PRSS8 and congenital diarrhea 5 with tufting enteropathy: Although rendering prostasin zymogen-locked allowed HAI-2-deficient embryos to successfully pass previously established HAI-2-dependent early and mid-term developmental milestones, these mice all developed a congenital tufting enteropathy (CTE)-like phenotype leading to an inability to gain weight after birth and an early postnatal lethality.