نتایج جستجو برای: arpkd

تعداد نتایج: 183  

A Azarfar Ag Keykhosravi MA Kiani Y Ravanshad

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...

2014
Xiang Shan Ren Yasunori Sato Kenichi Harada Motoko Sasaki Shinichi Furubo Jing Yu Song Yasuni Nakanuma

The polycystic kidney (PCK) rat is an animal model of Caroli's disease as well as autosomal recessive polycystic kidney disease (ARPKD). The signaling pathways involving the mammalian target of rapamycin (mTOR) are aberrantly activated in ARPKD. This study investigated the effects of inhibitors for the cell signaling pathways including mTOR on cholangiocyte proliferation of the PCK rat. Culture...

Journal: :American journal of physiology. Cell physiology 2006
Dragos Olteanu Bradley K Yoder Wen Liu Mandy J Croyle Elisabeth A Welty Kelley Rosborough J Michael Wyss P Darwin Bell Lisa M Guay-Woodford Mark O Bevensee Lisa M Satlin Erik M Schwiebert

The Tg737 degrees (rpk) autosomal recessive polycystic kidney disease (ARPKD) mouse carries a hypomorphic mutation in the Tg737 gene. Because of the absence of its protein product Polaris, the nonmotile primary monocilium central to the luminal membrane of ductal epithelia, such as the cortical collecting duct (CCD) principal cell (PC), is malformed. Although the functions of the renal monocili...

Journal: :Journal of the American Society of Nephrology : JASN 2006
Tamio Yamaguchi Scott J Hempson Gail A Reif Anne-Marie Hedge Darren P Wallace

Polycystic kidney disease (PKD) is a lethal disorder characterized by progressive expansion of renal cysts. Genetic mutations associated with PKD are thought to disrupt intracellular Ca2+ regulation, leading to abnormal proliferation of tubule epithelial cells. cAMP stimulates the B-Raf/MEK/extracellular signal-regulated kinase (B-Raf/MEK/ERK) pathway and accelerates the proliferation of cells ...

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