Polycystic kidney disease (PKD) is a leading cause of ESRD worldwide. In PKD, excessive
cell proliferation and fluid secretion, pathogenic interactions of mutated epithelial cells with …

Numerous murine (mouse and rat) models of polycystic kidney disease (PKD) have been
described in which the mutant phenotype results from a spontaneous mutation or …

Autosomal dominant polycystic kidney disease (ADPKD) is a common human genetic
disease characterized by cyst formation in kidney tubules and other ductular epithelia. Cells …

The primary cilium protrudes like an antenna from the cell surface, sensing mechanical and
chemical cues provided in the cellular environment. In some tissue types, ciliary orientation …

Extracellular matrix abnormalities have been found in both human and animal models of
polycystic kidney disease (PKD). A new mouse PKD model has been produced through …

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 …

Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenetic
disease predominantly caused by alteration or dysregulation of the PKD1 gene, which …

Mutations in the von Hippel-Lindau (VHL) gene are involved in the family cancer syndrome
for which it is named and the development of sporadic renal cell cancer (RCC) …

Mutations in the PKD1 gene are responsible for 85% of cases of autosomal dominant
polycystic kidney disease (ADPKD). This gene encodes a large membrane associated …

Renal cystogenesis is the pathological hallmark of autosomal dominant polycystic kidney
disease, caused by PKD1 and PKD2 mutations. The formation of renal cysts is a common …