Inactivation of integrin-β1 prevents the development of polycystic kidney disease after the loss of polycystin-1

K Lee, S Boctor, LMC Barisoni… - Journal of the American …, 2015 - journals.lww.com
Abstract Dysregulation of polycystin-1 (PC1) leads to autosomal dominant polycystic kidney
disease (ADPKD), a disorder characterized by the formation of multiple bilateral renal cysts …

Stable knockdown of polycystin-1 confers integrin-α2β1–mediated anoikis resistance

L Battini, E Fedorova, S Macip, X Li… - Journal of the …, 2006 - journals.lww.com
The mechanisms of action of polycystin-1 (PC1) have been difficult to dissect because of its
interaction with multiple factors, the heterogeneity of the genetic mutations, and the …

The cleaved cytoplasmic tail of polycystin-1 regulates Src-dependent STAT3 activation

JJ Talbot, X Song, X Wang, MM Rinschen… - Journal of the …, 2014 - journals.lww.com
Abstract Polycystin-1 (PC1) mutations result in proliferative renal cyst growth and
progression to renal failure in autosomal dominant polycystic kidney disease (ADPKD). The …

Extracellular matrix, integrins, and focal adhesion signaling in polycystic kidney disease

Y Zhang, G Reif, DP Wallace - Cellular signalling, 2020 - Elsevier
In autosomal dominant polycystic kidney disease (ADPKD), the inexorable growth of
numerous fluid-filled cysts leads to massively enlarged kidneys, renal interstitial damage …

Polycystin-2 regulates proliferation and branching morphogenesis in kidney epithelial cells

DH Grimm, A Karihaloo, Y Cai, S Somlo… - Journal of Biological …, 2006 - ASBMB
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the formation of
multiple fluid-filled cysts that expand over time and destroy the renal architecture. Loss or …

The polycystin-1 C-terminal fragment triggers branching morphogenesis and migration of tubular kidney epithelial cells

C Nickel, T Benzing, L Sellin, P Gerke… - The Journal of …, 2002 - Am Soc Clin Investig
Mutations of either PKD1 or PKD2 cause autosomal dominant polycystic kidney disease, a
syndrome characterized by extensive formation of renal cysts and progressive renal failure …

Translational research in ADPKD: lessons from animal models

H Happe, DJM Peters - Nature Reviews Nephrology, 2014 - nature.com
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in PKD1 or
PKD2, which encode polycystin-1 and polycystin-2, respectively. Rodent models are …

Mutant polycystin-2 induces proliferation in primary rat tubular epithelial cells in a STAT-1/p21-independent fashion accompanied instead by alterations in expression …

KN Felekkis, P Koupepidou, E Kastanos, R Witzgall… - BMC nephrology, 2008 - Springer
Abstract Background Autosomal Dominant Polycystic Kidney Disease (ADPKD) is
characterized by the formation of multiple fluid-filled cysts that destroy the kidney …

Fibrocystin/polyductin modulates renal tubular formation by regulating polycystin-2 expression and function

I Kim, Y Fu, K Hui, G Moeckel, W Mai, C Li… - Journal of the …, 2008 - journals.lww.com
Autosomal recessive polycystic kidney disease is caused by mutations in PKHD1, which
encodes the membrane-associated receptor-like protein fibrocystin/polyductin (FPC). FPC …

Polycystin-2 expression is regulated by a PC2-binding domain in the intracellular portion of fibrocystin

I Kim, C Li, D Liang, XZ Chen, RJ Coffy, J Ma… - Journal of biological …, 2008 - ASBMB
Autosomal dominant (ADPKD) and autosomal recessive (ARPKD) polycystic kidney disease
are caused by mutations in Pkd1/Pkd2 and Pkhd1, which encode polycystins (PCs) and …