E
En Kimura
Researcher at Kumamoto University
Publications - 90
Citations - 2513
En Kimura is an academic researcher from Kumamoto University. The author has contributed to research in topics: Duchenne muscular dystrophy & Muscular dystrophy. The author has an hindex of 24, co-authored 88 publications receiving 2071 citations. Previous affiliations of En Kimura include University of Washington.
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Journal ArticleDOI
The TREAT-NMD DMD Global Database: Analysis of More than 7,000 Duchenne Muscular Dystrophy Mutations
Catherine L. Bladen,David Salgado,Soledad Monges,Maria E. Foncuberta,Kyriaki Kekou,Konstantina Kosma,Konstantina Kosma,Hugh Dawkins,Leanne Lamont,Anna J. Roy,Teodora Chamova,Velina Guergueltcheva,Sophelia H. S. Chan,Lawrence Korngut,Craig Campbell,Yi Dai,Jen Wang,Nina Barišić,Petr Brabec,Jaana Lahdetie,Maggie C. Walter,Olivia Schreiber-Katz,Veronika Karcagi,Marta Garami,Venkatarman Viswanathan,Farhad Bayat,Filippo Buccella,En Kimura,Zaïda Koeks,Janneke C. van den Bergen,Miriam Rodrigues,Richard Roxburgh,Anna Lusakowska,Anna Kostera-Pruszczyk,Janusz Zimowski,Rosário Santos,Elena Neagu,Svetlana Artemieva,Vedrana Milic Rasic,Dina Vojinovic,Manuel Posada,Clemens Bloetzer,Pierre Yves Jeannet,Franziska Joncourt,Jordi Díaz-Manera,Eduard Gallardo,Ayse Karaduman,Haluk Topaloglu,Rasha El Sherif,Angela Stringer,Andriy V. Shatillo,Ann Martin,Holly L. Peay,Matthew I. Bellgard,Jan Kirschner,Kevin M. Flanigan,Volker Straub,Kate Bushby,Jan J.G.M. Verschuuren,Annemieke Aartsma-Rus,Annemieke Aartsma-Rus,Christophe Béroud,Christophe Béroud,Hanns Lochmüller +63 more
TL;DR: The development and analysis of the TREAT‐NMD DMD Global database is described and mutations were identified that would potentially benefit from novel genetic therapies for DMD including stop codon read‐through therapies and exon skipping therapy.
Journal ArticleDOI
Efficient and Reproducible Myogenic Differentiation from Human iPS Cells: Prospects for Modeling Miyoshi Myopathy In Vitro
Akihito Tanaka,Akihito Tanaka,Knut Woltjen,Katsuya Miyake,Akitsu Hotta,Makoto Ikeya,Takuya Yamamoto,Tokiko Nishino,Emi Shoji,Atsuko Sehara-Fujisawa,Yasuko Manabe,Nobuharu L. Fujii,Kazunori Hanaoka,Takumi Era,Satoshi Yamashita,Ken-ichi Isobe,En Kimura,Hidetoshi Sakurai +17 more
TL;DR: Using the induced differentiation technique, this work successfully recreated the pathological condition of MM in vitro, demonstrating defective membrane repair in hiPSC-derived myotubes from an MM patient and phenotypic rescue by expression of full-length DYSFERLIN (DYSF).
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Comparison of the Added Value of Contrast-Enhanced 3D Fluid-Attenuated Inversion Recovery and Magnetization-Prepared Rapid Acquisition of Gradient Echo Sequences in Relation to Conventional Postcontrast T1-Weighted Images for the Evaluation of Leptomeningeal Diseases at 3T
Hirofumi Fukuoka,Toshinori Hirai,T. Okuda,Yoshinori Shigematsu,Ako Sasao,En Kimura,Teruyuki Hirano,Shigetoshi Yano,R. Murakami,Y. Yamashita +9 more
TL;DR: For the depiction of leptomeningeal diseases, postcontrast 3D T2-FLAIR provides more additional information than postcont Contrast T1-weighted or MPRAGE imaging.
Journal ArticleDOI
Stable transduction of myogenic cells with lentiviral vectors expressing a minidystrophin.
Sheng Li,En Kimura,Brent Fall,Morayma Reyes,John C. Angello,Robert E. Welikson,Stephen D. Hauschka,Jeffrey S. Chamberlain +7 more
TL;DR: The ability of lentiviral vectors to transduce myogenic progenitors using a minidystrophin cassette regulated by a muscle-specific promoter suggests that this system could be useful for ex vivo gene therapy of muscular dystrophy.
Journal ArticleDOI
Clinical Outcomes in Duchenne Muscular Dystrophy: A Study of 5345 Patients from the TREAT-NMD DMD Global Database
Zaïda Koeks,Catherine L. Bladen,David Salgado,Erik W. van Zwet,Oksana Pogoryelova,Grace McMacken,Soledad Monges,Maria E. Foncuberta,Kyriaki Kekou,Konstantina Kosma,Hugh Dawkins,Leanne Lamont,Matthew I. Bellgard,Anna J. Roy,Teodora Chamova,Velina Guergueltcheva,Sophelia H. S. Chan,Lawrence Korngut,Craig Campbell,Yi Dai,Jen Wang,Nina Barišić,Petr Brabec,Jaana Lahdetie,Maggie C. Walter,Olivia Schreiber-Katz,Veronika Karcagi,Marta Garami,Agnes Herczegfalvi,Venkatarman Viswanathan,Farhad Bayat,Filippo Buccella,Alessandra Ferlini,En Kimura,Janneke C. van den Bergen,Miriam Rodrigues,Richard Roxburgh,Anna Lusakowska,Anna Kostera-Pruszczyk,Rosário Santos,Elena Neagu,Svetlana Artemieva,Vedrana Milic Rasic,Dina Vojinovic,Dina Vojinovic,Manuel Posada,Clemens Bloetzer,Andrea Klein,Jordi Díaz-Manera,Eduard Gallardo,Ayşe Karaduman,Tunca Oznur,Haluk Topaloglu,Rasha El Sherif,Angela Stringer,Andriy V. Shatillo,Ann Martin,Holly L. Peay,Jan Kirschner,Kevin M. Flanigan,Volker Straub,Kate Bushby,Christophe Béroud,Jan J.G.M. Verschuuren,Hanns Lochmüller +64 more
TL;DR: The data confirm the benefits of corticosteroid treatment on ambulation, need for scoliosis surgery, ventilation and, to a lesser extent, cardiomyopathy.