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ETR‐3 represses Tau exons 2/3 inclusion, a splicing event abnormally enhanced in myotonic dystrophy type I
Author(s) -
Leroy Olivier,
Dhaenens ClaireMarie,
SchraenMaschke Suzanna,
Belarbi Karim,
Delacourte André,
Andreadis Athena,
Sablonnière Bernard,
Buée Luc,
Sergeant Nicolas,
CailletBoudin MarieLaure
Publication year - 2006
Publication title -
journal of neuroscience research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.72
H-Index - 160
eISSN - 1097-4547
pISSN - 0360-4012
DOI - 10.1002/jnr.20980
Subject(s) - exon , rna splicing , myotonic dystrophy , alternative splicing , splicing factor , exonic splicing enhancer , biology , minigene , intron , genetics , rna , gene
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 CELF family member and MBNL transcripts in DM1 brains as assessed by RT‐PCR. By using cellular models with a control‐ or DM1‐like splicing pattern of Tau 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.