Inês do Carmo G. Gonçalves

TL;DR: It is reported that reduced SMN protein level alters miRNA expression and distribution in neurons, and it is demonstrated that miR-183 regulates translation of mTor via direct binding to its 3' UTR.

TL;DR: Current knowledge about the role of miRNAs in motor neuron disorders, especially about two common diseases: SMA and ALS is reviewed.

TL;DR: Results suggest decreased SMN leads to defective microRNA function via MEL-46 misregulation, followed by increased m2R expression, and neuronal dysfunction in SMA.

TL;DR: It is reported that expression of DROSHA, which is a critical enzyme in the microprocessor complex and essential for miRNA biogenesis, is reduced in motor neurons from an SMA mouse model, and this suggests that autophagy regulates RNA metabolism and neuronal growth via the DROSha/miRNA pathway and this pathway is dysregulated in SMA.

TL;DR: Current findings on ALS and SMA have similar cellular pathological mechanisms including mitochondrial dysfunction, oxidative stress and dysregulation in RNA-mediated gene expression, with specific focus on miRNA expression, pre-mRNA processing and RNA binding proteins.