Unknown,Transcriptomics,Genomics,Proteomics

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Transcription profiling of mouse dystrophin-deficient and dystrophin and utrophin double-deficient animalse crossed with mice with full-length hDMD genes


ABSTRACT: Crossing of hDMD mice that contain the full-length 2.3 Mb hDMD gene were crossed with dystrophin-deficient mdx mice and dystrophin and utrophin double-deficient mdx x utrn-/- mice resulted in a full rescue of the dystrophic features of these mice, as concluded from histological analysis. Analysis on Affymetrix gene chips demonstrated that also expression profiles of the dystrophic mice were normalized by crossing with transgenic hDMD mice. This confirms the full functionality of the hDMD transgene in mice. Experiment Overall Design: RNA from gastrocnemius muscle from individual mice was hybridized to Affymetrix U74Av2

ORGANISM(S): Mus musculus

SUBMITTER: Peter 't Hoen 

PROVIDER: E-GEOD-6790 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Generation and characterization of transgenic mice with the full-length human DMD gene.

't Hoen Peter A C PA   de Meijer Emile J EJ   Boer Judith M JM   Vossen Rolf H A M RH   Turk Rolf R   Maatman Ronald G H J RG   Davies Kay E KE   van Ommen Gert-Jan B GJ   van Deutekom Judith C T JC   den Dunnen Johan T JT  

The Journal of biological chemistry 20071213 9


We report the generation of mice with an intact and functional copy of the 2.3-megabase human dystrophin gene (hDMD), the largest functional stretch of human DNA thus far integrated into a mouse chromosome. Yeast spheroplasts containing an artificial chromosome with the full-length hDMD gene were fused with mouse embryonic stem cells and were subsequently injected into mouse blastocysts to produce transgenic hDMD mice. Human-specific PCR, Southern blotting, and fluorescent in situ hybridization  ...[more]

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