Presentation
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.
Projets
Cell cycle control and ribosome synthesis
Ribosome biogenesis disorders and ribosomopathies
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
Publications
- Fellmann F, Saunders C, O'Donohue MF, Reid DW, McFadden KA, Montel-Lehry N, Yu C, Fang M, Zhang J, Royer-Bertrand B, Farinelli P, Karboul N, Willer JR, Fievet L, Bhuiyan ZA, Kleinhenz AL, Jadeau J, Fulbright J, Rivolta C, Renella R, Katsanis N, Beckmann JS, Nicchitta CV, Da Costa L, Davis EE, Gleizes PE.
An atypical form of 60S ribosomal subunit in Diamond-Blackfan anemia linked to RPL17 variants
JCI Insight, 9(17):e172475
2024 Sep OPEN ACCESS, doi: 10.1172/jci.insight.1724 - Tagnères S, Santo PE, Radermecker J, Rinaldi D, Froment C, Provost Q, Bongers M, Capeille S, Watkins N, Marcoux J, Gleizes PE, Marcel V, Plisson-Chastang C, Lebaron S.
SURF2 is a MDM2 antagonist in triggering the nucleolar stress response
Nat Commun. 15(1):8404
2024 Sep doi: 10.1038/s41467-024-52659-x - Da Costa L, Mohandas N, David-NGuyen L, Platon J, Marie I, O'Donohue MF, Leblanc T, Gleizes PE.
Diamond-Blackfan anemia, the archetype of ribosomopathy: How distinct is it from the other constitutional ribosomopathies?
Blood Cells Mol Dis. 106:102838
2024 May doi: 10.1016/j.bcmd.2024.102838 - Wlodarski MW, Vlachos A, Farrar JE, Da Costa LM, Kattamis A, Dianzani I, Belendez C, Unal S, Tamary H, Pasauliene R, Pospisilova D, de la Fuente J, Iskander D, Wolfe L, Liu JM, Shimamura A, Albrecht K, Lausen B, Bechensteen AG, Tedgard U, Puzik A, Quarello P, Ramenghi U, Bartels M, Hengartner H, Farah RA, Al Saleh M, Hamidieh AA, Yang W, Ito E, Kook H, Ovsyannikova G, Kager L, Gleizes PE, Dalle JH, Strahm B, Niemeyer CM, Lipton JM, Leblanc TM; international Diamond-Blackfan anaemia syndrome guideline panel.
Diagnosis, treatment, and surveillance of Diamond-Blackfan anaemia syndrome: international consensus statement
Lancet Haematol. 11(5):e368-e382
2024 May doi: 10.1016/S2352-3026(24)00063-2 - Karim E, Park B, Marcelot C, Soldan V, Balor S, Bals S, Le Forestier A, Plisson-Chastang C, Gatel C, Gleizes PE, Snoeck E.
In-line and Off-axis Electron Holography for the Study of Biological Specimens
Microsc Microanal. 22;29(29 Suppl 1):1015-1016
2023 Jul doi: 10.1093/micmic/ozad067.512
View all publications
Funding
Alumni
PhDs: Jacques Rouquette, Almass-Houd Aguissa-Touré, Coralie Carron, Natacha Larburu, Ramtin Shayan, Laura Plassart, Maxime Aubert
Post-docs: Daniel Bacqueville, Milena Preti, Anirban Chakraborty
Researchers: Nicole Gas, Isabelle Léger, Jacqueline Depeyre, Guillaume Stahl, Célia Plisson-Chastang, Simon Lebaron
Technical staff: Valérie Choesmel-Cadamuro, Marc Delubac, Marlene Faubladier, Dana Rinaldi
