Figure 1.

Dysfunction of RNA-binding proteins has profound effects on the neuronal transcriptome. RNA-binding proteins precisely regulate mRNA processing, stability, transport and translation to meet critical functions in neurodevelopment, synaptic function, and plasticity. In this review, we discuss how the RNA-binding proteins, RBFOX1, FMRP, SMN, TDP-43 and FUS, regulate myriad aspects of RNA metabolism and impact neurodevelopment, synapse homeostasis, and the neuronal cytoskeleton. The figure depicts the nuclear localization and functions of RBFOX1, TDP-43 and FUS, whereas SMN and FMRP are mainly localized in the cytoplasm. SMN also is a component of Cajal bodies in the nucleus. RNA-binding proteins are components of neuronal ribonucleoprotein granules that function in the bidirectional transport, localization and/or translation of mRNAs in the dendrites and axon