The leading cause of death in autosomal dominant polycystic kidney disease (ADPKD) is
cardiovascular. However, little is known about the pathogenesis of these manifestations. The …

The expression of polycystin-1 in the vascular smooth muscle cells (VSMC) of elastic and
large distributive arteries suggests that some vascular manifestations of autosomal …

The kidney develops from direct interactions between the ureteric bud and the metanephric
mesenchyme. The ureteric bud gives rise to the collecting system and the metanephric …

Mutations in the PKD1 and PKD2 genes account for 85 and 15% of cases of autosomal
dominant polycystic kidney disease, respectively. Polycystin-2, the product of the PKD2 …

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 …

Autosomal-dominant polycystic kidney disease, one of the most frequent human genetic
disorders, is genetically heterogeneous. Most cases result from mutations of PKD1 or PKD2 …

Cellular interactions with collagen in a model of kidney tubulogenesis were investigated
using Madin-Darby canine kidney (MDCK) cells in an in vitro morphogenetic system. MDCK …

Background In a majority of cases, autosomal dominant polycystic kidney disease (ADPKD)
is caused by mutations within a putative open reading frame of the PKD1 gene. The …

Integrins mediate cell-cell and cell-extracellular matrix interactions and play key roles in
development. β 6 integrin expression has been demonstrated in human fetal kidney at a …

The polycystic kidney disease-1 gene, which is mutated in the majority of patients with
autosomal dominant polycystic kidney disease, has been identified. The protein encoded by …