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. 2022 Jun 15;132(12):e153792. doi: 10.1172/JCI153792

Figure 5. Anti-ACVR1 antibody activation of ACVR1[R206H] is type II receptor dependent.

Figure 5

(A) Acvr1[R206H]/+; GT(ROSA26)SorCreERT2/+ ([R206H]/+) mES cells lacking Acvr2a plus Acvr2b, or Bmpr2 or all 3 of these type II receptor genes were treated with 10 nM activin A, BMP7, BMP2, BMP10, or anti-ACVR1 mAb 1 for 1 hour. Activin A, BMP7, BMP2, BMP10, and anti-ACVR1 mAb 1 induced Smad1/5/8 phosphorylation in cells that lack Bmpr2 but retain Acvr2a and Acvr2b, but not in cells where Acvr2a and Acvr2b or all 3 type II receptors have been knocked out. (B) ACVR2B coimmunoprecipitates with both ACVR1 and ACVR1[R206H] from W20 cells expressing Myc-tagged ACVR1 and/or HA-tagged ACVR2B. Myc-ACVR1 was immunoprecipitated using an anti-Myc antibody. ACVR1 and ACVR2B were detected using an anti-ACVR1 or anti-HA antibody, respectively.