[HTML][HTML] A polycystin-centric view of cyst formation and disease: the polycystins revisited

ACM Ong, PC Harris - Kidney international, 2015 - Elsevier
It is 20 years since the identification of PKD1, the major gene mutated in autosomal
dominant polycystic kidney disease (ADPKD), followed closely by the cloning of PKD2 …

Polycystin-2 (TRPP2): Ion channel properties and regulation

M del Rocío Cantero, HF Cantiello - Gene, 2022 - Elsevier
Abstract Polycystin-2 (TRPP2, PKD2, PC2) is the product of the PKD2 gene, whose
mutations cause Autosomal Dominant Polycystic Kidney Disease (ADPKD). PC2 belongs to …

Toxic tubular injury in kidneys from Pkd1-deletion mice accelerates cystogenesis accompanied by dysregulated planar cell polarity and canonical Wnt signaling …

H Happé, WN Leonhard, A van der Wal… - Human molecular …, 2009 - academic.oup.com
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by large fluid-filled
cysts and progressive deterioration of renal function necessitating renal replacement …

Pkd1 haploinsufficiency increases renal damage and induces microcyst formation following ischemia/reperfusion

AP Bastos, K Piontek, AM Silva, D Martini… - Journal of the …, 2009 - journals.lww.com
Mutations in PKD1 cause the majority of cases of autosomal dominant polycystic kidney
disease (ADPKD). Because polycystin 1 modulates cell proliferation, cell differentiation, and …

Pkd2 dosage influences cellular repair responses following ischemia-reperfusion injury

S Prasad, JP McDaid, FWK Tam, JL Haylor… - The American journal of …, 2009 - Elsevier
Autosomal dominant polycystic kidney disease (ADPKD) results from mutations in either
PKD1 or PKD2 and accounts for 10% of all patients on renal replacement therapy. The …

Haploinsufficiency of Pkd2 is associated with increased tubular cell proliferation and interstitial fibrosis in two murine Pkd2 models

MY Chang, E Parker, S Ibrahim… - Nephrology Dialysis …, 2006 - academic.oup.com
Background. Autosomal dominant polycystic kidney disease (ADPKD) is the most common
inherited human kidney disease and is caused by germline mutations in PKD1 (85%) or …

G-protein signaling modulator 1 deficiency accelerates cystic disease in an orthologous mouse model of autosomal dominant polycystic kidney disease

M Kwon, TS Pavlov, K Nozu… - Proceedings of the …, 2012 - National Acad Sciences
Polycystic kidney diseases are the most common genetic diseases that affect the kidney.
There remains a paucity of information regarding mechanisms by which G proteins are …

Polycystin 2 is increased in disease to protect against stress-induced cell death

AL Brill, TT Fischer, JM Walters, A Marlier… - Scientific reports, 2020 - nature.com
Abstract Polycystin 2 (PC2 or TRPP1, formerly TRPP2) is a calcium-permeant Transient
Receptor Potential (TRP) cation channel expressed primarily on the endoplasmic reticulum …

TMEM33 regulates intracellular calcium homeostasis in renal tubular epithelial cells

M Arhatte, GS Gunaratne, C El Boustany, IY Kuo… - Nature …, 2019 - nature.com
Mutations in the polycystins cause autosomal dominant polycystic kidney disease (ADPKD).
Here we show that transmembrane protein 33 (TMEM33) interacts with the ion channel …

Polycystin 2-dependent cardio-protective mechanisms revealed by cardiac stress

E Giehl, FO Lemos, Y Huang, FJ Giordano… - … -European Journal of …, 2017 - Springer
Although autosomal dominant polycystic kidney disease (ADPKD) is characterized by the
development of multiple kidney cysts, the most frequent cause of death in ADPKD patients is …