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Biomolecules 2018, 8(4), 123; https://doi.org/10.3390/biom8040123

Pre-Ribosomal RNA Processing in Human Cells: From Mechanisms to Congenital Diseases

Laboratoire de Biologie Moléculaire Eucaryote, Centre de Biologie Intégrative (CBI), Université de Toulouse, CNRS, UPS, 31000 Toulouse, France
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Received: 27 September 2018 / Revised: 19 October 2018 / Accepted: 19 October 2018 / Published: 24 October 2018
(This article belongs to the Special Issue rRNA Biology)
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Abstract

Ribosomal RNAs, the most abundant cellular RNA species, have evolved as the structural scaffold and the catalytic center of protein synthesis in every living organism. In eukaryotes, they are produced from a long primary transcript through an intricate sequence of processing steps that include RNA cleavage and folding and nucleotide modification. The mechanisms underlying this process in human cells have long been investigated, but technological advances have accelerated their study in the past decade. In addition, the association of congenital diseases to defects in ribosome synthesis has highlighted the central place of ribosomal RNA maturation in cell physiology regulation and broadened the interest in these mechanisms. Here, we give an overview of the current knowledge of pre-ribosomal RNA processing in human cells in light of recent progress and discuss how dysfunction of this pathway may contribute to the physiopathology of congenital diseases. View Full-Text
Keywords: ribosomal RNAs (rRNAs); endonucleases; exonucleases; RNA processing; ribosomopathies; Diamond–Blackfan anemia; ribosomal stress ribosomal RNAs (rRNAs); endonucleases; exonucleases; RNA processing; ribosomopathies; Diamond–Blackfan anemia; ribosomal stress
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Aubert, M.; O’Donohue, M.-F.; Lebaron, S.; Gleizes, P.-E. Pre-Ribosomal RNA Processing in Human Cells: From Mechanisms to Congenital Diseases. Biomolecules 2018, 8, 123.

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