Hamzaoui, Mouad Groussard, Deborah Nezam, Dorian Djerada, Zoubir Lamy, Gaspard Tardif, Virginie Dumesnil, Anais Renet, Sylvanie Brunel, Valery Peters, Dorien J M
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Published in
Hypertension (Dallas, Tex. : 1979)
Autosomal dominant polycystic kidney disease is the most frequent hereditary kidney disease and is generally due to mutations in PKD1 and PKD2, encoding polycystins 1 and 2. In autosomal dominant polycystic kidney disease, hypertension and cardiovascular disorders are highly prevalent, but their mechanisms are partially understood. Since endothelia...
Wang, Jiali Lin, Yongda Chen, Xiutian Liu, Yiping Zhou, Tianbiao
Published in
Frontiers in Cell and Developmental Biology
Chronic kidney disease (CKD) has a major impact on public health, which could progress to end-stage kidney disease (ESRD) and consume many medical resources. Currently, the treatment for CKD has many flaws, so more effective treatment tools are urgently required for CKD. Mesenchymal stem cells (MSCs) are primitive cells with self-renewal and prolif...
Sundar, Shirin V Zhou, Julie Xia Magenheimer, Brenda S Reif, Gail A Wallace, Darren P Georg, Gunda I Jakkaraj, Sudhakar R Tash, Joseph S Yu, Alan S L Li, Xiaogang
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Published in
American journal of physiology. Renal physiology
Autosomal dominant polycystic kidney disease (ADPKD) is a debilitating renal neoplastic disorder with limited treatment options. It is characterized by the formation of large fluid-filled cysts that develop from kidney tubules through abnormal cell proliferation and cyst-filling fluid secretion driven by cAMP-dependent Cl- secretion. We tested the ...
Zheng, Qi Reid, Glen Eccles, Michael R. Stayner, Cherie
Published in
Frontiers in Physiology
Polycystic kidney disease (PKD) is a significant cause of end-stage kidney failure and there are few effective drugs for treating this inherited condition. Numerous aberrantly expressed non-coding RNAs (ncRNAs), particularly microRNAs (miRNAs), may contribute to PKD pathogenesis by participating in multiple intracellular and intercellular functions...
Yan, Ziyan Wang, Yuchen Deng, Wenfeng Zhou, Yi Hu, Yangcheng Qi, Ka Liu, Ding Xia, Renfei Liu, Rumin Zeng, Wenli
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Published in
Frontiers in Genetics
Background: Autosomal dominant polycystic kidney disease (ADPKD) is mainly caused by PKD1 and PKD2 mutations. However, only a few studies have investigated the genotype and phenotype characteristics of Asian patients with ADPKD. This study aimed to investigate the relationship between the natural course of ADPKD genotype and phenotype. Methods: Gen...
Iliuta, Ioan-Andrei Song, Xuewen Pickel, Lauren Haghighi, Amirreza Retnakaran, Ravi Scholey, James Sung, Hoon-Ki Steinberg, Gregory R. Pei, York
Published in
Frontiers in Molecular Biosciences
Autosomal dominant polycystic kidney disease (ADPKD) is the most common Mendelian kidney disease, affecting approximately one in 1,000 births and accounting for 5% of end-stage kidney disease in developed countries. The pathophysiology of ADPKD is strongly linked to metabolic dysregulation, which may be secondary to defective polycystin function. O...
Liu, Xiong Tang, Jingfeng Chen, Xing-Zhen
Published in
Frontiers in Physiology
Autosomal dominant polycystic kidney disease (ADPKD) is caused by mutations in the PKD1 or PKD2 gene which encodes membrane receptor PKD1 and cation channel PKD2, respectively. PKD2, also called transient receptor potential polycystin-2 (TRPP2), is a Ca2+-permeable channel located on the membrane of cell surface, primary cilia, and endoplasmic reti...
Xu, Jing Xue, Cheng Wang, Xiaodong Zhang, Lei Mei, Changlin Mao, Zhiguo
Published in
Frontiers in Medicine
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease worldwide and is one of the major causes of end-stage renal disease. PKD1 and PKD2 are two genes that mainly contribute to the development and progression of ADPKD. The precise mechanism is not fully understood. In recent years, epigenetic modification ...
Riddle, Heather A L Zhang, Shiqin Qian, Feng Williams, James C Jr Stubbs, Jason R Rowe, Peter Stanley N Parnell, Stephen C
Published in
American journal of physiology. Renal physiology
Individuals with autosomal dominant polycystic kidney disease have a higher incidence of stone formation than the general population. However, there are no cystic animal models known to develop stones. Cystic mice compound heterozygous for hypomorphic Pkd1V and Pkd1RC alleles develop cystic kidneys within a few weeks of birth but live beyond 20 wk ...
Shimoda, Naoko Ikeda, Mariko Yan, Tomohiro Kawasaki, Sayuri Hirama, Akio Kashiwagi, Tetsuya Sakai, Yukinao
Published in
Journal of Nippon Medical School = Nippon Ika Daigaku zasshi
Tolvaptan is the first effective drug treatment for autosomal dominant polycystic kidney disease (ADPKD) patients, but few long-term observations of the effects of tolvaptan have been reported. In this single center, retrospective cohort study, we investigated nine patients who participated in a phase 3 trial of tolvaptan for ADPKD patients at our ...