Distribution of α-integrin subunits in fetal polycystic kidney diseases

F Daïkha-Dahmane, F Narcy, M Dommergues… - Pediatric …, 1997 - Springer
An alteration in cell/matrix interactions is one of the suggested mechanisms leading to cyst
formation in polycystic kidney diseases. Most of these interactions are mediated by β1 …

The PKD1 gene product," polycystin-1," is a tyrosine-phosphorylated protein that colocalizes with alpha2beta1-integrin in focal clusters in adherent renal epithelia.

PD Wilson, L Geng, X Li, CR Burrow - Laboratory investigation; a …, 1999 - europepmc.org
Mutations in the PKD1 gene are responsible for autosomal dominant polycystic kidney
disease (ADPKD). Although PKD1 has been cloned and shown to be expressed at high …

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 …

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 …

Polycystin expression is temporally and spatially regulated during renal development

J Van Adelsberg, S Chamberlain… - American Journal of …, 1997 - journals.physiology.org
Mutations in PKD1 cause autosomal dominant polycystic kidney disease (ADPKD), a
common genetic disease in which cysts form from kidney tubules. The predicted product of …

The genes and proteins associated with poly-cystic kidney diseases.

PD Wilson - Minerva Urologica e Nefrologica= The Italian Journal of …, 2002 - europepmc.org
Genetically based polycystic kidney diseases include autosomal dominant (ADPKD) and
recessive (ARPKD) polycystic kidney diseases, nephronophthisis and medullary cystic …

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 …

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 …

Identification and localization of polycystin, the PKD1 gene product.

L Geng, Y Segal, B Peissel, N Deng… - The Journal of …, 1996 - Am Soc Clin Investig
Polycystin, the product of autosomal dominant polycystic kidney disease (ADPKD) 1 gene
(PKD1) is the cardinal member of a novel class of proteins. As a first step towards …

Characterisation and expression of the PKD-1 protein, polycystin, in renal and extrarenal tissues.

AC Ong, PC Harris, S Biddolph, C Bowker… - Kidney …, 1999 - europepmc.org
Studies of the nature and expression of the PKD-1 gene product, polycystin, have been
complicated by duplication of the PKD-1 gene, the low expression of the PKD-1 gene in …