Célia Plisson‐Chastang

The R2T(P) complex orchestrates the SHQ1 driven early steps of box H/ACA snoRNP maturation

Philipp Busse, Ana Catarina Paiva, Paulo E. Santo, Hugo Gizardin-Fredon et autres

Abstract The chaperones RuvBL1 and RuvBL2 are members of the AAA+ ATPase family and participate in diverse cellular processes, including DNA repair, transcriptional regulation, and assembly of macromolecular complexes such as snoRNPs. The biogenesis of box H/ACA snoRNPs additionally requires the assembly …

pt, fr, fi (code pays fourni par la source)

A RiboCancer cell line panel reveals that CLL‐associated Rps15 mutations translationally rewire transcription through codon‐specific tRNA accommodation defects

Anaïs Astier, Marino Caruso, Stijn Vereecke, Paulo E. Santo et autres

Abstract Recurrent point mutations in ribosomal proteins (RPs) RPL10 and RPS15 are found in T‐cell acute lymphoblastic leukemia (T‐ALL) and chronic lymphocytic leukemia (CLL), respectively. Furthermore, deletions of RPL5, RPL11, and RPL22 are frequent in hematologic diseases such as Diamond Blackfan Anemia, …

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An integrative structural biology approach reveals the dynamic organization of the R2SP quaternary chaperone complex

Paulo E. Santo, Marie-Eve Chagot, Hugo Gizardin-Fredon, Marie Ley et autres

R2SP belongs to the R2TP-like quaternary chaperone family and consists of RUVBL1/RUVBL2 AAA+ ATPases, which powers the machinery, and SPAG1 and PIH1D2 adapter proteins that engage specific clients to promote their quaternary assembly. However, little is known about the structure of R2SP …

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Organelle distribution and protein translation defects in radial glia cells with mutations in Eml1

Valeria Viola, Kaviya Chinnappa, R. Thompson, Carmen Cifuentes Diaz et autres

Abstract Cerebral cortical development is a finely regulated process, depending on key neuronal progenitors, giving rise to post-mitotic neurons that migrate to superficial regions in the developing brain. Disruptions can give rise to severe cortical malformations that are associated with intellectual disability …

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Translatome and translation dynamics analysis of a RiboCancer cell line panel reveals that leukemia-associated Rps15 mutations rewire translation through codon-specific tRNA accommodation defects

A. Astier, Marino Caruso, Stijn Vereecke, Paulo E. Santo et autres

ABSTRACT Deletions and point mutations targeting ribosomal proteins (RPs) have been identified in cancer. Yet, their role in translational dysregulation remains poorly understood. We performed an integrated genome-wide translatome analysis (proteome, Ribo-seq and total RNA-seq) as well as RiboMethSeq on an isogenic …

fr, be (code pays fourni par la source)

ZNHIT6, a major RUVBL1/2 ATPases Cofactor of C/D snoRNP Biogenesis

Elisa Vicq, Paulo E. Santo, Célia Plisson‐Chastang, Xavier Manival et autres

International audience

The snoRNP chaperone snR190 and the Npa1 complex form a macromolecular assembly required for 60S ribosomal subunit maturation

Hussein Hamze, Mariam Jaafar, Ali Khreiss, Carine Dominique et autres

The early steps of large-ribosomal-subunit assembly feature among the least understood steps of ribosome synthesis in eukaryotes. In Saccharomyces cerevisiae, the box C/D chaperone small nucleolar ribonucleoprotein (snoRNP) snR190 and the Npa1 complex, composed of the α-solenoid scaffold proteins Npa1 and Npa2, …

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R2TP-like Quaternary Chaperones: a comprehensive overview to understand the dynamic R2SP complex

Paulo E. Santo, Marie-Eve Chagot, Hugo Gizardin-Fredon, Marie Ley et autres

Abstract The human R2SP complex belongs to the R2TP-like quaternary chaperone family and consists of RUVBL1, RUVBL2, SPAG1 and PIH1D2. R2SP is crucial for the correct assembly of motile cilia (SPAG1 null mutations cause Primary Ciliary Dyskinesia) and the organization of the …

fr, us, pt (code pays fourni par la source)

Off-axis electron holography of unstained bacteriophages: Towards electrostatic potential measurement of biological samples

Elio Karim, Christophe Gatel, Amélie Leforestier, Stéphanie Balor et autres

fr (code pays fourni par la source)

SURF2 is a MDM2 antagonist in triggering the nucleolar stress response

Sophie Tagnères, Paulo E. Santo, Julie Radermecker, Dana Rinaldi et autres

Cancer cells rely on high ribosome production to sustain their proliferation rate. Many chemotherapies impede ribosome production which is perceived by cells as "nucleolar stress" (NS), triggering p53-dependent and independent pathways leading to cell cycle arrest and/or apoptosis. The 5S ribonucleoprotein (RNP) …

fr, gb (code pays fourni par la source)

SURF2 is a MDM2 antagonist in triggering the nucleolar stress response

Sophie Tagnères, Paulo E. Santo, Julie Radermecker, Dana Rinaldi et autres

Abstract Cancer cells are addicted to strong ribosome production to sustain their proliferation rate. Many chemotherapies impede ribosome production which is perceived by cells as “nucleolar stress” (NS), triggering p53-dependent and independent response pathways leading to cell cycle arrest and/or apoptosis. The …

fr, Maroc, au, gb, Mali (code pays fourni par la source)

Off-Axis Electron Holography of Unstained Bacteriophages: Toward Electrostatic Potential Measurement of Biological Samples

Elio Karim, Christophe Gatel, Amélie Leforestier, Stéphanie Balor et autres