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Neuronal RNA‐binding proteins in health and disease

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In mammalian cells in general and in neurons in particular, mRNA maturation, translation, and degradation are highly complex and dynamic processes. RNA‐binding proteins (RBPs) play crucial roles in all these events. First, they participate in the choice of pre‐mRNA splice sites and in the selection of the polyadenylation sites, determining which of the possible isoforms is produced from a given precursor mRNA. Then, once in the cytoplasm, the protein composition of the RNP particles determines whether the mature mRNA is transported along the dendrites or the axon of a neuron to the synapses, how efficiently it is translated, and how stable it is. In agreement with their importance for neuronal function, mutations in genes that code for RBPs are associated with various neurological diseases. In this review, we illustrate how individual RBPs determine the fate of an mRNA, and we discuss how mutations in RBPs or perturbations of the mRNA metabolism can cause neurodegenerative disorders. WIREs RNA 2014, 5:565–576. doi: 10.1002/wrna.1231 This article is categorized under: RNA Interactions with Proteins and Other Molecules > Protein–RNA Recognition RNA Interactions with Proteins and Other Molecules > Protein–RNA Interactions: Functional Implications RNA in Disease and Development > RNA in Disease

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RNA‐binding proteins (RNPs) determine the life of an mRNA. Specialized RBPs regulate mRNA biogenesis. In the nucleus, transcripts are cotranscriptionally capped, spliced, polyadenylated, and finally exported to the cytoplasm as ribonucleoprotein particles (mRNPs). In the mRNPs, the transcript is stabilized and protected from undesired degradation while translation is repressed. The mRNPs interact with cytoskeletal proteins and molecular motors contributing to mRNA trafficking and delivery to dendritic spines and synapses, where they finally participate in translation.
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Defects in RNA metabolism are observed in ribonucleopathies. Ribonucleopathies are a newly defined group of diseases that is characterized by defects in the protein components of ribonucleoprotein complexes. They can be divided into four groups according to their most relevant pathological mechanism (represented in colored boxes). In most cases, the alterations result in a loss of function of the RNPs, although in some cases a toxic gain of function cannot be ruled out. The figure shows selected examples of diseases for each category, as well as the RBP implicated in the pathology.
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RNA Interactions with Proteins and Other Molecules > Protein–RNA Interactions: Functional Implications
RNA Interactions with Proteins and Other Molecules > Protein–RNA Recognition
RNA in Disease and Development > RNA in Disease

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