Elucidation of osteoblast mineralization mechanism using McCunealbright syndrome iPS cells
Description: The pathophysiology of congenital skeletal diseases is diverse, and it has been clarified that disease-specific iPS cell application is important for elucidating the pathophysiological mechanism of the disease. GNAS-rich iPS cells (fibrous dysplasia and McCune-Albright syndrome) iPS cells were generated and compared with Gorlin syndrome ipS cells, with the aim of elucidating the relationship and pathophysiology of the Hedgehog pathway and the GNAS pathway. When GNAS-rich iPS cells were established and neoplastic lesions were reproduced, it was found that the undifferentiated state of diseased cells was enhanced. Therefore, it was clarified that the inhibition of osteoblast differentiation in fibrous dysplasia may be directly linked to the treatment by appropriately correcting the inhibition of Hedgehog by drug administration.
Amount: ¥17,810,000
Funding type: research
Funder scheme: Grant-in-Aid for Scientific Research (B)
Start date: 2018-04-01
End date: 2021-03-31
Investigator: TOSHIFUMI AZUMA
Institution: Tokyo Dental College
Funded outputs count: 11
Funder award ID: 18H03007
OpenAlex ID: G6689124391