Figure 5.

Circulating molecular species were displayed by an anti-C1-INH immunoblot assay. Citrate-plasma samples were collected from patients presenting with hereditary angioedema with C1 Inhibitor deficiency (C1-INH-HAE). Native plasma and plasma submitted to a 15-min incubation at 37°C with 0.1 nM C1s protease (plasma + C1s protease) were analyzed as described in (20). Four assays are displayed corresponding to healthy controls and patients with C1-INH-HAE: one type-I HAE (HAE-1) variant and three HAE-1/-2 variants, distributed in classes II and III. HAE-1/-2, C1-INH-HAE satisfying Rosen's criterion (18), with the expression of both alleles and presenting with a low antigenic C1-INH. C1-INH, C1 inhibitor.