Neuroscience Bulletin

, Volume 30, Issue 4, pp 610–626

RNA binding proteins: a common denominator of neuronal function and dysfunction

Authors

    • Laboratory of Molecular and Cellular Neuroscience, Center of Basic NeuroscienceBiomedical Research Foundation of the Academy of Athens
Review

DOI: 10.1007/s12264-014-1443-7

Cite this article as:
Doxakis, E. Neurosci. Bull. (2014) 30: 610. doi:10.1007/s12264-014-1443-7

Abstract

In eukaryotic cells, gene activity is not directly reflected by protein levels because mRNA processing, transport, stability, and translation are co- and post-transcriptionally regulated. These processes, collectively known as the ribonome, are tightly controlled and carried out by a plethora of trans-acting RNA-binding proteins (RBPs) that bind to specific cis elements throughout the RNA sequence. Within the nervous system, the role of RBPs in brain function turns out to be essential due to the architectural complexity of neurons exemplified by a relatively small somal size and an extensive network of projections and connections. Thus far, RBPs have been shown to be indispensable for several aspects of neurogenesis, neurite outgrowth, synapse formation, and plasticity. Consequently, perturbation of their function is central in the etiology of an ever-growing spectrum of neurological diseases, including fragile X syndrome and the neurodegenerative disorders frontotemporal lobar degeneration and amyotrophic lateral sclerosis.

Keywords

alternative polyadenylationalternative splicingamyotrophic lateral sclerosisanti-Hu syndromeCPEBELAVfragile X syndromeFMRPFUSHUHuBHuCHuDHuRneuronneurodegenerationNova-1Nova-2paraneoplastic opsoclonus-myoclonus ataxiaPTBP-2PTBP-1TDP-43FTLD

Copyright information

© Shanghai Institutes for Biological Sciences, CAS and Springer-Verlag Berlin Heidelberg 2014