Autosomal dominant polycystic kidney disease (ADPKD) is a progressive inherited disorder
in which renal tissue is gradually replaced with fluid-filled cysts, giving rise to chronic kidney …

Autosomal dominant polycystic kidney disease (ADPKD) is the most common potentially
lethal monogenic disorder, with more than 12 million cases worldwide. The two causative …

First identified in Drosophila, the Crumbs (Crb) proteins are important in epithelial polarity,
apical membrane formation, and tight junction (TJ) assembly. The conserved Crb …

Phosphoinositides, a family of phosphorylated derivatives of phosphatidylinositol (PtdIns),
are tightly regulated both temporally and spatially by PtdIns phosphatases and kinases …

The development of the kidney relies on the establishment and maintenance of a precise
tubular diameter of its functional units, the nephrons. This process is disrupted in polycystic …

The OFD1 gene was initially identified as the gene responsible for the X-linked dominant
male lethal OFD type I syndrome, a developmental disorder ascribed to cilia disfunction. The …

Excretory organs were acquired in the early phase of metazoan evolution, and they play a
crucial role in the maintenance of homeostasis of body fluids. In general, these organs …

The formation of polarized epithelial tubules is a hallmark of kidney development. One of the
fundamental principles in tubulogenesis is that epithelia coordinate the polarity of individual …

Polycystic kidney disease (PKD) is a common hereditary disorder which is characterized by
fluid-filled cysts in the kidney. Mutation in either PKD1, encoding polycystin-1 (PC1), or …

Mutations of the Pkhd1 gene cause autosomal recessive polycystic kidney disease
(ARPKD). Pkhd1 encodes fibrocystin/polyductin (FPC), a ciliary type I membrane protein of …