Repression of nuclear CELF activity can rescue CELF-regulated alternative splicing defects in skeletal muscle models of myotonic dystrophy

Myotonic dystrophy type 1 (DM1) is caused by the expansion of CUG repeats in the 3’ UTR of DMPK transcripts. DM1 pathogenesis has been attributed in part to alternative splicing dysregulation via elevation of CUG-BP, Elav-like family member 1 (CELF1). Several therapeutic approaches have been tested...

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Detalles Bibliográficos
Autores principales: Berger, Dara S., Ladd, Andrea N.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Public Library of Science 2012
Materias:
Muscular Dystrophy
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3286860/
https://www.ncbi.nlm.nih.gov/pubmed/22453899
http://dx.doi.org/10.1371/currents.RRN1305

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3286860/
https://www.ncbi.nlm.nih.gov/pubmed/22453899
http://dx.doi.org/10.1371/currents.RRN1305

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