Polycystic kidney disease

PD Wilson - New England Journal of Medicine, 2004 - Mass Medical Soc
Polycystic kidney diseases are inherited renal disorders due mainly to mutations in genes
that regulate the development and function of cells that line renal tubules. This review …

Genetics and pathogenesis of polycystic kidney disease

P Igarashi, S Somlo - Journal of the American Society of …, 2002 - journals.lww.com
Polycystic kidney disease (PKD), a common genetic cause of chronic renal failure in
children and adults, is characterized by the accumulation of fluid-filled cysts in the kidney …

Inversin, the gene product mutated in nephronophthisis type II, functions as a molecular switch between Wnt signaling pathways

M Simons, J Gloy, A Ganner, A Bullerkotte… - Nature …, 2005 - nature.com
Cystic renal diseases are caused by mutations of proteins that share a unique subcellular
localization: the primary cilium of tubular epithelial cells. Mutations of the ciliary protein …

β‐catenin signaling and cancer

PJ Morin - Bioessays, 1999 - Wiley Online Library
Since its discovery as a protein associated with the cytoplasmic region of E‐cadherin, β‐
catenin has been shown to perform two apparently unrelated functions: it has a crucial role …

Kidney-specific inactivation of the KIF3A subunit of kinesin-II inhibits renal ciliogenesis and produces polycystic kidney disease

F Lin, T Hiesberger, K Cordes… - Proceedings of the …, 2003 - National Acad Sciences
Polycystic kidney disease (PKD) is the most common genetic cause of renal failure in
humans. Several proteins that are encoded by genes associated with PKD have recently …

The state of the art of the zebrafish model for toxicology and toxicologic pathology research—advantages and current limitations

JM Spitsbergen, ML Kent - Toxicologic pathology, 2003 - journals.sagepub.com
The zebrafish (Danio rerio) is now the pre-eminent vertebrate model system for clarification
of the roles of specific genes and signaling pathways in development. The zebrafish …

A polycystic kidney-disease gene homologue required for male mating behaviour in C. elegans

MM Barr, PW Sternberg - Nature, 1999 - nature.com
The stereotyped mating behaviour of the Caenorhabditis elegans male is made up of
several substeps: response, backing, turning, vulva location, spicule insertion and sperm …

Nephronophthisis

F Hildebrandt - Genetic Diseases of the Kidney, 2009 - Elsevier
Publisher Summary Nephronophthisis (NPHP) is an autosomal recessive cystic kidney
disease that represents the most frequent monogenic cause of end-stage renal disease …

Primary cilia and signaling pathways in mammalian development, health and disease

IR Veland, A Awan, LB Pedersen, BK Yoder… - Nephron …, 2009 - karger.com
Although first described as early as 1898 and long considered a vestigial organelle of little
functional importance, the primary cilium has become one of the hottest research topics in …

Wnt/beta-catenin/tcf signaling: a critical pathway in gastrointestinal tumorigenesis

FT Kolligs, G Bommer, B Göke - Digestion, 2002 - karger.com
Cancers of the gastrointestinal tract, including the liver, bile ducts, and pancreas, constitute
the largest group of malignant tumors. Colorectal cancer is one of the most common …