Spatio/temporal regulation of mRNA processing
RNA processing in neurons emerges as a critical issue in neurodegeneration as exemplified with proteins like TDP-43, FUS and SMN which are RNA-binding proteins involved in amyotrophic lateral sclerosis (ALS), some types of frontotemporal lobar degeneration (FTLD) and spinal muscular atrophy (SMA), respectively. Interestingly, TDP-43, Fus and SMN are mostly nuclear RNA-binding proteins involved in RNA-splicing which shuttle in the cytoplasm and can associate with stress granules under stress conditions. These observations have boosted the investigations to probe the formation of stress granules in neurons and their putative relation with protein aggregation (TDP-43 and FUS can form cytoplasmic inclusions during neurodegeneration). The role of deregulated RNA splicing remains also to be clarified since proteins like FUS and TDP-43 can be trapped in cytoplasmic stress granules.