Ontology highlight
ABSTRACT:
SUBMITTER: Hayashi Y
PROVIDER: S-EPMC5135304 | biostudies-literature | 2016 Nov
REPOSITORIES: biostudies-literature
Proceedings of the National Academy of Sciences of the United States of America 20161028 46
Fibrodysplasia ossificans progressiva (FOP) patients carry a missense mutation in ACVR1 [617G > A (R206H)] that leads to hyperactivation of BMP-SMAD signaling. Contrary to a previous study, here we show that FOP fibroblasts showed an increased efficiency of induced pluripotent stem cell (iPSC) generation. This positive effect was attenuated by inhibitors of BMP-SMAD signaling (Dorsomorphin or LDN1931890) or transducing inhibitory SMADs (SMAD6 or SMAD7). In normal fibroblasts, the efficiency of i ...[more]