Assessment of the feasibility of exon 45–55 multiexon skipping for duchenne muscular dystrophy
2008

Feasibility of Multiexon Skipping for Duchenne Muscular Dystrophy

Sample size: 12 publication Evidence: low

Author Information

Author(s): Laura van Vliet, Christa L de Winter, Judith CT van Deutekom, Gert-Jan B van Ommen, Annemieke Aartsma-Rus

Primary Institution: Leiden University Medical Center

Hypothesis

The splicing order might favor the induction of multiexon 45–55 skipping.

Conclusion

Current state of the art does not sufficiently support clinical development of multiexon skipping for DMD.

Supporting Evidence

  • Antisense-mediated exon skipping is a promising therapeutic approach for Duchenne muscular dystrophy.
  • Different mutations require skipping of different exons, making the therapy mutation specific.
  • The study found that multiexon skipping levels could not be increased beyond naturally occurring levels.

Takeaway

The study tried to skip parts of a gene to help kids with a muscle disease, but it didn't work as well as hoped.

Methodology

The feasibility of inducing multiexon 45–55 skipping was tested in control and patient muscle cell cultures using various AON cocktails.

Limitations

The levels of intended multiexon skips were minimal and comparable to untreated cells.

Participant Demographics

Myotube cultures from a healthy individual and two Duchenne patients.

Digital Object Identifier (DOI)

10.1186/1471-2350-9-105

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