Employing in vitro pulse-chase techniques, we investigated the de novo synthesis and the kinetics of intracellular transport and extracellular matrix incorporation of proteoglycans (PG) by normal human renal epithelial cell and by epithelial cells isolated from cysts of autosomal dominant kidneys (ADPKD). Cell monolayers were pulsed either with (3H)leucine for 15 min and chased for seven interv...

Autosomal dominant polycystic kidney disease (ADPKD) is primarily associated with renal failure, but it also causes systemic diseases, including cysts of other systemic organs and cerebral or visceral aneurysm. To make matters worse, life-threatening aortic diseases are associated with ADPKD in some cases. However, only a few reports of ADPKD-associated with thoracic aortic dissection have been...

Renal cyst formation is the hallmark of autosomal dominant polycystic kidney disease (ADPKD). ADPKD cyst-lining cells have an increased proliferation rate and are surrounded by an abnormal extracellular matrix (ECM). We have previously shown that Laminin 5 (Ln-5, a alpha(3)beta(3)gamma(2) trimer) is aberrantly expressed in the pericystic ECM of ADPKD kidneys. We report that ADPKD cells in prima...

BACKGROUND/AIMS Early occurrence of hypertension is the prominent feature of autosomal dominant polycystic kidney disease (ADPKD). The role of angiotensin-converting enzyme (ACE) gene polymorphism and endothelial nitric oxide synthase (eNOS) gene polymorphism in the clinical course of ADPKD is not well understood. However, data about the expression of these genes are lacking. Thus, we aimed to ...

BACKGROUND Autosomal dominant polycystic kidney disease (ADPKD) is a systemic hereditary disorder characterized by bilateral diffuse renal cysts. Extrarenal involvement is a well known manifestation of ADPKD. Data relating to the association between seminal vesicle cysts and ADPKD are limited. The aims of this study are to evaluate the frequency of seminal vesicle cysts in ADPKD and to assess t...

Autosomal dominant polycystic kidney disease (ADPKD) constitutes the most common inherited kidney disease. Mutations in the PKD1 and PKD2 genes, encoding respective polycystin-1 and polycystin-2 Ca2+ ion channels, results in tubular epithelial cell-derived renal cysts. Recent clinical studies demonstrate oxidative stress as present early in ADPKD. Mitochondria comprise the primary reactive oxyg...

BACKGROUND Patients with autosomal dominant polycystic kidney disease (ADPKD) often develop hypertension before any abnormalities in renal function are detected clinically. Therefore, standard screening (serum creatinine and urinalysis) of young individuals with unexplained hypertension to exclude renal parenchymal disease would rarely detect ADPKD. METHODS Data from 516 subjects with ADPKD (...

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by a significant phenotypic variability in progression of the disease. Vascular endothelial growth factor (VEGF) has been reported to play a major role in renal pathophysiology. The aim of the present case-control study was to evaluate the association of two promoter polymorphisms (-2578C>A and -1154G>A) of VEGF gene and ADPK...

cation about genetic risk: a qualitative study, learning from families’ experiences. Eur J Hum Genet 2011; 19: 640–646 13. Chapman A. KIDIGO Controversies Conference on Autosomal Dominant Polycystic Kidney Disease. 2014. Available from: http://kdigo.org/home/ conferences/adpkd/ 14. European ADPKD Forum Report: Translating science into policy to improve ADPKD care. 2015. Available from: http://w...

BACKGROUND Autosomal dominant polycystic kidney disease (ADPKD) results in kidney cyst development and enlargement, resulting in chronic kidney disease (CKD) leading to renal failure. This study sought to determine if ADPKD patients in the early stages of CKD contribute to a sizable economic burden for the US health care system. METHODS This was a retrospective, matched cohort study, reviewin...