C
Christine Hilcenko
Researcher at University of Cambridge
Publications - 14
Citations - 600
Christine Hilcenko is an academic researcher from University of Cambridge. The author has contributed to research in topics: SBDS & Ribosome. The author has an hindex of 6, co-authored 11 publications receiving 464 citations. Previous affiliations of Christine Hilcenko include Laboratory of Molecular Biology.
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Journal ArticleDOI
Uncoupling of GTP hydrolysis from eIF6 release on the ribosome causes Shwachman-Diamond syndrome
Andrew J. Finch,Christine Hilcenko,Nicolas Basse,Lesley F Drynan,Beatriz Goyenechea,Tobias F. Menne,África González Fernández,Paul J. Simpson,Clive D'Santos,Mark J. Arends,Jean Donadieu,Christine Bellanné-Chantelot,Michael Costanzo,Charles Boone,Andrew N. J. McKenzie,Stefan M.V. Freund,Alan J. Warren +16 more
TL;DR: These findings establish a direct role for SBDS and EFL1 in catalyzing the translational activation of ribosomes in all eukaryotes, and define SDS as a ribosomopathy caused by uncoupling GTP hydrolysis from eIF6 release.
Journal ArticleDOI
Mechanism of eIF6 release from the nascent 60S ribosomal subunit
Felix Weis,Emmanuel Giudice,Mark J. Churcher,Li Jin,Christine Hilcenko,Christine Hilcenko,Chi C. Wong,David Traynor,Robert R. Kay,Alan J. Warren,Alan J. Warren +10 more
TL;DR: Cryo-EM structures of human SBDS and SBDS–EFL1 bound to Dictyostelium discoideum 60S ribosomal subunits with and without endogenous eIF 6 reveal the conserved mechanism of eIF6 release, which is corrupted in both inherited and sporadic leukemias.
Journal ArticleDOI
Aberrant 3' oligoadenylation of spliceosomal U6 small nuclear RNA in poikiloderma with neutropenia.
Christine Hilcenko,Christine Hilcenko,Paul J. Simpson,Paul J. Simpson,Andrew J. Finch,Andrew J. Finch,Frank R. Bowler,Mark J. Churcher,Mark J. Churcher,Li Jin,Li Jin,Len C. Packman,Adam Shlien,Peter J. Campbell,Peter J. Campbell,Michael Kirwan,Inderjeet Dokal,Alan J. Warren,Alan J. Warren +18 more
TL;DR: It is shown that human USB1 is a distributive 3'-5' exoribonuclease that posttranscriptionally removes uridine and adenosine nucleosides from the 3' end of spliceosomal U6 small nuclear RNA (snRNA).
Journal ArticleDOI
Mechanism of completion of peptidyltransferase centre assembly in eukaryotes.
Vasileios Kargas,Pablo Castro-Hartmann,Norberto Escudero-Urquijo,Kyle Dent,Christine Hilcenko,Carolin Sailer,Gertrude Zisser,Maria J Marques-Carvalho,Simone Pellegrino,Leszek Wawiórka,Stefan M.V. Freund,Jane L. Wagstaff,Antonina Andreeva,Alexandre Faille,Edwin Chen,Florian Stengel,Helmut Bergler,Alan J. Warren +17 more
TL;DR: The mechanism of peptidyltransferase centre (PTC) completion is presented that explains how integration of the last ribosomal proteins is coupled to release of the nuclear export adaptor Nmd3 and reveals how the central functional site of the ribosome is sculpted.
Journal ArticleDOI
Somatic genetic rescue of a germline ribosome assembly defect.
Shengjiang Tan,Laëtitia Kermasson,Christine Hilcenko,Vasileios Kargas,David Traynor,Ahmed Z. Boukerrou,Norberto Escudero-Urquijo,Alexandre Faille,Alexis Bertrand,M. Rossmann,Beatriz Goyenechea,Li Jin,Li Jin,Jonathan Moreil,Olivier Alibeu,Blandine Beaupain,Christine Bole-Feysot,Stefano Fumagalli,Sophie Kaltenbach,Sophie Kaltenbach,Jean-Alain Martignoles,Cécile Masson,Patrick Nitschke,Mélanie Parisot,Aurore Pouliet,Isabelle Radford-Weiss,Isabelle Radford-Weiss,Frédéric Tores,Jean-Pierre de Villartay,Mohammed Zarhrate,Ai Ling Koh,Ai Ling Koh,Kong Boo Phua,Kong Boo Phua,Bruno Reversade,Peter J. Bond,Peter J. Bond,Christine Bellanné-Chantelot,Isabelle Callebaut,François Delhommeau,Jean Donadieu,Alan J. Warren,Patrick Revy +42 more
TL;DR: In this article, acquired mutations in the EIF6 gene are a frequent mechanism of indirect somatic genetic rescue in Shwachman-Diamond syndrome (SDS), a leukemia predisposition disorder caused by a germline defect in ribosome assembly.