https://www.nature.com/articles/s41556-020-00577-7
https://doi.org/10.1038/s41556-020-00577-7
https://pubmed.ncbi.nlm.nih.gov/32958857/
TITLE:
SMN-primed ribosomes modulate the translation of transcripts related to spinal muscular atrophy
ALTERNATIVE TITLE:
None
DATE:
Verify – Tue, 22 Sep 2020 06:00:00 -0400
AUTHORS:
Fabio Lauria,Paola Bernabò,Toma Tebaldi,Ewout Joan Nicolaas Groen,Elena Perenthaler,Federica Maniscalco,Annalisa Rossi,Deborah Donzel,Massimiliano Clamer,Marta Marchioretto,Neža Omersa,Julia Orri,Mauro Dalla Serra,Gregor Anderluh,Alessandro Quattrone,Alberto Inga,Thomas Henry Gillingwater,Gabriella Viero
SOURCE:
Nature cell biology
DESCRIPTION:
The contribution of ribosome heterogeneity and ribosome-associated proteins to the molecular control of proteomes in health and disease remains unclear. Here, we demonstrate that survival motor neuron (SMN) protein-the 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 preferentially positioned within the first five codons of a set of mRNAs that are enriched for translational…
CONTENT:
Nat Cell Biol. 2020 Sep 21. doi: 10.1038/s41556-020-00577-7. Online ahead of print.
ABSTRACT
The contribution of ribosome heterogeneity and ribosome-associated proteins to the molecular control of proteomes in health and disease remains unclear. Here, we demonstrate that survival motor neuron (SMN) protein-the 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 preferentially positioned within the first five codons of a set of mRNAs that are enriched for translational enhancer sequences in the 5′ untranslated region (UTR) and rare codons at the beginning of their coding sequence. These SMN-specific mRNAs are associated with neurogenesis, lipid metabolism, ubiquitination, chromatin regulation and translation. Loss of SMN induces ribosome depletion, especially at the beginning of the coding sequence of SMN-specific mRNAs, leading to impairment of proteins that are involved in motor neuron function and stability, including acetylcholinesterase. Thus, SMN plays a crucial role in the regulation of ribosome fluxes along mRNAs encoding proteins that are relevant to SMA pathogenesis.
PMID:32958857 | DOI:10.1038/s41556-020-00577-7
PUBMED ID:
pubmed:32958857
OTHER ID:
pmid:32958857,doi:10.1038/s41556-020-00577-7
PUBLICATION DATE:
Tue, 22 Sep 2020 06:00:00 -0400
2020-09-22
RETRIEVAL DATE :
09/22/20 07:17AM
LINK – PUBMED:
https://pubmed.ncbi.nlm.nih.gov/32958857/?utm_source=Other&utm_medium=rss&utm_campaign=pubmed-2&utm_content=1l7lJQBQXfTBJkL86rnvYKzafMiKbgcUrlv_X8_D_H5EuRmkjR&fc=20200708141943&ff=20200922071706&v=2.11.5
LINK – DOI:
https://doi.org/10.1038/s41556-020-00577-7
LINK – FULL TEXT:
Pending
NOTES:
None