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. 2015 Sep 14;34(21):2601–2603. doi: 10.15252/embj.201592805

Figure 1. Potential link between dysregulated miRNA biogenesis and ALS .

Figure 1

(A) Under normal physiological conditions, pre‐miRNAs are by default processed by the Dicer complex in the cytosol. The resulting mature miRNA binds to specific target mRNAs, thereby blocking protein production. Interactions of Dicer with RBPs (e.g. TDP‐43) can enhance miRNA biogenesis. (B) In ALS, mutations in multiple genes (including TDP‐43, FUS, and SOD1) have been found to activate a stress response involving phosphorylation of eIF2α and SG formation. Remodeling of the Dicer complex leads to reduced pre‐miRNA processing and concomitantly decreased miRNA levels. As a consequence, the translation of target mRNAs is aberrantly relieved from inhibition by miRNAs, ending in degeneration of motor neurons.