Renal tubular dysgenesis (RTD) may result from genetic mutations in the gene encoding the major components of the renin-angiotensin-aldosterone system (RAAS), including angiotensinogen (AGT; MIM# 106150), renin (REN; MIM# 179820), angiotensin-converting enzyme, and angiotensin II receptor type 1 (AGTR1; MIM# 106165) (Gribouval et al., 2005, 2012). The gene discussed is ACE; the disease is renal tubular dysgenesis of genetic origin.