Induction of Noonan syndrome-specific human-induced pluripotent stem cells under serum-, feeder-, and integration-free conditions.

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Publisher: Springer
Document Type: Report; Brief article
Length: 279 words

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Keywords: Noonan syndrome; Disease-specific human-induced pluripotent stem cells; Disease modeling; Serum-free; Feeder-free; Integration-free Abstract Noonan syndrome is an autosomal dominant developmental disorder. Although it is relatively common, and its phenotypical variability is well documented, its pathophysiology is not fully understood. Previously, with the aim of revealing the pathogenesis of genetic disorders, we reported the induction of cleidocranial dysplasia-specific human-induced pluripotent stem cells (hiPSCs) from patient's dental pulp cells (DPCs) under serum-free, feeder-free, and integration-free conditions. Notably, these cells showed potential for application to genetic disorder disease models. Furthermore, using similar procedures, we reported the induction of hiPSCs derived from peripheral blood mononuclear cells (PBMCs) of healthy volunteers. These methods are beneficial, because they are carried out without invasive and painful biopsies. Using those procedures, we reprogrammed DPCs and PBMCs that were derived from a patient with Noonan syndrome (NS) to establish NS-specific hiPSCs (NS-DPC-hiPSCs and NS-PBMC-hiPSCs, respectively). The induction efficiency of NS-hiPSCs was higher than that of WT-hiPSCs. We hypothesize that this was caused by high NANOG expression. Here, we describe the experimental results and findings related to NS-hiPSCs. This is the first report on the establishment of NS-hiPSCs and their disease modeling. Author Affiliation: (1) Oral and Maxillofacial Surgery, Hiroshima University Hospital, Hiroshima, Japan (2) Department of Molecular Oral Medicine and Maxillofacial Surgery, Graduate School of Biomedical and Health Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, 734-8553, Hiroshima, Japan (3) TOKIWA-Bio, Inc., Tsukuba, Ibaraki, Japan (4) National Institute of Advanced Industrial Science and Technology (AIST), Tsukuba, Ibaraki, Japan (5) Laboratory of Gene Regulation, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan (k) tetsuok@hiroshima-u.ac.jp Article History: Registration Date: 09/18/2020 Received Date: 09/06/2020 Accepted Date: 09/17/2020 Online Date: 11/02/2020 Byline:

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Gale Document Number: GALE|A648054609