Thus, hyperactive FAM111A protease activity is the proposed pathomechanism underlying autosomal dominant FAM111A-related KCS and osteocraniostenosis and could explain reduced DNA replication, enhanced DNA replication stress and damage, and the induction of apoptotic cell death in cancer cells stably expressing FAM111A patient-variant proteins [4, 7]. The gene discussed is FAM111A; the disease is cancer.