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https://www.readbyqxmd.com/read/29351582/sco2-mutations-cause-early-onset-axonal-charcot-marie-tooth-disease-associated-with-cellular-copper-deficiency
#1
Adriana P Rebelo, Dimah Saade, Claudia P Pereira, Amjad Farooq, Tyler C Huff, Lisa Abreu, Carlos T Moraes, Diana Mnatsakanova, Kathy Mathews, Hua Yang, Eric A Schon, Stephan Zuchner, Michael E Shy
Recessive mutations in the mitochondrial copper-binding protein SCO2, cytochrome c oxidase (COX) assembly protein, have been reported in several cases with fatal infantile cardioencephalomyopathy with COX deficiency. Significantly expanding the known phenotypic spectrum, we identified compound heterozygous variants in SCO2 in two unrelated patients with axonal polyneuropathy, also known as Charcot-Marie-Tooth disease type 4. Different from previously described cases, our patients developed predominantly motor neuropathy, they survived infancy, and they have not yet developed the cardiomyopathy that causes death in early infancy in reported patients...
January 16, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29341623/is-subsurface-oxygen-necessary-for-the-electrochemical-reduction-of-co2-on-copper
#2
Alejandro Garza, Alexis T Bell, Martin Head-Gordon
It has recently been proposed that subsurface oxygen is crucial for the adsorption and subsequent electroreduction of CO2 on copper. Using density functional theory, we have studied the stability and diffusion of subsurface oxygen in single crystals of copper exposing (111) and (100) facets. Oxygen is at least 1.5 eV more stable on the surface than beneath it for both crystal orientations; interstitial sites are too small to accommodate oxygen. The rate of atomic oxygen diffusion from one layer below a Cu(111) surface to the surface is 5 × 103 s-1...
January 17, 2018: Journal of Physical Chemistry Letters
https://www.readbyqxmd.com/read/29335500/evidence-of-a-cubic-iron-sub-lattice-in-t-cufe2o4-demonstrated-by-x-ray-absorption-fine-structure
#3
Francesco Caddeo, Danilo Loche, Maria F Casula, Anna Corrias
Copper ferrite, belonging to the wide and technologically relevant class of spinel ferrites, was grown in the form of t-CuFe2O4 nanocrystals within a porous matrix of silica in the form of either an aerogel or a xerogel, and compared to a bulk sample. Extended X-ray absorption fine structure (EXAFS) spectroscopy revealed the presence of two different sub-lattices within the crystal structure of t-CuFe2O4, one tetragonal and one cubic, defined by the Cu2+ and Fe3+ ions respectively. Our investigation provides evidence that the Jahn-Teller distortion, which occurs on the Cu2+ ions located in octahedral sites, does not affect the coordination geometry of the Fe3+ ions, regardless of their location in octahedral or tetrahedral sites...
January 15, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29333859/chromatographic-separation-and-visual-detection-on-wicking-microfluidic-devices-quantitation-of-cu2-in-surface-ground-and-drinking-water
#4
Gayan C Bandara, Christopher Heist, Vincent T Remcho
Copper is widely applied in industrial and technological applications, and is an essential micro nutrient for humans and animals. However, exposure to high environmental levels of copper, especially through drinking water, can lead to copper toxicity, resulting in severe acute and chronic health effects. Therefore, regular monitoring of aqueous copper ions has become necessary as recent anthropogenic activities have led to elevated environmental concentrations of copper. On-site monitoring processes require an inexpensive, simple, and portable analytical approach capable of generating reliable qualitative and quantitative data efficiently...
January 15, 2018: Analytical Chemistry
https://www.readbyqxmd.com/read/29332954/determination-of-the-boltzmann-constant-with-cylindrical-acoustic-gas-thermometry-new-and-previous-results-combined
#5
X J Feng, J T Zhang, H Lin, K A Gillis, J B Mehl, M R Moldover, K Zhang, Y N Duan
We report a new determination of the Boltzmann constant kB using a cylindrical acoustic gas thermometer. We determined the length of the copper cavity from measurements of its microwave resonance frequencies. This contrasts with our previous work (Zhang et al 2011 Int. J. Thermophys.32 1297, Lin et al 2013 Metrologia50 417, Feng et al 2015 Metrologia52 S343) that determined the length of a different cavity using two-color optical interferometry. In this new study, the half-widths of the acoustic resonances are closer to their theoretical values than in our previous work...
October 2017: Metrologia
https://www.readbyqxmd.com/read/29331769/source-identification-of-uranium-containing-materials-at-mine-legacy-sites-in-portugal
#6
A C Keatley, P G Martin, K R Hallam, O D Payton, R Awbery, F Carvalho, J M Oliveira, L Silva, M Malta, T B Scott
Whilst prior nuclear forensic studies have focused on identifying signatures to distinguish between different uranium deposit types, this paper focuses on providing a scientific basis for source identification of materials from different uranium mine sites within a single region, which can then be potentially used within nuclear forensics. A number of different tools, including gamma spectrometry, alpha spectrometry, mineralogy and major and minor elemental analysis, have been utilised to determine the provenance of uranium mineral samples collected at eight mine sites, located within three different uranium provinces, in Portugal...
January 10, 2018: Journal of Environmental Radioactivity
https://www.readbyqxmd.com/read/29330900/overexpression-of-microrna408-enhances-photosynthesis-growth-and-seed-yield-in-diverse-plants
#7
Jiawei Pan, Dahui Huang, Zhonglong Guo, Zheng Kuang, He Zhang, Xinyu Xie, Zengfeng Ma, Shaopei Gao, Manuel T Lerdau, Chengcai Chu, Lei Li
The ability of a plant to produce grain, fruit, or forage depends ultimately on photosynthesis. There have been few attempts, however, to study microRNAs, which are a class of endogenous small RNAs post-transcriptionally programming gene expression, in relation to photosynthetic traits. We focused on miR408, one of the most conserved plant miRNAs, and overexpressed it in parallel in Arabidopsis, tobacco, and rice. The transgenic plants all exhibited increased copper content in the chloroplast, elevated abundance of plastocyanin, and an induction of photosynthetic genes...
January 13, 2018: Journal of Integrative Plant Biology
https://www.readbyqxmd.com/read/29329008/selective-formation-of-copper-nanoparticles-from-acid-mine-drainage-using-nanoscale-zerovalent-iron-particles
#8
R A Crane, D J Sapsford
Nanoscale zerovalent iron (nZVI) has been investigated for the selective formation of Cu nanoparticles from acid mine drainage (AMD) taken from a legacy mine site in the UK. Batch experiments were conducted containing unbuffered (pH 2.67 at t = 0) and pH buffered (pH < 3.1) AMD which were exposed to nZVI at 0.1-2.0 g/L. Results demonstrate that nZVI is selective for Cu, Cd and Al removal (>99.9% removal of all metals within 1 h when nZVI ≥ 1.0 g/L) from unbuffered AMD despite the coexistent of numerous other metals in the AMD, namely: Na, Ca, Mg, K, Mn and Zn...
December 18, 2017: Journal of Hazardous Materials
https://www.readbyqxmd.com/read/29325617/wilson-disease-and-related-copper-disorders
#9
Matthew T Lorincz
Copper is a required cofactor for enzymes in critical metabolic pathways. Mutations in copper metabolism genes or abnormalities in copper metabolism result in disease from copper excess or deficiency. Wilson disease (WD) is an autosomal-recessive disease caused by mutations in the ATP7B gene which encodes a copper-transporting ATPase. Over 500 different WD mutations throughout the ATP7B gene have been described, most of which are missense mutations. Mutations in both ATP7B alleles result in abnormal copper metabolism and subsequent toxic accumulation of copper...
2018: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/29316098/palladium-copper-dual-catalysis-for-cross-coupling-of-aryl-trialkyl-silanes-with-aryl-bromides
#10
Yasunori Minami, Takeshi Komiyama, Yuki Furuya, Tamejiro Hiyama
Although aryl(trialkyl)silanes are apparently ideal organometalic reagents for the cross-coupling reaction due to robustness, low toxicity, solubility, and easy accessibility, they are generally an inert cross-coupling nucleophile. Disclosed herein is that a palladium/copper catalytic system achieves the desired cross-coupling of aryl-SiMe3, -SiEt3, -SiMe2(t-Bu), and -Si(i-Pr)3 with aryl bromides, allowing application to the sequential C-H and C-Si bond arylation of thiophenes and the synthesis of poly(thiophene-fluorene)s...
January 5, 2018: Angewandte Chemie
https://www.readbyqxmd.com/read/29305420/in-vitro-reconstitution-functional-dissection-and-mutational-analysis-of-metal-ion-transport-by-mitoferrin-1
#11
Eric T Christenson, Austin S Gallegos, Anirban Banerjee
Iron is universally important to cellular metabolism and mitoferrins-1 and -2 have been proposed to be the iron importers of mitochondria, the cell's assembly plant of heme and iron-sulfur clusters. These iron-containing prosthetic groups are critical for a host of physiological processes ranging from oxygen transport and energy consumption to maintaining protein structural integrity. Mitoferrin-1 (Mfrn1) belongs to the mitochondrial carrier (MC) family and is atypical given its putative metallic cargo; most MCs transport nucleotides, amino acids, or other small to medium-size metabolites...
January 5, 2018: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29298484/looking-down-under-for-a-circular-economy-of-indium
#12
Tim T Werner, Luca Ciacci, Gavin Mark Mudd, Barbara K Reck, Stephen Alan Northey
Indium is a specialty metal crucial for modern technology, yet potentially critical due to its by-product status in mining. Measures to reduce its criticality typically focus on improving its recycling efficiency at end-of-life. This study quantifies primary and secondary indium resources ("stocks") for Australia through a dynamic material flow analysis. It is based on detailed assessments of indium mineral resources hosted in lead-zinc and copper deposits, respective mining activities from 1844-2013, and the trade of indium-containing products from 1988-2015...
January 4, 2018: Environmental Science & Technology
https://www.readbyqxmd.com/read/29289216/nanocalorimeter-platform-for-in-situ-specific-heat-measurements-and-x-ray-diffraction-at-low-temperature
#13
K Willa, Z Diao, D Campanini, U Welp, R Divan, M Hudl, Z Islam, W-K Kwok, A Rydh
Recent advances in electronics and nanofabrication have enabled membrane-based nanocalorimetry for measurements of the specific heat of microgram-sized samples. We have integrated a nanocalorimeter platform into a 4.5 T split-pair vertical-field magnet to allow for the simultaneous measurement of the specific heat and x-ray scattering in magnetic fields and at temperatures as low as 4 K. This multi-modal approach empowers researchers to directly correlate scattering experiments with insights from thermodynamic properties including structural, electronic, orbital, and magnetic phase transitions...
December 2017: Review of Scientific Instruments
https://www.readbyqxmd.com/read/29286645/oxygen-activation-and-radical-transformations-in-heme-proteins-and-metalloporphyrins
#14
Xiongyi Huang, John T Groves
As a result of the adaptation of life to an aerobic environment, nature has evolved a panoply of metalloproteins for oxidative metabolism and protection against reactive oxygen species. Despite the diverse structures and functions of these proteins, they share common mechanistic grounds. An open-shell transition metal like iron or copper is employed to interact with O2 and its derived intermediates such as hydrogen peroxide to afford a variety of metal-oxygen intermediates. These reactive intermediates, including metal-superoxo, -(hydro)peroxo, and high-valent metal-oxo species, are the basis for the various biological functions of O2-utilizing metalloproteins...
December 29, 2017: Chemical Reviews
https://www.readbyqxmd.com/read/29251804/a-study-of-neurite-orientation-dispersion-and-density-imaging-in-wilson-s-disease
#15
Yu-Kun Song, Xin-Bei Li, Xiao-Long Huang, Jing Zhao, Xiang-Xue Zhou, Yu-Liang Wang, Xu Yan, Jing-Yan Wang, Jian-Ping Chu
BACKGROUND: Previous studies have indicated that neurite orientation dispersion and density imaging (NODDI) could be used as a biomarker for detecting microstructural changes of brain. PURPOSE: To quantitatively evaluate the changes in basal ganglia (BG) and thalamus in Wilson's disease (WD) by NODDI and assess the correlation between parameters and disease severity. STUDY TYPE: Prospective case-control study. POPULATION: In total, 24 WD patients and 25 age- and sex-matched normal controls were involved in this study...
December 18, 2017: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29248947/variation-of-metallothionein-i-and-ii-gene-expression-in-the-bank-vole-clethrionomys-glareolus-under-environmental-zinc-and-cadmium-exposure
#16
Magdalena Mikowska, Barbara Dziublińska, Renata Świergosz-Kowalewska
The main idea of the study was to assess how environmental metal pollution activates defence responses at transcription levels in the tissues of bank voles (Clethrionomys glareolus). For this purpose, the metallothioneine (MT) genes expression (a well known biomarker of exposure and response to various metals) was measured. The real-time PCR method was used for relative quantification of metallothionein I and metallothionein II expressions in the livers, kidneys and testes of bank voles from six populations exposed to different contaminants, mainly zinc, cadmium and iron...
December 16, 2017: Archives of Environmental Contamination and Toxicology
https://www.readbyqxmd.com/read/29247867/physicochemical-antioxidant-dna-cleaving-properties-and-antimicrobial-activity-of-fisetin-copper-chelates
#17
Elżbieta Łodyga-Chruscińska, Maria Pilo, Antonio Zucca, Eugenio Garribba, Elżbieta Klewicka, Magdalena Rowińska-Żyrek, Marzena Symonowicz, Longin Chrusciński, Vitalij T Cheshchevik
Fisetin (3,3',4',7-tetrahydroxyflavone) metal chelates are of interest as this plant polyphenol has revealed broad prospects for its use as natural medicine in the treatment of various diseases. Metal interactions may change or enhance fisetin biological properties so understanding fisetin metal chelation is important for its application not only in medicine but also as a food additive in nutritional supplements. This work was aimed to determine and characterize copper complexes formed in different pH range at applying various metal/ligand ratios...
December 11, 2017: Journal of Inorganic Biochemistry
https://www.readbyqxmd.com/read/29247484/accurate-ionization-energies-for-mononuclear-copper-complexes-remain-a-challenge-for-density-functional-theory
#18
Christopher J Cramer, Büsra Dereli, Manual Ortuño
Cu is ubiquitous and its one-electron redox chemistry is central to many catalytic processes. Modeling such chemistry requires electronic structure methods capable of the accurate prediction of ionization energies (IEs) for compounds including copper in different oxidation states and supported by various ligands. Herein we estimate IEs for 12 mononuclear Cu species previously reported in the literature using 21 modern density functionals and the DLPNO-CCSD(T) wave function theory model; we consider extrapolated values of the latter to provide reference values of acceptable accuracy...
December 16, 2017: Chemphyschem: a European Journal of Chemical Physics and Physical Chemistry
https://www.readbyqxmd.com/read/29242872/phenylamino-derivatives-of-tris-2-pyridylmethyl-amine-hydrogen-bonded-peroxodicopper-complexes
#19
E W Dahl, H T Dong, N K Szymczak
A series of copper complexes bearing new 6-substituted tris(2-pyridylmethyl)amine ligands (LR) appended with NH(p-R-C6H4) groups (R = H, CF3, OMe) were prepared. These ligands are electronically tunable (ΔE1/2 = 160 mV) and CuI(LR)+ complexes react with oxygen to form hydrogen bonded (trans-1,2-peroxo)dicopper species.
December 15, 2017: Chemical Communications: Chem Comm
https://www.readbyqxmd.com/read/29239430/copper-i-complexes-based-on-ligand-systems-with-two-different-binding-sites-synthesis-structures-and-reaction-with-o2
#20
S T Li, B Braun-Cula, S Hoof, C Limberg
The synthesis of the ligand systems L1 and L2 with two different N3-binding sites linked through a dibenzofuran spacer and their coordination properties towards a variety of CuI precursors are reported. The reaction of L1 with copper halides leads to the formation of a bimetallic species [(L1)(CuICl)2] (1), and metallodimers [((L1)(CuIX)2)2(μ-(Cu)(μ-X)2)] (2: X = Br, 3: X = I) in which two dicopper complexes are bridged by a (μ-(Cu)(μ-X)2)-moiety whereas L2 reacts with copper chloride to afford {[Cu(L2)Cl2]}n (8)...
December 14, 2017: Dalton Transactions: An International Journal of Inorganic Chemistry
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