Cyst Reduction in a Polycystic Kidney Disease Drosophila Model Using Smac Mimics.

ABSTRACT: Autosomal dominant polycystic kidney disease (ADPKD) is an inherited malady affecting 12.5 million people worldwide. Therapeutic options to treat PKD are limited, due in part to lack of precise knowledge of underlying pathological mechanisms. Mimics of the second mitochondria-derived activator of caspases (Smac) have exhibited activity as antineoplastic agents and reported recently to ameliorate cysts in a murine ADPKD model, possibly by differentially targeting cystic cells and sparing the surrounding tissue. A first-in-kind Drosophila PKD model has now been employed to probe further the activity of novel Smac mimics. Substantial reduction of cystic defects was observed in the Malpighian (renal) tubules of treated flies, underscoring mechanistic conservation of the cystic pathways and pot

SUBMITTER: Millet-Boureima C 

PROVIDER: S-EPMC6966561 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature