Transfer of full-length Dmd to the diaphragm muscle of Dmd(mdx/mdx) mice through systemic administration of plasmid DNA

Mol Ther. 2001 Jul;4(1):45-51. doi: 10.1006/mthe.2001.0419.

Abstract

Mutations in the gene encoding dystrophin, a large cytoskeletal protein in muscle, lead to Duchenne muscular dystrophy (DMD). Affected individuals often die of respiratory failure resulting primarily from diaphragm muscle degeneration. Here we report a new procedure to transfer the full-length dystrophin cDNA into the diaphragm muscle of Dmd(mdx/mdx) mice, which carry a mutation in the dystrophin gene (Dmd). Significant gene transfer was found after intravenous injection of naked plasmid DNA followed by a brief (eight second) occlusion of blood flow at the vena cava. This is the first demonstration of gene transfer into the diaphragm muscle through systemic administration of naked plasmid DNA. The approach has potential application for treatment of DMD.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Diaphragm*
  • Dystrophin / genetics*
  • Gene Expression
  • Gene Transfer Techniques*
  • Genetic Therapy / methods
  • Genetic Vectors*
  • Immunohistochemistry
  • Injections, Intravenous
  • Male
  • Mice
  • Mice, Inbred mdx
  • Muscular Dystrophy, Animal / genetics
  • Muscular Dystrophy, Animal / metabolism
  • Muscular Dystrophy, Animal / pathology
  • Muscular Dystrophy, Animal / therapy*
  • Plasmids* / administration & dosage
  • Plasmids* / metabolism
  • Time Factors
  • Tissue Distribution

Substances

  • Dystrophin