Tulp3 Regulates Renal Cystogenesis by Trafficking of Cystoproteins to Cilia (Current Biology)

Hwang et al. show that embryonic-stage, nephron-specific Tulp3 knockouts develop cystic kidneys, while retaining intact cilia. The severity of cystogenesis is intermediate between that caused by loss of polycystin-1 or cilia. They uncover evidence that cystogenesis results from a reduction of ciliary levels of polycystins and the small GTPase Arl13b.