BACKGROUND Purinergic receptors are cell-surface molecules that bind extracellular nucleotides, notably ATP. The P2X family includes seven nonselective ion channels with one member, P2X(7), implicated in cytolytic pore formation and cell death. MATERIALS AND METHODS We sought P2X(7) expression in mouse nephrogenesis and cpk/cpk renal cyst growth, conditions in which both proliferation and apo...

20-Hydroxyeicosatetraenoic acid (20-HETE) has been implicated as a potential mediator in epithelial cell proliferation and cyst formation in polycystic kidney disease (PKD). In the present study, we studied the effects of chronic blockade of 20-HETE synthesis in an orthologous rodent model of autosomal recessive polycystic kidney disease (ARPKD), the PCK rat. RT-PCR analysis indicated that the ...

In congenital cystic malformations of the kidney, as in other groups of disorders, the accuracy of genetic counselling depends upon exact diagnosis. This requires the breakdown of the main heterogeneous group into specific entities, each of which is homogeneous from the genetic viewpoint and therefore carries a predictable recurrence risk within the family. It is particularly important to recog...

14 15 Autosomal-dominant polycystic kidney disease is a common cause of end-stage renal 16 disease, and no approved treatment is available in the US to slow disease progression. 17 The mTOR signaling pathway is aberrantly activated in renal cysts, and, while mTOR 18 inhibitors are highly effective in rodent models, clinical trials in ADPKD have been 19 disappointing due to dose-limiting extra-r...

Autosomal dominant polycystic kidney disease is a genetic disorder which results in the development of multiple cysts in the kidneys and other parenchymal organs. The two genes in which mutations are known to cause autosomal dominant polycystic kidney disease are PKD1 and PKD2. Approximately 50% of individuals with autosomal dominant polycystic kidney disease will develop end-stage renal diseas...

Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys.  Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. Affected individuals also have impaired renal function, usually caused, in children and teenagers, by an...

Abstract. The purpose of the study was to establish an ultrasonographic picture upper urinary system in cats with chronic kidney disease at different stages and their prognostic significance. Research methods. involved 40 sex, age breed established diagnosis from stage 1 4, 10 animals each group. staging based on concentration serum creatinine level. All underwent examination ChisonQBit 11 appa...

Cellular and extracellular (tubular basement membrane, TBM) alterations in the proteoglycans (PGs) of the rat renal tubules in diphenylthiazole-induced cystic disease were investigated. The PGs of normal and cystic kidneys were labeled with [35S]sulfate in an organ-perfusion system. Extracted cellular and TBM PGs were characterized by Sepharose CL-6B chromatography before or after treatment wit...

Polycystic kidney disease is a disorder marked by aberrant renal tubular epithelial cell proliferation and transport abnormalities. Sphingolipids are ubiquitous membrane components implicated in several cellular functions including cell membrane sorting, signaling, growth, ion transport, and adhesion. To investigate a potential pathogenic role for sphingolipids in cystic kidney disease, we stud...

Autosomal dominant polycystic kidney disease (ADPKD) results from mutations in either PKD1 or PKD2 and accounts for 10% of all patients on renal replacement therapy. The kidney disease phenotype is primarily characterized by cyst formation, but there are also prominent interstitial changes (inflammation, apoptosis, proliferation, and fibrosis). Using a model of unilateral ischemia-reperfusion i...