Induction of Noonan syndrome-specific human-induced pluripotent stem cells under serum-, feeder-, and integration-free conditions
| Autor: | Sachiko Yamasaki, Shigeaki Toratani, Atsuko Hamada, Eri Akagi, Tetsuji Okamoto, Manami Ohtaka, Mahito Nakanishi, Fumitaka Obayashi, Ken Nishimura, Koichi Koizumi |
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| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
Homeobox protein NANOG Induced Pluripotent Stem Cells Mutation Missense Peripheral blood mononuclear cell Article Culture Media Serum-Free Pathogenesis 03 medical and health sciences Young Adult 0302 clinical medicine Feeder-free medicine Humans Amino Acid Sequence RNA Messenger Induced pluripotent stem cell Base Sequence business.industry Noonan Syndrome Genetic disorder Feeder Cells Cell Biology General Medicine Nanog Homeobox Protein medicine.disease Alkaline Phosphatase Cellular Reprogramming Disease-specific human-induced pluripotent stem cells 030104 developmental biology Disease modeling Integration-free Cell culture 030220 oncology & carcinogenesis Cancer research Leukocytes Mononuclear Noonan syndrome Female Stem cell business Serum-free Developmental Biology |
| Zdroj: | In Vitro Cellular & Developmental Biology. Animal |
| ISSN: | 1543-706X 1071-2690 |
| Popis: | 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. Supplementary Information The online version of this article (10.1007/s11626-020-00515-9) contains supplementary material, which is available to authorized users. |
| Databáze: | OpenAIRE |
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