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Youn-Bok Lee
Researcher at King's College London
Publications - 32
Citations - 2214
Youn-Bok Lee is an academic researcher from King's College London. The author has contributed to research in topics: Trinucleotide repeat expansion & RNA. The author has an hindex of 21, co-authored 31 publications receiving 1911 citations. Previous affiliations of Youn-Bok Lee include Dongguk University & University of Bristol.
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Journal ArticleDOI
Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic.
Youn-Bok Lee,Han-Jou Chen,João N. Peres,Jorge Gomez-Deza,Jan Attig,Jan Attig,Maja Štalekar,Claire Troakes,Agnes L. Nishimura,Emma L. Scotter,Caroline Vance,Yoshitsugu Adachi,Valentina Sardone,Valentina Sardone,Jack W. Miller,Bradley N. Smith,Jean-Marc Gallo,Jernej Ule,Frank Hirth,Boris Rogelj,Corinne Houart,Christopher Shaw +21 more
TL;DR: It is demonstrated that the expression of 38× and 72× G4C2 repeats form intranuclear RNA foci that initiate apoptotic cell death in neuronal cell lines and zebrafish embryos and proposed that RNA toxicity and protein sequestration may disrupt RNA processing and contribute to neurodegeneration.
Journal ArticleDOI
Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species
Emma L. Scotter,Caroline Vance,Agnes L. Nishimura,Youn-Bok Lee,Han-Jou Chen,Hazel Urwin,Valentina Sardone,Valentina Sardone,Jacqueline C. Mitchell,Boris Rogelj,Boris Rogelj,David C. Rubinsztein,Christopher Shaw +12 more
TL;DR: It is found that soluble TDP-43 is degraded primarily by the UPS, whereas the clearance of aggregated T DP-43 requires autophagy, andTherapies for clearing excess TTP-43 should target a combination of these pathways.
Journal ArticleDOI
Twist-1 regulates the miR-199a/214 cluster during development
Youn-Bok Lee,Ioannis Bantounas,Do-Young Lee,Leonidas A. Phylactou,Maeve A. Caldwell,James B. Uney +5 more
TL;DR: This study shows the expression of the miR199a/214 cluster is controlled by Twist-1 via an E-Box promoter element and supports a role for these miRNAs as novel intermediates in the pathways controlling the development of specific neural cell populations.
Journal ArticleDOI
The C9ORF72 expansion mutation is a common cause of ALS+/-FTD in Europe and has a single founder.
Bradley N. Smith,Stephen Newhouse,Aleksey Shatunov,Caroline Vance,Simon Topp,Lauren Johnson,Jack W. Miller,Youn-Bok Lee,Claire Troakes,Kirsten M. Scott,Ashley R. Jones,Ian Gray,Jamie Wright,Tibor Hortobágyi,Safa Al-Sarraj,Boris Rogelj,John Powell,Michelle K. Lupton,Simon Lovestone,Peter C. Sapp,Markus Weber,Peter J. Nestor,Helenius J. Schelhaas,Anneloor ten Asbroek,Vincenzo Silani,Cinzia Gellera,Franco Taroni,Nicola Ticozzi,Leonard H. van den Berg,Jan H. Veldink,Phillip Van Damme,Wim Robberecht,Pamela J. Shaw,Janine Kirby,Hardev Pall,Karen E. Morrison,Alex G. Morris,Jacqueline de Belleroche,J. M. B. Vianney de Jong,Frank Baas,Peter M. Andersen,John Landers,Robert H. Brown,Michael E. Weale,Ammar Al-Chalabi,Christopher Shaw +45 more
TL;DR: It is concluded that the HREM has a single founder and is the most common mutation in familial and sporadic ALS in Europe.
Journal ArticleDOI
C9ORF72 repeat expansion causes vulnerability of motor neurons to Ca2+-permeable AMPA receptor-mediated excitotoxicity
Bhuvaneish T. Selvaraj,Matthew R. Livesey,Chen Zhao,Jenna M. Gregory,Owain T. James,Elaine M. Cleary,Amit K. Chouhan,Amit K. Chouhan,Angus B. Gane,Emma M. Perkins,Owen Dando,Simon G. Lillico,Youn-Bok Lee,Agnes L. Nishimura,Urjana Poreci,Sai Thankamony,Meryll Pray,Navneet A. Vasistha,Dario Magnani,Shyamanga Borooah,Karen Burr,David Story,Alexander McCampbell,Christopher Shaw,Peter C. Kind,Timothy J. Aitman,C. Bruce A. Whitelaw,Ian Wilmut,Colin Smith,Gareth B. Miles,Gareth B. Miles,Giles E. Hardingham,David J. A. Wyllie,Siddharthan Chandran +33 more
TL;DR: It is shown that increased expression of GluA1 AMPA receptor (AMPAR) subunit occurs in MNs with C9ORF72 mutations that leads to increased Ca2+-permeable AMPAR expression and results in enhanced selective MN vulnerability to excitotoxicity.