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Age of Onset of RNA Toxicity Influences Phenotypic Severity: Evidence from an Inducible Mouse Model of Myotonic Dystrophy (DM1)

Myotonic dystrophy type 1 (DM1) is the most common muscular dystrophy in adults. It is caused by an expanded (CTG)n tract in the 3′ UTR of the Dystrophia Myotonica Protein Kinase (DMPK) gene. This causes nuclear retention of the mutant mRNA into ribonuclear foci and sequestration of interacting RNA-...

Ausführliche Beschreibung

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Bibliographische Detailangaben
Hauptverfasser: Gladman, Jordan T., Mandal, Mahua, Srinivasan, Varadamurthy, Mahadevan, Mani S.
Format: Artigo
Sprache:Inglês
Veröffentlicht: Public Library of Science 2013
Schlagworte:
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC3764231/
https://ncbi.nlm.nih.gov/pubmed/24039817
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0072907
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