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https://www.readbyqxmd.com/read/28781846/excessive-homozygosity-identified-by-chromosomal-microarray-at-a-known-gcdh-mutation-locus-correlates-with-brain-mri-abnormalities-in-an-infant-with-glutaric-aciduria
#1
Abdul Ali Peer-Zada, Ali M Al-Asmari
Herein, we report a conceptually novel clinical case highlighting the diagnostic implications of excessive homozygosity and its correlation with brain MRI abnormalities in an infant with GA1. The case also points a need for an extra amount of caution to be exercised when evaluating patients with "negative exomes."
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28773127/ab-initio-investigations-of-magnetic-properties-and-induced-half-metallicity-in-ga1-xmnxp-x-0-03-0-25-0-5-and-0-75-alloys
#2
Amel Laref, Abeer AlMudlej, Slimane Laref, Jun Tao Yang, Yong-Chen Xiong, Shi Jun Luo
Ab-initio calculations are performed to examine the electronic structures and magnetic properties of spin-polarized Ga1-xMnxP (x = 0.03, 0.25, 0.5, and 0.75) ternary alloys. In order to perceive viable half-metallic (HM) states and unprecedented diluted magnetic semiconductors (DMSs) such as spintronic materials, the full potential linearized augmented plane wave method is utilized within the generalized gradient approximation (GGA). In order to tackle the correlation effects on 3d states of Mn atoms, we also employ the Hubbard U (GGA + U) technique to compute the magnetic properties of an Mn-doped GaP compound...
July 7, 2017: Materials
https://www.readbyqxmd.com/read/28759200/control-of-elemental-distribution-in-the-nanoscale-solid-state-reaction-that-produces-ga1-xznx-n1-xox-nanocrystals
#3
Pornthip Tongying, Ying-Gang Lu, Leah M G Hall, Kyureon Lee, Marta Sulima, Jim Ciston, Gordana Dukovic
Solid-state chemical transformations at the nanoscale can be a powerful tool for achieving compositional complexity in nanomaterials. It is desirable to understand the mechanisms of such reactions and characterize the local-level composition of the resulting materials. Here, we examine how reaction temperature controls the elemental distribution in (Ga1-xZnx)(N1-xOx) nanocrystals (NCs) synthesized via the solid-state nitridation of a mixture of nanoscale ZnO and ZnGa2O4 NCs. (Ga1-xZnx)(N1-xOx) is a visible-light absorbing semiconductor that is of interest for applications in solar photochemistry...
July 31, 2017: ACS Nano
https://www.readbyqxmd.com/read/28750086/gene-expression-and-metabolite-profiling-of-gibberellin-biosynthesis-during-induction-of-somatic-embryogenesis-in-medicago-truncatula-gaertn
#4
Rafał Igielski, Ewa Kępczyńska
Gibberellins (GAs) are involved in the regulation of numerous developmental processes in plants including zygotic embryogenesis, but their biosynthesis and role during somatic embryogenesis (SE) is mostly unknown. In this study we show that during three week- long induction phase, when cells of leaf explants from non-embryogenic genotype (M9) and embryogenic variant (M9-10a) were forming the callus, all the bioactive gibberellins from non-13-hydroxylation (GA4, GA7) and 13-hydroxylation (GA1, GA5, GA3, GA6) pathways were present, but the contents of only a few of them differed between the tested lines...
2017: PloS One
https://www.readbyqxmd.com/read/28716493/chemical-screening-and-development-of-novel-gibberellin-mimics
#5
Kai Jiang, Hiroaki Shimotakahara, Ming Luo, Masato Otani, Hidemitsu Nakamura, Said Salama Moselhy, Khalid Omer Abualnaja, Abdulrahman Labeed Al-Malki, Taha Abduallah Kumosani, Nobutaka Kitahata, Takeshi Nakano, Masatoshi Nakajima, Tadao Asami
Gibberellin (GA) plays versatile roles in the regulation of plant growth and development and therefore is widely used as a regulator in agriculture. We performed a chemical library screening and identified a chemical, named 67D, as a stimulator of seed germination that was suppressed by paclobutrazol (PAC), a GA biosynthesis inhibitor. In vitro binding assays indicated that 67D binds to the GID1 receptor. Further studies on the structure-activity relationship identified a chemical, named chemical 6, that strongly promoted seed germination suppressed by PAC...
July 4, 2017: Bioorganic & Medicinal Chemistry Letters
https://www.readbyqxmd.com/read/28713338/investigation-of-och1-in-the-virulence-of-candida-parapsilosis-using-a-new-neonatal-mouse-model
#6
Katalin Csonka, Máté Vadovics, Annamária Marton, Csaba Vágvölgyi, Erik Zajta, Adél Tóth, Renáta Tóth, Csaba Vizler, László Tiszlavicz, Héctor M Mora-Montes, Attila Gácser
Candida parapsilosis is an opportunistic human fungal pathogen that poses a serious threat to low birth weight neonates, particularly at intensive care units. In premature infants, the distinct immune responses to Candida infections are not well understood. Although several in vivo models exist to study systemic candidiasis, only a few are available to investigate dissemination in newborns. In addition, the majority of related studies apply intraperitoneal infection rather than intravenous inoculation of murine infants that may be less efficient when studying systemic invasion...
2017: Frontiers in Microbiology
https://www.readbyqxmd.com/read/28699143/glutaric-aciduria-type-1-and-acute-renal-failure-case-report-and-suggested-pathomechanisms
#7
Marcel du Moulin, Bastian Thies, Martin Blohm, Jun Oh, Markus J Kemper, René Santer, Chris Mühlhausen
Glutaric aciduria type 1 (GA1) is caused by deficiency of the mitochondrial matrix enzyme glutaryl-CoA dehydrogenase (GCDH), leading to accumulation of glutaric acid (GA) and 3-hydroxyglutaric acid (3OHGA) in tissues and body fluids. During catabolic crises, GA1 patients are prone to the development of striatal necrosis and a subsequent irreversible movement disorder during a time window of vulnerability in early infancy. Thus, GA1 had been considered a pure "cerebral organic aciduria" in the past. Single case reports have indicated the occurrence of acute renal dysfunction in children affected by GA1...
July 12, 2017: JIMD Reports
https://www.readbyqxmd.com/read/28687735/effect-of-carrier-confinement-on-effective-mass-of-excitons-and-estimation-of-ultralow-disorder-in-al-x-ga1-x-as-gaas-quantum-wells-by-magneto-photoluminescence
#8
S Haldar, V K Dixit, Geetanjali Vashisht, Shailesh Kumar Khamari, S Porwal, T K Sharma, S M Oak
Effect of charge carrier confinement and ultra-low disorder acquainted in AlGaAs/GaAs multi-quantum well system is investigated via Magneto-photoluminescence spectroscopy. Significant increase of effective mass is observed for the confined exciton in narrow QWs. The foremost reason behind such an observation is due to the induced non-parabolicity in bands. Moreover, as the thickness of the QW are reduced, confined excitons in QW experience atomic irregularities at the hetero-junctions and their effects are prominent in the photoluminescence linewidth...
July 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28674408/anisotropically-biaxial-strain-in-non-polar-112-0-plane-in-x-ga1-x-n-gan-layers-investigated-by-x-ray-reciprocal-space-mapping
#9
Guijuan Zhao, Huijie Li, Lianshan Wang, Yulin Meng, Zesheng Ji, Fangzheng Li, Hongyuan Wei, Shaoyan Yang, Zhanguo Wang
In this study, the indium composition x as well as the anisotropically biaxial strain in non-polar a-plane In x Ga1-x N on GaN is studied by X-ray diffraction (XRD) analysis. In accordance with XRD reciprocal lattice space mapping, with increasing indium composition, the maximum of the In x Ga1-x N reciprocal lattice points progressively shifts from a fully compressive strained to a fully relaxed position, then to reversed tensile strained. To fully understand the strain in the ternary alloy layers, it is helpful to grow high-quality device structures using a-plane nitrides...
July 3, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28646846/molecular-epidemiology-of-human-respiratory-syncytial-virus-in-iranian-%C3%A2-60-years-old-hospitalized-patients-with-acute-respiratory-symptoms
#10
Yaser Arjeyni, Ebrahim Faghihloo, Katayoun Samimi Rad, Vahid Salimi, Mahmood Mahmoudi, Talat Mokhtari-Azad
BACKGROUND: Human respiratory syncytial virus (HRSV) is an important cause of acute respiratory infection (ARI) and mortality in ≥60 years old population around the world. The aim of this study was to determine the frequency and circulating genotypes of HRSV in Iranian patients ≥ 60 years old. METHODS: In this cross-sectional molecular epidemiology study, we examined the C-terminal located hypervariable domain of G glycoprotein of HRSV in throat swabs from Tehran, Hormozgan, Boushehr, West Azarbayjan, Ghom and Alborz provinces of Iran which were addressed to national influenza center between October 2013 and March 2015...
June 2017: Archives of Iranian Medicine
https://www.readbyqxmd.com/read/28438223/extrastriatal-changes-in-patients-with-late-onset-glutaric-aciduria-type-i-highlight-the-risk-of-long-term-neurotoxicity
#11
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
#12
Déborah L N Poubel, Júlio César F Almeida, Ana P Dias Ribeiro, Guilherme B Maia, Jesús Maurício G Martinez, Fernanda Cristina P 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-hours rewetting period, respectively; GB1 and GB2: 24-hours dehydration and a 15-minutes or 24-hours rewetting period, respectively; and GC: 1-hour (GC1) or 24-hours (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
#13
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
#14
Julieta Fina, Romina Casadevall, Hamada AbdElgawad, Els Prinsen, Marios N Markakis, Gerrit T S Beemster, Paula Casati
Ultraviolet-B (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 inhibit maize (Zea mays) leaf growth without causing any other visible stress symptoms, including the 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)...
June 2017: Plant Physiology
https://www.readbyqxmd.com/read/28326241/modeling-of-the-growth-of-gaas-algaas-core-shell-nanowires
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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