Skip to main content
. 2022 Jun 15;132(12):e153792. doi: 10.1172/JCI153792

Figure 3. Ligand-independent dimerization of ACVR1[R206H], but not WT ACVR1, induces Smad1/5/8 signaling.

Figure 3

HEK293 cells harboring p-Smad1/5/8–responsive luciferase reporter (BRE) were transfected with hACVR1-DmrB (A) or hACVR1[R206H]-DmrB (B). Homodimerization of C-terminally DmrB-tagged ACVR1 was induced with 20 nM B/B homodimerizer for 16 hours. Activin A activated Smad1/5/8 signaling only in hACVR1[R206H]-DmrB cells, but BMP6 activated Smad1/5/8 signaling both in hACVR1-DmrB and hACVR1[R206H]-DmrB cells (A and B). Intracellular homodimerization of hACVR1[R206H] activated Smad1/5/8 signaling in the absence of exogenous ligands (C) as well as in the presence of 300 nM ACVR2B-Fc ligand trap (D). Data show the mean (n = 4) ± SEM. Three biological replicates were performed for the in vitro signaling assays.