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https://www.readbyqxmd.com/read/29925518/cilia-localized-lkb1-regulates-chemokine-signaling-macrophage-recruitment-and-tissue-homeostasis-in-the-kidney
#1
Amandine Viau, Frank Bienaimé, Kamile Lukas, Abhijeet P Todkar, Manuel Knoll, Toma A Yakulov, Alexis Hofherr, Oliver Kretz, Martin Helmstädter, Wilfried Reichardt, Simone Braeg, Tom Aschman, Annette Merkle, Dietmar Pfeifer, Verónica I Dumit, Marie-Claire Gubler, Roland Nitschke, Tobias B Huber, Fabiola Terzi, Jörn Dengjel, Florian Grahammer, Michael Köttgen, Hauke Busch, Melanie Boerries, Gerd Walz, Antigoni Triantafyllopoulou, E Wolfgang Kuehn
Polycystic kidney disease (PKD) and other renal ciliopathies are characterized by cysts, inflammation, and fibrosis. Cilia function as signaling centers, but a molecular link to inflammation in the kidney has not been established. Here, we show that cilia in renal epithelia activate chemokine signaling to recruit inflammatory cells. We identify a complex of the ciliary kinase LKB1 and several ciliopathy-related proteins including NPHP1 and PKD1. At homeostasis, this ciliary module suppresses expression of the chemokine CCL2 in tubular epithelial cells...
June 19, 2018: EMBO Journal
https://www.readbyqxmd.com/read/29925070/osmoregulation-in-polycystic-kidney-disease-relationship-with-cystogenesis-and-hypertension
#2
Conor F Underwood, Jacqueline K Phillips, Cara M Hildreth
Polycystic kidney disease (PKD) is a group of monogenetic conditions characterised by the progressive accumulation of multiple renal cysts and hypertension. One of the earliest features of PKD is a reduction in urinary concentrating capacity that impairs extracellular fluid conservation. Urinary concentrating impairment predisposes PKD patients to periods of hypohydration when fluid loss is not adequately compensated by fluid intake. The hypohydrated state provides a blood hyperosmotic stimulus for vasopressin release to minimise further water loss...
2018: Annals of Nutrition & Metabolism
https://www.readbyqxmd.com/read/29904385/dysregulation-of-b-cell-activity-during-proliferative-kidney-disease-in-rainbow-trout
#3
Beatriz Abos, Itziar Estensoro, Pedro Perdiguero, Marc Faber, Yehfang Hu, Patricia Díaz Rosales, Aitor G Granja, Christopher J Secombes, Jason W Holland, Carolina Tafalla
Proliferative kidney disease (PKD) is a widespread disease caused by the endoparasite Tetracapsuloides bryosalmonae (Myxozoa: Malacosporea). Clinical disease, provoked by the proliferation of extrasporogonic parasite stages, is characterized by a chronic kidney pathology with underlying transcriptional changes indicative of altered B cell responses and dysregulated T-helper cell-like activities. Despite the relevance of PKD to European and North American salmonid aquaculture, no studies, to date, have focused on further characterizing the B cell response during the course of this disease...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29901206/protein-kinase-d1-regulates-hypoxic-metabolism-through-hif-1%C3%AE-and-glycolytic-enzymes-incancer-cells
#4
Jiao Chen, Bomiao Cui, Yaping Fan, Xiaoying Li, Qian Li, Yue Du, Yun Feng, Ping Zhang
Protein kinase D1 (PKD1), one of the protein kinase D (PKD) family members, plays a prominent role in multiple bio-behaviors of cancer cells. Low pH and hypoxia are unique characteristics of the tumor microenvironment. The aim of this study was to investigate the role and mechanism of PKD1 in regulating metabolism in the human tongue squamous cell carcinoma (TSCC) cell line SCC25 under a hypoxic condition, as well as growth and apoptosis. Here, we found that hypoxia not only induced the expression of HIF-1α, but also induced the expression and activation of PKD1...
June 7, 2018: Oncology Reports
https://www.readbyqxmd.com/read/29897930/loss-of-fnip1-alters-kidney-developmental-transcriptional-program-and-synergizes-with-tsc1-loss-to-promote-mtorc1-activation-and-renal-cyst-formation
#5
Ryan Centini, Mark Tsang, Terri Iwata, Heon Park, Jeffrey Delrow, Daciana Margineantu, Brandon M Iritani, Haiwei Gu, H Denny Liggitt, Janella Kang, Lim Kang, David M Hockenbery, Daniel Raftery, Brian M Iritani
Birt-Hogg-Dube' Syndrome (BHDS) is a rare genetic disorder in humans characterized by skin hamartomas, lung cysts, pneumothorax, and increased risk of renal tumors. BHDS is caused by mutations in the BHD gene, which encodes for Folliculin, a cytoplasmic adapter protein that binds to Folliculin interacting proteins-1 and -2 (Fnip1, Fnip2) as well as the master energy sensor AMP kinase (AMPK). Whereas kidney-specific deletion of the Bhd gene in mice is known to result in polycystic kidney disease (PKD) and renal cell carcinoma, the roles of Fnip1 in renal cell development and function are unclear...
2018: PloS One
https://www.readbyqxmd.com/read/29892195/misdiagnosed-atypical-paroxysmal-kinesigenic-dyskinesia-a-case-report
#6
Fen Pan, Shangda Li, Haimei Li, Yi Xu, Manli Huang
Background: Paroxysmal kinesigenic dyskinesia (PKD) is characterized by sudden episodes of involuntary movements. PKD is a very rare movement disorder, and correct clinical diagnosis is often a challenge. Case: We present the case of a 23-year-old female with PKD. The patient showed episodes of twisting movements for 3 years. The symptoms lasted for about 5-10 minutes and subsided spontaneously. She was diagnosed as having epilepsy, and depressive and anxiety disorders successively...
2018: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/29883688/phosphoproteomic-identification-and-functional-characterization-of-protein-kinase-substrates-by-2d-dige-and-phos-tag-page
#7
REVIEW
Kou Motani, Hidetaka Kosako
Protein phosphorylation is one of the most common post-translational modifications in eukaryotes and can regulate diverse properties of proteins. Protein kinases are encoded by more than 500 genes in higher eukaryotes and play central roles in various cellular signaling pathways. Consequently, genetic abnormalities of protein kinases have been implicated in many diseases. To fully understand the complex phosphorylation-mediated signaling networks, it is important to globally identify and functionally characterize in vivo substrates of individual protein kinases...
June 5, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29880041/urinary-exosomal-expression-of-activator-of-g-protein-signaling-3-in-polycystic-kidney-disease
#8
Krishna C Keri, Kevin R Regner, Aaron T Dall, Frank Park
OBJECTIVE: PKD is a genetic disease that is characterized by abnormally proliferative epithelial cells in the kidney and liver. Urinary exosomes have been previously examined as a source of unique proteins that may be used to diagnose and monitor the progression of PKD. Previous studies by our group have shown that AGS3, which is a receptor-independent regulator G-proteins, was markedly upregulated in RTECs during kidney injury including PKD. In this study, our goal was to determine whether AGS3 could be measured in exosomes using animals and humans with PKD...
June 7, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29869165/polycystic-kidney-features-of-the-renal-pathology-in-glycogen-storage-disease-type-i-possible-evolution-to-renal-neoplasia
#9
Monika Gjorgjieva, Laure Monteillet, Julien Calderaro, Gilles Mithieux, Fabienne Rajas
Glycogen storage disease type I (GSDI) is a rare genetic pathology characterized by glucose-6 phosphatase (G6Pase) deficiency, translating in hypoglycemia during short fasts. Besides metabolic perturbations, GSDI patients develop long-term complications, especially chronic kidney disease (CKD). In GSDI patients, CKD is characterized by an accumulation of glycogen and lipids in kidneys, leading to a gradual decline in renal function. At a molecular level, the activation of the renin-angiotensin system is responsible for the development of renal fibrosis, eventually leading to renal failure...
June 4, 2018: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29860066/mutational-analysis-in-patients-with-autosomal-dominant-polycystic-kidney-disease-adpkd-identification-of-five-mutations-in-the-pkd1-gene
#10
Mayssa Abdelwahed, Pascale Hilbert, Asma Ahmed, Hichem Mahfoudh, Salem Bouomrani, Mouna Dey, Jamil Hachicha, Hassen Kamoun, Leila Keskes-Ammar, Neïla Belguith
Autosomal Dominant Polycystic Kidney Disease (ADPKD), the most frequent genetic disorder of the kidneys, is characterized by a typical presenting symptoms include cysts development in different organs and a non-cysts manifestations. ADPKD is caused by mutations in PKD1 or PKD2 genes. In this study, we aimed to search for molecular causative defects among PKD1 and PKD2 genes. Eighteen patients were diagnosed based on renal ultrasonography and renal/extra-renal manifestations. Then, Sanger sequencing was performed for PKD1 and PKD2 genes...
May 31, 2018: Gene
https://www.readbyqxmd.com/read/29859305/kunitz-type-serine-protease-inhibitor-is-a-novel-participator-in-anti-bacterial-and-anti-inflammatory-responses-in-japanese-flounder-paralichthys-olivaceus
#11
Xin Xu, Jinxiang Liu, Yujue Wang, Yu Si, Xuangang Wang, Zhigang Wang, Quanqi Zhang, Haiyang Yu, Xubo Wang
Kunitz-type serine protease inhibitor (KSPI) interacts with serine protease (SP) to regulate cascade reactions in vivo and plays essential roles in innate immunity. Theoretical considerations support various functions of kspi, but further studies are required for full characterization of these functions. In this study, a KSPI molecule was identified from Japanese flounder (Paralichthys olivaceus), and was named Pokspi. The full-length cDNA sequence of Pokspi was 2810 nt, containing an open reading frame of 1527 nt, which encoded a polypeptide of 509 amino acid residues...
May 31, 2018: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/29854969/longitudinal-assessment-of-left-ventricular-mass-in-autosomal-dominant-polycystic-kidney-disease
#12
Taimur Dad, Kaleab Z Abebe, K Ty Bae, Diane Comer, Vicente E Torres, Peter G Czarnecki, Robert W Schrier, Theodore I Steinman, Charity G Moore, Arlene B Chapman, Diana Kaya, Cheng Tao, William E Braun, Franz T Winklhofer, Godela Brosnahan, Marie C Hogan, Dana C Miskulin, Frederic Rahbari Oskoui, Michael F Flessner, Ronald D Perrone
Introduction: The high burden of cardiovascular morbidity and mortality in autosomal dominant polycystic kidney disease (ADPKD) is related to development of hypertension and left ventricular hypertrophy. Blood pressure reduction has been shown to reduce left ventricular mass in ADPKD; however, moderators and predictors of response to lower blood pressure are unknown. Methods: This was a post hoc cohort analysis of HALT PKD study A, a randomized placebo controlled trial examining the effect of low blood pressure and single versus dual renin-angiotensin blockade in early ADPKD...
May 2018: KI Reports
https://www.readbyqxmd.com/read/29854077/function-and-regulation-of-protein-kinase-d-in-oxidative-stress-a-tale-of-isoforms
#13
REVIEW
Mathias Cobbaut, Johan Van Lint
Oxidative stress is a condition that arises when cells are faced with levels of reactive oxygen species (ROS) that destabilize the homeostatic redox balance. High levels of ROS can cause damage to macromolecules including DNA, lipids, and proteins, eventually resulting in cell death. Moderate levels of ROS however serve as signaling molecules that can drive and potentiate several cellular phenotypes. Increased levels of ROS are associated with a number of diseases including neurological disorders and cancer...
2018: Oxidative Medicine and Cellular Longevity
https://www.readbyqxmd.com/read/29787614/outcome-of-polycystic-kidney-disease-patients-on-peritoneal-dialysis-systematic-review-of-literature-and-meta-analysis
#14
Vincent Dupont, Lukshe Kanagaratnam, Mickaël Sigogne, Clémence Bechade, Thierry Lobbedez, Jose Portoles, Philippe Rieu, Moustapha Drame, Fatouma Touré
BACKGROUND: Polycystic kidney disease (PKD) is the most frequent hereditary cause of chronic kidney disease. Peritoneal dialysis (PD) is often avoided for patients with PKD because of the suspected risk of mechanical and infectious complications. Only a few studies have analyzed the outcome of PKD patients on PD with sometimes conflicting results. The purpose of this meta-analysis was to investigate outcomes of patients with PKD treated by PD. METHODS: A systematic review and meta-analysis were performed examining all studies which included "Polycystic kidney disease" and "Peritoneal dialysis" in their titles, excluding commentaries, letters to the authors and abstracts...
2018: PloS One
https://www.readbyqxmd.com/read/29781735/protein-kinase-d-was-involved-in-vascular-remodeling-in-spontaneously-hypertensive-rats
#15
Jing Geng, Zhuo Zhao, Le Yang, Mingwei Zhang, Xiangjuan Liu
The present study was designed to determine the role of PKD in vascular remodeling (VR) in Spontaneously hypertensive rats (SHRs). Increased SBP, VR and PKD activation were prominent in SHRs. The SBP has a positive correlation with the activation of PKD in SHRs. The ratio of media to lumen (MT/LD), volume fraction of collagen (VFC), hydroxyproline, IL-6, TNF-α and nitrotyrosine content were significantly related to the activated PKD. It may be concluded that PKD plays a central role in VR, and the mechanism may be related to its regulation of hypertrophy, fibrosis, inflammation and oxidative stress...
May 21, 2018: Clinical and Experimental Hypertension: CHE
https://www.readbyqxmd.com/read/29775233/dialysis-modality-and-mortality-in-polycystic-kidney-disease
#16
Chenchen Zhou, Yaodong Gu, Changlin Mei, Bing Dai, Yi Wang, Cheng Xue
INTRODUCTION: Identifying the appropriate modality between hemodialysis (HD) and peritoneal dialysis (PD) is an unresolved issue in polycystic kidney disease (PKD) patients. This study aims to illustrate whether the mortality and survival are different among individuals receiving HD comparing PD. METHODS: We searched PubMed, EMBASE, and China National Knowledge Infrastructure about cohort studies involving PKD patients with end stage renal disease and comparing HD with PD...
May 18, 2018: Hemodialysis International
https://www.readbyqxmd.com/read/29770338/basic-fibroblast-growth-factor-fused-with-tandem-collagen-binding-domains-from-clostridium-histolyticum-collagenase-colg-increases-bone-formation
#17
Hiroyuki Sekiguchi, Kentaro Uchida, Osamu Matsushita, Gen Inoue, Nozomu Nishi, Ryo Masuda, Nana Hamamoto, Takaki Koide, Shintaro Shoji, Masashi Takaso
Basic fibroblast growth factor 2 (bFGF) accelerates bone formation during fracture healing. Because the efficacy of bFGF decreases rapidly following its diffusion from fracture sites, however, repeated dosing is required to ensure a sustained therapeutic effect. We previously developed a fusion protein comprising bFGF, a polycystic kidney disease domain (PKD; s2b), and collagen-binding domain (CBD; s3) sourced from the Clostridium histolyticum class II collagenase, ColH, and reported that the combination of this fusion protein with a collagen-like peptide, poly(Pro-Hyp-Gly)10 , induced mesenchymal cell proliferation and callus formation at fracture sites...
2018: BioMed Research International
https://www.readbyqxmd.com/read/29751520/young-adult-polycystic-kidney-disease-is-associated-with-major-cardiovascular-complications
#18
Ya-Wen Chuang, Tung-Min Yu, Shih-Ting Huang, Kuo-Ting Sun, Ying-Chih Lo, Pin-Kuei Fu, Bor-Jen Lee, Cheng-Hsu Chen, Cheng-Li Lin, Chia-Hung Kao
Background: Patients with polycystic kidney disease (PKD) might have a risk of cardiovascular diseases because several cardiovascular risk factors are occasionally associated with PKD patients. Data on the association between PKD and the risk of cardiovascular events, including acute coronary syndrome (ACS), stroke, and congestive heart failure (CHF), are scant. Methods: Patients aged ≥20 years who were newly diagnosed with PKD (International Classification of Diseases, Ninth Revision, Clinical Modification codes 753...
May 3, 2018: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/29743498/calretinin-participates-in-regulating-steroidogenesis-by-plc-ca-2-pkc-pathway-in-leydig-cells
#19
Wendan Xu, Qian Zhu, Shan Liu, Xiaonan Dai, Bei Zhang, Chao Gao, Li Gao, Jiayin Liu, Yugui Cui
Calretinin, a Ca2+ -binding protein, participates in many cellular events. Our previous studies found the high expression of calretinin in testicular Leydig cells. In this study, (MLTC-1 cells were infected with LV-calb2, R2C cells with LV-siRNA-calb2. The primary mouse Leydig cells were also used to confirm those data from cell lines. Testosterone level was significantly higher in the MLTC-1 cells with over-expressed calretinin than in the control, while progesterone was lower in the R2C cells in which down-regulated calretinin...
May 9, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29743334/randomised-controlled-trial-of-high-versus-ad-libitum-water-intake-in-patients-with-autosomal-dominant-polycystic-kidney-disease-rationale-and-design-of-the-drink-feasibility-trial
#20
Ragada El-Damanawi, Michael Lee, Tess Harris, Laura B Mader, Simon Bond, Holly Pavey, Richard N Sandford, Ian B Wilkinson, Alison Burrows, Przemyslaw Woznowski, Yoav Ben-Shlomo, Fiona E Karet Frankl, Thomas F Hiemstra
INTRODUCTION: Vasopressin stimulates cyst growth in autosomal dominant polycystic kidney disease (ADPKD) leading to enlarged kidneys, hypertension and renal failure. Vasopressin receptor blockade slows disease progression. Physiological suppression of vasopressin secretion through high water (HW) intake could achieve a similar effect, necessitating a definitive large-scale trial of HW intake in ADPKD. The objective of the DRINK trial is to answer the key design and feasibility questions required to deliver a successful definitive water intake trial...
May 9, 2018: BMJ Open
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