[HTML][HTML] Polycystin-1, STAT6, and P100 function in a pathway that transduces ciliary mechanosensation and is activated in polycystic kidney disease

SH Low, S Vasanth, CH Larson, S Mukherjee… - Developmental cell, 2006 - cell.com
Primary cilia are implicated in the pathogenesis of autosomal-dominant polycystic kidney
disease (ADPKD), which results from defects in polycystin-1 (PC1), but the function of PC1 …

Polycystins 1 and 2 mediate mechanosensation in the primary cilium of kidney cells

SM Nauli, FJ Alenghat, Y Luo, E Williams, P Vassilev… - Nature …, 2003 - nature.com
Several proteins implicated in the pathogenesis of polycystic kidney disease (PKD) localize
to cilia. Furthermore, cilia are malformed in mice with PKD with mutations in TgN737Rpw …

Polycystins and mechanosensation in renal and nodal cilia

SM Nauli, J Zhou - Bioessays, 2004 - Wiley Online Library
The external surfaces of the human body, as well as its internal organs, constantly
experience different kinds of mechanical stimulations. For example, tubular epithelial cells of …

[HTML][HTML] The exocyst protein Sec10 interacts with Polycystin-2 and knockdown causes PKD-phenotypes

B Fogelgren, SY Lin, X Zuo, KM Jaffe, KM Park… - PLoS …, 2011 - journals.plos.org
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by formation of
renal cysts that destroy the kidney. Mutations in PKD1 and PKD2, encoding polycystins-1 …

Ciliary mechanisms of cyst formation in polycystic kidney disease

M Ma, AR Gallagher, S Somlo - Cold Spring Harbor …, 2017 - cshperspectives.cshlp.org
Autosomal-dominant polycystic kidney disease (ADPKD) is a disease of defective tissue
homeostasis resulting in active remodeling of nephrons and bile ducts to form fluid-filled …

Polycystin-2 traffics to cilia independently of polycystin-1 by using an N-terminal RVxP motif

L Geng, D Okuhara, Z Yu, X Tian, Y Cai… - Journal of cell …, 2006 - journals.biologists.com
Primary cilia play a key role in the pathogenesis of autosomal dominant polycystic kidney
disease (ADPKD). The affected proteins, polycystin-1 (PC1) and polycystin-2 (PC2), interact …

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 …

Polycystin-2 immunolocalization and function in zebrafish

T Obara, S Mangos, Y Liu, J Zhao… - Journal of the …, 2006 - journals.lww.com
Polycystin-2 functions as a cation-permeable transient receptor potential ion channel in
kidney epithelial cells and when mutated results in human autosomal dominant polycystic …

Human ADPKD primary cyst epithelial cells with a novel, single codon deletion in the PKD1 gene exhibit defective ciliary polycystin localization and loss of flow …

C Xu, S Rossetti, L Jiang, PC Harris… - American Journal …, 2007 - journals.physiology.org
Autosomal dominant polycystic kidney disease (ADPKD) gene products polycystin-1 (PC1)
and polycystin-2 (PC2) colocalize in the apical monocilia of renal epithelial cells. Mouse and …

[HTML][HTML] Polycystin-2 localizes to kidney cilia and the ciliary level is elevated in orpk mice with polycystic kidney disease

GJ Pazour, JT San Agustin, JA Follit, JL Rosenbaum… - Current Biology, 2002 - cell.com
Defects in the PKD1 and PKD2 genes cause autosomal dominant polycystic kidney disease
(PKD) in~ 1 in 1000 adults worldwide. These genes encode polycystin-1 and polycystin-2 …