RNA toxicity in non‐coding repeat expansion disorders
نویسندگان
چکیده
منابع مشابه
RNA-mediated neurodegeneration in repeat expansion disorders.
Most neurodegenerative disorders are thought to result primarily from the accumulation of misfolded proteins, which interfere with protein homeostasis in neurons. For a subset of diseases, however, noncoding regions of RNAs assume a primary toxic gain-of-function, leading to degeneration in many tissues, including the nervous system. Here we review a series of proposed mechanisms by which nonco...
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Evidence for an RNA gain-of-function toxicity has now been provided for an increasing number of human pathologies. Myotonic dystrophies (DM) belong to a class of RNA-dominant diseases that result from RNA repeat expansion toxicity. Specifically, DM of type 1 (DM1), is caused by an expansion of CUG repeats in the 3'UTR of the DMPK protein kinase mRNA, while DM of type 2 (DM2) is linked to an exp...
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ژورنال
عنوان ژورنال: The EMBO Journal
سال: 2019
ISSN: 0261-4189,1460-2075
DOI: 10.15252/embj.2018101112