M
Marc-David Ruepp
Researcher at King's College London
Publications - 51
Citations - 1779
Marc-David Ruepp is an academic researcher from King's College London. The author has contributed to research in topics: RNA splicing & Gene. The author has an hindex of 18, co-authored 43 publications receiving 1236 citations. Previous affiliations of Marc-David Ruepp include University of Milano-Bicocca & University of Bern.
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Journal ArticleDOI
Phase Separation of FUS Is Suppressed by Its Nuclear Import Receptor and Arginine Methylation
Mario Hofweber,Saskia Hutten,Benjamin Bourgeois,Emil Spreitzer,Annika Niedner-Boblenz,Martina Schifferer,Martina Schifferer,Marc-David Ruepp,Mikael Simons,Mikael Simons,Dierk Niessing,Dierk Niessing,Tobias Madl,Dorothee Dormann +13 more
TL;DR: It is shown that Transportin and arginine methylation have a crucial function beyond nuclear import-namely to suppress RGG/RG-driven phase separation and stress granule association of FUS.
Journal ArticleDOI
The Solution Structure of FUS Bound to RNA Reveals a Bipartite Mode of RNA Recognition with Both Sequence and Shape Specificity.
Fionna E. Loughlin,Fionna E. Loughlin,Peter J. Lukavsky,Tamara Kazeeva,Stefan Reber,Eva Maria Hock,Martino Colombo,Christine von Schroetter,Phillip Pauli,Antoine Cléry,Oliver Mühlemann,Magdalini Polymenidou,Marc-David Ruepp,Frédéric H.-T. Allain +13 more
TL;DR: These structures show a bipartite binding mode of FUS comprising of sequence-specific recognition of a NGGU motif via the ZnF and an unusual shape Recognition of a stem-loop RNA via the RRM, and it is shown that disruption of theRRM andZnF domains abolishes FUS function in splicing.
Journal ArticleDOI
Comparison of EJC-enhanced and EJC-independent NMD in human cells reveals two partially redundant degradation pathways.
TL;DR: The surprising heterogeneity observed with only two NMD reporter pairs suggests the existence of several mechanistically distinct branches of NMD in human cells.
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Minor intron splicing is regulated by FUS and affected by ALS-associated FUS mutants
Stefan Reber,Jolanda Stettler,Giuseppe Filosa,Martino Colombo,Daniel Jutzi,Silvia C. Lenzken,Christoph Schweingruber,Rémy Bruggmann,Angela Bachi,Silvia M.L. Barabino,Oliver Mühlemann,Marc-David Ruepp +11 more
TL;DR: It is shown that FUS interacts with the minor spliceosome constituent U11 snRNP, binds preferentially to minor introns and directly regulates their removal, and a FUS knockout in neuroblastoma cells strongly disturbs the splicing of minor intron‐containing m RNAs, among them mRNAs required for action potential transmission and for functional spinal motor units.
Journal ArticleDOI
Rescue of a severe mouse model for spinal muscular atrophy by U7 snRNA-mediated splicing modulation
Kathrin Meyer,Julien Marquis,Judith Trüb,Rachel Nlend Nlend,Sonia Verp,Marc-David Ruepp,Hans Imboden,Isabelle Barde,Didier Trono,Daniel Schümperli +9 more
TL;DR: It is shown that a bifunctional U7 snRNA that stimulates exon 7 inclusion, when introduced by germline transgenesis, can efficiently complement the most severe mouse SMA model.