V
Volker Straub
Researcher at Newcastle University
Publications - 461
Citations - 26289
Volker Straub is an academic researcher from Newcastle University. The author has contributed to research in topics: Muscular dystrophy & Duchenne muscular dystrophy. The author has an hindex of 76, co-authored 393 publications receiving 22440 citations. Previous affiliations of Volker Straub include Boston Children's Hospital & Fraunhofer Society.
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Journal ArticleDOI
Nusinersen versus Sham Control in Infantile-Onset Spinal Muscular Atrophy
Richard S. Finkel,Eugenio Mercuri,Basil T. Darras,Anne M. Connolly,Nancy L. Kuntz,Janbernd Kirschner,Claudia A. Chiriboga,Kayoko Saito,Laurent Servais,Eduardo F. Tizzano,Haluk Topaloglu,Mar Tulinius,Jacqueline Montes,Allan M. Glanzman,Kathie M. Bishop,Z. John Zhong,Sarah Gheuens,C. Frank Bennett,Eugene Schneider,Wildon Farwell,Darryl C. De Vivo,Walter G. Bradley,M. K. Schroth,J. B. Bodensteriner,C. S. Davis,R. Shell,J. Hen,E. D. Austin,S. Aziz-Zaman,J. Cappell,Andrei Constantinescu,Rosangel Cruz,Jahannaz Dastgir,Sally Dunaway,K. Engelstad,M. Gormley,N. Holuba La Marca,Alexander G. Khandji,S. Kramer,Jonathan Marra,C. Ortiz-Miller,M. Popolizio,Rachel Salazar,L. Sanabria,L. Weimer,Pallavi Anand,R. Gadeken,Paul T. Golumbek,Catherine Siener,Craig M. Zaidman,Fouad Al-Ghamdi,C. Berde,Partha S. Ghosh,Robert J. Graham,Tim Harrington,A. Koka,R. Laine,W. Liew,Elizabeth Mirek,G. Ordonez,Amy Pasternak,Janet Quigley,Navil F. Sethna,M. Souris,Heather Szelag,L. Wand,John W. Day,Genevieve D'Souza,T. T. Duong,Richard Gee,J. Kitsuwa-Lowe,D. McFall,S. Patnaik,S. Paulose,Jennifer Perez,Christopher Proud,B. Purse,R. J. Ramamurthi,Sarada Sakamuri,Jacinda B. Sampson,B. Sanjanwala,A. C. Tesi Rocha,K. Watson,L. Welsh,L.D.M. Peña,Laura E. Case,J. Coates,Stephanie DeArmey,M. M. Homi,C. Milleson,N. Nelson,Alexandra C. Ross,Edward C. Smith,B. Taicher,J. Wootton,Erika Finanger,D. Benjamin,A. Frank,Catherine E. Roberts,Barry S. Russman,K. Zilke,D. Berry,Matthew Civitello,D. Cook,J. D. Endsley,C. Johnson,M. Kasper,W. Leon,A. Lim,K. O'Reardon,L. Y. Sigurdardottir,J. Turner,F. Weber-Guzman,M. Zinn,Susan T. Iannaccone,Diana Castro,M. Cowie,Alan C Farrow-Gillespie,A. Herbert,M. Kauk,D. McElroy,N. Miller,Leslie Nelson,Luke Smith,T. Spain,S. Trest,Nicholas E. Johnson,Russell J. Butterfield,D. DiBella,K. Mayne,T. M. Newcomb,N. Rausch,C. Blomgren,H. W. Choi,Leon G. Epstein,S. Goldman,Kristin J. Krosschell,Jena M. Krueger,Jonathan E. Kurz,Vamshi K. Rao,Julie A. Parsons,V. Allen,Alan Bielsky,K. Booker,A. Camuto,Terri Carry,Peter G. Fuhr,Melissa Gibbons,J. Janas,H. Johnson,C. Kelly,L. S. Lord-Halvorson,S. Nicolarsen,S. Shea,V. Tran,G. VanderVeen,M. Yang,C. Zimmerman,Perry B. Shieh,N. Parziale,L. Rao,J. W. Said,F. Shu,C. Skura,L. Staudt,Gihan Tennekoon,L. Adang,John F. Brandsema,M. Chadehumbe,Jean Flickinger,Elizabeth Kichula,D. Stanford,M. Toms,J. Zigmont,Maryam Oskoui,S. Arpin,P. Dinunzio,P. M. Ingelmo,C. Poulin,G. Rivera,C. Sabapathy,M. Srour,S. Turgeon-Desilet,D. Zielinski,Kathryn Selby,C. King,J. Lee,A. Michoulas,E. Roland,Jiri Vajsar,V. Chau,James J. Dowling,R. Haldenby,M. Miki,S. So,S. I. Pascual Pascual,A. Martinez Bermejo,S. Epinosa Garcia,S. Garcia Guixot,M. M. Martinez Moreno,M. del Pilar Tirado Requero,M. del Mar Garcia Romero,C. Aguilar,F. Munell Casadesus,M. B. Gomez Garcia de la Banda,M. Gallardo,G. Gili,M. Alavarez Molinero,M. de Los Angeles Tormos Munoz,N. J. Palacios,B. Planas Pascual,M. del Mar Melendez Plumed,A. F. Rucian,E. Toro Tamargo,M. Gratacos Vinola,S. Borell,Matthias Eckenweiler,Marcus Krüger,Astrid Pechmann,B. Rippberger,S. Stein,Sibylle Vogt,S. Wider,Ulrike Schara,B. Andres,A. Della Marina,A. Ganfuss,P. Jachertz,Heike Koelbel,K. Rupprich,E. S. Schroers,N. Sponemann,Claudio Bruno,Chiara Fiorillo,Alberto Garaventa,Paola Lanteri,Valentina Lanzillotta,C. Manzitti,M. Pedermonte,Paola Tacchetti,Federica Trucco,A. Zuffi,R. De Sanctis,Lavinia Fanelli,Marco Luigetti,Concetta Palermo,Marika Pane,Marco Piastra,Serena Sivo,E. Gargaun,Teresa Gidaro,S. Gilabert,P. L. Léger,A. G. Le Moing,C. Lilien,Michèle Mayer,Q. Ollievier,J. Rambaud,Jessica Taytard,R. Vialle,T. Voit,Francesco Muntoni,L. D'Argenzio,PH Lister,Adnan Y. Manzur,J. Pisco Domingos,Danielle Ramsey,Valeria Ricotti,L. Schottlaender,Mariacristina Scoto,S. M. Scuplak,V. Selby,Volker Straub,S. Baily,Marta Bertoli,A. Mayhew,Robert Muni Lofra,Adrian Murphy,Claire L Wood,Niklas Darin,J. Eldblom,E. Kimber,A. K. Kroksmark,A. Lindstedt,E. Michael,Kalliopi Sofou,Nicolas Deconinck,A. Christiaens,Sandra Coppens,K. DeCock,E. De Vos voor,F. Dorban,G. Gilbert,S. Rooze,V. Tahon,R. Van Coster,R. Van Der Looven,Arnaud Vanlander,D. Vens,Helene Verhelst,B. Wenderickx,S. Wittevrongel,Michelle A. Farrar,N. Berthon-Jones,Michael Doumit,Karen Herbert,Tejaswi Kandula,Margot Morrison,J. O'Brien,S. Richardson,H. A. Ferreira Sampaio,H. L. Teoh,Monique M. Ryan,K. Carroll,K. L. De Valle,D. Villano,Ian R. Woodcock,E. M. Yiu,Didem Ardicli,C. Gunbey,V. G. Haliloglu,Ayşe Karaduman,Bahadır Konuşkan,F. G. Yildiz Sarikaya,E. Serdaroglu,M. Tanyildiz,C. M. Temucin,Mirac Yildirim,Öznur Yilmaz,R. Arakawa,Y. Chiba,K. Eto,K. Hirasawa,T. Ikai,S. Ito,Y. Ito,Y. Kaburagi,H. Kaneko,S. Matsumaru,N. Matsushima,K. Mizuochi,S. Nagata,H. Nakatsukasa,A. Nishikawa,Y. Otani,T. Sato,M. Shichiji,K. Sugimoto,A. Takeshita,T. Yanagishita,A. Yamauchi,Y. Takeshima,T. Fujino,N. Fukuda,T. Lee,K. Oriyama,T. Shibano,H. Shimomura,T. Tachikawa,Y. Tanaka,N. Taniguchi,Jong-Hee Chae,S. A. Choi,S. M. Chun,H. Jo,H. Kim,S. Y. Kim,J. S. Lee,B. C. Lim,Hyung Ik Shin,W. S. Son,Sophelia H. S. Chan,A. C. Chung,C. S. Yan,C. Stella,C. K.W. Joseph,C. S. Ng,H. C.C. Alvin,I. J.K. Janice,L. W.M. Wendy,M. N. Chui-San,N. Y. Ki,T. N. Shun,W. Y. Connie,W. C. Virginia,Y. Yvonne,Y. J. Jong,Tai-Heng Chen,P. C. Chou,Y. H. Chou,H. W. Chung,J. H. Hsu,Y. H. Ju,W. C. Liang,H. H. Shih,H. Y. Wang,Y. C. Wu,Y. S. Zeng +397 more
TL;DR: Those who received nusinersen were more likely to be alive and have improvements in motor function than those in the control group and infants with a shorter disease duration at screening wereMore likely than those with a longer disease duration to benefit from nusineren.
Journal ArticleDOI
Exon skipping and dystrophin restoration in patients with Duchenne muscular dystrophy after systemic phosphorodiamidate morpholino oligomer treatment: an open-label, phase 2, dose-escalation study.
Sebahattin Cirak,Virginia Arechavala-Gomeza,Michela Guglieri,Lucy Feng,Silvia Torelli,Karen Anthony,Stephen Abbs,M. E. Garralda,John P. Bourke,Dominic J. Wells,George Dickson,Matthew J.A. Wood,Steve D. Wilton,Volker Straub,Ryszard Kole,Stephen B. Shrewsbury,Caroline Sewry,Jennifer E. Morgan,Kate Bushby,Francesco Muntoni +19 more
TL;DR: The safety and biochemical efficacy presented show the potential of AVI-4658 to become a disease-modifying drug for Duchenne muscular dystrophy.
Journal ArticleDOI
Post-translational disruption of dystroglycan-ligand interactions in congenital muscular dystrophies
Daniel E. Michele,Rita Barresi,Motoi Kanagawa,Fumiaki Saito,Ronald D. Cohn,Jakob S. Satz,James Dollar,Ichizo Nishino,Richard I. Kelley,Hannu Somer,Volker Straub,Katherine D. Mathews,Steven A. Moore,Kevin P. Campbell +13 more
TL;DR: The results suggest that at least three distinct mammalian genes function within a convergent post-translational processing pathway during the biosynthesis of dystroglycan, and that abnormal dystoglycan–ligand interactions underlie the pathogenic mechanism of muscular dystrophy with brain abnormalities.
Journal ArticleDOI
Muscular dystrophy and neuronal migration disorder caused by mutations in a glycosyltransferase, POMGnT1.
Aruto Yoshida,Kazuhiro Kobayashi,Hiroshi Manya,Kiyomi Taniguchi,Hiroki Kano,Mamoru Mizuno,Toshiyuki Inazu,Hideyo Mitsuhashi,Seiichiro Takahashi,Makoto Takeuchi,Ralf Herrmann,Volker Straub,Beril Talim,Thomas Voit,Haluk Topaloglu,Tatsushi Toda,Tamao Endo +16 more
TL;DR: It is suggested that interference in O-mannosyl glycosylation is a new pathomechanism for muscular dystrophy as well as neuronal migration disorder.
Journal ArticleDOI
Local restoration of dystrophin expression with the morpholino oligomer AVI-4658 in Duchenne muscular dystrophy: a single-blind, placebo-controlled, dose-escalation, proof-of-concept study
Maria Kinali,Maria Kinali,Virginia Arechavala-Gomeza,Lucy Feng,Sebahattin Cirak,D. Hunt,C. Adkin,Michela Guglieri,Emma J. Ashton,Stephen Abbs,Petros Nihoyannopoulos,M. E. Garralda,Mary A. Rutherford,Caroline McCulley,Linda Popplewell,Linda Popplewell,Ian R. Graham,Ian R. Graham,George Dickson,George Dickson,Matthew J.A. Wood,Dominic J. Wells,Steve D. Wilton,Ryszard Kole,Volker Straub,Kate Bushby,Caroline Sewry,Jennifer E. Morgan,Francesco Muntoni,Francesco Muntoni +29 more
TL;DR: A single-blind, placebo-controlled, dose-escalation study in patients with Duchenne muscular dystrophy to assess the safety and biochemical efficacy of an intramuscular morpholino splice-switching oligonucleotide (AVI-4658) that skips exon 51 in dystrophin mRNA.