Molecular Structure of the PKD Protein Complex Finally Solved
Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic form of kidney failure worldwide.1 The disorder is characterized by the formation and enlargement of renal cysts that distort renal architecture, resulting in massively enlarged kidneys and end-stage kidney disease (ESKD). The disorder is also associated with numerous extrarenal complications, including polycystic liver disease and intracranial aneurysms.2 The most common form of ADPKD is caused by mutations in 2 genes: ∼85% of affected individuals have mutations in the PKD1 gene, while the rest have mutations in PKD2.