Figure 1.

Protease activity of missense HTRA1s identified in symptomatic carriers. (A) SDS-PAGE of WT and missense mutant HTRA1 proteins used in the protease assay. Black arrows indicate the full-length band of HTRA1 tagged with myc-His₆. (B) Protease activities of missense HTRA1s identified in symptomatic carriers and CARASIL patients. Activities were calculated from the slope of the linear portion of the normalized fluorescence vs. time (30, 60, 90 min) plots. Mean values from 3 independent experiments are shown. Red and blue bars indicate protease activities of WT and S328A, the positive and negative controls, respectively. Green bars indicate protease activities of missense HTRA1s identified in symptomatic carriers. Purple bars indicate protease activities of missense HTRA1s identified only in CARASIL patients. I-bars indicate standard errors (SE). Statistical comparisons of protease activities between WT and each missense HTRA1 protein were performed with one-way analysis of variance followed by the Dunnett's post hoc test. ^***P < 0.0001 for protease activities of each HTRA1 relative to WT. ^##P < 0.0001 for protease activities of HTRA1 relative to WT.