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 …

[HTML][HTML] Molecular pathogenesis of ADPKD: the polycystin complex gets complex

ACM Ong, PC Harris - Kidney international, 2005 - Elsevier
Molecular pathogenesis of ADPKD: The polycystin complex gets complex. Autosomal-
dominant polycystic kidney disease (ADPKD) is one of the most common human monogenic …

Polycystins and primary cilia: primers for cell cycle progression

J Zhou - Annual review of physiology, 2009 - annualreviews.org
Polycystins are a family of eight-transmembrane proteins united by sequence homology.
The name stems from the identification of mutations in genes encoding polycystin-1 and-2 in …

Human ADPKD primary cyst epithelial cells with a novel, single codon deletion in the PKD1 gene exhibit defective ciliary polycystin localization and loss of flow …

C Xu, S Rossetti, L Jiang, PC Harris… - American Journal …, 2007 - journals.physiology.org
Autosomal dominant polycystic kidney disease (ADPKD) gene products polycystin-1 (PC1)
and polycystin-2 (PC2) colocalize in the apical monocilia of renal epithelial cells. Mouse and …

Polycystin: new aspects of structure, function, and regulation

PD Wilson - Journal of the American Society of Nephrology, 2001 - journals.lww.com
Polycystin-1 is a modular membrane protein with a long extracellular N-terminal portion that
bears several ligand-binding domains, 11 transmembrane domains, and a≥ 200 amino …

The genetics and physiology of polycystic kidney disease

JP Calvet, JJ Grantham - Seminars in nephrology, 2001 - seminarsinnephrology.org
Autosomal dominant polycystic kidney disease (ADPKD) is a major, inherited disorder that is
characterized by the growth of large, fluid-filled cysts from the tubules and collecting ducts of …

Molecular genetics and pathogenesis of autosomal dominant polycystic kidney disease

MA Arnaout - Annual review of medicine, 2001 - annualreviews.org
▪ Abstract Autosomal dominant polycystic kidney disease (ADPKD) is a common and
systemic disease characterized by formation of focal cysts. Of the three potential causes of …

[HTML][HTML] The mechanosensory role of primary cilia in vascular hypertension

SM Nauli, X Jin, BP Hierck - International journal of vascular …, 2011 - hindawi.com
Local regulation of vascular tone plays an important role in cardiovascular control of blood
pressure. Aside from chemical or hormonal regulations, this local homeostasis is highly …

[HTML][HTML] Polycystin-1 Interacting Protein-1 (CU062) Interacts with the Ectodomain of Polycystin-1 (PC1)

WA Lea, T Winklhofer, L Zelenchuk, M Sharma… - Cells, 2023 - mdpi.com
The PKD1 gene, encoding protein polycystin-1 (PC1), is responsible for 85% of cases of
autosomal dominant polycystic kidney disease (ADPKD). PC1 has been shown to be …

A polycystin-2-like large conductance cation channel in rat left ventricular myocytes

T Volk, AP Schwoerer, S Thiessen… - Cardiovascular …, 2003 - academic.oup.com
Objective: Several members of the PKD gene family (PKD2, PKDL and PKD2L2) are
expressed in the heart. Polycystin-2 and its homologues, which are encoded by these …