligomer can induce removal of this exon, and result in an in-frame mRNA transcript encoding a shortened but functional dystrophin protein. In-order to further investigate the molecular processes associated with dystrophin restoration, we used Genechip Exon Arrays to compare expression levels in the diaphragm of 10 week-old treated mdx mice to that of age-matched mdx and C57Bl/10 control mice. Quality control and statistical analysis were necessary to normalise microarray data. A one-way ANOVA and fold changes were used to compile gene lists. These genes were categorized based on gene ontology classification; for example, signal transduction, ion transport etc, and then analysed in more detail. The data arising from this study should provide a greater understanding about gene interactions and their affects on pathways that are involved in muscle regeneration, and may contribute to the development of new therapies for DMD.
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