Establishment of Gorin syndrome specific iPSC and elucidation of the mechanism of cystic odontogenic tumor by genome operation

2018 Fiscal Year Final Research Report

• Project/Area Number: 17K17252
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Surgical dentistry
• Research Institution: Hiroshima University
• Principal Investigator: Hamada Atsuko 広島大学, 病院(歯), 歯科診療医 (30760318)
• Project Period (FY): 2017-04-01 – 2019-03-31
• Keywords: 疾患特異的iPSC

Outline of Final Research Achievements

In this study, we aimed to elucidate the onset mechanism of hereditary diseases causing lesions in the maxillofacial region and establish a diagnosis and treatment method, and successfully induced and established NBCCS disease specific iPSCs under serum- and feeder-free culture conditions. Furthermore, to create a pathological condition model under serum-free conditions, we performed inductions of epithelial stem cells and mesenchymal stem cells using wild type iPSCs and NBCCS-iPSCs, and compared. As a result, in the proliferation ability of the induced epithelial stem cells, the NBCCS-iPSC-derived one is higher than that of the wild type-iPSC-derived one, which indicates that a part of the pathological condition could be reproduced in vitro.

Free Research Field

口腔外科

Academic Significance and Societal Importance of the Research Achievements

顎顔面口腔領域に病変を生じる遺伝性疾患においては、その発症機構や診断・治療法が十分に確立されていない。本研究では基底細胞母斑症候群（NBCCS）をモデルとしてNBCCS疾患特異的iPSCの樹立に成功した。さらに、本疾患の主要な症状である顎骨多発嚢胞の発症機序を明らかにする目的で、NBCCS-iPSCを上皮細胞に分化誘導し健常人iPS由来のそれと比較することで、NBCCS-iPSC由来上皮細胞は増殖能が高いことを明らかとした。本研究より得られる知見は、顎顔面口腔疾患の病態解明や発症機序の解析、さらには治療法の開発研究に寄与するものと考えられる。