Interestingly, vasculopathy-associated mutations in HtrA1 that abolish its protease activity (Hara et al., 2009) and ability to oligomerize (Nozaki et al., 2016), but harbor the anti-TGFβ-like KI domain, are unable to antagonize TGFβ signaling. The gene discussed is TGFB1; the disease is vascular disorder.