Creation of a Novel Humanized Dystrophic Mouse Model of Duchenne Muscular Dystrophy and Application of a CRISPR/Cas9 Gene Editing Therapy

May 17, 2017Journal of neuromuscular diseases

New Human-Like Mouse Model for Duchenne Muscular Dystrophy and Testing of a Gene Editing Treatment

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Abstract

A novel dystrophic mouse model was developed by deleting exon 45 in the human DMD gene using CRISPR/Cas9.

  • Mutations in the DMD gene disrupt the reading frame, leading to Duchenne muscular dystrophy.
  • Therapeutic strategies, including exon skipping and CRISPR/Cas9, aim to restore the DMD reading frame.
  • The new mouse model allows for testing of these therapeutic strategies in a living organism.
  • Application of CRISPR/Cas9 targeting exons 45-55 in the model may restore dystrophin production.

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