Fig. 3

Effect of SERPING1 mutations on the expression of extracellular and extracellular C1-INH. (A) SERPING1 gene mutation plasmid sequencing: The red box shows c.550G > A, c.566C > A and c.708T > G mutant bases. (B) Detection of FLAG and C1-INH protein expression after plasmid transfection by Western blot. (C) Protein subcellular localization of WT and mutant of SERPING1 gene: Red indicates the location of the endoplasmic reticulum, green indicates the location of the C1-INH protein through the FLAG tag, blue indicates the nucleus, and the white arrow indicates the accumulation of proteins in the cell. (D-E) Western blot detection of protein expression of extracellular C1-INH after single transfection of mutant plasmid or co-transfection of mutant plasmid + WT. (F) Left: C1-INH protein concentration standard curve; Right: The difference of C1-INH protein concentration between mutant and wild type detected by ELISA. These experiments in B-F were replicated 3 times with similar results