The recessive inheritance of TULP3 mutations is also consistent with our genetic studies in the mouse where the conditional knockout of both copies of Tulp3 in renal epithelial cells drive kidney cysts, while deletion of a single copy of Tulp3 failed to show phenotypes (Hwang et al., 2019; Legue and Liem, 2019). This evidence concerns the gene TULP3 and Complex Cyst of Kidney.