Defects in muscle maturation contribute to spinal muscular atrophy.

What is it about?

In this article, we show that the muscle cells of spinal muscular atrophy mice show defective muscle-building mechanisms when observed in the petri dish and in muscles extracted. There is evidence that there is a delay in the developmental steps of muscle maturation using a various array of techniques. Treatment with Trichostatin A, an HDAC inhibitor similar to valproic acid once considered as a potential treatment, improves the maturation of the molecular markers involved in the maturation of the muscles.

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Why is it important?

At the time of the publication of the study, it offered the most comprehensive evidence that muscle defects independent of the neural defects may contribute to SMA pathogenesis.