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. 2018 Dec 10;129(1):388–405. doi: 10.1172/JCI98869

Figure 9. Formation of C1INH aggregates containing associated normal and mutant C1INH induced by dominant-negative SERPING1 gene variants causing HAE.

Figure 9

(A) Live widefield microscopy of HeLa cells cultured for 48 hours after cotransfection with 450 ng pSERPING1[WT]-mCherry and 450 ng pSERPING1[Variant]. Aggregates are indicated with arrows. Scale bars: 10 μm. (B) Western blot analysis of medium, soluble and insoluble C1INH derived from HeLa cells cultured for 72 hours after cotransfection with 450 ng pSERPING1[WT]-HA and 450 ng pSERPING1[Variant]. The experiment was carried out as described in Figure 6, B and C. (C) Coimmunoprecipitation on cell lysate from HeLa cells cultured for 48 hours after cotransfection with 20 μg pSERPING1[WT]-V5 and 20 μg pSERPING1[Variant]-HA. The experiment was carried out as described in Figure 6D. (A and B) Data are representative of findings from more than 3 biological replicates and similar results were seen in at least 2 independent experiments. Transfections were carried out in triplicate (n = 3; B) or duplicate (n = 2; C).