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https://www.readbyqxmd.com/read/28435998/a-6-year-old-boy-with-wilson-disease-a-diagnostic-dilemma
#1
Ramaswamy Ganesh, N Suresh, T Vasanthi, Malathi Sathiyasekaran, R Thulasiraman
A 6-year-old boy presented with 2 months history of progressive abdominal distension and jaundice. He was deeply icteric with ascites, hepatosplenomegaly, hyperbilirubinemia, raised transaminases, and coagulopathy. Viral markers and slit lamp examination for Kayser-Fleischer ring were negative. Serum ceruloplasmin and 24-h urinary copper post-D-pencillamine challenge were normal. Anti-smooth muscle antibody was positive 1:20, and liver biopsy showed micronodular cirrhosis with abundant Mallory hyaline and stainable copper deposits...
April 24, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28430106/atomic-motions-in-the-layered-copper-pseudochalcogenide-cuncn-indicative-of-a-quantum-spin-liquid-scenario
#2
Andrei L Tchougreeff, Ralf Stoffel, Andreas Houben, Philipp Jacobs, Richard Dronskowski, Matej Pregelj, Andrej Zorko, Denis Arcon, Oksana Zaharko
We explore the thermodynamic properties of the layered copper(II) carbodiimide CuNCN by heat-capacity measurements and investigate the corresponding thermal atomic motions by means of neutron powder diffraction as well as inelastic neutron scattering. The experiments are complemented by a combination of density-functional calculations, phonon analysis and analytic theory. The existence of a soft flexural mode-bending of the layers, characteristic for the material structure-is established in the phonon spectrum of CuNCN by giving characteristic temperature-dependent contributions to the heat capacity and atomic displacement parameters...
April 21, 2017: Journal of Physics. Condensed Matter: An Institute of Physics Journal
https://www.readbyqxmd.com/read/28425513/regioselective-copper-catalyzed-direct-arylation-of-benzodithiophene-s-s-tetraoxide
#3
D P Khambhati, K A N Sachinthani, A L Rheingold, T L Nelson
An efficient copper-catalyzed direct arylation reaction for the regioselective functionalization of benzodithiophene-S,S-tetraoxide has been developed. The method demonstrates a broad scope with isolated yields ranging from good to excellent. Furthermore, the reaction specificity for aryl iodides over the unreactive aryl bromides provide a opportunity to generate a new donor-acceptor-donor triad.
April 20, 2017: Chemical Communications: Chem Comm
https://www.readbyqxmd.com/read/28421430/imbalance-of-morphofunctional-responses-of-jurkat-t-lymphoblasts-at-short-term-culturing-with-relief-zinc-or-copper-containing-calcium-phosphate-coating-on-titanium
#4
L S Litvinova, V V Shupletsova, N A Dunets, O G Khaziakhmatova, K A Yurova, M Yu Khlusova, G B Slepchenko, E G Cherempey, Yu P Sharkeev, E G Komarova, M B Sedelnikova, I A Khlusov
Morphofunctional response of Jurkat T cells that were cultured for 24 h on substrates prepared from commercially pure titanium with relief microarc bilateral calcium phosphate coating containing copper or zinc was studied. Changes in the concentration of essential trace elements contained in this coating can cause significant imbalance of molecular processes of differentiation, secretion, apoptosis, and necrosis and reduce tumor cell survival.
January 2017: Doklady. Biochemistry and Biophysics
https://www.readbyqxmd.com/read/28419456/time-lapse-lab-based-x-ray-nano-ct-study-of-corrosion-damage
#5
R S Bradley, Y Liu, T L Burnett, X Zhou, S B Lyon, P J Withers, A Gholinia, T Hashimoto, D Graham, S R Gibbon, B Hornberger
An experimental protocol (workflow) has been developed for time-lapse x-ray nanotomography (nano-CT) imaging of environmentally driven morphological changes to materials. Two case studies are presented. First, the leaching of nanoparticle corrosion inhibitor pigment from a polymer coating was followed over 14 days, while in the second case the corrosion damage to an AA2099 aluminium alloy was imaged over 12 hours. The protocol includes several novel aspects relevant to nano-CT with the use of a combination of x-ray absorption and phase contrast data to provide enhanced morphological and composition information, and hence reveal the best information to provide new insights into the changes of different phases over time...
April 17, 2017: Journal of Microscopy
https://www.readbyqxmd.com/read/28415304/measurement-of-the-equation-of-state-of-solid-density-copper-heated-with-laser-accelerated-protons
#6
S Feldman, G Dyer, D Kuk, T Ditmire
We present equation of state (EOS) measurements of solid-density copper heated to 5-10 eV. A copper sample was heated isochorically by hydrogen ions accelerated from an adjacent foil by a high intensity pulsed laser, and probed optically. The measured temperature and expansion are compared against simulations using the most up-to-date wide range EOS tables available.
March 2017: Physical Review. E
https://www.readbyqxmd.com/read/28411891/recombinant-expression-of-thermobifida-fusca-e7-lpmo-in-pichia-pastoris-and-escherichia-coli-and-their-functional-characterization
#7
Kelly B Rodrigues, Jéssica K A Macêdo, Tallyta Teixeira, Jéssica S Barros, Ana C B Araújo, Fernanda P Santos, Betânia F Quirino, Bruno S A F Brasil, Thaís F C Salum, Patrícia V Abdelnur, Léia C L Fávaro
The discovery of lytic polysaccharides monooxygenases copper dependent (LPMOs) revolutionized the classical concept that the cleavage of cellulose is a hydrolytic process in recent years. These enzymes carry out oxidative cleavage of cellulose (and other polysaccharides), acting synergistically with cellulases and other hydrolases. In fact, LPMOs have the potential for increasing the efficiency of the lignocellulosic biomass conversion in biofuels and high value chemicals. Among a small number of microbial LPMOs that have been characterized, some LPMOs were expressed and characterized biochemically from the bacteria Thermobifida fusca, using the host Escherichia coli...
April 9, 2017: Carbohydrate Research
https://www.readbyqxmd.com/read/28411822/label-free-and-sensitive-assay-for-deoxyribonuclease-i-activity-based-on-enzymatically-polymerized-superlong-poly-thymine-hosted-fluorescent-copper-nanoparticles
#8
Lan Luo, Fengzhou Xu, Hui Shi, Xiaoxiao He, Taiping Qing, Yanli Lei, Jinlu Tang, Dinggeng He, Kemin Wang
Deoxyribonuclease I (DNase I) is an important physiological indicator and diagnostic biomarker, but traditional methods for assessing its activity are time-consuming, laborious, and usually radioactive. Herein, by effectively combining the special functions of DNase I and terminal deoxynucleotidyl transferase (TdT), a simple, green, cost-effective, label-free and ultrasensitive assay for DNase I activity has been constructed based on superlong poly(thymine)-hosted copper nanoparticles (poly T-CuNPs). In this strategy, a 3'-phosphorylated DNA primer is designed to block TdT polymerization...
July 1, 2017: Talanta
https://www.readbyqxmd.com/read/28409271/unique-presentation-of-cutis-laxa-with-leigh-like-syndrome-due-to-echs1-deficiency
#9
S Balasubramaniam, L G Riley, D Bratkovic, D Ketteridge, N Manton, M J Cowley, V Gayevskiy, T Roscioli, M Mohamed, T Gardeitchik, E Morava, J Christodoulou
Clinical finding of cutis laxa, characterized by wrinkled, redundant, sagging, nonelastic skin, is of growing significance due to its occurrence in several different inborn errors of metabolism (IEM). Metabolic cutis laxa results from Menkes syndrome, caused by a defect in the ATPase copper transporting alpha (ATP7A) gene; congenital disorders of glycosylation due to mutations in subunit 7 of the component of oligomeric Golgi (COG7)-congenital disorders of glycosylation (CDG) complex; combined disorder of N- and O-linked glycosylation, due to mutations in ATPase H+ transporting V0 subunit a2 (ATP6VOA2) gene; pyrroline-5-carboxylate reductase 1 deficiency; pyrroline-5-carboxylate synthase deficiency; macrocephaly, alopecia, cutis laxa, and scoliosis (MACS) syndrome, due to Ras and Rab interactor 2 (RIN2) mutations; transaldolase deficiency caused by mutations in the transaldolase 1 (TALDO1) gene; Gerodermia osteodysplastica due to mutations in the golgin, RAB6-interacting (GORAB or SCYL1BP1) gene; and mitogen-activated pathway (MAP) kinase defects, caused by mutations in several genes [protein tyrosine phosphatase, non-receptor-type 11 (PTPN11), RAF, NF, HRas proto-oncogene, GTPase (HRAS), B-Raf proto-oncogene, serine/threonine kinase (BRAF), MEK1/2, KRAS proto-oncogene, GTPase (KRAS), SOS Ras/Rho guanine nucleotide exchange factor 2 (SOS2), leucine rich repeat scaffold protein (SHOC2), NRAS proto-oncogene, GTPase (NRAS), and Raf-1 proto-oncogene, serine/threonine kinase (RAF1)], which regulate the Ras-MAPK cascade...
April 13, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/28409194/heavy-metals-in-the-blue-crab-callinectes-sapidus-in-mersin-bay-turkey
#10
H Y Çoğun, Ö Firat, T Aytekin, G Firidin, Ö Firat, H Varkal, Ö Temiz, F Kargin
The concentrations of four metals (copper, cadmium, zinc and lead) were quantified in blue crabs (Callinectes sapidus) tissues collected in January, April, June and September at two stations in Mersin Bay, northeastern Mediterranean Sea, using ICP-MS. The metal concentrations in crabs from the two stations differed significantly. The hepatopancreas was the major organ accumulating metals, followed by gill and muscle tissues. Except for lead, the highest concentrations of metals were measured in the hepatopancreas...
April 13, 2017: Bulletin of Environmental Contamination and Toxicology
https://www.readbyqxmd.com/read/28402308/phyto-dewatering-of-sewage-sludge-using-panicum-repens-l
#11
A S El-Gendy, H I El-Kassas, T M A Razek, H Abdel-Latif
Experiments in the field environment have been conducted to study the growth of Panicum repens L., an aquatic plant, in the sewage sludge matrix. The experiments were also carried out to investigate the ability of this plant to dewater sewage sludge to increase the capacity of conventional drying beds. In addition, the ability of Panicum repens L. to reduce the sludge contents of certain elements (copper (Cu), Iron (Fe), Sodium (Na), lead (Pb), and Zinc (Zn)) was also investigated. All experiments were carried out in batch reactors...
April 2017: Water Science and Technology: a Journal of the International Association on Water Pollution Research
https://www.readbyqxmd.com/read/28399452/design-synthesis-and-evaluation-of-curcumin-derivatives-as-nrf2-activators-and-cytoprotectors-against-oxidative-death
#12
Zhi-Shan Tu, Qi Wang, Dan-Dan Sun, Fang Dai, Bo Zhou
Activation of nuclear factor erythroid-2-related factor 2 (Nrf2) has been proven to be an effective means to prevent the development of cancer, and natural curcumin stands out as a potent Nrf2 activator and cancer chemopreventive agent. In this study, we synthesized a series of curcumin analogs by introducing the geminal dimethyl substituents on the active methylene group to find more potent Nrf2 activators and cytoprotectors against oxidative death. The geminally dimethylated and catechol-type curcumin analog (compound 3) was identified as a promising lead molecule in terms of its increased stability and cytoprotective activity against the tert-butyl hydroperoxide (t-BHP)-induced death of HepG2 cells...
April 5, 2017: European Journal of Medicinal Chemistry
https://www.readbyqxmd.com/read/28394586/dicopper-cu-i-cu-i-and-cu-i-cu-ii-complexes-in-copper-catalyzed-azide-alkyne-cycloaddition
#13
Micah S Ziegler, K V Lakshmi, T Don Tilley
A discrete, dicopper μ-alkynyl complex, [Cu2(μ-η(1):η(1)-C≡C(C6H4)CH3)DPFN]NTf2 (DPFN = 2,7-bis(fluoro-di(2-pyridyl)methyl)-1,8-naphthyridine; NTf2(-) = N(SO2CF3)2(-)), reacts with p-tolylazide to yield a dicopper complex with a symmetrically bridging 1,2,3-triazolide, [Cu2(μ-η(1):η(1)-(1,4-bis(4-tolyl)-1,2,3-triazolide))DPFN]NTf2. This transformation exhibits bimolecular reaction kinetics and represents a key step in a proposed, bimetallic mechanism for copper-catalyzed azide-alkyne cycloaddition (CuAAC)...
April 10, 2017: Journal of the American Chemical Society
https://www.readbyqxmd.com/read/28394445/3d-map-distribution-of-metallic-nanoparticles-in-whole-cells-using-mev-ion-microscopy
#14
M S Vasco, L C Alves, V Corregidor, A Bettiol, F Watt, T Pinheiro
In this work, a new tool was developed, the MORIA program that readily translates Rutherford backscattering spectrometry (RBS) output data into visual information, creating a display of the distribution of elements in a true three-dimensional (3D) environment. The program methodology is illustrated with the analysis of yeast Saccharomyces cerevisiae cells, exposed to copper oxide nanoparticles (CuO-NP) and HeLa cells in the presence of gold nanoparticles (Au-NP), using different beam species, energies and nuclear microscopy systems...
April 10, 2017: Journal of Microscopy
https://www.readbyqxmd.com/read/28392828/screening-of-wilson-s-disease-in-a-psychiatric-population-difficulties-and-pitfalls-a-preliminary-study
#15
Caroline Demily, François Parant, David Cheillan, Emmanuel Broussolle, Alice Pavec, Olivier Guillaud, Lioara Restier, Alain Lachaux, Muriel Bost
BACKGROUND: Wilson's disease (WD) is a rare autosomal-recessive, inherited disorder caused by a mutation in the copper-transporting gene ATP7B affecting the liver and nervous system. About 30% of patients with WD may initially present with psychiatric symptoms, and diagnosis can be difficult to establish. The objectives of the present preliminary study were [1] to evaluate the relevance of serum copper (Cu) and ceruloplasmin (Cp) measures in hospitalized patients with psychiatric disorders; and [2] to identify possible mutations in the ATP7B gene in patients with abnormal biological copper profile...
2017: Annals of General Psychiatry
https://www.readbyqxmd.com/read/28390091/inhibition-of-tyrosinase-by-4h-chromene-analogues-synthesis-kinetic-studies-and-computational-analysis
#16
Edikarlos M Brasil, Luciana M Canavieira, Érica T C Cardoso, Edilene O Silva, Jerônimo Lameira, José L M Nascimento, Vera L Eifler-Lima, Barbarella de Matos Macchi, Dharmarajan Sriram, Paul V Bernhardt, José Rogério Araújo Silva, Craig M Williams, Cláudio N Alves
Inhibition of mushroom tyrosinase was observed with synthetic dihydropyrano[3,2-b]chromenediones. Among them, DHPC04 displayed the most potent tyrosinase inhibitory activity with a Ki value of 4μM, comparable to the reference standard inhibitor kojic acid. A kinetic study suggested that these synthetic heterocyclic compounds behave as competitive inhibitors for the L-DOPA binding site of the enzyme. Furthermore, molecular modeling provided important insight into the mechanism of binding interactions with the tyrosinase copper active site...
April 8, 2017: Chemical Biology & Drug Design
https://www.readbyqxmd.com/read/28387405/supramolecular-chemistry-of-pillar-n-arenes-functionalised-by-a-copper-i-catalysed-alkyne-azide-cycloaddition-click-reaction
#17
T Kakuta, T Yamagishi, T Ogoshi
Since we discovered pillar[n]arenes in 2008, many chemists have developed a strong interest in pillar[n]arene chemistry because of the many advantages associated with these materials, including their facile and high yielding synthesis, versatile functionality, planar chirality and unique host-guest properties. In this feature article, we discuss recent advances in the field of supramolecular chemistry based on the use of pillar[n]arenes as substrates for copper(i)-catalysed alkyne-azide cycloaddition (CuAAC) "click" chemistry...
April 7, 2017: Chemical Communications: Chem Comm
https://www.readbyqxmd.com/read/28386624/mutated-pet117-causes-complex-iv-deficiency-and-is-associated-with-neurodevelopmental-regression-and-medulla-oblongata-lesions
#18
G H Renkema, G Visser, F Baertling, L T Wintjes, V M Wolters, J van Montfrans, G A P de Kort, P G J Nikkels, P M van Hasselt, S N van der Crabben, R J T Rodenburg
The genetic basis of the many progressive, multi systemic, mitochondrial diseases that cause a lack of cellular ATP production is heterogeneous, with defects found both in the mitochondrial genome as well as in the nuclear genome. Many different mutations have been found in the genes encoding subunits of the enzyme complexes of the oxidative phosphorylation system. In addition, mutations in genes encoding proteins involved in the assembly of these complexes are known to cause mitochondrial disorders. Here we describe two sisters with a mitochondrial disease characterized by lesions in the medulla oblongata, as demonstrated by brain magnetic resonance imaging, and an isolated complex IV deficiency and reduced levels of individual complex IV subunits...
April 6, 2017: Human Genetics
https://www.readbyqxmd.com/read/28381075/thermal-analysis-of-copper-titanium-multiwall-carbon-nanotube-composites
#19
Smail Hamamda, Ahmed Jari, S Revo, K Ivanenko, Youcef Jari, T Avramenko
The aim of this research is the thermostructural study of Cu-Ti, Cu-Ti 1 vol% multiwall carbon nanotubes (MWCNTs) and Cu-Ti 3 vol% MWCNTs. Several investigation techniques were used to achieve this objective. Dilatometric data show that the coefficient of thermal expansion of the nanocomposite containing less multiwall carbon nanotubes is linear and small. The same nanocomposite exhibits regular heat transfer and weak mass exchange with the environment. Raman spectroscopy shows that the nanocomposite with more MWCNTs contains more defects...
December 2017: Nanoscale Research Letters
https://www.readbyqxmd.com/read/28379978/integrated-control-of-wood-destroying-basidiomycetes-combining-cu-based-wood-preservatives-and-trichoderma-spp
#20
Javier Ribera, Siegfried Fink, Maria Del Carmen Bas, Francis W M R Schwarze
The production of new generation of wood preservatives (without addition of a co-biocide) in combination with an exchange of wood poles on identical sites with high fungal inoculum, has resulted in an increase of premature failures of wood utility poles in the last decades. Wood destroying basidiomycetes inhabiting sites where poles have been installed, have developed resistance against wood preservatives. The objective of the in vitro studies was to identify a Trichoderma spp. with a highly antagonistic potential against wood destroying basidiomycetes that is capable of colonizing Cu-rich environments...
2017: PloS One
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