Transcription profiling of mouse expressing toxic UGexp RNA vs. Mbnl1 knockout and Clcn1 null mice to assess the post-transcriptional impact of toxic RNA in myotonic dystrophy

ABSTRACT: Myotonic dystrophy type 1 (DM1) is an RNA dominant disease in which mutant transcripts containing an expanded CUG repeat (CUGexp) cause muscle dysfunction by interfering with biogenesis of other mRNAs. The toxic effects of mutant RNA are mediated partly through sequestration of splicing regulator Muscleblind-like 1 (Mbnl1), a protein that binds to CUGexp RNA. A gene that is prominently affected encodes chloride channel 1 (Clcn1), resulting in hyperexcitability of muscle (myotonia). To identify DM1-affected genes and study mechanisms for dysregulation, we performed global mRNA profiling in transgenic mice that express CUGexp RNA, as compared to Mbnl1 knockout and Clcn1 null mice. We found that the majority of changes induced by CUGexp RNA in skeletal muscle can be explained by reduced activ

ORGANISM(S): Mus musculus

SUBMITTER: Stephen Welle 

PROVIDER: E-GEOD-14691 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress