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TDP-43 toxicity and the usefulness of junk

A new study shows that loss of the lariat debranching enzyme Dbr1 suppresses TDP-43 toxicity. The accumulated intronic lariat RNAs, which are normally degraded after splicing, likely act as decoys to sequester TDP-43 away from binding to and disrupting functions of other RNAs.

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Figure 1: Model for suppression of TDP-43 toxicity following loss of Dbr1.

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Correspondence to Don W Cleveland.

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Sun, S., Cleveland, D. TDP-43 toxicity and the usefulness of junk. Nat Genet 44, 1289–1291 (2012). https://doi.org/10.1038/ng.2473

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