ACE and renal tubular dysgenesis: Gribouval et al. ([34] performed immunostaining to identify whether mutations in REN (Renin), then ACE (Angiotensin Converting Enzyme), AGT (angiotensinogen), and AGTR1 (angiotensin II receptor type 1) were present in Renal Tubular Dysgenesis affected tissue hypothesizing all four genes were causal, and that dysfunction of any biomolecules involved in the renin-angiotensin system could potentially lead to Renal Tubular Dysgenesis.