نتایج جستجو برای: polycystic kidney diseases pkd

تعداد نتایج: 1000149  

2015
Jogi Madhuprakash Nour Eddine El Gueddari Bruno M. Moerschbacher Appa Rao Podile

Chitin is an abundant renewable polysaccharide, next only to cellulose. Chitinases are important for effective utilization of this biopolymer. Chitinase D from Serratia proteamaculans (SpChiD) is a single domain chitinase with both hydrolytic and transglycosylation (TG) activities. SpChiD had less of hydrolytic activity on insoluble polymeric chitin substrates due to the absence of auxiliary bi...

2014
Sarmed H. Kathem Ashraf M. Mohieldin Shakila Abdul-Majeed Sajida H. Ismail Qaiss H. Altaei Ibrahim K. Alshimmari Mohanned M. Alsaidi Hussein Khammas Andromeda M. Nauli Bina Joe Surya M. Nauli

BACKGROUND Ciliopathies are a group of diseases associated with abnormal structure or function of primary cilia. Ciliopathies include polycystic kidney disease (PKD), a pathology associated with vascular hypertension. We previously showed that cilia length regulates cilia function, and cilia function is required for nitric oxide (NO) biosynthesis in endothelial cells. Because patients with PKD ...

Journal: :Journal of the American Society of Nephrology : JASN 2009
Peter C Harris

Polycystic kidney diseases (PKD) are a group of inherited disorders characterized by morbidity-associated development of renal cysts. Three forms of PKD are described here: The common, late onset, autosomal dominant PKD (ADPKD); the mainly infantile, autosomal recessive PKD (ARPKD); and the lethal, syndromic, Meckel syndrome that also includes central nervous system and digital defects. Positio...

2017
SONAM RAJ RANA GOPAL SINGH PARIMAL DAS

Background: Polycystic kidney disease (PKD) is a systemic disorder which adds majority of renal patients to end stage renal disease. Autosomal dominant polycystic kidney disease (ADPKD) is more prevalent and leading cause of dialysis and kidney transplant. Linkage analysis revealed some closely linked loci two of which are identified as PKD1, PKD2 and an unidentified locus to ADPKD. Methods: Th...

2011
Surya M. Nauli Xingjian Jin Beerend P. Hierck

Local regulation of vascular tone plays an important role in cardiovascular control of blood pressure. Aside from chemical or hormonal regulations, this local homeostasis is highly regulated by fluid-shear stress. It was previously unclear how vascular endothelial cells were able to sense fluid-shear stress. The cellular functions of mechanosensory cilia within vascular system have emerged rece...

Journal: :Biochemical and biophysical research communications 2009
Emily E Stagner Denise J Bouvrette Jianlin Cheng Elizabeth C Bryda

Mutations in either the Bicaudal-C or the Anks6 gene which encode the Bicc1 and SamCystin proteins respectively cause formation of renal cysts in rodent models of polycystic kidney disease, however their role in the mammalian kidney is unknown. Immunolocalization studies demonstrated that, unlike many other PKD-related proteins, SamCystin and Bicc1 do not localize to the primary cilia of cultur...

2016
Hervé Husson Sarah Moreno Laurie A. Smith Mandy M. Smith Ryan J. Russo Rose Pitstick Mikhail Sergeev Steven R. Ledbetter Nikolay O. Bukanov Monica Lane Kate Zhang Katy Billot George Carlson Jagesh Shah Laurent Meijer David R. Beier Oxana Ibraghimov-Beskrovnaya

Polycystic kidney diseases (PKDs) comprise a subgroup of ciliopathies characterized by the formation of fluid-filled kidney cysts and progression to end-stage renal disease. A mechanistic understanding of cystogenesis is crucial for the development of viable therapeutic options. Here, we identify CDK5, a kinase active in post mitotic cells, as a new and important mediator of PKD progression. We...

Journal: :American journal of physiology. Renal physiology 2015
Vicki J Hwang Jeffrey Kim Amy Rand Chaozhe Yang Steve Sturdivant Bruce Hammock P Darwin Bell Lisa M Guay-Woodford Robert H Weiss

Since polycystic kidney disease (PKD) was first noted over 30 years ago to have neoplastic parallels, there has been a resurgent interest in elucidating neoplasia-relevant pathways in PKD. Taking a nontargeted metabolomics approach in the B6(Cg)-Cys1(cpk/)J (cpk) mouse model of recessive PKD, we have now characterized metabolic reprogramming in these tissues, leading to a glutamine-dependent TC...

Journal: :Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2011
Thierry Lobbedez Malick Touam David Evans Jean-Philippe Ryckelynck Bertrand Knebelman Christian Verger

BACKGROUND It is commonly believed that polycystic kidney disease (PKD) patients on peritoneal dialysis (PD) are over-exposed to technique failure and peritonitis compared with other patients. This study was carried out to assess whether PKD is associated with technique failure and to evaluate the outcome of PKD patients on PD. METHODS This was a retrospective cohort study based on the data o...

2012
Constantinos Deltas Kyriacos Felekkis

The autosomal dominant form of polycystic kidney disease (ADPKD) is one of the most frequent monogenic disorders and the most frequent among inherited kidney disorders. In fact it has a prevalence in the population of about 1/1,000 individuals, therefore it does not even satisfy the definition for rare diseases. It is mainly characterized by the formation of multiple cysts filled with fluid tha...

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