[HTML][HTML] Metabolic reprogramming and the role of mitochondria in polycystic kidney disease

C Podrini, L Cassina, A Boletta - Cellular signalling, 2020 - Elsevier
Abstract Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a slowly progressive
disease characterized by the relentless growth of renal cysts throughout the life of affected …

Metabolism and mitochondria in polycystic kidney disease research and therapy

V Padovano, C Podrini, A Boletta… - Nature Reviews …, 2018 - nature.com
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common,
potentially lethal, monogenic diseases and is caused predominantly by mutations in …

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

CC Lin, M Kurashige, Y Liu, T Terabayashi… - Scientific reports, 2018 - nature.com
Recent studies have reported intrinsic metabolic reprogramming in Pkd1 knock-out cells,
implicating dysregulated cellular metabolism in the pathogenesis of polycystic kidney …

Increased mitochondrial fragmentation in polycystic kidney disease acts as a modifier of disease progression

L Cassina, M Chiaravalli, A Boletta - The FASEB Journal, 2020 - Wiley Online Library
Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenic disorder,
characterized by bilateral renal cyst formation. Multiple pathways are de‐regulated in cystic …

Dissection of metabolic reprogramming in polycystic kidney disease reveals coordinated rewiring of bioenergetic pathways

C Podrini, I Rowe, R Pagliarini, ASH Costa… - Communications …, 2018 - nature.com
Abstract Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a genetic disorder
caused by loss-of-function mutations in PKD1 or PKD2. Increased glycolysis is a prominent …

Metabolic reprogramming in autosomal dominant polycystic kidney disease: evidence and therapeutic potential

KL Nowak, K Hopp - Clinical Journal of the American Society of …, 2020 - journals.lww.com
Autosomal dominant polycystic kidney disease is characterized by progressive development
and enlargement of kidney cysts, leading to ESKD. Because the kidneys are under high …

The pathobiology of polycystic kidney disease from a metabolic viewpoint

LF Menezes, GG Germino - Nature Reviews Nephrology, 2019 - nature.com
Autosomal dominant polycystic kidney disease (ADPKD) affects an estimated 1 in 1,000
people and slowly progresses to end-stage renal disease (ESRD) in about half of these …

The cpk model of recessive PKD shows glutamine dependence associated with the production of the oncometabolite 2-hydroxyglutarate

VJ Hwang, J Kim, A Rand, C Yang… - American Journal …, 2015 - journals.physiology.org
Since polycystic kidney disease (PKD) was first noted over 30 years ago to have neoplastic
parallels, there has been a resurgent interest in elucidating neoplasia-relevant pathways in …

[HTML][HTML] Metabolic reprogramming in a slowly developing orthologous model of polycystic kidney disease

K Hopp, EK Kleczko, BY Gitomer… - American Journal …, 2022 - journals.physiology.org
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited
kidney disease and affects 1 in 1,000 individuals. There is accumulating evidence …

Polycystin 2 regulates mitochondrial Ca2+ signaling, bioenergetics, and dynamics through mitofusin 2

IY Kuo, AL Brill, FO Lemos, JY Jiang, JL Falcone… - Science …, 2019 - science.org
Mitochondria and the endoplasmic reticulum (ER) have an intimate functional relationship
due to tethering proteins that bring their membranes in close (~ 30 nm) apposition. One …