Abnormalities in focal adhesion complex formation, regulation, and function in human autosomal recessive polycystic kidney disease epithelial cells

S Israeli, K Amsler, N Zheleznova… - American Journal of …, 2010 - journals.physiology.org
Integrin-associated focal adhesion complex formation and turnover plays an essential role in
directing interactions between epithelial cells and the extracellular matrix during …

Cystic diseases of the kidney: role of adhesion molecules in normal and abnormal tubulogenesis

PD Wilson, CR Burrow - Experimental nephrology, 1999 - karger.com
This short review summarizes some information concerning what is known about matrix
adhesion molecules, focal adhesion proteins, and cell-cell adhesion molecules in normal …

[HTML][HTML] The polycystin 1-C-terminal fragment stimulates ERK-dependent spreading of renal epithelial cells

D Joly, S Ishibe, C Nickel, Z Yu, S Somlo… - Journal of biological …, 2006 - ASBMB
Polycystin 1, the product of the PKD1 gene, is mutated in autosomal dominant polycystic
kidney disease, a disease characterized by renal cyst formation and progressive renal …

Functional analysis of PKD1 transgenic lines reveals a direct role for polycystin-1 in mediating cell-cell adhesion

AJ Streets, LJ Newby, MJ O'Hare… - Journal of the …, 2003 - journals.lww.com
The PKD1 protein, polycystin-1, is a large transmembrane protein of uncertain function and
topology. To study the putative functions of polycystin-1, conditionally immortalized kidney …

[HTML][HTML] Polycystins, focal adhesions and extracellular matrix interactions

IA Drummond - Biochimica et Biophysica Acta (BBA)-Molecular Basis …, 2011 - Elsevier
Polycystic kidney disease is the most common heritable disease in humans. In addition to
epithelial cysts in the kidney, liver and pancreas, patients with autosomal dominant …

A polycystin-1 multiprotein complex is disrupted in polycystic kidney disease cells

T Roitbak, CJ Ward, PC Harris, R Bacallao… - Molecular biology of …, 2004 - Am Soc Cell Biol
Autosomal dominant polycystic kidney disease (ADPKD) is typified by the accumulation of
fluid-filled cysts and abnormalities in renal epithelial cell function. The disease is principally …

Impaired formation of desmosomal junctions in ADPKD epithelia

RJ Russo, H Husson, D Joly, NO Bukanov… - Histochemistry and cell …, 2005 - Springer
Abstract Mutations in polycystin-1 (PC-1) are responsible for autosomal dominant polycystic
kidney disease (ADPKD), characterized by formation of fluid-filled tubular cysts. The PC-1 is …

Mispolarization of desmosomal proteins and altered intercellular adhesion in autosomal dominant polycystic kidney disease

M Silberberg, AJ Charron, R Bacallao… - American Journal …, 2005 - journals.physiology.org
Polycystin-1, the product of the major gene mutated in autosomal dominant polycystic kidney
disease (ADPKD), has been shown to associate with multiple epithelial cell junctions. Our …

Homophilic and heterophilic polycystin 1 interactions regulate E-cadherin recruitment and junction assembly in MDCK cells

AJ Streets, BE Wagner, PC Harris… - Journal of cell …, 2009 - journals.biologists.com
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited
human renal disease and is caused by mutations in two genes, PKD1 (85%) and PKD2 …

[HTML][HTML] Fibrocystin is essential to cellular control of adhesion and epithelial morphogenesis

WH Ziegler, B Soetje, LP Marten, J Wiese… - International journal of …, 2020 - mdpi.com
Mutations of the Pkhd1 gene cause autosomal recessive polycystic kidney disease
(ARPKD). Pkhd1 encodes fibrocystin/polyductin (FPC), a ciliary type I membrane protein of …