Ontology highlight
ABSTRACT:
SUBMITTER: Onodera S
PROVIDER: S-EPMC7363748 | biostudies-literature | 2020 Jul
REPOSITORIES: biostudies-literature
Onodera Shoko S Saito Akiko A Hojo Hironori H Nakamura Takashi T Zujur Denise D Watanabe Katsuhito K Morita Nana N Hasegawa Daigo D Masaki Hideki H Nakauchi Hiromitsu H Nomura Takeshi T Shibahara Takahiko T Yamaguchi Akira A Chung Ung-Il UI Azuma Toshifumi T Ohba Shinsuke S
Stem cell reports 20200611 1
Two genetic diseases, Gorlin syndrome and McCune-Albright syndrome (MAS), show completely opposite symptoms in terms of bone mineral density and hedgehog (Hh) activity. In this study, we utilized human induced pluripotent stem cell (iPSC)-based models of the two diseases to understand the roles of Hh signaling in osteogenesis. Gorlin syndrome-derived iPSCs showed increased osteoblastogenesis and mineralization with Hh signaling activation and upregulation of a set of transcription factors in an ...[more]