Divergent function of polycystin 1 and polycystin 2 in cell size regulation

A Viau, F Kotsis, C Boehlke, S Braeg, M Klein… - Biochemical and …, 2020 - Elsevier
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 or
PKD2, the genes encoding polycystin 1 (PC1) and polycystin 2 (PC2), respectively. PC1 and …

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 …

Polycystin: In vitro synthesis, in vivo tissue expression, and subcellular localization identifies a large membrane-associated protein

O Ibraghimov-Beskrovnaya… - Proceedings of the …, 1997 - National Acad Sciences
The primary structure of polycystin predicts a large integral membrane protein with multiple
cell recognition motifs, but its function remains unknown. Insight into polycystin's normal …

Polycystin-1 induces resistance to apoptosis through the phosphatidylinositol 3-kinase/Akt signaling pathway

M Boca, G Distefano, F Qian, AK Bhunia… - Journal of the …, 2006 - journals.lww.com
Abstract Polycystin-1 (PC-1), the PKD1 gene product, is a large receptor whose expression
in renal epithelial cells results in resistance to apoptosis and tubulogenesis, a model …

Regulation of STATs by polycystin-1 and their role in polycystic kidney disease

T Weimbs, EE Olsan, JJ Talbot - Jak-Stat, 2013 - Taylor & Francis
Autosomal-dominant polycystic kidney disease (ADPKD) is a common genetic disease
caused by mutations in the gene coding for polycystin-1 (PC1). PC1 can regulate STAT …

Autosomal dominant polycystic kidney disease: recent advances in pathogenesis and treatment

MY Chang, ACM Ong - Nephron physiology, 2008 - karger.com
Autosomal dominant polycystic kidney disease (ADPKD) is a common genetic disorder
affecting 1 in 1,000 people in the general population and accounts for up to 10% of all …

Autosomal dominant polycystic kidney disease: recent advances in pathogenesis and potential therapies

T Mochizuki, K Tsuchiya, K Nitta - Clinical and experimental nephrology, 2013 - Springer
Autosomal dominant polycystic kidney disease (ADPKD) is the most common progressive
hereditary kidney disease. In 85–90% of cases, ADPKD results from a mutation in the PKD1 …

Interaction of the leucine-rich repeats of polycystin-1 with extracellular matrix proteins: possible role in cell proliferation

AN Malhas, RA Abuknesha… - Journal of the American …, 2002 - journals.lww.com
Polycystin-1, the product of the PKD1 gene, is a membrane-bound multidomain protein with
a unique structure and a molecular weight of≈ 460 kD. The purpose of this study is to …

Murine polycystic kidney epithelial cell lines have increased integrin-mediated adhesion to collagen

J van Adelsberg - American Journal of Physiology-Renal …, 1994 - journals.physiology.org
Polycystic kidney disease (PKD), in which epithelial cysts arise from or instead of normal
renal tubules, is one of the most common genetic diseases. It has both autosomal dominant …

[HTML][HTML] Human polycystin-2 transgene dose-dependently rescues ADPKD phenotypes in Pkd2 mutant mice

A Li, X Tian, X Zhang, S Huang, Y Ma, D Wu… - The American Journal of …, 2015 - Elsevier
Although much is known about the molecular genetic mechanisms of autosomal-dominant
polycystic kidney disease (ADPKD), few effective treatment is currently available. Here, we …