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Tubuler Disorder

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https://www.readbyqxmd.com/read/28770116/cisplatin-induced-nephrotoxicity-and-hiv-associated-nephropathy-mimickers-of-myeloma-like-cast-nephropathy
#1
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
#2
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...
July 27, 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
#3
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
#4
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
#5
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
#6
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
#7
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 if 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 knockout mice...
July 20, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28715431/colony-collapse-disorder-ccd-and-bee-age-impact-honey-bee-pathophysiology
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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...
June 1, 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
#15
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
https://www.readbyqxmd.com/read/28598689/mathematical-rationalization-for-the-renal-tubular-transport-revised-concepts
#16
Roberto Mioni, Alessandra Marega, Giulio Romano, Domenico Montanaro
The current emphasis on kinetics and in situ control of molecular exchanges, across the tubular membrane, has not been paralleled by corresponding improvements in our understanding of tubular behaviour at the macroscopic level of classical physiology. In this paper, we propose a mathematical rationalization of macroscopic tubular transport by means of a principal transport equation, originating from the law of mass action between substrate and carrier. The other equations, derived from the main one, demonstrate the possibility of distinguishing between transporters with low affinity and high capacity and transporters with high affinity and low capacity...
June 9, 2017: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/28551791/mitochondrial-mechanosensor-microdomains-in-cardiovascular-disorders
#17
Michele Miragoli, Aderville Cabassi
The cardiomyocytes populating the 'working myocardium' are highly organized and such organization ranges from macroscale (e.g. the geometrical rod shape) to microscale (dyad/t-tubules) domains. This meticulous level of organization is imperative for assuring the normal and physiological pump-function of the heart. In the pathological cardiac tissue, the domains-related architecture is partially lost, resulting in morphological, electrical and metabolic remodeling and promoting cardiovascular diseases including heart failure and arrhythmias...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28530841/the-increased-expression-of-toll-like-receptor-4-in-renal-and-skin-lesions-in-lupus-erythematosus
#18
Nesrine Elloumi, Raouia Fakhfakh, Lobna Ayadi, Khadija Sellami, Olfa Abida, Mariem Ben Jmaa, Tahya Sellami, Khawla Kammoun, Hatem Masmoudi
Toll-like receptor 4 (TLR-4), a bacterial lipopolysaccharide sensor, is an innate immunity essential modulator. It is expressed on both immune and non-immune cells and may contribute to the cutaneous and renal manifestations during lupus erythematosus (LE). Our purpose is to evaluate TLR-4 expression and analyzing its role in lupus nephritis (LN) and chronic cutaneous lupus erythematosus (CLE) pathogenesis. TLR-4 immunohistochemical staining was performed on 30 LN renal biopsies compared with 11 healthy renal tissues and 30 skin biopsies from CLE patients compared with 15 normal individuals...
July 2017: Journal of Histochemistry and Cytochemistry: Official Journal of the Histochemistry Society
https://www.readbyqxmd.com/read/28495475/ameliorative-effects-of-achillea-millefolium-inflorescences-alcoholic-extract-against-nicotine-induced-reproductive-failure-in-rat
#19
M Hallaj Salahipour, Sh Hasanzadeh, H Malekinejad
BACKGROUND: Nicotine (Nic) is a major risk factor in the development of functional disorders of male reproductive system. Achillea millefolium; is highly regarded for medicinal activities, due to its antioxidant and anti-inflammatory properties. This study was carried out to evaluate whether Achillea millefolium (Achm) inflorescences alcoholic extract could serve as a protective agent in male reproductive male failures during Nic exposure in a rat model. METHODS: Twenty-five adult male Wistar rats were categorized into the five groups...
May 8, 2017: Experimental and Toxicologic Pathology: Official Journal of the Gesellschaft Für Toxikologische Pathologie
https://www.readbyqxmd.com/read/28490965/protective-effects-of-fennel-oil-extract-against-sodium-valproate-induced-hepatorenal-damage-in-albino-rats
#20
Wael M Al-Amoudi
Foeniculum vulgare (Apiaceae) is commonly known as fennel. This herb is well-known worldwide and traditionally used as curative herbal therapy for the treatment of epileptic disease, seizurescarminative, digestive, lactogogue, diuretic, treating respiratory and gastrointestinal disorders. The aim of present study is to investigate the possible effect of fennel oil against the toxicity of Sodium-Valproic (SVP) in albino rats. In order to assess the protection of fennel oil on SVP induced hepato- and nephro-toxicity, male albino rats were treated with 1 ml/kg b...
May 2017: Saudi Journal of Biological Sciences
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