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https://www.readbyqxmd.com/read/28438223/extrastriatal-changes-in-patients-with-late-onset-glutaric-aciduria-type-i-highlight-the-risk-of-long-term-neurotoxicity
#1
Nikolas Boy, Jana Heringer, Renate Brackmann, Olaf Bodamer, Angelika Seitz, Stefan Kölker, Inga Harting
BACKGROUND: Without neonatal initiation of treatment, 80-90% of patients with glutaric aciduria type 1 (GA1) develop striatal injury during the first six years of life resulting in a complex, predominantly dystonic movement disorder. Onset of motor symptoms may be acute following encephalopathic crisis or insidious without apparent crisis. Additionally, so-called late-onset GA1 has been described in single patients diagnosed after the age of 6 years. With the aim of better characterizing and understanding late-onset GA1 we analyzed clinical findings, biochemical phenotype, and MRI changes of eight late-onset patients and compared these to eight control patients over the age of 6 years with early diagnosis and start of treatment...
April 24, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28419716/effect-of-dehydration-and-rehydration-intervals-on-fracture-resistance-of-reattached-tooth-fragments-using-a-multimode-adhesive
#2
Déborah Lousan do Nascimento Poubel, Júlio César Franco Almeida, Ana Paula Dias Ribeiro, Guilherme Brasil Maia, Jesús Maurício Gonzalez Martinez, Fernanda Cristina Pimentel Garcia
BACKGROUND/AIMS: The tooth fragment bonding technique, frequently used to restore traumatized teeth, may be affected by dehydration/rehydration periods. The aim of this study was to evaluate the effects of different dry and wet storage intervals on multimode adhesive bonding between reattached fragments and teeth. MATERIALS AND METHODS: Eighty-four bovine incisors were fractured and randomized into groups (n=12). After teeth fracturing, each specimen was assigned to one of the following groups: G0: control group (sound tooth); GA1 and GA2: 1-h dehydration and a 15-min or 24-h rewetting period, respectively; GB1 and GB2: 24-h dehydration and a 15-min or 24-h rewetting period, respectively; and GC: 1-h (GC1) or 24-h (GC2) dehydration period only...
April 17, 2017: Dental Traumatology: Official Publication of International Association for Dental Traumatology
https://www.readbyqxmd.com/read/28414749/sequence-variability-of-the-respiratory-syncytial-virus-rsv-fusion-gene-among-contemporary-and-historical-genotypes-of-rsv-a-and-rsv-b
#3
Anne M Hause, David M Henke, Vasanthi Avadhanula, Chad A Shaw, Lorena I Tapia, Pedro A Piedra
BACKGROUND: The fusion (F) protein of RSV is the major vaccine target. This protein undergoes a conformational change from pre-fusion to post-fusion. Both conformations share antigenic sites II and IV. Pre-fusion F has unique antigenic sites p27, ø, α2α3β3β4, and MPE8; whereas, post-fusion F has unique antigenic site I. Our objective was to determine the antigenic variability for RSV/A and RSV/B isolates from contemporary and historical genotypes compared to a historical RSV/A strain...
2017: PloS One
https://www.readbyqxmd.com/read/28400494/uv-b-inhibits-leaf-growth-through-changes-in-growth-regulating-factors-and-gibberellin-levels
#4
Julieta Fina, Romina Casadevall, Hamada AbdElgawad, Els Prinsen, Marios Nektarios Markakis, Gerrit T S Beemster, Paula Casati
UV-B radiation affects leaf growth in a wide range of species. In this work, we demonstrate that UV-B levels present in solar radiation inhibits maize leaf growth without causing any other visible stress symptoms, including accumulation of DNA damage. We conducted kinematic analyses of cell division and expansion to understand the impact of UV-B radiation on these cellular processes. Our results demonstrate that the decrease in leaf growth in UV-B irradiated leaves is a consequence of a reduction in cell production, and a shortened growth zone (GZ)...
April 11, 2017: Plant Physiology
https://www.readbyqxmd.com/read/28326241/modeling-of-the-growth-of-gaas-algaas-core-shell-nanowires
#5
Qian Zhang, Peter W Voorhees, Stephen H Davis
Heterostructured GaAs-AlGaAs core-shell nanowires with have attracted much attention because of their significant advantages and great potential for creating high performance nanophotonics and nanoelectronics. The spontaneous formation of Al-rich stripes along certain crystallographic directions and quantum dots near the apexes of the shell are observed in AlGaAs shells. Controlling the formation of these core-shell heterostructures remains challenging. A two-dimensional model valid on the wire cross section, that accounts for capillarity in the faceted surface limit and deposition has been developed for the evolution of the shell morphology and concentration in Al x Ga1-x As alloys...
2017: Beilstein Journal of Nanotechnology
https://www.readbyqxmd.com/read/28281424/a-review-of-patients-with-glutaric-aciduria-type-1-at-inkosi-albert-luthuli-central-hospital-durban-south-africa
#6
R Govender, A Mitha, L Mubaiwa
Glutaric aciduria type 1 (GA1) is an organic acidaemia. The objective of this study was to describe the profile of patients diagnosed with GA1 at Inkosi Albert Luthuli Central Hospital, Durban, South Africa from 2007 to 2015. We identified 6 children (4 girls, 2 boys) in a retrospective review. The mean age at diagnosis was 12 months. Clinical findings on presentation were encephalopathic crises (n=4), hypotonia (n=4) and macrocephaly (n=5). Other complications included seizures (n=4), dystonia (n=3) and bulbar dysfunction (n=4)...
February 27, 2017: South African Medical Journal, Suid-Afrikaanse Tydskrif Vir Geneeskunde
https://www.readbyqxmd.com/read/28263586/unusually-flexible-indium-iii-metal-organic-polyhedra-materials-for-detecting-trace-amounts-of-water-in-organic-solvents-and-high-proton-conductivity
#7
Xi Du, Ruiqing Fan, Liangsheng Qiang, Yang Song, Kai Xing, Wei Chen, Ping Wang, Yulin Yang
Humidity-induced single-crystal transformation was observed in the indium metal-organic polyhedra [In2(TCPB)2]·2H2O (In1), where H3TCPB is 1,3,5-tri(4-carboxyphenoxy)benzene. When the humidity is above 58% relative humidity (RH) at room temperature, the neutral compound In1 could be successfully converted into the positively charged compound In1-H along with the color change from yellow to deep red, which also undergoes a reversible transformation into In1 driven by thermal dehydration. Notably, the color of In1 takes only 5 min to change under 58% RH at room temperature, which is much quicker than common desiccant bluestone...
March 6, 2017: Inorganic Chemistry
https://www.readbyqxmd.com/read/28235370/low-temperature-pld-growth-of-ultrathin-zno-nanowires-by-using-zn-x-al1-x-o-and-zn-x-ga1-x-o-seed-layers
#8
Alexander Shkurmanov, Chris Sturm, Helena Franke, Jörg Lenzner, Marius Grundmann
ZnO nanowires (NWs) are used as building blocks for a wide range of different devices, e.g. light emitters, resonators, and sensors. Integration of the NWs into such structures requires a high level of NWs' diameter control. Here, we present that the doping concentration of Zn x Al1-x O and Zn x Ga1-x O seed layers has a strong impact on the NW growth and allows to tune the diameter of the NWs by two orders of magnitude down to less than 7 nm. These ultrathin NWs exhibit a well-oriented vertical growth and thus are promising for the investigation of quantum effects...
December 2017: Nanoscale Research Letters
https://www.readbyqxmd.com/read/28150798/fermi-level-and-bands-offsets-determination-in-insulating-ga-mn-n-gan-structures
#9
L Janicki, G Kunert, M Sawicki, E Piskorska-Hommel, K Gas, R Jakiela, D Hommel, R Kudrawiec
The Fermi level position in (Ga,Mn)N has been determined from the period-analysis of GaN-related Franz-Keldysh oscillation obtained by contactless electroreflectance in a series of carefully prepared by molecular beam epitaxy GaN/Ga1-xMnxN/GaN(template) bilayers of various Mn concentration x. It is shown that the Fermi level in (Ga,Mn)N is strongly pinned in the middle of the band gap and the thickness of the depletion layer is negligibly small. For x > 0.1% the Fermi level is located about 1.25-1.55 eV above the valence band, that is very close to, but visibly below the Mn-related Mn(2+)/Mn(3+) impurity band...
February 2, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28143689/reversible-brain-atrophy-in-glutaric-aciduria-type-1
#10
Yurika Numata-Uematsu, Osamu Sakamoto, Yosuke Kakisaka, Yukimune Okubo, Yoshitsugu Oikawa, Natsuko Arai-Ichinoi, Shigeo Kure, Mitsugu Uematsu
Glutaric aciduria type 1 (GA1) is a rare metabolic disorder caused by a deficiency of glutaryl-CoA dehydrogenase. The typical clinical onset features an acute encephalopathic crisis developed in early childhood, causing irreversible striatal injury. Recently, tandem mass spectrometry of spots of dried blood has allowed pre-symptomatic detection of GA1 in newborns. Early treatment can prevent irreversible neurological injury. We report the case of a girl with GA1 who exhibited a characteristic reversible change upon brain magnetic resonance imaging (MRI)...
June 2017: Brain & Development
https://www.readbyqxmd.com/read/28096969/dendrosomal-nanocurcumin-and-p53-overexpression-synergistically-trigger-apoptosis-in-glioblastoma-cells
#11
Reihaneh Keshavarz, Babak Bakhshinejad, Sadegh Babashah, Narges Baghi, Majid Sadeghizadeh
OBJECTIVES: Glioblastoma is the most lethal tumor of the central nervous system. Here, we aimed to evaluate the effects of exogenous delivery of p53 and a nanoformulation of curcumin called dendrosomal curcumin (DNC), alone and in combination, on glioblastoma tumor cells. MATERIALS AND METHODS: MTT assay was exploited to measure the viability of U87-MG cells against DNC treatment. Cells were separately subjected to DNC treatment and transfected with p53-containing vector and then were co-exposed to DNC and p53 overexpression[A GA1][B2]...
December 2016: Iranian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28065924/gibberellins-regulate-iron-deficiency-response-by-influencing-iron-transport-and-translocation-in-rice-seedlings-oryza-sativa
#12
Baolan Wang, Haifang Wei, Zhen Xue, Wen-Hao Zhang
Background and aims: Gibberellins (GAs) are a class of plant hormones with diverse functions. However, there has been little information on the role of GAs in response to plant nutrient deficiency. Methods: To evaluate the roles of GAs in regulation of Fe homeostasis, the effects of GA on Fe accumulation and Fe translocation in rice seedlings were investigated using wild-type, a rice mutant ( eui1 ) displaying enhnaced endogenous GA concentrations due to a defect in GA deactivation, and transgenic rice plants overexpressing OsEUI ...
April 1, 2017: Annals of Botany
https://www.readbyqxmd.com/read/28062662/disease-causing-mutations-affecting-surface-residues-of-mitochondrial-glutaryl-coa-dehydrogenase-impair-stability-heteromeric-complex-formation-and-mitochondria-architecture
#13
Jessica Schmiesing, Benjamin Lohmöller, Michaela Schweizer, Henning Tidow, Søren W Gersting, Ania C Muntau, Thomas Braulke, Chris Mühlhausen
The neurometabolic disorder glutaric aciduria type 1 (GA1) is caused by mutations in the GCDH gene encoding the mitochondrial matrix protein glutaryl-CoA dehydrogenase (GCDH), which forms homo- and heteromeric complexes. Twenty percent of all pathogenic mutations affect single amino acid residues on the surface of GCDH resulting in a severe clinical phenotype. We report here on heterologous expression studies of 18 missense mutations identified in GA1 patients affecting surface amino acids. Western blot and pulse chase experiments revealed that the stability of half of the GCDH mutants was significantly reduced...
February 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28057895/gibberellin-signaling-requires-chromatin-remodeler-pickle-to-promote-vegetative-growth-and-phase-transitions
#14
Jeongmoo Park, Dong-Ha Oh, Maheshi Dassanayake, Khoa Thi Nguyen, Joe Ogas, Giltsu Choi, Tai-Ping Sun
PICKLE (PKL) is an ATP-dependent chromodomain-helicase-DNA-binding domain (CHD3) chromatin remodeling enzyme in Arabidopsis (Arabidopsis thaliana). Previous studies showed that PKL promotes embryonic-to-vegetative transition by inhibiting expression of seed-specific genes during seed germination. The pkl mutants display a low penetrance of the "pickle root" phenotype, with a thick and green primary root that retains embryonic characteristics. The penetrance of this pickle root phenotype in pkl is dramatically increased in gibberellin (GA)-deficient conditions...
February 2017: Plant Physiology
https://www.readbyqxmd.com/read/28009157/solar-water-splitting-and-nitrogen-fixation-with-layered-bismuth-oxyhalides
#15
Jie Li, Hao Li, Guangming Zhan, Lizhi Zhang
Hydrogen and ammonia are the chemical molecules that are vital to Earth's energy, environmental, and biological processes. Hydrogen with renewable, carbon-free, and high combustion-enthalpy hallmarks lays the foundation of next-generation energy source, while ammonia furnishes the building blocks of fertilizers and proteins to sustain the lives of plants and organisms. Such merits fascinate worldwide scientists in developing viable strategies to produce hydrogen and ammonia. Currently, at the forefronts of hydrogen and ammonia syntheses are solar water splitting and nitrogen fixation, because they go beyond the high temperature and pressure requirements of methane stream reforming and Haber-Bosch reaction, respectively, as the commercialized hydrogen and ammonia production routes, and inherit the natural photosynthesis virtues that are green and sustainable and operate at room temperature and atmospheric pressure...
December 23, 2016: Accounts of Chemical Research
https://www.readbyqxmd.com/read/27997362/elucidating-the-relationship-between-crystallo-chemistry-and-optical-properties-of-cigs-nanocrystals
#16
Mahshid Ahmadi, Stevin S Pramana, Chris Boothroyd, Yeng Ming Lam
The performance of solar cells fabricated using Cu(In,Ga)(S,Se)2 nanocrystal (NC) inks synthesized using the hot injection method has yielded efficiencies up to 12% recently. The efficiency of these devices is highly dependent on the chemical composition and crystallographic quality of the NCs. The former has been extensively discussed as it can be easily correlated to the optical properties of the film, but detailed crystallographic structure of these NCs has scarcely been discussed and it can influence both the optical and electrical properties...
January 27, 2017: Nanotechnology
https://www.readbyqxmd.com/read/27966463/growth-of-wurtzite-al-x-ga1-x-p-nanowire-shells-and-characterization-by-raman-spectroscopy
#17
Alexander Berg, Magnus Heurlin, Stylianos Tsopanidis, Mats-Erik Pistol, Magnus T Borgström
The phonon energies of AlGaP in wurtzite crystal structure are generally not known, as opposed to their zincblende counterparts, because AlGaP crystallizes in zincblende phase in bulk and thin films structures. However, in nanowires AlGaP can be grown in wurtzite crystal structure. In this work we have grown wurtzite GaP/AlGaP/GaP core-shell nanowires by use of MOVPE. After developing suitable growth conditions, the Al composition was determined by STEM-EDX measurements and the wurtzite AlGaP phonon energies by Raman spectroscopy...
January 20, 2017: Nanotechnology
https://www.readbyqxmd.com/read/27936573/impurity-compensation-effect-induced-by-tin-valence-change-in-%C3%AE-ga1-4sn0-6o3-thin-films
#18
Xiaolong Zhao, Zhenping Wu, Wei Cui, Yusong Zhi, Daoyou Guo, Linghong Li, Weihua Tang
Corundum-structured α-phase Ga1.4Sn0.6O3 thin films have been deposited on m-plane Al2O3(300) substrates using laser molecular beam epitaxy technology. With increasing of the oxygen partial pressure, the crystal lattice of Ga1.4Sn0.6O3 films expands due to tin ions valence changes from Sn(4+) to Sn(2+). The resistivity of the film deposited under 3 × 10(-5) Pa is 3.54 × 10(4) Ω·cm, which decreases by about 2 orders of magnitude than that fabricated under 3 × 10(-1) Pa. The mixture valence of Sn(2+) and Sn(4+) ions leads to the impurity altitude compensation effect...
January 11, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/27878724/production-of-recombinant-polypeptides-containing-one-ga-module-and-analysis-of-their-ability-to-bind-to-human-albumin
#19
E A Bormotova, T V Gupalova
Surface proteins of many bacterial species interact with human serum albumin (HSA) via a special region of amino acid sequence termed GA module. For instance, surface peptostreptococcal albumin-binding protein of anaerobic bacteria Peptostreptococcus magnus contains one HSA-binding GA-module. Protein G from group G and C Streptococcus strains isolated from humans has HSA-binding region consisting of three GA-modules. HSA-binding protein containing two GA-modules was found in strains of group G Streptococcus of animal origin...
November 2016: Bulletin of Experimental Biology and Medicine
https://www.readbyqxmd.com/read/27858203/enhanced-fucosylation-of-ga1-in-the-digestive-tracts-of-x-ray-irradiated-mice
#20
Masao Iwamori, Kyoko Tanaka, Shigeki Adachi, Daisuke Aoki, Taisei Nomura
In mice at 4 days after X-ray-irradiation at 0.5 Gy/min for 16 min, the tissue weights of immune organs, i.e., thymus and spleen, were decreased due to injury to lymphocytes by the X-rays. The resulting immunosuppressive condition allowed the growth of lactobacilli, i.e., L. murinus, which contained LacβTH-DG and possessed the ability to induce transcription of the fucosyltransferase gene for synthesis of FGA1. LacβTH-DG was detected in the jejunal and ileal contents of X-ray-irradiated mice, but not in those of control mice, whereas LacTetH-DG of L...
November 18, 2016: Glycoconjugate Journal
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