Transcription profiling of mouse wild type embryonic stem cells after blastocytst injection revealglobal corrections in MDX mice.

ABSTRACT: Duchenne muscular dystrophy (DMD) is an incurable neuromuscular degenerative disease, caused by a mutation in the dystrophin gene. Mdx mice recapitulate DMD features. Here we show that injection of wild-type (WT) embryonic stem cells (ESCs) into mdx blastocysts produces mice with improved pathology. A small fraction of WT ESCs incorporates into the mdx mouse nonuniformly to upregulate protein levels of dystrophin in the skeletal muscle. The chimeric muscle shows reduced regeneration and restores dystrobrevin, a dystrophin-related protein, in areas with high and with low dystrophin content. WT ESC injection also normalizes the amount of fat, a tissue that does not express dystrophin. ESC injection without dystrophin does not prevent the appearance of phenotypes in the skeletal muscle or in

ORGANISM(S): Mus musculus

SUBMITTER: Agnes Viale 

PROVIDER: E-GEOD-12580 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress