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The RNA editing enzymes ADARs: mechanism of action and human disease

Cell and Tissue Research volume 356, pages 527–532 (2014)Cite this article

Abstract

A-to-I RNA editing is a ubiquitous and crucial molecular mechanism able to convert adenosines into inosines (then read as guanosines by several intracellular proteins/enzymes) within RNA molecules, changing the genomic information. The A-to-I deaminase enzymes (ADARs), which modify the adenosine, can alter the splicing and translation machineries, the double-stranded RNA structures and the binding affinity between RNA and RNA-binding proteins. ADAR activity is an essential mechanism in mammals and altered editing has been associated with several human diseases. Many efforts are now being concentrated on modifying ADAR activity in vivo in an attempt to correct RNA editing dysfunction. Concomitantly, ongoing studies aim to show the way that the ADAR deaminase domain can be used as a possible new tool, an intracellular Trojan horse, for the correction of heritage diseases not related to RNA editing events.

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Affiliations

  1. RNA Editing Laboratory, Oncohaematology Department, Bambino Gesù Children’s Hospital IRCCS, Piazza S. Onofrio 4, 00165, Rome, Italy

    Sara Tomaselli, Franco Locatelli & Angela Gallo

  2. Department of Pediatric Science, Università di Pavia, Strada Nuova 65, 27100, Pavia, Italy

    Franco Locatelli

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  1. Sara Tomaselli
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  2. Franco Locatelli
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  3. Angela Gallo
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Correspondence to Angela Gallo.

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Tomaselli, S., Locatelli, F. & Gallo, A. The RNA editing enzymes ADARs: mechanism of action and human disease. Cell Tissue Res 356, 527–532 (2014). https://doi.org/10.1007/s00441-014-1863-3

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  • DOI: https://doi.org/10.1007/s00441-014-1863-3

Keywords

  • RNA editing
  • ADARs
  • Inosine
  • Heterochromatin
  • Human diseases