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https://www.readbyqxmd.com/read/28435156/polycystic-kidney-disease-pmm2-mutation-causes-pkd-and-hyperinsulinism
#1
Ellen F Carney
No abstract text is available yet for this article.
April 24, 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28429822/what-goes-around-comes-around-an-investigation-of-resistance-to-proliferative-kidney-disease-in-rainbow-trout-oncorhynchus-mykiss-walbaum-following-experimental-re-exposure
#2
C Bailey, H Segner, T Wahli
Rainbow trout Oncorhynchus mykiss surviving proliferative kidney disease (PKD) are reported not to develop the disease upon re-exposure. However, the mechanisms involved in the immune response to re-exposure are unknown. We examined disease susceptibility and the immune response of naive 1+ rainbow trout when first exposed to Tetracapsuloides bryosalmonae in comparison with that of 1+ rainbow trout re-exposed to T. bryosalmonae. PKD pathogenesis, parasite burden and transcriptional signatures of the host immune response were assessed at 10, 25 and 40 d...
April 21, 2017: Journal of Fish Diseases
https://www.readbyqxmd.com/read/28428613/differential-regulation-of-pkd-isoforms-in-oxidative-stress-conditions-through-phosphorylation-of-a-conserved-tyr-in-the-p-1-loop
#3
Mathias Cobbaut, Rita Derua, Heike Döppler, Hua Jane Lou, Sandy Vandoninck, Peter Storz, Benjamin E Turk, Thomas Seufferlein, Etienne Waelkens, Veerle Janssens, Johan Van Lint
Protein kinases are essential molecules in life and their crucial function requires tight regulation. Many kinases are regulated via phosphorylation within their activation loop. This loop is embedded in the activation segment, which additionally contains the Mg(2+) binding loop and a P + 1 loop that is important in substrate binding. In this report, we identify Abl-mediated phosphorylation of a highly conserved Tyr residue in the P + 1 loop of protein kinase D2 (PKD2) during oxidative stress. Remarkably, we observed that the three human PKD isoforms display very different degrees of P + 1 loop Tyr phosphorylation and we identify one of the molecular determinants for this divergence...
April 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28416225/b-type-natriuretic-peptide-overexpression-ameliorates-hepatorenal-fibrocystic-disease-in%C3%A2-a%C3%A2-rat%C3%A2-model-of-polycystic-kidney-disease
#4
Sara J Holditch, Claire A Schreiber, Peter C Harris, Nicholas F LaRusso, Marina Ramirez-Alvarado, Alessandro Cataliotti, Vicente E Torres, Yasuhiro Ikeda
Polycystic kidney disease (PKD) involves progressive hepatorenal cyst expansion and fibrosis, frequently leading to end-stage renal disease. Increased vasopressin and cAMP signaling, dysregulated calcium homeostasis, and hypertension play major roles in PKD progression. The guanylyl cyclase A agonist, B-type natriuretic peptide (BNP), stimulates cGMP and shows anti-fibrotic, anti-hypertensive, and vasopressin-suppressive effects, potentially counteracting PKD pathogenesis. Here, we assessed the impacts of guanylyl cyclase A activation on PKD progression in a rat model of PKD...
April 14, 2017: Kidney International
https://www.readbyqxmd.com/read/28409351/inflammation-and-fibrosis-in-polycystic-kidney-disease
#5
Cheng Jack Song, Kurt A Zimmerman, Scott J Henke, Bradley K Yoder
Polycystic kidney disease (PKD) is a commonly inherited disorder characterized by cyst formation and fibrosis (Wilson, N Engl J Med 350:151-164, 2004) and is caused by mutations in cilia or cilia-related proteins, such as polycystin 1 or 2 (Oh and Katsanis, Development 139:443-448, 2012; Kotsis et al., Nephrol Dial Transplant 28:518-526, 2013). A major pathological feature of PKD is the development of interstitial inflammation and fibrosis with an associated accumulation of inflammatory cells (Grantham, N Engl J Med 359:1477-1485, 2008; Zeier et al...
2017: Results and Problems in Cell Differentiation
https://www.readbyqxmd.com/read/28408623/protein-kinase-d1-pkd1-phosphorylation-on-ser-203-by-type-i-p21-activated-kinase-pak-regulates-pkd1-localization
#6
Jen-Kuan Chang, Yang Ni, Liang Han, James Sinnett-Smith, Rodrigo Jacamo, Osvaldo Rey, Steven H Young, Enrique Rozengurt
While PKC-mediated phosphorylation of protein kinase D1 (PKD1) has been extensively characterized, little is known about PKD1 regulation by other upstream kinases. Here, we report that stimulation of epithelial or fibroblastic cells with G protein-coupled receptor (GPCR) agonists, including angiotensin II or bombesin induced rapid and persistent PKD1 phosphorylation at Ser(203), a highly conserved residue located within the PKD1 N-terminal domain. Exposure to PKD or PKC family inhibitors did not prevent PKD1 phosphorylation at Ser(203), indicating that it is not mediated by autophosphorylation...
April 13, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28406902/bicaudal-c-mutation-causes-myc-and-tor-pathway-up-regulation-and-polycystic-kidney-disease-like-phenotypes-in-drosophila
#7
Chiara Gamberi, David R Hipfner, Marie Trudel, William D Lubell
Progressive cystic kidney degeneration underlies diverse renal diseases, including the most common cause of kidney failure, autosomal dominant Polycystic Kidney Disease (PKD). Genetic analyses of patients and animal models have identified several key drivers of this disease. The precise molecular and cellular changes underlying cystogenesis remain, however, elusive. Drosophila mutants lacking the translational regulator Bicaudal C (BicC, the fly ortholog of vertebrate BICC1 implicated in renal cystogenesis) exhibited progressive cystic degeneration of the renal tubules (so called "Malpighian" tubules) and reduced renal function...
April 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28397578/paroxysmal-kinesigenic-dyskinesia-like-phenotype-in-multiple-sclerosis
#8
Roxana Pop, Stefan Kipfer
In April 2015, a 20-year-old woman with multiple sclerosis (MS) presented with acute onset of repetitive abnormal postures and choreatic movements of the right arm, precipitated by voluntary movements (online video 1 and 2). Brain magnetic resonance imaging (MRI) showed a new active MS lesion involving the basal ganglia on the left side (Figure 1(a)). Intravenous steroid treatment resulted in rapid regression of this paroxysmal kinesigenic dyskinesia (PKD)-like hyperkinetic movement disorder. The patient became asymptomatic within 3 months...
April 1, 2017: Multiple Sclerosis: Clinical and Laboratory Research
https://www.readbyqxmd.com/read/28387829/hereditary-polycystic-kidney-disease-is-characterized-by-lymphopenia-across-all-stages-of-kidney-dysfunction-an-observational-study
#9
Steven Van Laecke, Tessa Kerre, Evi V Nagler, Bart Maes, Rogier Caluwe, Eva Schepers, Griet Glorieux, Wim Van Biesen, Francis Verbeke
Background: Polycystic kidney disease (PKD) is characterized by urinary tract infections and extrarenal abnormalities such as an increased risk of cancer. As mutations in polycystin-1 and -2 are associated with decreased proliferation of immortalized lymphoblastoid cells in PKD, we investigated whether lymphopenia could be an unrecognized trait of PKD. Methods: We studied 700 kidney transplant recipients with ( n  = 126) or without PKD at the time of kidney transplantation between 1 January 2003 and 31 December 2014 at Ghent University Hospital...
April 6, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28386880/long-term-effect-of-coffee-consumption-on-autosomal-dominant-polycystic-kidneys-disease-progression-results-from-the-suisse-adpkd-a-prospective-longitudinal-cohort-study
#10
Laura Girardat-Rotar, Milo A Puhan, Julia Braun, Andreas L Serra
BACKGROUND: Previous in vitro experiments of human polycystic kidney disease (PKD) cells reported that caffeine is a risk factor for the promotion of cyst enlargement in patients with autosomal dominant PKD (ADPKD). The relentless progression of ADPKD inclines the majority of physicians to advocate minimization of caffeine consumption despite the absence of clinical data supporting such a recommendation so far. This is the first clinical study to assess prospectively the association between coffee consumption and disease progression in a longitudinal ADPKD cohort...
April 6, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/28373276/polycystic-kidney-disease-with-hyperinsulinemic-hypoglycemia-caused-by-a-promoter-mutation-in-phosphomannomutase-2
#11
Oscar Rubio Cabezas, Sarah E Flanagan, Horia Stanescu, Elena García-Martínez, Richard Caswell, Hana Lango-Allen, Montserrat Antón-Gamero, Jesús Argente, Anna-Marie Bussell, Andre Brandli, Chris Cheshire, Elizabeth Crowne, Simona Dumitriu, Robert Drynda, Julian P Hamilton-Shield, Wesley Hayes, Alexis Hofherr, Daniela Iancu, Naomi Issler, Craig Jefferies, Peter Jones, Matthew Johnson, Anne Kesselheim, Enriko Klootwijk, Michael Koettgen, Wendy Lewis, José María Martos, Monika Mozere, Jill Norman, Vaksha Patel, Andrew Parrish, Celia Pérez-Cerdá, Jesús Pozo, Sofia A Rahman, Neil Sebire, Mehmet Tekman, Peter D Turnpenny, William Van't Hoff, Daan H H M Viering, Michael N Weedon, Patricia Wilson, Lisa Guay-Woodford, Robert Kleta, Khalid Hussain, Sian Ellard, Detlef Bockenhauer
Hyperinsulinemic hypoglycemia (HI) and congenital polycystic kidney disease (PKD) are rare, genetically heterogeneous disorders. The co-occurrence of these disorders (HIPKD) in 17 children from 11 unrelated families suggested an unrecognized genetic disorder. Whole-genome linkage analysis in five informative families identified a single significant locus on chromosome 16p13.2 (logarithm of odds score 6.5). Sequencing of the coding regions of all linked genes failed to identify biallelic mutations. Instead, we found in all patients a promoter mutation (c...
April 3, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28367092/pkd-knockdown-inhibits-pressure-overload-induced-cardiac-hypertrophy-by-promoting-autophagy-via-akt-mtor-pathway
#12
Di Zhao, Wei Wang, Hao Wang, Honghai Peng, Xiangjuan Liu, Weixing Guo, Guohai Su, Zhuo Zhao
Growing evidence shows that protein kinase D (PKD) plays an important role in the development of pressure overload-induced cardiac hypertrophy. However, the mechanisms involved are not clear. This study tested our hypothesis that PKD might mediate cardiac hypertrophy by negatively regulating autophagy using the technique of PKD knockdown by siRNA. Cardiac hypertrophy was induced in 8-week old male C57BL/6 mice by transverse aortic constriction (TAC). TAC mice were then divided into five groups receiving the treatments of vehicle (DMSO), an autophagy inducer rapamycin (1 mg/kg/day, i...
2017: International Journal of Biological Sciences
https://www.readbyqxmd.com/read/28363424/higher-pkd3-expression-in-hepatocellular-carcinoma-hcc-tissues-predicts-poorer-prognosis-for-hcc-patients
#13
Haiyun Yang, Ming Xu, Xiufang Chi, Qun Yan, Yadong Wang, Wen Xu, Kangmin Zhuang, Aimin Li, Side Liu
AIM: Protein kinase D (PKD) acts as a key mediator in several cancer development signaling pathways. The aim of this study was to investigate the clinical significance and prognostic value of PKD3 expression in hepatocellular carcinoma (HCC) patients after hepatectomy. METHODS: PKD3 mRNA and protein expression levels in tumor and matched non-tumoral (NT) tissues, HCC cell lines were evaluated by quantitative PCR (qRT-PCR), western blotting and immunohistochemical staining (IHC)...
March 28, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/28360038/insulin-receptor-and-gpcr-crosstalk-stimulates-yap-via-pi3k-and-pkd-in-pancreatic-cancer-cells
#14
Fang Hao, Qinhong Xu, Yinglan Zhao, Jan V Stevens, Steven H Young, James Sinnett-Smith, Enrique Rozengurt
We examined the impact of crosstalk between the insulin receptor (INSR) and G protein-coupled receptor (GPCR) signaling pathways on the regulation of Yes-associated Protein (YAP) localization, phosphorylation and transcriptional activity in the context of human pancreatic ductal adenocarcinoma (PDAC). Stimulation of PANC-1 or MiaPaCa-2 cells with insulin and neurotensin, a potent mitogenic combination of agonists for these cells, promoted striking YAP nuclear localization and decreased YAP phosphorylation at Ser127 and Ser397...
March 30, 2017: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/28341240/overexpression-of-exogenous-kidney-specific-ngal-attenuates-progressive-cyst-development-and-prolongs-lifespan-in-a-murine-model-of-polycystic-kidney-disease
#15
Ellian Wang, Yuan-Yow Chiou, Wen-Yih Jeng, Hsiu-Kuan Lin, Hsi-Hui Lin, Hsian-Jean Chin, Chi-Kuang Leo Wang, Shang-Shiuan Yu, Shih-Chieh Tsai, Chih-Ying Chiang, Po-Hao Cheng, Hong-Jie Lin, Si-Tse Jiang, Sou-Tyau Chiu, Hsiu Mei Hsieh-Li
Neutrophil gelatinase-associated lipocalin (Ngal) is a biomarker for acute and chronic renal injuries, including polycystic kidney disease (PKD). However, the effect of Ngal on PKD progression remains unexplored. To study this, we generated 3 strains of mice with different expression levels of Ngal within an established PKD model (Pkd1(L3/L3)): Pkd1(L3/L3) (with endogenous Ngal), Pkd1(L3/L3); Ngal(Tg/Tg) (with endogenous and overexpression of exogenous kidney-specific Ngal) and Pkd1(L3/L3); Ngal(-/-) mice (with Ngal deficiency)...
February 2017: Kidney International
https://www.readbyqxmd.com/read/28332126/klotho-suppresses-high-phosphate-induced-osteogenic-responses-in-human-aortic-valve-interstitial-cells-through-inhibition-of-sox9
#16
Fei Li, Qingzhou Yao, Lihua Ao, Joseph C Cleveland, Nianguo Dong, David A Fullerton, Xianzhong Meng
Elevated level of blood phosphate (Pi) associated with chronic kidney disease (CKD) is a risk factor of aortic valve calcification. Aortic valve interstitial cells (AVICs) display osteogenic responses to high Pi although the underlying mechanism is incompletely understood. Sox9 is a pro-chondrogenic factor and may play a role in ectopic tissue calcification. Circulating and kidney levels of Klotho are reduced in patients with CKD. We hypothesized that Sox9 mediates high Pi-induced osteogenic responses in human AVICs and that Klotho inhibits the responses...
March 22, 2017: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/28323891/characterization-of-baff-and-april-subfamily-receptors-in-rainbow-trout-oncorhynchus-mykiss-potential-role-of-the-baff-april-axis-in-the-pathogenesis-of-proliferative-kidney-disease
#17
Aitor G Granja, Jason W Holland, Jaime Pignatelli, Christopher J Secombes, Carolina Tafalla
Proliferative kidney disease (PKD) is a parasitic infection of salmonid fish characterized by hyper-secretion of immunoglobulins in response to the presence of the myxozoan parasite, Tetracapsuloides bryosalmonae. In this context, we hypothesized that the BAFF/APRIL axis, known to play a major role in B cell differentiation and survival in mammals, could be affected by the parasite and consequently be involved in the apparent shift in normal B cell activity. To regulate B cell activity, BAFF and APRIL bind to transmembrane activator and calcium modulator and cyclophilin ligand interactor (TACI) and B cell maturation antigen (BCMA), whereas BAFF also binds to BAFF receptor (BAFF-R)...
2017: PloS One
https://www.readbyqxmd.com/read/28322206/comparative-study-of-proliferative-kidney-disease-in-grayling-thymallus-thymallus-and-brown-trout-salmo-trutta-fario-an-exposure-experiment
#18
Heike Schmidt-Posthaus, Albert Ros, Regula Hirschi, Ernst Schneider
Proliferative kidney disease (PKD) is an emerging disease threatening wild salmonid populations, with the myxozoan parasite Tetracapsuloides bryosalmonae as the causative agent. Species differences in parasite susceptibility and disease-induced mortality seem to exist. The aim of the present study was to compare incidence, pathology and mortality of PKD in grayling Thymallus thymallus and brown trout Salmo trutta under identical semi-natural conditions. Young-of-the-year grayling and brown trout, free of T...
March 21, 2017: Diseases of Aquatic Organisms
https://www.readbyqxmd.com/read/28293419/cilia-before-and-after
#19
REVIEW
Peter Satir
This is a history of cilia research before and after the discovery of intraflagellar transport (IFT) and the link between primary cilia ciliogenesis and polycystic kidney disease (PKD). Before IFT, ca. the beginning of the new millennium, although sensory and primary cilia were well described, research was largely focused on motile cilia, their structure, movement, and biogenesis. After IFT and the link to PKD, although work on motile cilia has continued to progress, research on primary cilia has exploded, leading to new insights into the role of cilia in cell signaling and development...
2017: Cilia
https://www.readbyqxmd.com/read/28291603/proliferative-kidney-disease-in-rainbow-trout-oncorhynchus-mykiss-under-intensive-breeding-conditions-pathogenesis-and-haematological-and-immune-parameters
#20
Miroslava Palikova, Ivana Papezikova, Zdenka Markova, Stanislav Navratil, Jan Mares, Lukas Mares, Libor Vojtek, Pavel Hyrsl, Eva Jelinkova, Heike Schmidt-Posthaus
Proliferative kidney disease (PKD) is an endoparasitic disease of salmonid fish caused by Tetracapsuloides bryosalmonae (Myxozoa: Malacosporea). This study presents a comprehensive view on PKD development in rainbow trout (Oncorhynchus mykiss) reared at an intensive fish breeding facility, with focus on mortality, pathology/histopathology, haematological findings and immune functions. Diseased and reference fish were sampled monthly and time course of natural infection was followed up from the onset of clinical signs (September 2014) to full recovery (January 2015)...
March 6, 2017: Veterinary Parasitology
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