ETR-3 represses Tau exons 2/3 inclusion, a splicing event abnormally enhanced in myotonic dystrophy type I
Authors
Leroy, OlivierDhaenens, Claire-Marie
Schraen-Maschke, Suzanna
Belarbi, Karim
Delacourte, Andre
Andreadis, Athena
Sablonniere, Bernard
Buee, Luc
Sergeant, Nicolas
Caillet-Boudin, Marie-Laure
UMass Chan Affiliations
Department of Cell BiologyDocument Type
Journal ArticlePublication Date
2006-07-25Keywords
Alternative SplicingBlotting, Western
Brain
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
Cell Biology
Metadata
Show full item recordAbstract
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.Source
J Neurosci Res. 2006 Sep;84(4):852-9. Link to article on publisher's siteDOI
10.1002/jnr.20980Permanent Link to this Item
http://hdl.handle.net/20.500.14038/25691PubMed ID
16862542Related Resources
Link to Article in PubMedae974a485f413a2113503eed53cd6c53
10.1002/jnr.20980