A cleavage product of Polycystin-1 is a mitochondrial matrix protein that affects mitochondria morphology and function when heterologously expressed.

ABSTRACT: Recent studies have reported intrinsic metabolic reprogramming in Pkd1 knock-out cells, implicating dysregulated cellular metabolism in the pathogenesis of polycystic kidney disease. However, the exact nature of the metabolic changes and their underlying cause remains controversial. We show herein that Pkd1 ^k o ^/ko renal epithelial cells have impaired fatty acid utilization, abnormal mitochondrial morphology and function, and that mitochondria in kidneys of ADPKD patients have morphological alterations. We further show that a C-terminal cleavage product of polycystin-1 (CTT) translocates to the mitochondria matrix and that expression of CTT in Pkd1 ^ko/ko cells rescues some of the mitochondrial phenotypes. Using Drosophila to model in vivo effects, we find t

SUBMITTER: Lin CC 

PROVIDER: S-EPMC5807443 | biostudies-literature | 2018 Feb

REPOSITORIES: biostudies-literature