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https://www.readbyqxmd.com/read/29169004/idiopathic-atypical-haemolytic-uraemic-syndrome-presenting-with-acute-dystonia
#1
M Rizwan, K E Maduemem
Hemolytic Uremic Syndrome (HUS), a triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The atypical HUS (aHUS) results from over activation of complement system with formation of micro thrombi and damage to endothelial cells resulting in renal impairment in 50 % and death in 25 %, commonly in untreated patients. We report an intriguing case of aHUS presenting with acute onset of movement disorder and fluctuating delirium.
August 12, 2017: Irish Medical Journal
https://www.readbyqxmd.com/read/29168908/the-rickiols-20-22-and-24-membered-macrolides-from-the-ascomycete-hypoxylon-rickii
#2
Frank Surup, Eric Kuhnert, Andreas Böhm, Vincent Wiebach, Danny Solga, Hauke Engler, Tim Pendzialek, Albrecht Berkessel, Marc Stadler, Markus Kalesse
In preceding studies the neotropical ascomycete Hypoxylon rickii turned out to be a prolific source of new secondary metabolites since we had obtained terpenoids with five different scaffolds besides a series of terphenyls. From the mycelial extracts of a 70 L scale fermentation of this strain we additionally isolated nine new macrolides (1 - 9) by RP-HPLC. Their planar structures were elucidated by NMR spectroscopy complemented by HR-ESIMS. The relative configurations were assigned by J-based configuration analyses and confirmed by Kishi's universal database...
November 23, 2017: Chemistry: a European Journal
https://www.readbyqxmd.com/read/29168564/grade-related-differences-in-strategy-use-in-multidigit-division-in-two-instructional-settings
#3
Marian Hickendorff, Joke Torbeyns, Lieven Verschaffel
We aimed to investigate upper elementary children's strategy use in the domain of multidigit division in two instructional settings: the Netherlands and Flanders (Belgium). A cross-sectional sample of 119 Dutch and 122 Flemish fourth to sixth graders solved a varied set of multidigit division problems. With latent class analysis, three distinct strategy profiles were identified: children consistently using number-based strategies, children combining the use of column-based and number-based strategies, and children combining the use of digit-based and number-based strategies...
November 23, 2017: British Journal of Developmental Psychology
https://www.readbyqxmd.com/read/29168351/complement-5a-mediated-trophoblasts-dysfunction-is-involved-in-the-development-of-pre-eclampsia
#4
Yu Ma, Ling-Ran Kong, Qian Ge, Yuan-Yuan Lu, Mo-Na Hong, Yu Zhang, Cheng-Chao Ruan, Ping-Jin Gao
Pre-eclampsia (PE) is a life-threatening multisystem disorder leading to maternal and neonatal mortality and morbidity. Emerging evidence showed that activation of the complement system is implicated in the pathological processes of PE. However, little is known about the detailed cellular and molecular mechanism of complement activation in the development of PE. In this study, we reported that complement 5a (C5a) plays a pivotal role in aberrant placentation, which is essential for the onset of PE. We detected an elevated C5a deposition in macrophages and C5a receptor (C5aR) expression in trophoblasts of pre-eclamptic placentas...
November 23, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29168053/the-sociobiology-of-genes-the-gene-s-eye-view-as-a-unifying-behavioural-ecological-framework-for-biological-evolution
#5
Alexis De Tiège, Yves Van de Peer, Johan Braeckman, Koen B Tanghe
Although classical evolutionary theory, i.e., population genetics and the Modern Synthesis, was already implicitly 'gene-centred', the organism was, in practice, still generally regarded as the individual unit of which a population is composed. The gene-centred approach to evolution only reached a logical conclusion with the advent of the gene-selectionist or gene's eye view in the 1960s and 1970s. Whereas classical evolutionary theory can only work with (genotypically represented) fitness differences between individual organisms, gene-selectionism is capable of working with fitness differences among genes within the same organism and genome...
November 22, 2017: History and Philosophy of the Life Sciences
https://www.readbyqxmd.com/read/29168031/neural-cells-generated-from-human-induced-pluripotent-stem-cells-as-a-model-of-cns-involvement-in-mucopolysaccharidosis-type-ii
#6
Jitka Rybová, Jana Ledvinová, Jakub Sikora, Ladislav Kuchař, Robert Dobrovolný
Mucopolysaccharidosis type II (MPSII) is a rare X-linked lysosomal storage disorder caused by mutations in the iduronate-2-sulfatase (IDS) gene (IDS, Xq28). MPSII is characterized by skeletal deformities, hearing loss, airway obstruction, hepatosplenomegaly, cardiac valvular disease, and progressive neurological impairment. At the cellular level, IDS deficiency leads to lysosomal storage of glycosaminoglycans (GAGs), dominated by accumulation of dermatan and heparan sulfates. Human induced pluripotent stem cells (iPSC) represent an alternative system that complements the available MPSII murine model...
November 22, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29168029/the-chinese-version-of-the-sleqol-is-a-reliable-assessment-of-health-related-quality-of-life-in-han-chinese-patients-with-systemic-lupus-erythematosus
#7
Hai-Zhi Jiang, Zhi-Guo Lin, Hong-Juan Li, Qing -Du, Wei -Tian, Shu-Ya Wang, Shang-Qi Guan, Yi-Fang Mei
To assess the health-related quality of life (HRQOL) of Han Chinese people with systemic lupus erythematosus (SLE) using a Chinese version of the Systemic Lupus Erythematosus-Specific Quality of Life Questionnaire (SLEQOL-C) and explore the factors influencing HRQOL of people with SLE. Participants were Han Chinese people with SLE. The SLEQOL-C and 36-item Short Form Health Survey (SF-36) were used to estimate the HRQOL. Disease activity was determined using the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) and fatigue using the Functional Assessment of Chronic Illness Therapy-Fatigue (FACIT-F)...
November 22, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29167939/the-properdin-pathway-an-alternative-activation-pathway-or-a-critical-amplification-loop-for-c3-and-c5-activation
#8
REVIEW
Richard A Harrison
This review is not intended to cover in detail all aspects of the discovery and evolution of our understanding of the "alternative pathway" of complement activation, there are many excellent reviews that do this (see Fearon (CRC Crit Rev Immunol 1:1-32, 1979), Pangburn and Müller-Eberhard (Springer Semin Immunopathol 7:163-192, 1984)), but instead to give sufficient background for current concepts to be put in context. The prevailing textbook view, of components having a primary role as an alternative "pathway" for C3 activation, is challenged, with an argument developed for the primary role of the system being that of providing a surface-dependent amplification loop for both C3 and C5 activation...
November 22, 2017: Seminars in Immunopathology
https://www.readbyqxmd.com/read/29167800/cross-species-complementation-of-bacterial-and-eukaryotic-type-cardiolipin-synthases
#9
Petra Gottier, Mauro Serricchio, Rita Vitale, Angela Corcelli, Peter Bütikofer
The glycerophospholipid cardiolipin is a unique constituent of bacterial and mitochondrial membranes. It is involved in forming and stabilizing high molecular mass membrane protein complexes and in maintaining membrane architecture. Absence of cardiolipin leads to reduced efficiency of the electron transport chain, decreased membrane potential, and, ultimately, impaired respiratory metabolism. For the protozoan parasite Trypanosoma brucei cardiolipin synthesis is essential for survival, indicating that the enzymes involved in cardiolipin production represent potential drug targets...
November 3, 2017: Microbial Cell
https://www.readbyqxmd.com/read/29167795/managing-rheumatoid-arthritis-with-dietary-interventions
#10
REVIEW
Shweta Khanna, Kumar Sagar Jaiswal, Bhawna Gupta
Self-help by means of dietary interventions can help in management of various disorders including rheumatoid arthritis (RA), a debilitating autoimmune disease. Dietary interventions necessitate a widespread appeal for both patients as well as clinicians due to factors including affordability, accessibility, and presence of scientific evidences that demonstrate substantial benefits in reducing disease symptoms such as pain, joint stiffness, swelling, tenderness and associated disability with disease progression...
2017: Frontiers in Nutrition
https://www.readbyqxmd.com/read/29167678/a-radish-basic-helix-loop-helix-transcription-factor-rstt8-acts-a-positive-regulator-for-anthocyanin-biosynthesis
#11
Sun-Hyung Lim, Da-Hye Kim, Jae K Kim, Jong-Yeol Lee, Sun-Hwa Ha
The MYB-bHLH-WDR (MBW) complex activates anthocyanin biosynthesis through the transcriptional regulation. RsMYB1 has been identified as a key player in anthocyanin biosynthesis in red radish (Raphanus sativus L.), but its partner bHLH transcription factor (TF) remains to be determined. In this study, we isolated a bHLH TF gene from red radish. Phylogenetic analysis indicated that this gene belongs to the TT8 clade of the IIIF subgroup of bHLH TFs, and we thus designated this gene RsTT8. Subcellular localization analysis showed that RsTT8-sGFP was localized to the nuclei of Arabidopsis thaliana protoplasts harboring the RsTT8-sGFP construct...
2017: Frontiers in Plant Science
https://www.readbyqxmd.com/read/29167450/mafb-is-a-critical-regulator-of-complement-component-c1q
#12
Mai Thi Nhu Tran, Michito Hamada, Hyojung Jeon, Risako Shiraishi, Keigo Asano, Motochika Hattori, Megumi Nakamura, Yuki Imamura, Yuki Tsunakawa, Risa Fujii, Toshiaki Usui, Kaushalya Kulathunga, Christina-Sylvia Andrea, Ryusuke Koshida, Risa Kamei, Yurina Matsunaga, Makoto Kobayashi, Hisashi Oishi, Takashi Kudo, Satoru Takahashi
The transcription factor MafB is expressed by monocytes and macrophages. Efferocytosis (apoptotic cell uptake) by macrophages is important for inhibiting the development of autoimmune diseases, and is greatly reduced in Mafb-deficient macrophages. Here, we show the expression of the first protein in the classical complement pathway C1q is important for mediating efferocytosis and is reduced in Mafb-deficient macrophages. The efferocytosis defect in Mafb-deficient macrophages can be rescued by adding serum from wild-type mice, but not by adding serum from C1q-deficient mice...
November 22, 2017: Nature Communications
https://www.readbyqxmd.com/read/29167370/uncertainty-epistemics-and-active-inference
#13
Thomas Parr, Karl J Friston
Biological systems-like ourselves-are constantly faced with uncertainty. Despite noisy sensory data, and volatile environments, creatures appear to actively maintain their integrity. To account for this remarkable ability to make optimal decisions in the face of a capricious world, we propose a generative model that represents the beliefs an agent might possess about their own uncertainty. By simulating a noisy and volatile environment, we demonstrate how uncertainty influences optimal epistemic (visual) foraging...
November 2017: Journal of the Royal Society, Interface
https://www.readbyqxmd.com/read/29167320/mapks-influence-pollen-tube-growth-by-controlling-the-formation-of-phosphatidylinositol-4-5-bisphosphate-in-an-apical-plasma-membrane-domain
#14
Franziska Hempel, Irene Stenzel, Mareike Heilmann, Praveen Krishnamoorthy, Wilhelm Menzel, Ralph Golbik, Stefan Helm, Dirk Dobritzsch, Sacha Baginsky, Justin Lee, Wolfgang Hoehenwarter, Ingo Heilmann
An apical plasma membrane domain enriched in the regulatory phospholipid phosphatidylinositol 4,5-bisphosphate (PtdIns(4,5)P2) is critical for polar tip growth of pollen tubes. How the biosynthesis of PtdIns(4,5)P2 by phosphatidylinositol 4-phosphate 5-kinases (PI4P 5-kinases) is controlled by upstream signaling is currently unknown. The pollen-expressed PI4P 5-kinase PIP5K6 is required for clathrin-mediated endocytosis and polar tip growth in pollen tubes. Here, we identify PIP5K6 as a target of the pollen-expressed mitogen-activated protein kinase MPK6 and characterize the regulatory effects...
November 22, 2017: Plant Cell
https://www.readbyqxmd.com/read/29167230/absence-of-cd59-in-guinea-pigs-analysis-of-the-cavia-porcellus-genome-suggests-the-evolution-of-a-cd59-pseudogene
#15
Hani Boshra, Wioleta M Zelek, Timothy R Hughes, Santiago Rodriguez de Cordoba, B Paul Morgan
CD59 is a membrane-bound regulatory protein that inhibits the assembly of the terminal membrane attack complex (C5b-9) of complement. From its original discovery in humans almost 30 years ago, CD59 has been characterized in a variety of species, from primates to early vertebrates, such as teleost fish. CD59 is ubiquitous in mammals; however, we have described circumstantial evidence suggesting that guinea pigs (Cavia porcellus) lack CD59, at least on erythrocytes. In this study, we have used a combination of phylogenetic analyses with syntenic alignment of mammalian CD59 genes to identify the only span of genomic DNA in C...
November 22, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29167171/advances-in-our-understanding-of-the-pathogenesis-of-hemolytic-uremic-syndromes
#16
Emily Elizabeth Bowen, Richard John Mark Coward
Hemolytic uremic syndrome (HUS) is major global health care issue as it is the leading cause of acute kidney injury in children. It is a triad of acute kidney injury, microangiopathic hemolytic anemia and thrombocytopenia. It is classified as a glomerular thrombotic microangiopathy. In recent years major advances in our understanding of complement driven inherited rare forms of HUS have been achieved. However, 90% of cases of HUS are associated with a Shiga toxin producing enteric pathogen. The precise pathological mechanisms in this setting are yet to be elucidated...
November 22, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29166869/multimodal-imaging-of-retinal-pigment-epithelial-detachments-in-patients-with-c3-glomerulopathy-case-report-and-review-of-the-literature
#17
Valeria Kheir, Ali Dirani, Matthieu Halfon, Jean-Pierre Venetz, Georges Halabi, Yan Guex-Crosier
BACKGROUND: To describe the optical coherence tomography angiograhy (OCTA) of drusenoid pigment epithelial detachments (PEDs) in a woman affected by Complement 3 (C3) glomerulopathy, which represents a spectrum of glomerular diseases characterized on fluorescent microscopy by C3 accumulation with absent, or scanty, immunoglobulin deposits. It is due to acquired or genetically defective alternative pathway control and is generally associated with drusen-like deposits in Bruch's membrane, as well as choriocapillaris...
November 22, 2017: BMC Ophthalmology
https://www.readbyqxmd.com/read/29166860/msaabcr-operon-is-involved-in-persister-cell-formation-in-staphylococcus-aureus
#18
Gyan S Sahukhal, Shanti Pandey, Mohamed O Elasri
BACKGROUND: Persister cells comprise a phenotypic variant that shows extreme antibiotic tolerance resulting in treatment failures of bacterial infections. While this phenomenon has posed a great threat in public health, mechanisms underlying their formation in Staphylococcus aureus remain largely unknown. Increasing evidences of the presence of persister cells in recalcitrant infections underscores the great urgency to unravel the mechanism by which these cells develop. Previously, we characterized msaABCR operon that plays roles in regulation of virulence, biofilm development and antibiotic resistance...
November 22, 2017: BMC Microbiology
https://www.readbyqxmd.com/read/29166697/-patient-unrelated-risk-factors-for-the-development-and-treatment-course-of-anastomotic-failure-in-colorectal-surgery-results-of-structured-m-m-conferences
#19
Peter Alexander Vogel
Background There are numerous published studies on patient-related risk factors for the development of anastomotic failure. We therefore investigated the influence of patient-unrelated risk factors for the development and course of treatment of anastomotic failure in colorectal surgery. Patient Sample From May 1, 2015, until December, 31, 2016, n = 179 post-colorectal surgery patients were analysed. Overall, n = 14 patients suffered from anastomotic failure. These patients' course of treatment was analysed in a Morbidity and Mortality Conference (M+M conference) structured according to the London Protocol...
November 22, 2017: Zentralblatt Für Chirurgie
https://www.readbyqxmd.com/read/29166655/how-well-do-you-know-your-mutation-complex-effects-of-genetic-background-on-expressivity-complementation-and-ordering-of-allelic-effects
#20
Christopher H Chandler, Sudarshan Chari, Alycia Kowalski, Lin Choi, David Tack, Michael DeNieu, William Pitchers, Anne Sonnenschein, Leslie Marvin, Kristen Hummel, Christian Marier, Andrew Victory, Cody Porter, Anna Mammel, Julie Holms, Gayatri Sivaratnam, Ian Dworkin
For a given gene, different mutations influence organismal phenotypes to varying degrees. However, the expressivity of these variants not only depends on the DNA lesion associated with the mutation, but also on factors including the genetic background and rearing environment. The degree to which these factors influence related alleles, genes, or pathways similarly, and whether similar developmental mechanisms underlie variation in the expressivity of a single allele across conditions and among alleles is poorly understood...
November 22, 2017: PLoS Genetics
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