bluuebunny/crossref_metadata_2025_split huggingface dataset:
Photo and Thermo‐luminescence Properties of Mn4+, Ce4+ and Sm3+ in MgAl2Si2O8 Phosphor
Authors: Öztürk Esra , Kalaycioglu Nilgun Ozpozan , Uzun Erdem 
Published: December 2015

AbstractMn4+, Ce4+ and Sm3+ doped MgAl2Si2O8‐based phosphors were synthesized at 1300 °C by solid state reaction and characterized by thermogravimetry (TG), differential thermal analysis (DTA), X‐ray powder diffraction (XRD), photoluminescence (PL), thermoluminescence (TL) and scanning electron microscopy (SEM). The phosphors showed broad red emission bands in the range of 610–715 nm and different maximum intensity when activated by UV illumination. Such a red emission can be attributed to the intrinsic 2E→4A2 transitions of Mn4+.
Efficient Synthesis of 3,3‐diaryloxindoles Catalyzed by L‐prolinate Anion Immobilized onto Amberlite as a Novel Heterogeneous Organocatalyst
Authors: Keshavarz Mosadegh , Iravani Nasir , Ahmady Amanollah Zarei , Vafaei‐Nezhad Masoumeh 
Published: December 2015

AbstractL‐prolinate anion immobilized onto amberlite IRA900OH ([Amb]L‐Prolinate) is reported as a new, reusable and cheap organocatalyst for the condensation of indoles with isatins to afford the corresponding 3,3′‐diaryloxindol derivatives in ethanol under reflux condition. [Amb]L‐Prolinate was achieved by the treatment of a MeOH/H2O solution of L‐proline with amberlite IRA900OH at 60 °C. The procedure for heterogenization of L‐proline organocatalyst is based on non‐covalent ion‐pair immobilization of L‐proline on the surface of anion‐exchange resin amberlite IRA900OH (AmbIRA900OH) as a commercially accessible cationic polymer support. The prepared heterogeneous organocatalyst was well characterized by using of FTIR, TGA, DTG, XRD and elemental analysis techniques. The catalytic activity of the catalyst was also examined in the reaction of indole and isatin. The catalyst has been recovered and its reusability confirmed in 8 runs
Nano‐TiO2 as an Efficient Catalyst for Tandem Knoevenagel–Michael‐Cyclocondensation Reaction of Dimedone with Aromatic Aldehydes and Ammonium Acetate or Aromatic Amines under Solvent‐free Conditions
Authors: Khazaei Ardeshir , Moosavi‐Zare Ahmad Reza , Mohammadi Zahra , Khakyzadeh Vahid , Afsar Javad 
Published: December 2015

AbstractTiO2 nanoparticles in anatase and rutile forms was characterized and studied by several techniques including X‐ray diffraction (XRD), scanning electron microscope (SEM), transmission electron microscopy (TEM) and successfully applied as an efficient and heterogeneous catalyst in the synthesis of 1,8‐dioxo‐decahydroacridines via the one‐pot multi‐component condensation reaction of dimedone with aromatic aldehydes and ammonium acetate or aromatic amines under mild and solvent‐free conditions.
Longitudinal magnetic resonance imaging study shows progressive pyramidal and callosal damage in Friedreich's ataxia
Authors: Rezende Thiago J.R. , Silva Cynthia B. , Yassuda Clarissa L. , Campos Brunno M. , D'Abreu Anelyssa , Cendes Fernando , Lopes‐Cendes Iscia , França Marcondes C. 
Published: December 2015

ABSTRACTIntroductionSpinal cord and peripheral nerves are classically known to be damaged in Friedreich's ataxia, but the extent of cerebral involvement in the disease and its progression over time are not yet characterized. The aim of this study was to evaluate longitudinally cerebral damage in Friedreich's ataxiaMethodsWe enrolled 31 patients and 40 controls, which were evaluated at baseline and after 1 and 2 years. To assess gray matter, we employed voxel‐based morphometry and cortical thickness measurements. White matter was evaluated using diffusion tensor imaging. Statistical analyses were both cross‐sectional and longitudinal (corrected for multiple comparisons).ResultsGroup comparison between patients and controls revealed widespread macrostructural differences at baseline: gray matter atrophy in the dentate nuclei, brainstem, and precentral gyri; and white matter atrophy in the cerebellum and superior cerebellar peduncles, brainstem, and periventricular areas. We did not identify any longitudinal volumetric change over time. There were extensive microstructural alterations, including superior cerebellar peduncles, corpus callosum, and pyramidal tracts. Longitudinal analyses identified progressive microstructural abnormalities at the corpus callosum, pyramidal tracts, and superior cerebellar peduncles after 1 year of follow‐up.ConclusionPatients with Friedreich's ataxia present more widespread gray and white matter damage than previously reported, including not only infratentorial areas, but also supratentorial structures. Furthermore, patients with Friedreich's ataxia have progressive microstructural abnormalities amenable to detection in a short‐term follow‐up. © 2015 International Parkinson and Movement Disorder Society
The clinical phenotype of early‐onset isolated dystonia caused by recessive COL6A3 mutations (DYT27)
Authors: Jochim Angela , Zech Michael , Gora‐Stahlberg Gina , Winkelmann Juliane , Haslinger Bernhard 
Published: December 2015

ABSTRACTBackground and PurposeWe recently identified mutations in the a3 (VI) collagen gene COL6A3 that cause autosomal‐recessive isolated dystonia (DYT27). This article gives a detailed description of the clinical phenotype associated with this new type of dystonia.MethodsA total of 5 recessive COL6A3 mutation carriers underwent clinical examinations, and case histories were recorded on videotape.ResultsBiallelic COL6A3 mutations cause isolated dystonia with interindividual heterogeneity of distribution and severity. Dystonia was generalized in 3 patients, pronounced in the cranio‐cervical region, upper limbs, and trunk; segmental in 1 patient, with the neck and upper limbs affected; and focal with cervical involvement in another patient. Symptoms began in childhood, adolescence, or early adulthood, initially affecting the neck as cervical dystonia or the hand as writer's cramp.ConclusionCOL6A3‐associated dystonia represents a newly identified autosomal‐recessive entity characterized clinically by an early symptom onset with variable distribution. © 2015 Movement Disorder Society
GATA5 mutation homozygosity linked to a double outlet right ventricle phenotype in a Lebanese patient
Authors: Kassab Kameel , Hariri Hadla , Gharibeh Lara , Fahed Akl C. , Zein Manal , El‐Rassy Inaam , Nemer Mona , El‐Rassi Issam , Bitar Fadi , Nemer Georges 
Published: December 2015

AbstractBackgroundGATA transcription factors are evolutionary conserved zinc finger proteins with multiple roles in cell differentiation/proliferation and organogenesis. GATA5 is only transiently expressed in the embryonic heart, and the inactivation of both Gata5 alleles results in a partially penetrant bicuspid aortic valve (BAV) phenotype in mice. We hypothesized that only biallelic mutations in GATA5 could be disease causing.MethodsA total of 185 patients with different forms of congenital heart disease (CHD) were screened along 150 healthy individuals for GATA4, 5, and 6. All patients' phenotypes were diagnosed with echocardiography.ResultsSequencing results revealed eight missense variants (three of which are novel) in cases with various conotruncal and septal defects. Out of these, two were inherited in recessive forms: the p.T67P variant, which was found both in patients and in healthy individuals, and the previously described p.Y142H variant which was only found in a patient with a double outlet right ventricle (DORV). We characterized the p.Y142H variant and showed that it significantly reduced the transcriptional activity of the protein over cardiac promoters by 30–40%.ConclusionOur results do prove that p.Y142H is associated with DORV and suggests including GATA5 as a potential gene to be screened in patients with this phenotype.
Trans‐11 vaccenic acid improves insulin secretion in models of type 2 diabetes in vivo and in vitro
Authors: Wang Xiaofeng , Gupta Joel , Kerslake Matthew , Rayat Gina , Proctor Spencer D. , Chan Catherine B. 
Published: December 2015

ScopeTrans‐11 vaccenic acid (VA) is a fatty acid produced by ruminants entering the human food supply through meat and dairy products, which appears not to have the health risks associated with industrially produced trans‐fatty acids. In this study, we investigated the effect of VA on insulin secretion in vivo in rats and in vitro in human and rat islets after diabetogenic insult.Methods and resultsHyperglycemic clamp showed that VA dietary supplementation for 8 weeks significantly increased glucose turnover in rats with type 2 diabetes (T2D), accompanied by an elevated plasma C‐peptide concentration, indicating improved insulin secretion. The β‐cell area and proliferation rate were higher in T2D+VA than T2D group. Isolated islets from T2D+VA rats had higher glucose‐stimulated insulin secretion (GSIS) than T2D group. In vitro, VA treatment for 24 and 48 h significantly enhanced GSIS in rat and human islets after diabetogenic challenges. The mRNA expression of G‐protein‐coupled receptor 40 (GPR40) and regenerating islet‐derived 1α (REG‐1α) were consistently increased by VA in both rat and human islets.ConclusionThese results indicate that VA may improve insulin secretion and growth of islets in T2D, at least partly by altering GPR40 and REG‐1α mRNA expression.
Folate deficiency and over‐supplementation causes impaired folate metabolism: Regulation and adaptation mechanisms in Caenorhabditis elegans
Authors: Ortbauer Martina , Ripper Doris , Fuhrmann Thomas , Lassi Maximilian , Auernigg‐Haselmaier Sandra , Stiegler Christina , König Jürgen 
Published: December 2015

ScopeImpaired folate metabolism increases the risk of birth defects, neurodegenerative and cardiovascular disease, osteoporosis and cancer. We used Caenorhabditis elegans to investigate impaired folate metabolism by RNA interference of key enzymes in the methionine synthase (MS) and thymidylate synthase (TS) cycle and by folate deficiency and over‐supplementation feeding studies.Methods and resultsFolate status is influenced by genetic variations (polymorphisms), folate deficiency and supplementation. Single RNAi of dihydrofolate reductase (DHFR), methylenetetrahydrofolate reductase (MTHFR) and MS revealed that gene regulation is largely affected in both folate cycles. Adaptation requires a close transcriptional connection between TS and MS cycle. Coupled DHFR and MS expression is required to balance both cycles, but seems to reduce the overall rate of folate conversion. Feeding studies showed that folate over‐supplementation to functioning metabolism inactivates MS and MTHFR expression and enhances TS activity, which favors DNA synthesis over methylation reactions. Folate deficiency disrupted homeostasis by favoring TS cycle and led to malformation in C. elegans offspring. Embryos show aneuploidy and are nonviable lacking DNA repair during meiotic stage of diakinesis.ConclusionSingle gene silencing alters gene expression in both cycles and disrupts folate homeostasis. Folate over‐supplementation and deficiency favors TS over MS cycle and causes prophase DNA damage.
Confronting a Trinity of Institutional Barriers: Denial, Cover‐Up, and Secrecy
Authors: Barker Holly M. 
Published: December 2015

AbstractDiscourses of government and the nuclear industry erase previous fear and knowledge about the damages and injuries from nuclear weapons that impact indigenous communities. This cover‐up enables nuclear states and nuclear industries to promote nuclear energy as a viable response to climate change. Many people, including environmentalists, consider nuclear energy to be the only viable energy source for the future global economy in that it does not contribute to climate change. It is difficult for us to consider the full scope of the risks that accompany decisions to embrace nuclear energy as a means to reduce greenhouse gas emissions, however, because lessons learned about radiation remain secret or silenced.
Testing theZimbardoTimePerspectiveInventory in theChinese context
Authors: Wang Ya , Chen Xing‐jie , Cui Ji‐fang , Liu Lu‐lu 
Published: December 2015

AbstractIn this study, the authors evaluated theChinese version of theZimbardoTimePerspectiveInventory (ZTPI). TheZTPIwas tested among a sample of 303 university students. A subsample of 51 participants was then asked to complete theZTPIagain along with another set of questionnaires. The five‐factor model of a 20‐item short version of theZTPIshowed good model fit, internal consistency, and test‐retest reliability. The 20‐itemChinese version of theZTPIalso provided good validity, showing correlations with other variables in expected directions.Past‐Positive was positively correlated with reappraisal and negatively correlated with suppression emotion regulation strategies, andPresent‐Hedonistic was positively correlated with reappraisal emotion regulation strategies. These findings indicate that theZTPIis a reliable and valid instrument for measuring time perspective in theChinese setting.
Grapiprant: an EP4 prostaglandin receptor antagonist and novel therapy for pain and inflammation
Authors: Kirkby Shaw Kristin , Rausch‐Derra Lesley C. , Rhodes Linda 
Published: December 2015

AbstractThere are five active prostanoid metabolites of arachidonic acid (AA) that have widespread and varied physiologic functions throughout the body, including regulation of gastrointestinal mucosal blood flow, renal haemodynamics and primary haemostasis. Each prostanoid has at least one distinct receptor that mediates its action. Prostaglandin E2 (PGE2) is a prostanoid that serves important homeostatic functions, yet is also responsible for regulating pain and inflammation. PGE2 binds to four receptors, of which one, the EP4 receptor, is primarily responsible for the pain and inflammation associated with osteoarthritis (OA). The deleterious and pathologic actions of PGE2 are inhibited in varying degrees by steroids, aspirin and cyclo‐oxygenase inhibiting NSAIDs; however, administration of these drugs causes decreased production of PGE2, thereby decreasing or eliminating the homeostatic functions of the molecule. By inhibiting just the EP4 receptor, the homeostatic function of PGE2 is better maintained. This manuscript will introduce a new class of pharmaceuticals known as the piprant class. Piprants are prostaglandin receptor antagonists (PRA). This article will include basic physiology of AA, prostanoids and piprants, will review available evidence for the relevance of EP4 PRAs in rodent models of pain and inflammation, and will reference available data for an EP4 PRA in dogs and cats. Piprants are currently in development for veterinary patients and the purpose of this manuscript is to introduce veterinarians to the class of drugs, with emphasis on an EP4 PRA and its potential role in the control of pain and inflammation associated with OA in dogs and cats.
Biosynthesis of glycosaminoglycans: associated disorders and biochemical tests
Authors: Sasarman Florin , Maftei Catalina , Campeau Philippe M. , Brunel‐Guitton Catherine , Mitchell Grant A. , Allard Pierre 
Published: December 2015

AbstractGlycosaminoglycans (GAG) are long, unbranched heteropolymers with repeating disaccharide units that make up the carbohydrate moiety of proteoglycans. Six distinct classes of GAGs are recognized. Their synthesis follows one of three biosynthetic pathways, depending on the type of oligosaccharide linker they contain. Chondroitin sulfate, dermatan sulfate, heparan sulfate, and heparin sulfate contain a common tetrasaccharide linker that is O‐linked to specific serine residues in core proteins. Keratan sulfate can contain three different linkers, either N‐linked to asparagine or O‐linked to serine/threonine residues in core proteins. Finally, hyaluronic acid does not contain a linker and is not covalently attached to a core protein. Most inborn errors of GAG biosynthesis are reported in small numbers of patients. To date, in 20 diseases, convincing evidence for pathogenicity has been presented for mutations in a total of 16 genes encoding glycosyltransferases, sulfotransferases, epimerases or transporters. GAG synthesis defects should be suspected in patients with a combination of characteristic clinical features in more than one connective tissue compartment: bone and cartilage (short long bones with or without scoliosis), ligaments (joint laxity/dislocations), and subepithelial (skin, sclerae). Some produce distinct clinical syndromes. The commonest laboratory tests used for this group of diseases are analysis of GAGs, enzyme assays, and molecular testing. In principle, GAG analysis has potential as a general first‐line diagnostic test for GAG biosynthesis disorders.
Impact of age at onset and newborn screening on outcome in organic acidurias
Authors: Heringer Jana , Valayannopoulos Vassili , Lund Allan M. , Wijburg Frits A. , Freisinger Peter , Barić Ivo , Baumgartner Matthias R. , Burgard Peter , Burlina Alberto B. , Chapman Kimberly A. , i Saladelafont Elisenda Cortès , Karall Daniela , Mühlhausen Chris , Riches Victoria , Schiff Manuel , Sykut‐Cegielska Jolanta , Walter John H. , Zeman Jiri , Chabrol Brigitte , Kölker Stefan ,   additional individual contributors of the E‐IMD consortium
Published: December 2015

AbstractBackground and aimTo describe current diagnostic and therapeutic strategies in organic acidurias (OADs) and to evaluate their impact on the disease course allowing harmonisation.MethodsDatasets of 567 OAD patients from the E‐IMD registry were analysed. The sample includes patients with methylmalonic (MMA, n = 164), propionic (PA, n = 144) and isovaleric aciduria (IVA, n = 83), and glutaric aciduria type 1 (GA1, n = 176). Statistical analysis included description and recursive partitioning of diagnostic and therapeutic strategies, and odds ratios (OR) for health outcome parameters. For some analyses, symptomatic patients were divided into those presenting with first symptoms during (i.e. early onset, EO) or after the newborn period (i.e. late onset, LO).ResultsPatients identified by newborn screening (NBS) had a significantly lower median age of diagnosis (8 days) compared to the LO group (363 days, p < 0.001], but not compared to the EO group. Of all OAD patients 71 % remained asymptomatic until day 8. Patients with cobalamin‐nonresponsive MMA (MMA‐Cbl−) and GA1 identified by NBS were less likely to have movement disorders than those diagnosed by selective screening (MMA‐Cbl−: 10 % versus 39 %, p = 0.002; GA1: 26 % versus 73 %, p < 0.001). For other OADs, the clinical benefit of NBS was less clear. Reported age‐adjusted intake of natural protein and calories was significantly higher in LO patients than in EO patients reflecting different disease severities. Variable drug combinations, ranging from 12 in MMA‐Cbl− to two in isovaleric aciduria, were used for maintenance treatment. The effects of specific metabolic treatment strategies on the health outcomes remain unclear because of the strong influences of age at onset (EO versus LO), diagnostic mode (NBS versus selective screening), and the various treatment combinations used.ConclusionsNBS is an effective intervention to reduce time until diagnosis especially for LO patients and to prevent irreversible cerebral damage in GA1 and MMA‐Cbl−. Huge diversity of therapeutic interventions hampers our understanding of optimal treatment.
Conserved Curvature of RNA Polymerase I Core Promoter Beyond rRNA Genes: The Case of the Tritryps
Authors: Smircich Pablo , Duhagon María Ana , Garat Beatriz 
Published: December 2015

Abstract
In trypanosomatids, the RNA polymerase I (RNAPI)-dependent promoters controlling the ribosomal RNA (rRNA) genes have been well identified. Although the RNAPI transcription machinery recognizes the DNA conformation instead of the DNA sequence of promoters, no conformational study has been reported for these promoters. Here we present the in silico analysis of the intrinsic DNA curvature of the rRNA gene core promoters in Trypanosoma brucei, Trypanosoma cruzi, and Leishmania major. We found that, in spite of the absence of sequence conservation, these promoters hold conformational properties similar to other eukaryotic rRNA promoters. Our results also indicated that the intrinsic DNA curvature pattern is conserved within the Leishmania genus and also among strains of T. cruzi and T. brucei. Furthermore, we analyzed the impact of point mutations on the intrinsic curvature and their impact on the promoter activity. Furthermore, we found that the core promoters of protein-coding genes transcribed by RNAPI in T. brucei show the same conserved conformational characteristics. Overall, our results indicate that DNA intrinsic curvature of the rRNA gene core promoters is conserved in these ancient eukaryotes and such conserved curvature might be a requirement of RNAPI machinery for transcription of not only rRNA genes but also protein-coding genes.
Clostridium difficile Infections in Veterans Health Administration Long-Term Care Facilities
Authors: Reeves Jeffrey S. , Evans Martin E. , Simbartl Loretta A. , Kralovic Stephen M. , Kelly Allison A. , Jain Rajiv , Roselle Gary A. 
Published: December 2015

OBJECTIVEA nationwide initiative was implemented in February 2014 to decrease Clostridium difficile infections (CDI) in Veterans Affairs (VA) long-term care facilities. We report a baseline of national CDI data collected during the 2 years before the Initiative.METHODSPersonnel at each of 122 reporting sites entered monthly retrospective CDI case data from February 2012 through January 2014 into a national database using case definitions similar to those used in the National Healthcare Safety Network Multidrug-Resistant Organism/CDI module. The data were evaluated using Poisson regression models to examine infection occurrences over time while accounting for admission prevalence and type of diagnostic test.RESULTSDuring the 24-month analysis period, there were 100,800 admissions, 6,976,121 resident days, and 1,558 CDI cases. The pooled CDI admission prevalence rate (including recurrent cases) was 0.38 per 100 admissions, and the pooled nonduplicate/nonrecurrent community-onset rate was 0.17 per 100 admissions. The pooled long-term care facility–onset rate and the clinically confirmed (ie, diarrhea or evidence of pseudomembranous colitis) long-term care facility–onset rate were 1.98 and 1.78 per 10,000 resident days, respectively. Accounting for diagnostic test type, the long-term care facility–onset rate declined significantly (P=.05), but the clinically confirmed long-term care facility–onset rate did not.CONCLUSIONSVA long-term care facility CDI rates were comparable to those in recent reports from other long-term care facilities. The significant decline in the long-term care facility-onset rate but not in the clinically confirmed long-term care facility–onset rate may have been due to less testing of asymptomatic patients. Efforts to decrease CDI rates in long-term care facilities are necessary as part of a coordinated approach to decrease healthcare-associated infections.Infect. Control Hosp. Epidemiol. 2016;37(3):295–300