Thus, we conclude that expression of Nrf2, and some Nrf2 target genes, are elevated in those kidney tubule cells that have lost Fh1 and line renal cysts, and that, at least in the mouse model, this occurs independently of Hif-1α and Phds and at an early stage of renal cyst formation. The gene discussed is HIF1A; the disease is cystic kidney disease.