Abstract:
:In ribosomopathies, the Diamond-Blackfan anemia (DBA) or 5q- syndrome, ribosomal protein (RP) genes are affected by mutation or deletion, resulting in bone marrow erythroid hypoplasia. Unbalanced production of ribosomal subunits leading to a limited ribosome cellular content, regulates translation at the expense of the master erythroid transcription factor GATA1. In RPS14-deficient cells mimicking 5q- syndrome erythroid defects, we show that the transcript length, codon bias of the coding sequence (CDS) and 3'UTR structure are the key determinants of translation. In these cells, short transcripts with a structured 3'UTR and high CAI showed a decreased translation efficiency. Quantitative analysis of the whole proteome confirmed that the post-transcriptional changes depended on the transcript characteristics that governed the translation efficiency in conditions of low ribosome availability. In addition, proteins involved in normal erythroid differentiation share most determinants of translation selectivity. Our findings thus indicate that impaired erythroid maturation due to 5q- syndrome may proceed from a translational selectivity at the expense of the erythroid differentiation program and suggest that an interplay between the CDS and UTRs may regulate mRNA translation.
journal_name
Haematologicajournal_title
Haematologicaauthors
Boussaid I,Le Goff S,Floquet C,Gautier EF,Raimbault A,Viailly PJ,Al Dulaimi D,Burroni B,Dusanter-Fourt I,Hatin I,Mayeux P,Cosson B,Fontenay Mdoi
10.3324/haematol.2019.239970subject
Has Abstractpub_date
2020-04-23 00:00:00eissn
0390-6078issn
1592-8721pii
haematol.2019.239970pub_type
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