Polycystic kidney disease

C Bergmann, LM Guay-Woodford, PC Harris… - Nature reviews Disease …, 2018 - nature.com
Cystic kidneys are common causes of end-stage renal disease, both in children and in
adults. Autosomal dominant polycystic kidney disease (ADPKD) and autosomal recessive …

The endoplasmic reticulum (ER) chaperone BiP is a master regulator of ER functions: Getting by with a little help from ERdj friends

KFR Pobre, GJ Poet, LM Hendershot - Journal of Biological Chemistry, 2019 - ASBMB
The endoplasmic reticulum (ER) represents the entry point into the secretory pathway where
nascent proteins encounter a specialized environment for their folding and maturation …

Mutations in GANAB, encoding the glucosidase IIα subunit, cause autosomal-dominant polycystic kidney and liver disease

B Porath, VG Gainullin, E Cornec-Le Gall… - The American Journal of …, 2016 - cell.com
Autosomal-dominant polycystic kidney disease (ADPKD) is a common, progressive, adult-
onset disease that is an important cause of end-stage renal disease (ESRD), which requires …

Isolated polycystic liver disease genes define effectors of polycystin-1 function

W Besse, K Dong, J Choi, S Punia… - The Journal of …, 2017 - Am Soc Clin Investig
Dominantly inherited isolated polycystic liver disease (PCLD) consists of liver cysts that are
radiologically and pathologically identical to those seen in autosomal dominant polycystic …

Pharmacological targeting of MYC-regulated IRE1/XBP1 pathway suppresses MYC-driven breast cancer

NA Zhao, J Cao, L Xu, Q Tang… - The Journal of …, 2018 - Am Soc Clin Investig
The unfolded protein response (UPR) is a cellular homeostatic mechanism that is activated
in many human cancers and plays pivotal roles in tumor progression and therapy resistance …

Research progress on endoplasmic reticulum homeostasis in kidney diseases

D Wu, LF Huang, XC Chen, XR Huang, HY Li… - Cell Death & …, 2023 - nature.com
The endoplasmic reticulum (ER) plays important roles in biosynthetic and metabolic
processes, including protein and lipid synthesis, Ca2+ homeostasis regulation, and …

ER stress, the unfolded protein response and osteoclastogenesis: a review

W Huang, Y Gong, L Yan - Biomolecules, 2023 - mdpi.com
Endoplasmic reticulum (ER) stress and its adaptive mechanism, the unfolded protein
response (UPR), are triggered by the accumulation of unfolded and misfolded proteins …

IRE1 signaling regulates chondrocyte apoptosis and death fate in the osteoarthritis

R Huang, Z Hui, S Wei, D Li, W Li… - Journal of cellular …, 2022 - Wiley Online Library
IRE1 is an important central regulator of unfolded protein response (UPR) in the
endoplasmic reticulum (ER) because of its ability to regulate cell fate as a function of stress …

Polycystic kidney disease: Novel insights into polycystin function

L Luo, S Roy, L Li, M Ma - Trends in Molecular Medicine, 2023 - cell.com
Autosomal dominant polycystic kidney disease (ADPKD) is a life-threatening monogenic
disease caused by mutations in PKD1 and PKD2 that encode polycystin 1 (PC1) and …

Cilia and polycystic kidney disease

M Ma - Seminars in Cell & Developmental Biology, 2021 - Elsevier
Polycystic kidney disease (PKD), comprising autosomal dominant polycystic kidney disease
(ADPKD) and autosomal recessive polycystic kidney d isease (ARPKD), is characterized by …