نتایج جستجو برای: pkd2

تعداد نتایج: 596  

Journal: :Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2008
Stéphane Burtey Marta Riera Emilie Ribe Petra Pennekamp Edith Passage Roselyne Rance Bernd Dworniczak Michel Fontés

Polycystin-2 (PC-2), a cation channel of the Trp family, is involved in autosomal dominant polycystic kidney disease (ADPKD) type 2 (ADPKD2). This protein has recently been localized to the primary cilium where its channel function seems to be involved in a mechanosensory phenomenon. However, its biological function is not totally understood, especially in tubule formation. In the present paper...

2005
Sertac N. Kip Larry W. Hunter Qun Ren Peter C. Harris Stefan Somlo Vicente E. Torres Gary C. Sieck Qi Qian

Cardiovascular complications are the leading cause of morbidity and mortality in autosomal dominant polycystic kidney disease. Pkd2 / vascular smooth muscle cells (VSMCs) have an abnormal phenotype and defective intracellular Ca ([Ca ]i) regulation. We examined cAMP content in vascular smooth muscles from Pkd2 / mice because cAMP is elevated in cystic renal epithelial cells. We found cAMP conce...

Journal: :Journal of the American Society of Nephrology : JASN 2011
Ming-Yang Chang Jenn-Kan Lu Ya-Chung Tian Yung-Chang Chen Cheng-Chieh Hung Yi-Hui Huang Yau-Hung Chen Mai-Szu Wu Chih-Wei Yang Yi-Chuan Cheng

The P2X7 receptor participates in purinergic signaling, which may promote the progression of ADPKD. We examined the effects of a P2X7 receptor antagonist and a P2X7 receptor agonist on cyst development in a zebrafish model of polycystic kidney disease in which we knocked down pkd2 by morpholinos. We used live wt-1b pronephric-specific GFP-expressing zebrafish embryos to directly observe changes...

Journal: :Development 2011
Keiichiro Kamura Daisuke Kobayashi Yuka Uehara Sumito Koshida Norio Iijima Akira Kudo Takahiko Yokoyama Hiroyuki Takeda

The internal organs of vertebrates show distinctive left-right asymmetry. Leftward extracellular fluid flow at the node (nodal flow), which is generated by the rotational movement of node cilia, is essential for left-right patterning in the mouse and other vertebrates. However, the identity of the pathways by which nodal flow is interpreted remains controversial as the molecular sensors of this...

Journal: :The Journal of biological chemistry 2009
Maya T Kunkel Erin L Garcia Taketoshi Kajimoto Randy A Hall Alexandra C Newton

Protein kinase D (PKD) transduces an abundance of signals downstream of diacylglycerol production. The mammalian PKD family consists of three isoforms, PKD1, PKD2, and PKD3; of these PKD1 and PKD2 contain PDZ-binding motifs at their carboxyl termini. Here we show that membrane-localized NHERF scaffold proteins provide a nexus for tightly controlled PKD signaling via a PDZ domain interaction. Us...

2018
Xiaowen Liu Thuy Vien Jingjing Duan Shu-Hsien Sheu Paul G DeCaen David E Clapham

Mutations in the polycystin genes, PKD1 or PKD2, results in Autosomal Dominant Polycystic Kidney Disease (ADPKD). Although a genetic basis of ADPKD is established, we lack a clear understanding of polycystin proteins' functions as ion channels. This question remains unsolved largely because polycystins localize to the primary cilium - a tiny, antenna-like organelle. Using a new ADPKD mouse mode...

2014
Binu M. Paul Mark B. Consugar Moonnoh Ryan Lee Jamie L. Sundsbak Christina M. Heyer Sandro Rossetti Vickie J. Kubly Katharina Hopp Vicente E. Torres Eliecer Coto Maurizio Clementi Nadja Bogdanova Edgar de Almeida Daniel G. Bichet Peter C. Harris

Mutations to PKD1 and PKD2 are associated with autosomal dominant polycystic kidney disease (ADPKD). The absence of apparent PKD1/PKD2 linkage in five published European or North American families with ADPKD suggested a third locus, designated PKD3. Here we re-evaluated these families by updating clinical information, re-sampling where possible, and mutation screening for PKD1/PKD2. In the Fren...

Journal: :Disease models & mechanisms 2010
Steve Mangos Pui-ying Lam Angela Zhao Yan Liu Sudha Mudumana Aleksandr Vasilyev Aiping Liu Iain A Drummond

Mutations in polycystin1 (PKD1) account for the majority of autosomal dominant polycystic kidney disease (ADPKD). PKD1 mutations are also associated with vascular aneurysm and abdominal wall hernia, suggesting a role for polycystin1 in extracellular matrix (ECM) integrity. In zebrafish, combined knockdown of the PKD1 paralogs pkd1a and pkd1b resulted in dorsal axis curvature, hydrocephalus, car...

Journal: :The American journal of pathology 2009
Sony Prasad John Patrick McDaid Frederick Wai Keung Tam John Lionel Haylor Albert Chee Meng Ong

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...

Journal: :Journal of the American Society of Nephrology : JASN 2009
York Pei James Obaji Annie Dupuis Andrew D Paterson Riccardo Magistroni Elizabeth Dicks Patrick Parfrey Benvon Cramer Eliecer Coto Roser Torra Jose L San Millan Robert Gibson Martijn Breuning Dorien Peters David Ravine

Individuals who are at risk for autosomal dominant polycystic kidney disease are often screened by ultrasound using diagnostic criteria derived from individuals with mutations in PKD1. Families with mutations in PKD2 typically have less severe disease, suggesting a potential need for different diagnostic criteria. In this study, 577 and 371 at-risk individuals from 58 PKD1 and 39 PKD2 families,...

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