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Tubuler Disorder

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https://www.readbyqxmd.com/read/29351417/zinc-regulates-vascular-endothelial-activities-through-zinc-sensing-receptor-znr-gpr39
#1
Donghui Zhu, Yingchao Su, Yufeng Zheng, Bingmei M Fu, Liping Tang, Yi-Xian Qin
Zn2+ is an essential element for cell survival/growth, and its deficiency is linked to many disorders. Extracellular Zn2+ concentration changes participate in modulating fundamental cellular processes such as proliferation, secretion, ion transport, and cell signal transduction in a mechanism that is not well understood. Here, we hypothesize that the Zn-sensing receptor ZnR/GPR39, found in tissues where dynamic Zn2+ homeostasis takes place, enables extracellular Zn2+ to trigger intracellular signaling pathways regulating key cell functions in vascular cells...
December 13, 2017: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/29323117/impaired-autophagy-bridges-lysosomal-storage-disease-and-epithelial-dysfunction-in-the-kidney
#2
Beatrice Paola Festa, Zhiyong Chen, Marine Berquez, Huguette Debaix, Natsuko Tokonami, Jenny Ann Prange, Glenn van de Hoek, Cremonesi Alessio, Andrea Raimondi, Nathalie Nevo, Rachel H Giles, Olivier Devuyst, Alessandro Luciani
The endolysosomal system sustains the reabsorptive activity of specialized epithelial cells. Lysosomal storage diseases such as nephropathic cystinosis cause a major dysfunction of epithelial cells lining the kidney tubule, resulting in massive losses of vital solutes in the urine. The mechanisms linking lysosomal defects and epithelial dysfunction remain unknown, preventing the development of disease-modifying therapies. Here we demonstrate, by combining genetic and pharmacologic approaches, that lysosomal dysfunction in cystinosis results in defective autophagy-mediated clearance of damaged mitochondria...
January 11, 2018: Nature Communications
https://www.readbyqxmd.com/read/29321950/manifestations-of-renal-impairment-in-fructose-induced-metabolic-syndrome
#3
Kameliya Bratoeva, George S Stoyanov, Albena Merdzhanova, Mariya Radanova
Introduction International studies show an increased incidence of chronic kidney disease (CKD) in patients with metabolic syndrome (MS). It is assumed that the major components of MS - obesity, insulin resistance, dyslipidemia, and hypertension - are linked to renal damage through the systemic release of several pro-inflammatory mediators, such as uric acid (UA), C-reactive protein (CRP), and generalized oxidative stress. The aim of the present study was to investigate the extent of kidney impairment and manifestations of dysfunction in rats with fructose-induced MS...
November 7, 2017: Curēus
https://www.readbyqxmd.com/read/29287783/effects-of-telmisartan-and-losartan-on-irradiated-testes
#4
Naira da Silva Mansano, Isabela Fernandes Jorge, Agnaldo Bruno Chies, Gustavo Arruda Viani, Maria Angélica Spadella
AIMS: To analyze the effects of radiation on the reproductive tissue of male Wistar rats and to evaluate whether treatment with the Ang II AT1 receptor antagonists telmisartan and losartan mitigate the dysfunctions resulting from this exposure. MAIN METHODS: Rats were randomly divided into groups: Control, Irradiated, Telmisartan, Losartan, Irradiated+Telmisartan, and Irradiated+Losartan. Single dose of 5Gy was administered directly into the scrotum, followed by treatment with telmisartan (12mg/kg/day) or losartan (34mg/kg/two times/day) for 60days...
December 26, 2017: Life Sciences
https://www.readbyqxmd.com/read/29275531/renal-phosphate-handling-and-inherited-disorders-of-phosphate-reabsorption-an-update
#5
REVIEW
Carsten A Wagner, Isabel Rubio-Aliaga, Nati Hernando
Renal phosphate handling critically determines plasma phosphate and whole body phosphate levels. Filtered phosphate is mostly reabsorbed by Na+-dependent phosphate transporters located in the brush border membrane of the proximal tubule: NaPi-IIa (SLC34A1), NaPi-IIc (SLC34A3), and Pit-2 (SLC20A2). Here we review new evidence for the role and relevance of these transporters in inherited disorders of renal phosphate handling. The importance of NaPi-IIa and NaPi-IIc for renal phosphate reabsorption and mineral homeostasis has been highlighted by the identification of mutations in these transporters in a subset of patients with infantile idiopathic hypercalcemia and patients with hereditary hypophosphatemic rickets with hypercalciuria...
December 23, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29242247/chip-regulates-aquaporin-2-quality-control-and-body-water-homeostasis
#6
Qi Wu, Hanne B Moeller, Donté A Stevens, Rebekah Sanchez-Hodge, Gabrielle Childers, Marleen L A Kortenoeven, Lei Cheng, Lena L Rosenbaek, Carrie Rubel, Cam Patterson, Trairak Pisitkun, Jonathan C Schisler, Robert A Fenton
The importance of the kidney distal convoluted tubule (DCT) and cortical collecting duct (CCD) is highlighted by various water and electrolyte disorders that arise when the unique transport properties of these segments are disturbed. Despite this critical role, little is known about which proteins have a regulatory role in these cells and how these cells can be regulated by individual physiologic stimuli. By combining proteomics, bioinformatics, and cell biology approaches, we found that the E3 ubiquitin ligase CHIP is highly expressed throughout the collecting duct; is modulated in abundance by vasopressin; interacts with aquaporin-2 (AQP2), Hsp70, and Hsc70; and can directly ubiquitylate the water channel AQP2 in vitro shRNA knockdown of CHIP in CCD cells increased AQP2 protein t1/2 and reduced AQP2 ubiquitylation, resulting in greater levels of AQP2 and phosphorylated AQP2...
December 14, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29237739/salt-losing-tubulopathies-in-children-what-s-new-what-s-controversial
#7
Robert Kleta, Detlef Bockenhauer
Renal tubulopathies provide insights into the inner workings of the kidney, yet also pose therapeutic challenges. Because of the central nature of sodium in tubular transport physiology, disorders of sodium handling may affect virtually all aspects of the homeostatic functions of the kidney. Yet, owing to the rarity of these disorders, little clinical evidence regarding treatment exists. Consequently, treatment can vary widely between individual physicians and centers and is based mainly on understanding of renal physiology, reported clinical observations, and individual experiences...
December 13, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29223554/oral-administration-of-nigella-sativa-oil-and-thymoquinone-attenuates-long-term-cisplatin-treatment-induced-toxicity-and-oxidative-damage-in-rat-kidney
#8
Zeba Farooqui, Faaiza Shahid, Aijaz Ahmed Khan, Farah Khan
Cisplatin (CP) is an effective anti-cancer drug which causes remarkable toxicity to the kidney, particularly to proximal tubules, by generating reactive oxygen species. Nigella sativa (NS), commonly known as "black cumin" reduces the progression of various kidney disorders. Thymoquinone (TQ), the major bioactive constituent of NS seeds, has been credited for various pharmacological effects of NS. Since, a typical clinical CP dosing regimen involves CP administration in multiple cycles over a long time duration, hence the present study aimed to evaluate the renoprotective efficacy of NS oil and TQ against multiple dose CP treatment induced deleterious biochemical and histological alterations in rat kidney...
December 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/29202762/myocyte-membrane-and-microdomain-modifications-in-diabetes-determinants-of-ischemic-tolerance-and-cardioprotection
#9
REVIEW
Jake Russell, Eugene F Du Toit, Jason N Peart, Hemal H Patel, John P Headrick
Cardiovascular disease, predominantly ischemic heart disease (IHD), is the leading cause of death in diabetes mellitus (DM). In addition to eliciting cardiomyopathy, DM induces a 'wicked triumvirate': (i) increasing the risk and incidence of IHD and myocardial ischemia; (ii) decreasing myocardial tolerance to ischemia-reperfusion (I-R) injury; and (iii) inhibiting or eliminating responses to cardioprotective stimuli. Changes in ischemic tolerance and cardioprotective signaling may contribute to substantially higher mortality and morbidity following ischemic insult in DM patients...
December 4, 2017: Cardiovascular Diabetology
https://www.readbyqxmd.com/read/29181661/role-of-sertoli-and-leydig-cells-in-the-regulation-of-spermatogonial-stem-cell-and-development-of-reproductive-disorders-in-male-c57bl-6-mice-with-type-1-diabetes-mellitus
#10
E G Skurikhin, A V Pakhomova, O V Pershina, V A Krupin, N N Ermakova, E S Pan, A I Kudryashova, L A Ermolaeva, E S Khmelevskaya, V E Goldberg, V V Zhdanov, A M Dygai
Course administration streptozotocin to male C57Bl/6 mice induces a complex of symptoms typical of type 1 diabetes mellitus: hyperglycemia and insulin deficiency, focal inflammatory infiltration of the pancreas, destructive changes in the Langerhans islets, damage to the insular apparatus (reduced number of PDX1+ cells and insulin expression by the secreting cells). Male reproductive disorder are serious complications of type 1 diabetes mellitus. In "diabetic" mice, interstitial edema with inflammatory infiltration and microvascular disorders in the testicular tissue are observed, the number of endothelial precursors (CD45-/CD31+) and the total number and percentage of motile spermatozoa decreased, immature spermatogenic epithelium cells are desquamated of into the lumen of the tubules...
November 27, 2017: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/29180453/a-hereditary-spastic-paraplegia-associated-atlastin-variant-exhibits-defective-allosteric-coupling-in-the-catalytic-core
#11
John P O'Donnell, Laura J Byrnes, Richard B Cooley, Holger Sondermann
The dynamin-related GTPase atlastin (ATL) catalyzes membrane fusion of the endoplasmic reticulum (ER) and thus establishes a network of branched membrane tubules. When ATL function is compromised, the morphology of the ER deteriorates, and these defects can result in neurological disorders such as hereditary spastic paraplegia (HSP) and hereditary sensory neuropathy (HSN). ATLs harness the energy of GTP hydrolysis to initiate a series of conformational changes that enable homodimerization and subsequent membrane fusion...
November 27, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29177110/advances-in-the-understanding-and-treatment-of-mitochondrial-fatty-acid-oxidation-disorders
#12
Eric S Goetzman
Purpose of review: This review focuses on advances made in the past three years with regards to understanding the mitochondrial fatty acid oxidation (FAO) pathway, the pathophysiological ramifications of genetic lesions in FAO enzymes, and emerging therapies for FAO disorders. Recent findings: FAO has now been recognized to play a key energetic role in pulmonary surfactant synthesis, T-cell differentiation and memory, and the response of the proximal tubule to kidney injury...
September 2017: Current Genetic Medicine Reports
https://www.readbyqxmd.com/read/29173229/prothymosin-alpha-expression-in-the-vertebrate-testis-a-comparative-review
#13
Massimo Venditti, Sergio Minucci
Prothymosin alpha (PTMA) is a highly acidic, intrinsically disordered protein that was first extracted from rat thymus and characterized as an immunogenic factor but soon detected in a variety of mammalian tissues. The presence of a nuclear localization signal and the adoption of a peculiar random-coil conformation are among the reasons behind its interaction with several molecular partners, hence at this time PTMA is known to be a very conserved and widely expressed molecule, involved in numerous and diverse biological processes...
November 27, 2017: Zygote: the Biology of Gametes and Early Embryos
https://www.readbyqxmd.com/read/29136753/-metabolomics-study-of-tris-2-chloroethyl-phosphate-induced-hepaotoxicity-and-nephrotoxicity-in-sprague-dawley-rats
#14
W Q Yang, F Zhao, L Li, Y J Fang
Objective: To discuss the potential toxic target organ and the toxic effects and mechanisms of tris (2-chloroethyl) phosphate (TCEP) on SD rats. Methods: 40 female SD rats weaning from milk for 21 days, weighted (50±2.3)g were selected as subjects and marked by the weight. They were randomly divided into 4 groups, namely control group, 50 (L), 100 (M) and 250 (H) mg·kg(-1)·d(-1) dose of TCEP group. Each group has 10 rats, and administrated the corresponding dose of drug or vehicle by mouth, quaque die for 60 days...
November 6, 2017: Zhonghua Yu Fang Yi Xue za Zhi [Chinese Journal of Preventive Medicine]
https://www.readbyqxmd.com/read/29102933/mesenchymal-stem-cells-contribute-to-improvement-of-renal-function-in-a-canine-kidney-injury-model
#15
Seung-Jun Lee, Min-Ok Ryu, Min-Soo Seo, Sang-Bum Park, Jin-Ok Ahn, Sei-Myoung Han, Kyung-Sun Kang, Dong-Ha Bhang, Hwa-Young Youn
BACKGROUND/AIM: The kidney excretes waste materials and regulates important metabolic functions, and renal disorders constitute a significant medical problem and can result in fatalities. In the present study, mesenchymal stem cells derived from canine umbilical cord blood (cUCB-MSCs) were isolated and evaluated for their ability to improve renal function in a canine model of acute kidney injury (AKI). MATERIALS AND METHODS: The canine AKI model was developed by i...
November 2017: In Vivo
https://www.readbyqxmd.com/read/29101530/sudden-death-due-to-malignant-hyperthermia-with-a-mutation-of-ryr1-autopsy-morphology-and-genetic-analysis
#16
Wenhe Li, Lin Zhang, Yue Liang, Fang Tong, Yiwu Zhou
Malignant hyperthermia (MH) is an autosomal dominant disorder characterized by abnormal calcium homeostasis in skeletal muscles in response to triggering agents. Autopsy, morphology, and genetic analysis were performed on a 19-year-old man who died rapidly after exposure to sevoflurane during maxillofacial surgery. Muscle spasm around the operation area and limb rigidity occurred and renal tubules full of myoglobin casts were observed by microscopy. Ultrastructural changes in the skeletal muscles and the myocardium were detected by transmission electron microscopy (TEM)...
December 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/29072386/-genetic-disorders-of-renal-phosphate-handling
#17
REVIEW
Daniella Magen
Hereditary disorders of renal phosphate handling comprise a diverse group of genetic diseases, usually characterized by excessive urinary phosphate wasting and a negative phosphate balance. In the minority of cases, perturbations of renal phosphate handling are associated with excessive urinary phosphate reabsorption, leading to pathological hyperphosphatemia. Inorganic phosphate is an essential mineral in the human body, playing a crucial role in cellular metabolism and skeletal mineralization. Whole body phosphate balance is maintained by a highly controlled equilibrium between intestinal uptake, skeletal deposition and renal excretion...
October 2017: Harefuah
https://www.readbyqxmd.com/read/29064803/morphological-substantiation-for-acute-immobilization-stress-related-disorders-of-adaptation-mechanisms
#18
Mykhailo M Koptev, Nataliia I Vynnyk
INTRODUCTION: Nowadays, an individual is being constantly accompanied by stresses in his/her everyday life. Stress reactions, produced in the process of evolution, have become the organisms' response to emergency action or pathological factors and are the important link in adaptation process. However, the adverse course of stress reaction can lead to derangement of the adaptation mechanisms in the body and become the element of the pathogenesis of various diseases. THE AIM: The study was aimed at morphological substantiation of derangement of adaptation mechanisms in white Wistar rats caused by the acute immobilization stress...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29031361/treatment-of-disorders-of-sodium-balance-in-chronic-kidney-disease
#19
REVIEW
David H Ellison
Extracellular fluid volume expansion is nearly universal in patients with CKD. Such volume expansion has features similar to the syndrome of heart failure with preserved ejection fraction, which not only leads to symptoms but can also lead to further organ damage. Unique treatment challenges are present in this patient population, including low glomerular filtration, which limits sodium chloride filtration, intrinsic tubule predisposition to sodium chloride retention, and proteinuria. In addition, pharmacokinetic considerations alter the disposition of diuretics in patients with CKD and nephrotic syndrome...
September 2017: Advances in Chronic Kidney Disease
https://www.readbyqxmd.com/read/29029242/germ-cell-neoplasia-in-situ-and-preserved-fertility-despite-suppressed-gonadotropins-in-a-patient-with-testotoxicosis
#20
Li Juel Mortensen, Martin Blomberg Jensen, Peter Christiansen, Ann-Margrethe Rønholt, Anne Jørgensen, Hanne Frederiksen, John E Nielsen, Anand C Loya, Birgitte Grønkær Toft, Niels E Skakkebæk, Ewa Rajpert-De Meyts, Anders Juul
Context: Testotoxicosis is an autosomal dominant, male-limited disorder. Activating mutations in the Luteinizing Hormone Receptor gene (LHCGR) cause high autonomous testosterone secretion resulting in early-onset peripheral precocious puberty. Little is known about long-term consequences of testotoxicosis. Case Description: We present a rare case of a patient followed for 25 years with two remarkable outcomes; preserved fertility and germ cell neoplasia in situ (GCNIS)...
October 5, 2017: Journal of Clinical Endocrinology and Metabolism
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