< Back to previous page

Project

Clinical and Fundamental Aspects of Autosomal Dominant Polycystic Kidney Disease (ADPKD) (FWOTM937)

ADPKD accounts for 10% of the dialysis population and is characterized by the progressive enlargement of the kidneys due to cysts. The disease can be slowed in adults, but no cure exists. Kidney function often remains normal until adulthood, while cysts start to grow before birth. Normal renal tissue is destroyed, and remaining nephrons increase their function. This exhaust them and accelerates further loss of function. Starting treatment before damage has occurred could improve long-term outcomes. Half of the children with ADPKD will develop kidney failure as adults and might benefit from early treatment, but we cannot identify them. A proteomics analysis in young patients revealed several proteins that merit further study. We will investigate circulating proteins and the urinary concentration capacity in a unique cohort of children with ADPKD. We think these markers can predict the risk for progression to kidney failure and allow timely start of treatment, improving long-term outcomes. Second, we will develop cell lines from the urine of young patients. Currently, cells used to study ADPKD are derived from non-functioning end stage kidneys and from cysts. Cyst in ADPKD arise from all segments of the kidney but mostly from the distal portion. Existing cells from the proximal nephron will be compared with newly developed cells from the distal portion and we will study proteins that were identified by proteomics in various cells to gain new mechanistic insights.
Date:1 Oct 2018 →  30 Sep 2020
Keywords:adpkd, kidney
Disciplines:Kidney diseases