ACE and cyst: Upregulation of intrarenal RAS components including renin, angiotensin-converting enzyme (ACE) and Ang II has been described in a rodent model of autosomal recessive PKD (ARPKD)10 (in the absence of any changes in systemic Ang) and similar findings were seen in mouse models with loss of cilia or polycystin 1, where cyst formation and increased blood pressure were associated with increased kidney angiotensinogen (AGT) levels11.