Figure 2 | Scientific Reports

Figure 2

From: Intermittent C1-Inhibitor Deficiency Associated with Recessive Inheritance: Functional and Structural Insight

Figure 2

Plasma C1-INH conformers versus function. (a) Non-reducing SDS-PAGE and immunoblot analysis of plasma from patient T.M. (378 R/C) at different functional values of C1-INH, showing the native (105 kd), and the aberrant conformers of 96 kd and 250 kd. Equal plasma volumes were loaded in each lane. Normal human plasma (NHP) and the Arg378Cys homozygote plasma (378C/C) are shown as reference. The primary Ab was the 12-27-15 monoclonal antibody, revealed with a secondary Ab designed for fluorescent detection. The figure represents part of the samples evaluated in panel b. (b) The relative amount of the different conformers is plotted vs C1-INH function measured in the same sample. The values are the mean of three independent experiments.

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