{"doi":"10.1002/jnr.20980","title":"ETR‐3 represses Tau exons 2/3 inclusion, a splicing event abnormally enhanced in myotonic dystrophy type I","abstract":"<jats:title>Abstract</jats:title><jats:p>Altered splicing of transcripts, including the insulin receptor (IR) and the cardiac troponin (cTNT), is a key feature of myotonic dystrophy type I (DM1). CELF and MBNL splicing factor members regulate the splicing of those transcripts. We have previously described an alteration of Tau exon 2 splicing in DM1 brain, resulting in the favored exclusion of exon 2. However, the factors required for alternative splicing of Tau exon 2 remain undetermined. Here we report a decreased expression of <jats:italic>CELF</jats:italic> family member and <jats:italic>MBNL</jats:italic> transcripts in DM1 brains as assessed by RT‐PCR. By using cellular models with a control‐ or DM1‐like splicing pattern of <jats:italic>Tau</jats:italic> transcripts, we demonstrate that ETR‐3 promotes selectively the exclusion of Tau exon 2. These results together with the analysis of Tau exon 6 and IR exon 11 splicing in brain, muscle, and cell models suggest that DM1 splicing alteration of several transcripts involves various factors. © 2006 Wiley‐Liss, Inc.</jats:p>","journal":"Journal of Neuroscience 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Neurodegenerative Diseases","Cardiomyopathy and Myosin Studies","Muscle Physiology and Disorders","Biology","Medicine","Alternative Splicing","Blotting, Western","Brain","CELF Proteins","Cell Line, Tumor","Electrophoresis, Polyacrylamide Gel","Exons","Humans","Middle Aged","Muscle, Skeletal","Myotonic Dystrophy","Nerve Tissue Proteins","Neuroblastoma","Nuclear Proteins","RNA, Messenger","RNA-Binding Proteins","Reverse Transcriptase Polymerase Chain Reaction","Transfection","tau Proteins"],"mesh_terms":["CELF Proteins","Brain","Electrophoresis, Polyacrylamide Gel","Exons","Humans","Middle Aged","Myotonic Dystrophy","Nerve Tissue Proteins","Neuroblastoma","Nuclear Proteins","RNA, Messenger","Transfection","Blotting, Western","RNA-Binding Proteins","tau Proteins","Alternative Splicing","Muscle, Skeletal","Reverse Transcriptase Polymerase Chain Reaction","Cell Line, Tumor"],"keywords":["Exon","RNA splicing","Myotonic dystrophy","Alternative splicing","Splicing factor","Exonic splicing 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