Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a hereditary disease that affects 1 in 500 people in New Zealand. Kidneys of ADPKD patients are filled with thousands of fluid filled cysts which are supported by an extensive network of scar-like tissue called interstitial fibrosis.

Primary cilia (arrows) in renal fibroblasts derived from Pkd mutant mice.

Researchers have recently discovered that defects in kidney epithelial cilia may be a potential cause of polycystic kidney disease. While the formation of cysts is thought to be the main cause of the disease, there has been little emphasis on the critical relationship between the appearance of interstitial fibrosis and the progression of the polycystic kidney disease.

This study aims to examine the fibrosis associated with ADPKD and investigate whether the primary cilium plays a role in the development of the highly destructive fibrosis.

People involved:

Assoc Professor Cynthia Jensen (PI)
Assoc Professor Tony Poole (PI)
Dr Sue McGlashan (AI)
Mrs Sarah Kennedy

Collaborators:

Prof Mike Eccles (University of Otago)
Dr Cherie Stayner (University of Otago)
Dr Alistair Johnstone (Massey University)
Assoc Prof Bradley Yoder (University of Alabama at Birmingham)

Funded by:

Auckland Medical Research Foundation
Royal Society of NZ Marsden Fund