Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
8625071 | Bone | 2018 | 7 Pages |
Abstract
The mutated FOP receptor [ACVR1 (R206H)] is hypersensitive to BMP4 and uniquely sensitive (compared to the wild type receptor) to Act A. Both canonical and non-canonical ligands have a synergistic effect on BMP-pSmad1/5/8 signaling in FOP CTPCs and may cooperate to alter the threshold for HO in FOP. Our findings in primary human FOP CTPCs have important implications for the design of clinical trials to inhibit dysregulated BMP pathway signaling in humans who have FOP.
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Authors
Haitao Wang, Eileen M. Shore, Robert J. Pignolo, Frederick S. Kaplan,