Our long-term commitment to Diamond-Blackfan anemia

We are part of two European consortia: RiboEurope and DBAGeneCure aiming to understand the molecular basis and to develop therapeutics (gene therapy) for Diamond-Blackfan anemia (DBA). Diamond-Blackfan anemia is a bone marrow failure syndrome manifested notably by failure to produce red blood cells. Please watch our two educational video clips…

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Glutamine & Nucleolar surveillance

Nucleolar surveillance is a p53-dependent anti-tumoral pathway activated upon ribosome biogenesis dysfunction. It had remained unclear why liquid tumours respond better to ribosome biogenesis inhibitors than solid ones. In collaboration with the team of Tsuyoshi Osawa (U. Tokyo), we have revealed the importance of the intra tumoral concentration of the…

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Dihydrouridine on messenger RNAs slows ribosomes!

On the cover: In this issue of Molecular Cell, Finet et al. (pp. 404–419) use a transcriptome-wide approach to reveal that the dihydrouridine RNA modification (the snail) is present on eukaryotic mRNAs. They show that dihydrouridylated codons are translated more slowly by the ribosome (blue shapes) and that modification of the…

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Fibrillarin is required for Neural Crest Cell maturation

Ribosomes are essential nanomachines responsible for protein production in all cells. Ribosomopathies are diseases caused by improper ribosome formation due to mutations in ribosomal proteins or ribosome assembly factors. Such diseases primarily affect the brain and blood, and it is unclear how malfunctioning of a process as general as ribosome…

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The Nucleolus as a Multiphase Liquid Condensate

The nucleolus is the most prominent nuclear body and serves a fundamentally important biological role as a site of ribonucleoprotein particle assembly, primarily dedicated to ribosome biogenesis. Despite being one of the first intracellular structures visualized historically, the biophysical rules governing its assembly and function are only starting to become…

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The “iNo score”: Towards bringing the nucleolus in the clinics

Using the iNo score to discriminate normal from altered nucleolar morphology, with applications in basic cell biology and potential in human disease diagnostics             Ribosome biogenesis is initiated in the nucleolus, a cell condensate essential to gene expression, whose morphology informs cancer pathologists on the health…

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Curing cancer with Daffodils?

Pellegrino et al., have shown that haemanthamine inhibits both ribosome biogenesis and function, providing for the first time the molecular basis of the anticancer properties of this natural compound used for centuries in folk medicine. The ribosome biogenesis inhibition triggers an antitumoral surveillance response leading to p53 stabilization (nucleolar stress).…

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Involvement of U3 and U8 box C/D snoRNAs in tumorigenesis

Small nucleolar RNAs (snoRNAs) are emerging as a novel class of proto-oncogenes and tumor suppressors; their involvement in tumorigenesis remains unclear. The box C/D snoRNAs U3 and U8 are upregulated in breast cancers. Here we characterize the function of human U3 and U8 in ribosome biogenesis, nucleolar structure, and tumorigenesis.…

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“Ki-67 organizes heterochromatin and is required for nucleolar genesis”

  In Collaboration with Daniel Fisher (Montpellier Institute of Molecular Genetics – CNRS, Université de Montpellier), we showed that Ki-67 plays an important role during nucleologenesis. Specifically, we showed that Ki-67 is important for PR (Perichromosomal Region) formation: During mitosis, the nucleolus undergoes a dramatic cycle of disassembly and reassembly.…

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“Kill The Sammson, Kill The Melanoma”

In a work led by Jean-Christophe Marine (VIB, KU Leuven) we contributed to the characterization of the lineage-specific long non-coding RNA Sammson. Sammson is only expressed in melanoma cells. When Sammson’ expression is shut, melanoma cells die, principally of loss of mitochondrial function. Focal amplifications of chromosome 3p13–3p14 occur in…

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10th Ribosome Synthesis Meeting in Brussels, Belgium, 19-23 August 2015

Organizing committee: Sander GRANNEMAN (University of Edinburgh)Katrin KARBSTEIN (The SCRIPPS Research Institute, Florida)Denis LAFONTAINE (University of Brussels)Tom MEIER (Albert Einstein College of Medicine, New York)Joost ZOMERDIJK (University of Dundee) Keynote lectures:Prof David TOLLERVEY, Wellcome Trust Center for Cell Biology, U. EdinburghProf Nenad BAN, ETH, Zurich

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