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Expression of a Dominant Negative CELF Protein In Vivo Leads to Altered Muscle Organization, Fiber Size, and Subtype

By Dara S. Berger, Michelle Moyer, Gregory M. Kliment, Erik van Lunteren and Andrea N. Ladd
Topics: Research Article
Publisher: Public Library of Science
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Provided by: PubMed Central

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  24. (2007). Ladd A
  25. (2002). Loss of the muscle-specific chloride channel in type 1 myotonic dystrophy lead to misregulated alternative splicing.
  26. (1992). Mapping of myogenin transcription during embryogenesis using transgenes linked to the myogenin control region.
  27. (2001). Mice transgenic for the human myotonic dystrophy region with expanded CTG repeats display muscular and brain abnormalities.
  28. (2010). MicroRNAs coordinate an alternative splicingnetwork duringmouse postnatalheart development.GenesDev.
  29. (2006). Misregulation of alternative splicing causes pathogenesis in myotonic dystrophy.
  30. (2001). Molecular basis for impaired muscle differentiation in myotonic dystrophy.
  31. (1992). Molecular basis of myotonic dystrophy - expansion of a trinucleotide (CTG) repeat at the 3’ end of a transcript encoding a protein kinase family member.
  32. (1994). Muscle histopathology in myotonic dystrophy in relation to age and muscular weakness.
  33. (1992). Myotonic dystrophy mutation - an unstable CTG repeat in the 3’ untranslated region of the gene.
  34. (2004). Overexpression of CUG triplet repeat-binding protein, CUGBP1, in mice inhibits myogenesis.
  35. (2007). Physical continuity of the perimysium from myofibers to tendons: Involvement in lateral force transmission in skeletal muscle.
  36. (2001). RNA CUG repeats sequester CUGBP1 and alter protein levels and activity of CUGBP1.
  37. (2001). The CELF family of RNA binding proteins is implicated in cell-specific and developmentally regulated alternative splicing.
  38. (2005). Transgenic mice expressing CUG-BP1 reproduce splicing mis-regulation observed in myotonic dystrophy.