In every living organism, the production of ribosomes, the protein factories of the cell, is central to gene expression and cell metabolism. Tackling the high complexity of ribosome synthesis in human cells has been made more urgent by the recent linkage of congenital diseases and cancer predisposition to ribosome production defects. Our group focuses on the mechanisms of ribosome synthesis in human cells under several angles: pre-ribosomal RNA processing, nuclear export of pre-ribosomal particles, structure of the pre-ribosomes. This mechanistic framework allows us to characterize the functional impact of mutations affecting ribosome biogenesis in rare diseases (ribosomopathies), especially Diamond-Blackfan anemia.
Visualizing the translocation of pre-ribosomal particles through the nuclear pore complex
Determination of the 3D structure of human pre-40S particles by electron microscopy
Specific mechanisms of pre-ribosomal RNA processing in human cells
- Larburu N, Montellese C, O'Donohue MF, Kutay U, Gleizes PE, Plisson-Chastang C.
Structure of a human pre-40S particle points to a role for RACK1 in the final steps of 18S rRNA processing
Nucleic Acids Res
2016 Sep DOI: 10.1093/nar/gkw714
- Bailly A, Perrin A, Bou Malhab LJ, Pion E, Larance M, Nagala M, Smith P, O'Donohue MF, Gleizes PE, Zomerdijk J, Lamond AI, Xirodimas DP.
The NEDD8 inhibitor MLN4924 increases the size of the nucleolus and activates p53 through the ribosomal-Mdm2 pathway
Oncogene, 35, 415-426
- Sloan KE, Gleizes PE, Bohnsack MT.
Nucleocytoplasmic transport of RNAs and RNA-protein complexes
J Mol Biol, 35, 415-426
- Plisson-Chastang C, Larburu N, Gleizes PE.
Ribosomal 60S-subunit production: the final scene
Nat Struct Mol Biol. 22, 837–838
- Henras A, O’Donohue MF, Plisson-Chastang C, Chakraborty A, Gleizes PE.
An overview of pre-rRNA processing in eukaryotes
WIREs RNA 6: 225-42
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PhDs: Jacques Rouquette, Almass-Houd Aguissa-Touré, Coralie Carron, Natacha Larburu
Post-docs: Daniel Bacqueville, Milena Preti, Anirban Chakraborty
Researchers: Nicole Gas, Isabelle Léger, Jacqueline Depeyre, Guillaume Stahl
Technical staff: Valérie Choesmel-Cadamuro, Marc Delubac, Marlene Faubladier