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Tubuler Disorder

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https://www.readbyqxmd.com/read/28919595/a-case-of-hepatorenal-syndrome-and-abdominal-compartment-syndrome-with-high-renal-congestion
#1
Hiroteru Kamimura, Takayuki Watanabe, Tomoyuki Sugano, Nao Nakajima, Junji Yokoyama, Kenya Kamimura, Atsunori Tsuchiya, Masaaki Takamura, Hirokazu Kawai, Takashi Kato, Gen Watanabe, Satoshi Yamagiwa, Shuji Terai
BACKGROUND Hepatorenal syndrome (HRS) is a reversible renal impairment that occurs in patients with acute liver failure and advanced liver cirrhosis. HRS is due to a renal vasoconstriction that results from extreme vasodilatation. It is therefore a functional disorder, not associated with structural kidney damage. On the other hand, end-stage liver diseases are often complicated by massive ascites. Massive ascites may cause abdominal compartment syndrome (ACS), which includes impairment of renal blood flow, but there are no reports indicating that kidney lesions caused by ACS may pathologically contribute to end-stage liver diseases...
September 18, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28889026/gnas-mutations-and-heterotopic-ossification
#2
Murat Bastepe
GNAS is a complex imprinted gene encoding the alpha-subunit of the stimulatory heterotrimeric G protein (Gsα). GNAS gives rise to additional gene products that exhibit exclusively maternal or paternal expression, such as XLαs, a large variant of Gsα that shows exclusively paternal expression and is partly identical to the latter. Gsα itself is expressed biallelically in most tissues, although the expression occurs predominantly from the maternal allele in a small set of tissues, such as renal proximal tubules...
September 6, 2017: Bone
https://www.readbyqxmd.com/read/28851737/cholesterol-metabolism-and-cx43-cx46-cx50-gap-junction-protein-expression-and-localisation-in-normal-and-diabetic-and-obese-ob-ob-and-db-db-mouse-testes
#3
R-Marc Pelletier, Casimir D Akpovi, Li Chen, Maria Leiza Vitale
Decreased fertility and birth rates are afflictions arising from metabolic disorders. This study assesses cholesterol metabolism and Cx46, Cx50, Cx43 expression in interstitium- and seminiferous tubule-enriched fractions of leptin-deficient (ob/ob) and leptin receptor-deficient (db/db) mice, two type 2 diabetes and obesity models associated with infertility. Testosterone decreased, glucose, free and esterified cholesterol increased in serum whereas in interstitium, free and esterified cholesterol decreased in ob/ob and db/db...
August 29, 2017: American Journal of Physiology. Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28835935/protective-effect-of-hydroalcoholic-extract-of-teucrium-polium-on-diabetes-induced-testicular-damage-and-serum-testosterone-concentration
#4
Ramin Salimnejad, Ghasem Sazegar, Mohammad Javad Saeedi Borujeni, Seyed Mojtaba Mousavi, Fatemeh Salehi, Fatemeh Ghorbani
BACKGROUND: Diabetes has an adverse effect on spermatogenesis by rising oxidative stress. OBJECTIVE: The aim of this study was to investigate the effect of Teucrium Polium extract administration on spermatogenesis and testicular structure in diabetic rats induced with Streptozotocin. MATERIALS AND METHODS: 32 male Wistar rats were randomly divided into four groups (n=8/each): control group, diabetic group received distilled water, and two experimental groups included diabetic rats treated with 50 and 100 mg/body weigh of Teucrium Polium extract for 6 six weeks...
April 2017: International Journal of Reproductive Biomedicine (Yazd, Iran)
https://www.readbyqxmd.com/read/28835760/testicular-adrenal-rests-tumors-and-testicular-microlithiasis-in-a-brazilian-case-series-with-classic-congenital-adrenal-hyperplasia
#5
Laura Ohana Marques Coelho de Carvalho, Raymundo Miguel Garcia Lora, Claudia Renata Rezende Penna, Izabel Calland Ricarte Beserra
BACKGROUND: Testicular adrenal rest tumors are a benign condition characterized by the presence of remnants of adrenal tissue within the testes that can lead to infertility. Testicular microlithiasis are calculus deposits within the seminiferous tubules. Both are described in congenital adrenal hyperplasia. OBJECTIVES: Describe the frequency of testicular adrenal rest tumors and testicular microlithiasis in a Brazilian case series of patients with classic congenital adrenal hyperplasia and to also relate these changes to disease control and hypothalamic-pituitary-gonadal axis disorders...
January 2017: International Journal of Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28770116/cisplatin-induced-nephrotoxicity-and-hiv-associated-nephropathy-mimickers-of-myeloma-like-cast-nephropathy
#6
Muhammad Siddique Khurram, Ahmed Alrajjal, Warda Ibrar, Jacob Edens, Umer Sheikh, Ameer Hamza, Hong Qu
Myeloma cast nephropathy is an obstructing disorder of renal tubules, caused by precipitation of Bence Jones proteins. Myeloma-like cast nephropathy (MLCN) has been reported in the literature to occur in various primary renal and nonrenal diseases. We present a series of three rare cases of cast nephropathy, two of which are HIV patients, and the third patient is receiving cisplatin-based chemotherapy. However, in all three patients plasma cell dyscrasia has been ruled out. A 30-year-old male was admitted to the hospital with facial cellulitis...
2017: Case Reports in Nephrology
https://www.readbyqxmd.com/read/28765879/mass-spectrometric-identification-of-dystrophin-the-protein-product-of-the-duchenne-muscular-dystrophy%C3%A2-gene-in-distinct-muscle-surface-membranes
#7
Sandra Murphy, Kay Ohlendieck
Supramolecular membrane complexes of low abundance are difficult to study by routine bioanalytical techniques. The plasmalemmal complex consisting of sarcoglycans, dystroglycans, dystrobrevins and syntrophins, which is closely associated with the membrane cytoskeletal protein dystrophin, represents such a high‑molecular‑mass protein assembly in skeletal muscles. The almost complete loss of the dystrophin isoform Dp427‑M and concomitant reduction in the dystrophin‑associated glycoprotein complex is the underlying cause of the highly progressive neuromuscular disorder named Duchenne muscular dystrophy...
October 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28763101/relevant-genetic-polymorphisms-and-kidney-expression-of-tlr5-and-tlr9-in-lupus-nephritis
#8
Nesrine Elloumi, Raouia Fakhfakh, Olfa Abida, Lobna Ayadi, Sameh Marzouk, Hend Hachicha, Mohamed Fourati, Zouhir Bahloul, Mohamed Nabil Mhiri, Khawla Kammoun, Hatem Masmoudi
Toll-like receptor (TLR) genetic polymorphisms may modify their expression causing inflammatory disorders and influencing both susceptibility and severity of lupus erythematosus. We aim to determine whether TLR5 and TLR9 genes polymorphisms are implicated in the susceptibility to systemic lupus erythematosus (SLE) and lupus nephritis (LN) and to evaluate their expressions and distributions in renal LN patients' biopsies. The frequencies of 2 SNP in TLR9 gene and 1 in TLR5 gene was examined in 106 SLE patients (among them 37 LN patients) and in 200 matched controls by PCR-RFLP analysis...
August 1, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28757000/characteristics-of-vitamin-b12-deficiency-in-patients-with-plasma-cell-disorders
#9
Caitlyn Braschi, John Doucette, Ajai Chari
BACKGROUND: Although increased rates of vitamin B12 deficiency have been reported in patients with plasma cell dyscrasias (PCDs), no mechanism has been identified. Excess free light chains (FLCs) could disrupt the renal proximal tubule receptors where B12 is reabsorbed. We sought to characterize the relationship between B12 deficiency and PCDs. We hypothesized that rates of B12 deficiency would be highest in patients with PCDs with high FLC burdens. METHODS: We reviewed the electronic medical records of 501 patients who met inclusion criteria (diagnosed PCD with documented serum B12 and FLC levels) to obtain clinical data recorded prior to patients' lowest B12 levels...
July 12, 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28752231/parasites-and-pathological-conditions-in-the-edible-oyster-crassostrea-madrasensis-preston-from-the-east-and-west-coasts-of-india
#10
G Suja, V Kripa, K Sunil Mohamed, J Lijo, K G Mini, N K Sanil
A detailed pathological survey was carried out on the commercially important edible oyster, Crassostrea madrasensis (Preston), from two distinct coastal/brackish water ecosystems of south India. Samples were collected twice a year during wet and dry seasons from 2009 to 2012. Bacterial colonies in the form of prokaryotic inclusions, protozoans (Perkinsus beihaiensis, Nematopsis sp. and ciliates Sphenophrya sp. and Stegotricha sp.), metazoans (trematodes, turbellaria, cestodes and crustaceans) and shell parasites (Polydora spp...
July 27, 2017: Parasitology Research
https://www.readbyqxmd.com/read/28739648/hepatocyte-nuclear-factor-1%C3%AE-controls-mitochondrial-respiration-in-renal-tubular-cells
#11
Audrey Casemayou, Audren Fournel, Alessia Bagattin, Joost Schanstra, Julie Belliere, Stéphane Decramer, Dimitri Marsal, Marion Gillet, Nicolas Chassaing, Antoine Huart, Marco Pontoglio, Claude Knauf, Jean-Loup Bascands, Dominique Chauveau, Stanislas Faguer
AKI is a frequent condition that involves renal microcirculation impairment, infiltration of inflammatory cells with local production of proinflammatory cytokines, and subsequent epithelial disorders and mitochondrial dysfunction. Peroxisome proliferator-activated receptor γ coactivator 1-α (PPARGC1A), a coactivator of the transcription factor PPAR-γ that controls mitochondrial biogenesis and function, has a pivotal role in the early dysfunction of the proximal tubule and the subsequent renal repair. Here, we evaluated the potential role of hepatocyte nuclear factor-1β (HNF-1β) in regulating PPARGC1A expression in AKI...
July 24, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28729419/the-exocyst-is-required-for-photoreceptor-ciliogenesis-and-retinal-development
#12
Glenn P Lobo, Diana Fulmer, Lilong Guo, Xiaofeng Zuo, Yujing Dang, Seok-Hyung Kim, Yanhui Su, Kola George, Elisabeth Obert, Ben Fogelgren, Deepak Nihalani, Russell A Norris, Bärbel Rohrer, Joshua H Lipschutz
We previously have shown that the highly conserved eight-protein exocyst trafficking complex is required for ciliogenesis in kidney tubule cells. We hypothesized here that ciliogenic programs are conserved across organs and species. To determine whether renal primary ciliogenic programs are conserved in the eye, and to characterize the function and mechanisms by which the exocyst regulates eye development in zebrafish, we focused on exoc5, a central component of the exocyst complex, by analyzing both exoc5 zebrafish mutants, and photoreceptor-specific Exoc5 knock-out mice...
September 8, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28715431/colony-collapse-disorder-ccd-and-bee-age-impact-honey-bee-pathophysiology
#13
Dennis vanEngelsdorp, Kirsten S Traynor, Michael Andree, Elinor M Lichtenberg, Yanping Chen, Claude Saegerman, Diana L Cox-Foster
Honey bee (Apis mellifera) colonies continue to experience high annual losses that remain poorly explained. Numerous interacting factors have been linked to colony declines. Understanding the pathways linking pathophysiology with symptoms is an important step in understanding the mechanisms of disease. In this study we examined the specific pathologies associated with honey bees collected from colonies suffering from Colony Collapse Disorder (CCD) and compared these with bees collected from apparently healthy colonies...
2017: PloS One
https://www.readbyqxmd.com/read/28712002/mutations-in-gfpt1-related-congenital-myasthenic-syndromes-are-associated-with-synaptic-morphological-defects-and-underlie-a-tubular-aggregate-myopathy-with-synaptopathy
#14
Stéphanie Bauché, Geoffroy Vellieux, Damien Sternberg, Marie-Joséphine Fontenille, Elodie De Bruyckere, Claire-Sophie Davoine, Guy Brochier, Julien Messéant, Lucie Wolf, Michel Fardeau, Emmanuelle Lacène, Norma Romero, Jeanine Koenig, Emmanuel Fournier, Daniel Hantaï, Nathalie Streichenberger, Veronique Manel, Arnaud Lacour, Aleksandra Nadaj-Pakleza, Sylvie Sukno, Françoise Bouhour, Pascal Laforêt, Bertrand Fontaine, Laure Strochlic, Bruno Eymard, Frédéric Chevessier, Tanya Stojkovic, Sophie Nicole
Mutations in GFPT1 (glutamine-fructose-6-phosphate transaminase 1), a gene encoding an enzyme involved in glycosylation of ubiquitous proteins, cause a limb-girdle congenital myasthenic syndrome (LG-CMS) with tubular aggregates (TAs) characterized predominantly by affection of the proximal skeletal muscles and presence of highly organized and remodeled sarcoplasmic tubules in patients' muscle biopsies. We report here the first long-term clinical follow-up of 11 French individuals suffering from LG-CMS with TAs due to GFPT1 mutations, of which nine are new...
August 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28705828/crispr-induced-null-alleles-show-that-frost-protects-drosophila-melanogaster-reproduction-after-cold-exposure
#15
Claire E Newman, Jantina Toxopeus, Hiroko Udaka, Soohyun Ahn, David M Martynowicz, Steffen P Graether, Brent J Sinclair, Anthony Percival-Smith
The ability to survive and reproduce after cold exposure is important in all kingdoms of life. However, even in a sophisticated genetic model system like Drosophila melanogaster, few genes have been identified as functioning in cold tolerance. The accumulation of the Frost (Fst) gene transcript increases after cold exposure, making it a good candidate for a gene that has a role in cold tolerance. However, despite extensive RNAi knockdown analysis, no role in cold tolerance has been assigned to Fst CRISPR is an effective technique for completely knocking down genes, and less likely to produce off-target effects than GAL4-UAS RNAi systems...
July 13, 2017: Journal of Experimental Biology
https://www.readbyqxmd.com/read/28699114/reversible-hypokalemia-and-bartter-like-syndrome-during-prolonged-systemic-therapy-with-colistimethate-sodium-in-an-adult-patient
#16
Tarek Kamal Eldin, Grazia Tosone, Alfredo Capuano, Raffaele Orlando
We present the case of a 58-year-old woman who developed hypokalaemia and metabolic alkalosis 2 weeks after therapy with colistimethate sodium for the treatment of chronic lower limb ulcer infection by extensively drug-resistant (XDR) Pseudomonas aeruginosa. The metabolic changes observed resembled Bartter syndrome, a group of congenital disorders affecting the distal segments of the renal tubules. The metabolic abnormalities reversed spontaneously 6 days after drug discontinuation. Acquired forms of Bartter syndrome have been reported during courses of antibiotic therapy; however, to our knowledge, this is the first documented case associated with colistimethate therapy in an adult...
December 2017: Drug Safety—Case Reports
https://www.readbyqxmd.com/read/28694431/key-role-for-the-organic-anion-transporters-oat1-and-oat3-in-the-in-vivo-handling-of-uremic-toxins-and-solutes
#17
Wei Wu, Kevin T Bush, Sanjay K Nigam
In vitro data indicates that the kidney proximal tubule (PT) transporters of uremic toxins and solutes (e.g., indoxyl sulfate, p-cresol sulfate, kynurenine, creatinine, urate) include two "drug" transporters of the organic anion transporter (OAT) family: OAT1 (SLC22A6, originally NKT) and OAT3 (SLC22A8). Here, we have examined new and prior metabolomics data from the Oat1KO and Oat3KO, as well as newly obtained metabolomics data from a "chemical double" knockout (Oat3KO plus probenecid). This gives a picture of the in vivo roles of OAT1 and OAT3 in the regulation of the uremic solutes and supports the centrality of these "drug" transporters in independently and synergistically regulating uremic metabolism...
July 10, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28680856/sertoli-cells-are-susceptible-to-zikv-infection-in-mouse-testis
#18
Zi-Yang Sheng, Na Gao, Zhao-Yang Wang, Xiao-Yun Cui, De-Shan Zhou, Dong-Ying Fan, Hui Chen, Pei-Gang Wang, Jing An
Flaviviruses including Dengue virus (DENV), Yellow fever virus (YFV), West Nile virus (WNV), and Japanese encephalitis virus (JEV) are global health problems that caused several serious diseases such as fever, hemorrhagic fever, and encephalitis in the past century. Recently, Zika virus (ZIKV) which spreads from Asia to American and causes millions of infections emerges as a new dangerous member of the genus of Flavivirus. Unlike other well-known flaviviruses, ZIKV can be transmitted sexually and infect testes in murine models...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28644734/high-throughput-phenotypic-screening-of-kinase-inhibitors-to-identify-drug-targets-for-polycystic-kidney-disease
#19
Tijmen H Booij, Hester Bange, Wouter N Leonhard, Kuan Yan, Michiel Fokkelman, Steven J Kunnen, Johannes G Dauwerse, Yu Qin, Bob van de Water, Gerard J P van Westen, Dorien J M Peters, Leo S Price
Polycystic kidney disease (PKD) is a prevalent disorder characterized by renal cysts that lead to kidney failure. Various signaling pathways have been targeted to stop disease progression, but most interventions still focus on alleviating PKD-associated symptoms. The mechanistic complexity of the disease, as well as the lack of functional in vitro assays for compound testing, has made drug discovery for PKD challenging. To identify modulators of PKD, Pkd1(-/-) kidney tubule epithelial cells were applied to a scalable and automated 3D cyst culture model for compound screening, followed by phenotypic profiling to determine compound efficacy...
September 2017: SLAS Discovery
https://www.readbyqxmd.com/read/28636607/three-dimensional-cell-shapes-and-arrangements-in-human-sweat-glands-as-revealed-by-whole-mount-immunostaining
#20
Ryuichiro Kurata, Sugiko Futaki, Itsuko Nakano, Fumitaka Fujita, Atsushi Tanemura, Hiroyuki Murota, Ichiro Katayama, Fumihiro Okada, Kiyotoshi Sekiguchi
Because sweat secretion is facilitated by mechanical contraction of sweat gland structures, understanding their structure-function relationship could lead to more effective treatments for patients with sweat gland disorders such as heat stroke. Conventional histological studies have shown that sweat glands are three-dimensionally coiled tubular structures consisting of ducts and secretory portions, although their detailed structural anatomy remains unclear. To better understand the details of the three-dimensional (3D) coiled structures of sweat glands, a whole-mount staining method was employed to visualize 3D coiled gland structures with sweat gland markers for ductal luminal, ductal basal, secretory luminal, and myoepithelial cells...
2017: PloS One
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