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. 2017 Sep 14;26(24):4786–4798. doi: 10.1093/hmg/ddx358

Figure 6.

Figure 6.

Tacrolimus activates Smad1/5/8 in cells expressing ALK1 HHT mutants. (A, B) C2C12 cells transfected with human WT ALK1 or ALK1 HHT mutants R411P (A) and T372fsX (B) were serum-starved for 3 h (0.1% FBS medium) and then treated for 3 h with the indicated concentrations of BMP9 or tacrolimus (FK-506). Please note that two immunoreactive bands were detected for WT ALK1, and that the R411P mutation increased the abundance of the low molecular weight band. Asterisk in (B) denotes a nonspecific band. (C) Partial genomic DNA sequences of the HHT patient ACVRL1 gene. Arrow indicates the c.1112dup insertion (T372fsX mutation) in the mutated allele. (D) HHT BOECs were treated with the indicated concentrations of tacrolimus for 24 h in depleted medium (0.1% FBS medium). Cell extracts in (A, B and D) were analyzed by WB using antibodies directed against the indicated proteins.