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PreprintCited by 98RNA and protein synthesis mechanismsNeurogenetic and Muscular Disorders ResearchAmyotrophic Lateral Sclerosis Research

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SMN-primed ribosomes modulate the translation of transcripts related to Spinal Muscular Atrophy

Open access
Lauria, F, Bernabò, P, Tebaldi, T, Groen, EJN, Perenthaler, E, Clamer, M, Maniscalco, F, Marchioretto, M, Orri, Julia, Dalla Serra, M, Inga, A, Quattrone, A, Gillingwater, TH, Viero, G·2019·Nature Cell Biology
DOI 10.1101/751701PMC PMC7610479

Preprint — published as 10.1038/s41556-020-00577-7 · Paper page

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Abstract

The contribution of ribosome heterogeneity and ribosome-associated factors to the molecular control of proteomes in health and disease remains enigmatic. We demonstrate that Survival Motor Neuron (SMN) protein, loss of which causes the neuromuscular disease spinal muscular atrophy (SMA), binds to ribosomes and that this interaction is tissue-dependent. SMN-primed ribosomes are positioned within the first five codons of a set of mRNAs which are enriched in IRES-like sequences in the 5’UTR and rare codons at the beginning of their coding sequence. Loss of SMN at early-stages of SMA induces translational defects in vivo, characterized by ribosome depletion in rare codons at the third and fifth position of the coding sequence. These positional defects cause ribosome depletion from mRNAs bound by SMN-primed ribosomes and translational impairment of proteins involved in motor neuron function and stability, including acetylcholinesterase. Thus, SMN plays a crucial role in the regulation of ribosome fluxes along mRNAs which encode proteins relevant to SMA pathogenesis.

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Metadata

Authors
Lauria, F., Bernabò, P., Tebaldi, T., Groen, E., Perenthaler, E., Clamer, M., Maniscalco, F., Marchioretto, M., Orri, J., Dalla Serra, M., Inga, A., Quattrone, A., Gillingwater, T., & Viero, G.
Affiliations
  • National Research Council (IT)
  • University of Trento (IT)
  • Erasmus MC (NL)
  • Fundació Jesuïtes Educació (ES)
Corresponding author
Viero, G
ORCID
Venue
Nature Cell Biology
Publisher
openRxiv
Published
2019-09-01
Volume
22
Issue
10
Pages
1239-1251
ISSN
1465-7392 (ISSN-L)
DOI
10.1101/751701
PMC
PMC7610479
OpenAlex
W3087754470
Cited by
98
References
1
Citations by year
2026: 8 · 2025: 20 · 2024: 20 · 2023: 16 · 2022: 19 · 2021: 13 · 2020: 2
FWCI
4.05
Type
journal article
Language
en
Open access
Locations
  • Nature Cell Biology (publishedVersion)
  • bioRxiv (Cold Spring Harbor Laboratory) (acceptedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
  • EUR Research Repository (Erasmus University Rotterdam) (submittedVersion)
  • Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
  • Institutional Research Information System (Università degli Studi di Trento) (submittedVersion)
  • Edinburgh Research Explorer (University of Edinburgh) (submittedVersion, other-oa)
Funders
  • University of Bern
  • Wellcome Trust (106098/Z/14/Z, 106098)
  • University of Oxford
  • University of Edinburgh
  • European Cooperation in Science and Technology (CA15126)
  • Fondazione Telethon (GGP19115)
  • Università degli Studi di Trento
  • Javna Agencija za Raziskovalno Dejavnost RS (P1-0391)
  • AFM-Téléthon (22129)
  • Fondazione Cassa Di Risparmio Di Trento E Rovereto
  • Provincia Autonoma di Trento
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
  • Health Sciences › Medicine › Genetics (clinical) › Neurogenetic and Muscular Disorders Research
  • Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Keywords
survival motor neuron protein, spinal muscular atrophy, ribosome heterogeneity, ribosome-associated proteins, translational regulation, 5' untranslated region, rare codons, translational enhancer, acetylcholinesterase, motor neuron function
MeSH terms
Animals, Disease Models, Animal, Gene Expression Regulation, Motor Neurons, Muscular Atrophy, Spinal, Ribosomes, RNA, Messenger, Protein Biosynthesis, Proteome, Mice, Survival of Motor Neuron 1 Protein, Transcriptome

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