期刊
JOURNAL OF NEUROSCIENCE RESEARCH
卷 84, 期 4, 页码 852-859出版社
WILEY
DOI: 10.1002/jnr.20980
关键词
myotonic dystrophy; splicing; microtubule-associated Tau; insulin receptor; ETR-3
资金
- NICHD NIH HHS [P01 HD05515] Funding Source: Medline
- NINDS NIH HHS [R01 NS38051] Funding Source: Medline
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. (c) 2006 Wiley-Liss, Inc.
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