Derick G. Wansink
Radboud University Nijmegen
Publications 49
#1Remco T. P. van Cruchten (Radboud University Nijmegen)
#2Derick G. Wansink (Radboud University Nijmegen)H-Index: 17
#1Cornelia Rosanne Maria Ausems (Radboud University Nijmegen)
#2Renée H.L. Raaijmakers (Radboud University Nijmegen)H-Index: 1
Last.H. van Bokhoven (Radboud University Nijmegen)H-Index: 67
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Pericytes are multipotent, vessel-associated progenitors that exhibit high proliferative capacity, can cross the blood-muscle barrier, and have the ability to home to muscle tissue and contribute to myogenesis. Consequently, pericyte-based therapies hold great promise for muscular dystrophies. A complex multi-system disorder exhibiting muscular dystrophy for which pericytes might be a valuable cell source is myotonic dystrophy type 1 (DM1). DM1 is caused by an unstable (CTG)n repeat in the DMPK ...
#1Wiljan Hendriks (Radboud University Nijmegen)H-Index: 38
#2Nicole Bakker (Radboud University Nijmegen)
Last.Peter H.M. Klaren (Radboud University Nijmegen)H-Index: 20
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Background Study motivation and knowledge retention benefit from regular student self-assessments. Inclusion of certainty-based learning (CBL) in computer-assisted formative tests may further enhance this by enabling students to identify whether they are uninformed or misinformed regarding the topics tested, which may trigger future study actions including instructor consultation.
The congenital form of myotonic dystrophy type 1 (cDM) is caused by the large-scale expansion of a (CTG•CAG)n repeat in DMPK and DM1-AS. The production of toxic transcripts with long trinucleotide tracts from these genes results in impairment of the myogenic differentiation capacity as cDM’s most prominent morpho-phenotypic hallmark. In the current in vitro study, we compared the early differentiation programs of isogenic cDM myoblasts with and without a (CTG)2600 repeat obtained by gene editing...
#1M. Leontien van der Bent (Radboud University Nijmegen)H-Index: 1
#2Omar Paulino da Silva Filho (Radboud University Nijmegen)H-Index: 1
Last.Roland Brock (Radboud University Nijmegen)H-Index: 28
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Antisense oligonucleotides (ASOs) are a promising class of therapeutics that are starting to emerge in the clinic. Determination of intracellular concentrations required for biologic effects and id...
CRISPR/Cas technology holds promise for the development of therapies to treat inherited diseases. Myotonic dystrophy type 1 (DM1) is a severe neuromuscular disorder with a variable multisystemic character for which no cure is yet available. Here, we review CRISPR/Cas-mediated approaches that target the unstable (CTG•CAG)n repeat in the DMPK/DM1-AS gene pair, the autosomal dominant mutation that causes DM1. Expansion of the repeat results in a complex constellation of toxicity at the DNA level, a...
1 CitationsSource
#1Laurène M. André (Radboud University Nijmegen)H-Index: 2
#2Remco T. P. van Cruchten (Radboud University Nijmegen)
Last.Derick G. Wansink (Radboud University Nijmegen)H-Index: 17
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Myotonic dystrophy type 1 (DM1) is a severe neuromuscular disorder caused by the expression of trinucleotide repeat-containing DMPK transcripts. Abnormally expanded (CUG)n repeats in these transcripts form hairpin-like structures that cause the RNA to accumulate in the cell nucleus by sequestering isoforms of the Muscleblind (MBNL) family, tissue-specific regulators of developmentally programmed, post-transcriptional processes in RNA metabolism. Through this mechanism, the function of MBNL in RN...
#1S. Yanovsky-Dagan (HUJI: Hebrew University of Jerusalem)H-Index: 1
#2E. Bnaya (HUJI: Hebrew University of Jerusalem)H-Index: 1
Last.Rachel Eiges (HUJI: Hebrew University of Jerusalem)H-Index: 11
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Myotonic dystrophy type 1 (DM1) results from a CTG repeat expansion in the 3-UTR of DMPK. When the repeat extensively expands, this results in DMPK aberrant methylation, reduction in SIX5 transcription and the development of the congenital form of the disease. To explore whether hypermethylation could be reversed in DM1 embryonic stem cells (hESCs) and patient myoblasts, we monitored methylation levels following removal of the expanded repeat by CRISPR/Cas9-mediated editing. Excision of the repe...
1 CitationsSource
#2Bé WieringaH-Index: 51
Last.Derick G. WansinkH-Index: 17
view all 3 authors...
2 CitationsSource