The main feature of polycystic kidney diseases (PKD) is formation and progressive enlargement of renal cysts. Alterations in epithelial cell proliferation, extracellular matrix, and ion transport are thought to contribute to cyst enlargement and loss of renal function. Abnormal Cl- secretion is implicated in cyst enlargement in autosomal dominant PKD (ADPKD), but little is known about transport...

BACKGROUND Activation of the mTOR pathway has been implicated in the mediation of the progression of polycystic kidney disease (PKD). Whereas targeted inhibition of mTOR has been proven to be effective in various animal models of autosomal dominant PKD, its efficacy in autosomal recessive PKD (ARPKD) remains to be elucidated. We examined the effects of sirolimus in PCK rats, an orthologous anim...

Pan-caspase inhibition reduces tubular apoptosis and proliferation and slows progression of disease in a rat model of polycystic kidney disease (PKD). It is unknown, however, which specific caspases are involved in PKD progression. Because caspase-3 is a major mediator of apoptosis, its role in autosomal recessive PKD was determined. Mice with caspase-3 gene deletion were crossed with mice harb...

Increased proliferative activity of the renal tubular epithelium is thought to be a prerequisite for renal cyst formation by many investigators. However, in humans, the exact in vivo proliferation rate of epithelial cells lining these cysts is not known. In this study, which used immunohistochemical methods with an antibody to proliferating cell nuclear antigen (PCNA), the proliferation index (...

Human autosomal recessive polycystic kidney disease (ARPKD) produces kidneys which are massively enlarged due to multiple cysts, hypertension, and congenital hepatic fibrosis characterized by dilated bile ducts and portal hypertension. The PCK rat is an orthologous model of human ARPKD with numerous fluid-filled cysts caused by stimulated cellular proliferation in the renal tubules and hepatic ...

A MAJORITY of growth factors, including members of the epidermal growth factors (EGF) family, are synthesized as proproteins, requiring cleavage for the mediation of some of their functions. This pro-protein cleavage of the EGF family members is performed by disintegrin and metalloprotease (ADAM) family members and matrix metalloproteinases (MMPs) and is tightly regulated by various factors (9)...

POLYCYSTIC KIDNEY DISEASE comprises a number of genetically disparate disorders including autosomal dominant polycystic kidney disease (ADPKD), autosomal recessive polycystic kidney disease (ARPKD), and nephronophthisis (NPHP). These cystic disorders are characterized by the development and expansion of numerous renal cysts with ultimate loss of renal function. While ARPKD and NPHP are relative...

Immunotherapy is a potential way to save the lives of patients with bladder cancer, but it only benefits approximately 20% them. A total 4,028 cancer were collected for this study. Unsupervised non-negative matrix factorization and nearest template prediction algorithms employed classification. We identified immune non-immune classes from The Cancer Genome Atlas Bladder Urothelial Carcinoma (TC...