Neuronal <scp>RNA</scp>‐binding proteins in health and disease

  • Silvia Carolina Lenzken
    Department of Biotechnology and Biosciences University of Milano‐Bicocca Milan Italy
  • Tilmann Achsel
    VIB Center for the Biology of Disease and Department of Molecular and Developmental Genetics KU Leuven Leuven Belgium
  • Maria Teresa Carrì
    Laboratory of Neurochemistry Fondazione Santa Lucia IRCCS Rome Italy
  • Silvia M.L. Barabino
    Department of Biotechnology and Biosciences University of Milano‐Bicocca Milan Italy

説明

<jats:p>In mammalian cells in general and in neurons in particular, <jats:styled-content style="fixed-case">mRNA</jats:styled-content> maturation, translation, and degradation are highly complex and dynamic processes. <jats:styled-content style="fixed-case">RNA</jats:styled-content>‐binding proteins (<jats:styled-content style="fixed-case">RBPs</jats:styled-content>) play crucial roles in all these events. First, they participate in the choice of pre‐<jats:styled-content style="fixed-case">mRNA</jats:styled-content> splice sites and in the selection of the polyadenylation sites, determining which of the possible isoforms is produced from a given precursor <jats:styled-content style="fixed-case">mRNA</jats:styled-content>. Then, once in the cytoplasm, the protein composition of the <jats:styled-content style="fixed-case">RNP</jats:styled-content> particles determines whether the mature <jats:styled-content style="fixed-case">mRNA</jats:styled-content> 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 <jats:styled-content style="fixed-case">RBPs</jats:styled-content> are associated with various neurological diseases. In this review, we illustrate how individual <jats:styled-content style="fixed-case">RBPs</jats:styled-content> determine the fate of an <jats:styled-content style="fixed-case">mRNA</jats:styled-content>, and we discuss how mutations in <jats:styled-content style="fixed-case">RBPs</jats:styled-content> or perturbations of the <jats:styled-content style="fixed-case">mRNA</jats:styled-content> metabolism can cause neurodegenerative disorders. <jats:italic>WIREs RNA</jats:italic> 2014, 5:565–576. doi: 10.1002/wrna.1231</jats:p><jats:p>This article is categorized under: <jats:list list-type="explicit-label"> <jats:list-item><jats:p>RNA Interactions with Proteins and Other Molecules > Protein–RNA Recognition</jats:p></jats:list-item> <jats:list-item><jats:p>RNA Interactions with Proteins and Other Molecules > Protein–RNA Interactions: Functional Implications</jats:p></jats:list-item> <jats:list-item><jats:p>RNA in Disease and Development > RNA in Disease</jats:p></jats:list-item> </jats:list></jats:p>

収録刊行物

  • WIREs RNA

    WIREs RNA 5 (4), 565-576, 2014-03-28

    Wiley

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