C
Cornelis K. van der Ent
Researcher at Utrecht University
Publications - 239
Citations - 11047
Cornelis K. van der Ent is an academic researcher from Utrecht University. The author has contributed to research in topics: Cystic fibrosis & Medicine. The author has an hindex of 45, co-authored 217 publications receiving 8969 citations. Previous affiliations of Cornelis K. van der Ent include Boston Children's Hospital & University Medical Center Utrecht.
Papers
More filters
Journal ArticleDOI
Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR
Jennifer L. Taylor-Cousar,Anne Munck,Edward F. McKone,Cornelis K. van der Ent,Alexander Moeller,Christopher Simard,Linda T Wang,Edward P. Ingenito,Charlotte M. McKee,Yimeng Lu,J Lekstrom-Himes,J. Stuart Elborn +11 more
TL;DR: These data show that lumacaftor in combination with ivacaftors provided a benefit for patients with cystic fibrosis homozygous for the Phe508del CFTR mutation.
Journal ArticleDOI
Functional Repair of CFTR by CRISPR/Cas9 in Intestinal Stem Cell Organoids of Cystic Fibrosis Patients
Gerald Schwank,Bon-Kyoung Koo,Valentina Sasselli,Johanna F. Dekkers,Inha Heo,Turan Demircan,Nobuo Sasaki,Sander Boymans,Edwin Cuppen,Cornelis K. van der Ent,Edward E. S. Nieuwenhuis,Jeffrey M. Beekman,Hans Clevers +12 more
TL;DR: The CRISPR/Cas9 genome editing system is used to correct the CFTR locus by homologous recombination in cultured intestinal stem cells of CF patients and the corrected allele is expressed and fully functional as measured in clonally expanded organoids.
Journal ArticleDOI
A functional CFTR assay using primary cystic fibrosis intestinal organoids
Johanna F. Dekkers,Caroline L. Wiegerinck,Hugo R. de Jonge,Inez Bronsveld,Hettie M. Janssens,Karin M. de Winter-de Groot,Arianne M. Brandsma,Nienke W M de Jong,Marcel J. C. Bijvelds,Bob J. Scholte,Edward E. S. Nieuwenhuis,Stieneke van den Brink,Hans Clevers,Cornelis K. van der Ent,Sabine Middendorp,Jeffrey M. Beekman +15 more
TL;DR: This relatively simple and robust assay will facilitate diagnosis, functional studies, drug development and personalized medicine approaches in cystic fibrosis.
Journal ArticleDOI
Long-term expanding human airway organoids for disease modeling
Norman Sachs,Angelos Papaspyropoulos,Domenique D. Zomer-van Ommen,Inha Heo,Lena Böttinger,Dymph Klay,Fleur Weeber,Guizela Huelsz-Prince,Nino Iakobachvili,Gimano D. Amatngalim,Joep de Ligt,Arne Van Hoeck,Natalie Proost,Marco C. Viveen,Anna Lyubimova,Luc Teeven,Sepideh Derakhshan,Jeroen Korving,Harry Begthel,Johanna F. Dekkers,Kuldeep Kumawat,Emilio Ramos,Matthijs F.M. van Oosterhout,G. Johan A. Offerhaus,Dominique J Wiener,Eduardo P. Olimpio,Krijn K. Dijkstra,Egbert F. Smit,Maarten van der Linden,Sridevi Jaksani,Marieke van de Ven,Jos Jonkers,Anne C. Rios,Emile E. Voest,Coline H.M. van Moorsel,Cornelis K. van der Ent,Edwin Cuppen,Alexander van Oudenaarden,Frank E. J. Coenjaerts,Linde Meyaard,Louis Bont,Peter J. Peters,Sander J. Tans,Jeroen S. van Zon,Sylvia F. Boj,Robert G.J. Vries,Jeffrey M. Beekman,Hans Clevers +47 more
TL;DR: It is concluded that human airway organoids represent versatile models for the in vitro study of hereditary, malignant, and infectious pulmonary disease.
Journal ArticleDOI
Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis
Johanna F. Dekkers,Gitte Berkers,Evelien Kruisselbrink,Annelotte M. Vonk,Hugo R. de Jonge,Hettie M. Janssens,Inez Bronsveld,Eduard A. van de Graaf,Edward E. S. Nieuwenhuis,Roderick H. J. Houwen,Frank P. Vleggaar,Johanna C. Escher,Yolanda B. de Rijke,Christof J. Majoor,Harry G.M. Heijerman,Karin M. de Winter-de Groot,Hans Clevers,Cornelis K. van der Ent,Jeffrey M. Beekman +18 more
TL;DR: In vitro measurements of CFTR function in patient-derived rectal organoids may be useful for identifying subjects who would benefit from CFTR-correcting treatment, independent of their CFTR mutation.