Targeting the Hippo Pathway Is a New Potential Therapeutic Modality for Malignant Mesothelioma.

Science.gov (United States)

Sekido, Yoshitaka

2018-03-22

Malignant mesothelioma (MM) constitutes a very aggressive tumor that arises from the pleural or peritoneal cavities and is highly refractory to conventional therapies. Several key genetic alterations are associated with the development and progression of MM including mutations of the CDKN2A/ARF , NF2 , and BAP1 tumor-suppressor genes. Notably, activating oncogene mutations are very rare; thus, it is difficult to develop effective inhibitors to treat MM. The NF2 gene encodes merlin, a protein that regulates multiple cell-signaling cascades including the Hippo pathway. MMs also exhibit inactivation of Hippo pathway components including LATS1/2, strongly suggesting that merlin-Hippo pathway dysregulation plays a key role in the development and progression of MM. Furthermore, Hippo pathway inactivation has been shown to result in constitutive activation of the YAP1/TAZ transcriptional coactivators, thereby conferring malignant phenotypes to mesothelial cells. Critical YAP1/TAZ target genes, including prooncogenic CCDN1 and CTGF , have also been shown to enhance the malignant phenotypes of MM cells. Together, these data indicate the Hippo pathway as a therapeutic target for the treatment of MM, and support the development of new strategies to effectively target the activation status of YAP1/TAZ as a promising therapeutic modality for this formidable disease.

Ilexgenin A exerts anti-inflammation and anti-angiogenesis effects through inhibition of STAT3 and PI3K pathways and exhibits synergistic effects with Sorafenib on hepatoma growth

Energy Technology Data Exchange (ETDEWEB)

Yang, Hao [Medical and Pharmaceutical Institute, Yangzhou University, Yangzhou 225001, Jiangsu (China); College of Medicine, Yangzhou University, Yangzhou 225001, Jiangsu (China); Institute of Pharmacology and Toxicology, Jiangsu Kanion Pharmaceutical Co., Ltd., Lianyungang 222000, Jiangsu (China); Wang, Juan [Medical and Pharmaceutical Institute, Yangzhou University, Yangzhou 225001, Jiangsu (China); College of Medicine, Yangzhou University, Yangzhou 225001, Jiangsu (China); Fan, Jin-hong; Zhang, Ya-qi; Zhao, Jun-xian [Medical and Pharmaceutical Institute, Yangzhou University, Yangzhou 225001, Jiangsu (China); Dai, Xiao-jun [Medical and Pharmaceutical Institute, Yangzhou University, Yangzhou 225001, Jiangsu (China); Chinese Medicine Hospital of Yangzhou City, Yangzhou 225009, Jiangsu (China); Liu, Qi; Shen, Yan-jun [Medical and Pharmaceutical Institute, Yangzhou University, Yangzhou 225001, Jiangsu (China); College of Medicine, Yangzhou University, Yangzhou 225001, Jiangsu (China); Liu, Chang [Medical and Pharmaceutical Institute, Yangzhou University, Yangzhou 225001, Jiangsu (China); Sun, Wei-dong, E-mail: zyykjc@sina.com [Medical and Pharmaceutical Institute, Yangzhou University, Yangzhou 225001, Jiangsu (China); Chinese Medicine Hospital of Yangzhou City, Yangzhou 225009, Jiangsu (China); Sun, Yun, E-mail: ysun@yzu.edu.cn [Medical and Pharmaceutical Institute, Yangzhou University, Yangzhou 225001, Jiangsu (China); College of Medicine, Yangzhou University, Yangzhou 225001, Jiangsu (China)

2017-01-15

Recently, we reported that Ilexgenin A exhibits anti-cancer activities and induces cell arrest. Here, we investigated the effect of Ilexgenin A on the inflammation, angiogenesis and tumor growth of hepatocellular carcinoma (HCC). Our current study revealed that Ilexgenin A significantly inhibited the inflammatory cytokines TNF-α and IL-6 levels and downregulated pro-angiogenic factor VEGF production and transcription in HepG2 cells. The underlying mechanism for Ilexgenin A effects appears to be through inhibiting STAT3 and PI3K pathways. Furthermore, we found that not only Ilexgenin A inhibited STAT3 and PI3K pathways in HepG2 cells but also blocked these signaling pathways in HUVECs. Most importantly, by employing two HCC xenografts models - HepG2 and H22, we showed that Ilexgenin A reduced tumor growth and exhibited synergy effect with Sorafenib. ELISA assay, histological analysis and immunohistochemistry examination revealed that the expression of VEGF and MVD was significantly decreased after the treatment with Ilexgenin A and the combination. Moreover, Ilexgenin A could enhance caspase-3/7 activity in vitro and transmission electron microscope indicated that the combination induced evident apoptosis of tumor cells and caused the structural changes of mitochondria in vivo. Although no apparent adverse effects occurred during the treatment period, Sorafenib monotherapy elicited hepatotoxicity for specific expression in the increased level of AST and the ratio of AST/ALT. However, the combination could remedy this adverse effect. In conclusion, the results described in the present study identifies Ilexgenin A as a promising therapeutic candidate that modulates inflammation, angiogenesis, and HCC growth. - Highlights: • Ilexgenin A exerts anti-inflammatory and anti-angiogenesis effects in hepatoma. • Ilexgenin A may exert these effects through inhibition of STAT3 and PI3K pathways. • Ilexgenin A exhibits synergistic effects with Sorafenib on hepatoma growth

Transcriptomic analyses of tributyltin-induced sexual dimorphisms in rare minnow (Gobiocypris rarus) brains.

Science.gov (United States)

Zhang, Ji-Liang; Liu, Min; Zhang, Chun-Nuan; Li, Er-Chao; Fan, Ming-Zhen; Huang, Mao-Xian

2018-07-30

The brain of fish displays sexual dimorphisms and exhibits remarkable sexual plasticity throughout their life span. Although reproductive toxicity of tributyltin (TBT) in fish is well documented in fish, it remains unknown whether TBT interrupts sexual dimorphisms of fish brains. In this work, brain transcriptomic profiles of rare minnow (Gobiocypris rarus) was characterized and sex-biased genes were identified using RNA sequencing. Functional annotation and enrichment analysis were performed to reveal differences of gene products and pathways between the brains of male and female fish. Furthermore, transcriptomic responses of male and female brains to TBT at 10 ng/L were also investigated to understand effects of TBT on brain sexual dimorphisms. Only 345 male-biased and 273 female-biased genes were found in the brains. However, significant female-biased pathways of circadian rhythm and phototransduction were identified in the brains by enrichment analysis. Interestingly, following TBT exposure in the female fish, the circadian rhythm pathway was significantly disrupted based on enrichment analysis, while in the male fish, the phototransduction pathway was significantly disrupted. In the female fish, expression of genes (Per, Cry, Rev-Erb α, Ror, Dec and CK1δ/ε) in the circadian rhythm pathway was down-regulated after TBT exposure; while in the male fish, expression of genes (Rec, GNAT1_2, GNGT1, Rh/opsin, PDE and Arr) in the phototransduction pathway was up-regulated after TBT exposure. Overall, our results not only provide key data on the molecular basis of brain sexual dimorphisms in fish, but also offer valuable resources for investigating molecular mechanisms by which environmental chemicals might influence brain sexual plasticity. Copyright © 2018 Elsevier Inc. All rights reserved.

The 3-hydroxyacyl-CoA dehydratases HACD1 and HACD2 exhibit functional redundancy and are active in a wide range of fatty acid elongation pathways.

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Sawai, Megumi; Uchida, Yukiko; Ohno, Yusuke; Miyamoto, Masatoshi; Nishioka, Chieko; Itohara, Shigeyoshi; Sassa, Takayuki; Kihara, Akio

2017-09-15

Differences among fatty acids (FAs) in chain length and number of double bonds create lipid diversity. FA elongation proceeds via a four-step reaction cycle, in which the 3-hydroxyacyl-CoA dehydratases (HACDs) HACD1-4 catalyze the third step. However, the contribution of each HACD to 3-hydroxyacyl-CoA dehydratase activity in certain tissues or in different FA elongation pathways remains unclear. HACD1 is specifically expressed in muscles and is a myopathy-causative gene. Here, we generated Hacd1 KO mice and observed that these mice had reduced body and skeletal muscle weights. In skeletal muscle, HACD1 mRNA expression was by far the highest among the HACDs However, we observed only an ∼40% reduction in HACD activity and no changes in membrane lipid composition in Hacd1 -KO skeletal muscle, suggesting that some HACD activities are redundant. Moreover, when expressed in yeast, both HACD1 and HACD2 participated in saturated and monounsaturated FA elongation pathways. Disruption of HACD2 in the haploid human cell line HAP1 significantly reduced FA elongation activities toward both saturated and unsaturated FAs, and HACD1 HACD2 double disruption resulted in a further reduction. Overexpressed HACD3 exhibited weak activity in saturated and monounsaturated FA elongation pathways, and no activity was detected for HACD4. We therefore conclude that HACD1 and HACD2 exhibit redundant activities in a wide range of FA elongation pathways, including those for saturated to polyunsaturated FAs, with HACD2 being the major 3-hydroxyacyl-CoA dehydratase. Our findings are important for furthering the understanding of the molecular mechanisms in FA elongation and diversity. © 2017 by The American Society for Biochemistry and Molecular Biology, Inc.

An Asymptomatic Case of Wolff-Parkinson-White Syndrome with Right-sided Free-wall Accessory Pathway and Left Ventricular Dysfunction

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Takanao Mine, MD

2011-01-01

Full Text Available A 16-year-old girl with a known history of asymptomatic Wolff-Parkinson-White syndrome exhibited signs of left ventricular (LV septal akinesia and LV dysfunction during routine follow-up. A 12-lead surface ECG showed pre-excitation, a predominantly negative delta wave in V1 and left axis deviation, which was consistent with the presence of a right free-wall accessory pathway. Radiofrequency ablation of the anterolateral right atrium around the local shortest atrium-to-ventricle interval created the accessory pathway block. An echocardiogram taken one month after the procedure revealed that LV septal wall motion had normalized and that LV ejection fraction had improved from 50% before the ablation to 64% after the ablation. Most previous reports of asymptomatic patients of WPW with LV septal dyskinesia and dysfunction have described right septal or posteroseptal accessory pathways. This patient reported here represents a rare case with right free-wall accessory pathway and LV dysfunction without tachycardia.

Exome sequencing in schizophrenic patients with high levels of homozygosity identifies novel and extremely rare mutations in the GABA/glutamatergic pathways.

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Edoardo Giacopuzzi

Full Text Available Inbreeding is a known risk factor for recessive Mendelian diseases and previous studies have suggested that it could also play a role in complex disorders, such as psychiatric diseases. Recent inbreeding results in the presence of long runs of homozygosity (ROHs along the genome, which are also defined as autozygosity regions. Genetic variants in these regions have two alleles that are identical by descent, thus increasing the odds of bearing rare recessive deleterious mutations due to a homozygous state. A recent study showed a suggestive enrichment of long ROHs in schizophrenic patients, suggesting that recent inbreeding could play a role in the disease. To better understand the impact of autozygosity on schizophrenia risk, we selected, from a cohort of 180 Italian patients, seven subjects with extremely high numbers of large ROHs that were likely due to recent inbreeding and characterized the mutational landscape within their ROHs using Whole Exome Sequencing and, gene set enrichment analysis. We identified a significant overlap (17%; empirical p-value = 0.0171 between genes inside ROHs affected by low frequency functional homozygous variants (107 genes and the group of most promising candidate genes mutated in schizophrenia. Moreover, in four patients, we identified novel and extremely rare damaging mutations in the genes involved in neurodevelopment (MEGF8 and in GABA/glutamatergic synaptic transmission (GAD1, FMN1, ANO2. These results provide insights into the contribution of rare recessive mutations and inbreeding as risk factors for schizophrenia. ROHs that are likely due to recent inbreeding harbor a combination of predisposing low-frequency variants and extremely rare variants that have a high impact on pivotal biological pathways implicated in the disease. In addition, this study confirms that focusing on patients with high levels of homozygosity could be a useful prioritization strategy for discovering new high-impact mutations in

Exome sequencing in schizophrenic patients with high levels of homozygosity identifies novel and extremely rare mutations in the GABA/glutamatergic pathways.

Science.gov (United States)

Giacopuzzi, Edoardo; Gennarelli, Massimo; Minelli, Alessandra; Gardella, Rita; Valsecchi, Paolo; Traversa, Michele; Bonvicini, Cristian; Vita, Antonio; Sacchetti, Emilio; Magri, Chiara

2017-01-01

Inbreeding is a known risk factor for recessive Mendelian diseases and previous studies have suggested that it could also play a role in complex disorders, such as psychiatric diseases. Recent inbreeding results in the presence of long runs of homozygosity (ROHs) along the genome, which are also defined as autozygosity regions. Genetic variants in these regions have two alleles that are identical by descent, thus increasing the odds of bearing rare recessive deleterious mutations due to a homozygous state. A recent study showed a suggestive enrichment of long ROHs in schizophrenic patients, suggesting that recent inbreeding could play a role in the disease. To better understand the impact of autozygosity on schizophrenia risk, we selected, from a cohort of 180 Italian patients, seven subjects with extremely high numbers of large ROHs that were likely due to recent inbreeding and characterized the mutational landscape within their ROHs using Whole Exome Sequencing and, gene set enrichment analysis. We identified a significant overlap (17%; empirical p-value = 0.0171) between genes inside ROHs affected by low frequency functional homozygous variants (107 genes) and the group of most promising candidate genes mutated in schizophrenia. Moreover, in four patients, we identified novel and extremely rare damaging mutations in the genes involved in neurodevelopment (MEGF8) and in GABA/glutamatergic synaptic transmission (GAD1, FMN1, ANO2). These results provide insights into the contribution of rare recessive mutations and inbreeding as risk factors for schizophrenia. ROHs that are likely due to recent inbreeding harbor a combination of predisposing low-frequency variants and extremely rare variants that have a high impact on pivotal biological pathways implicated in the disease. In addition, this study confirms that focusing on patients with high levels of homozygosity could be a useful prioritization strategy for discovering new high-impact mutations in genetically

Cryptotanshinone exhibits therapeutical effects on cerebral stroke through the PI3K/AKT‑eNOS signaling pathway.

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Zhu, Weixin; Qiu, Weihong; Lu, Ailan

2017-12-01

Cerebral stroke is a kind of acute cerebrovascular disease with high incidence, morbidity and disability. Treatments against various types of cerebral stroke are limited at preventive measurements due to the lack of effective therapeutic method. The present study aimed to investigate the protective effect of cryptotanshinone (CPT) on cerebral stroke, and investigate the possible mechanism involved in order to develop a novel therapy against stoke. The phosphoinositide 3‑kinase membrane translocation of cerebral stroke rats pretreated with CPT at various concentrations were measured, as well as the phosphorylation of protein kinase B (AKT) and endothelial nitric oxide synthase (eNOS). Additionally, the expression level of B‑cell lymphoma 2 (Bcl‑2), Bcl‑2‑associated X protein (Bax) and vascular endothelial growth factor were also assessed using western blotting and reverse transcription‑quantitative polymerase chain reaction. Furthermore, biochemical tests were used to measure the activity of superoxide dismutase (SOD), malondialdehyde (MDA) and nitric oxide (NO) in both the cerebral cortex and peripheral blood. As a result, CPT‑pretreated rats presented declined phosphoinositide 3‑kinase (PI3K) and AKT expression levels, indicating that the PI3K/AKT signaling pathway was inhibited. Increased Bcl‑2 and NO levels in both the cerebral cortex and peripheral blood demonstrated the anti‑apoptosis and blood vessel protection effect of CPT. Furthermore, increased SOD activity and declined MDA levels demonstrated suppressed lipid peroxidation. In conclusion, CPT exhibited a protective effect against cerebral stroke through inhibition of the PI3K/AKT‑eNOS signaling pathway. These results suggested the potential of CPT as a promising agent in the treatment of cerebral stroke.

Lipoleiomyoma of the Uterus and Primary Ovarian Leiomyoma in a Postmenopausal Woman: Two Rare Entities in the Same Individual

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Sefa Kelekci

2015-01-01

Full Text Available Uterine lipoleiomyomas are rare benign tumours that are composed of various mixtures of smooth muscle and mature fat tissue. Leiomyomas, which arise primarily in the ovary, are extremely rare tumours that account for 0.5–1% of all benign ovarian tumours. To the best of our knowledge, we present the first case of an ovarian leiomyoma coexisting with a uterine lipoleiomyoma in the postmenopausal period. A 59-year-old, gravida 4, para 3, postmenopausal woman exhibited pelvic discomfort and increased frequency of micturition. A pelvic examination revealed a solid, tender mass on the left side that could not be clearly separated from the uterus. She underwent a laparotomy with an initial diagnosis of a left ovarian mass. She had previously undergone a total abdominal hysterectomy and bilateral salpingo-oophorectomy. A histopathological examination revealed a uterine lipoleiomyoma, composed of variable amounts of smooth muscle cells and mature adipocytes and a right ovarian leiomyoma composed of interlacing bundles and fascicles of spindle cells. The coexistence of these two rare entities in the same individual may represent a common pathway as a stimulating agent. This case may help to clarify the pathogenesis of these lesions.

Metabolic engineering of the phenylpropanoid pathway enhances the antioxidant capacity of Saussurea involucrata.

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Jian Qiu

Full Text Available The rare wild species of snow lotus Saussurea involucrata is a commonly used medicinal herb with great pharmacological value for human health, resulting from its uniquely high level of phenylpropanoid compound production. To gain information on the phenylpropanid biosynthetic pathway genes in this critically important medicinal plant, global transcriptome sequencing was performed. It revealed that the phenylpropanoid pathway genes were well represented in S. involucrata. In addition, we introduced two key phenylpropanoid pathway inducing transcription factors (PAP1 and Lc into this medicinal plant. Transgenic S. involucrata co-expressing PAP1 and Lc exhibited purple pigments due to a massive accumulation of anthocyanins. The over-expression of PAP1 and Lc largely activated most of the phenylpropanoid pathway genes, and increased accumulation of several phenylpropanoid compounds significantly, including chlorogenic acid, syringin, cyanrine and rutin. Both ABTS (2,2'-azinobis-3-ethylbenzotiazo-line-6-sulfonic acid and FRAP (ferric reducing anti-oxidant power assays revealed that the antioxidant capacity of transgenic S. involucrata lines was greatly enhanced over controls. In addition to providing a deeper understanding of the molecular basis of phenylpropanoid metabolism, our results potentially enable an alternation of bioactive compound production in S. involucrata through metabolic engineering.

Resources, challenges and way forward in rare mitochondrial diseases research.

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Rajput, Neeraj Kumar; Singh, Vipin; Bhardwaj, Anshu

2015-01-01

Over 300 million people are affected by about 7000 rare diseases globally. There are tremendous resource limitations and challenges in driving research and drug development for rare diseases. Hence, innovative approaches are needed to identify potential solutions. This review focuses on the resources developed over the past years for analysis of genome data towards understanding disease biology especially in the context of mitochondrial diseases, given that mitochondria are central to major cellular pathways and their dysfunction leads to a broad spectrum of diseases. Platforms for collaboration of research groups, clinicians and patients and the advantages of community collaborative efforts in addressing rare diseases are also discussed. The review also describes crowdsourcing and crowdfunding efforts in rare diseases research and how the upcoming initiatives for understanding disease biology including analyses of large number of genomes are also applicable to rare diseases.

New Trident Molecule with Phosphoric Acid Functionality for Trivalent Rare Earth Extraction

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Keisuke Ohto

2017-11-01

Full Text Available Tripodal extraction reagent with three phosphoric acid groups, together with the corresponding monopodal molecule has been prepared to investigate some metals extraction behavior, in particular, trivalent rare earth elements (REEs. The tripodal reagent exhibited extremely high selectivity for metals with high valency such as Zr(IV, In(III, Lu(III, and Fe(III. Tripodal reagent also exhibited exceptionally high extraction ability compared with the corresponding monopodal one in the extraction of trivalent rare earths. The result for the stoichiometry of tripodal reagent to heavy rare earths showed the inflection point between Er (2:1 for a ligand with ion and Tm (1:1. The extraction reactions were determined for all rare earths with both reagents. The extraction equilibrium constants (Kex, the separation factors (β, half pH values (pH1/2, difference half pH values (ΔpH1/2 for extraction of REEs with both reagents are estimated.

Bisphenol A induces spermatocyte apoptosis in rare minnow Gobiocypris rarus

International Nuclear Information System (INIS)

Zhang, Yingying; Cheng, Mengqian; Wu, Lang; Zhang, Guo; Wang, Zaizhao

2016-01-01

Highlights: • Adult male G. rarus were exposed to 225 μg/L BPA for 7, 21 and 63 days. • BPA could induce spermatocyte apoptosis in rare minnow testis. • The mitochondrial apoptotic pathway participated in the germ cell apoptosis. • The spermatocyte apoptosis was likely initiated by BPA induced meiosis arrest. - Abstract: Bisphenol A (BPA) is an endocrine disruptor, and could induce germ cells apoptosis in the testis of mammals. But whether it could affect fish in the same mechanism has not’ been studied till now. In the present study, to investigate the influence of BPA on testis germ cells in fish, adult male rare minnow Gobiocypris rarus were exposed to 225 μg L"−"1 (0.99 μM) BPA for 1, 3 and 9 weeks. Through TdT-mediated dUTP nick end labeling (TUNEL) and transmission electron microscope (TEM) analysis, we found that the amount of apoptotic spermatocytes significantly increased in a time dependent manner following BPA exposure. Western Blot results showed that the ratio of Bcl2/Bax, the important apoptosis regulators in intrinsic mitochondrial apoptotic pathway, was significantly decreased. qPCR showed that mRNA expression of several genes in mitochondrial apoptotic pathway including bcl2, bax, casp9, cytc and mcl1b were significantly changed following BPA exposure. In addition, mRNA expression of meiosis regulation genes (kpna7 and wee2), and genes involved in both apoptosis and meiosis (birc5, ccna1, and gsa1a) were also affected by BPA. Taken together, the present study demonstrated that BPA could induce spermatocytes apoptosis in rare minnow testis, and the apoptosis was probably under regulation of intrinsic mitochondrial apoptotic pathway. Moreover, the spermatocyte apoptosis was likely initiated by BPA induced meiosis arrest.

Bisphenol A induces spermatocyte apoptosis in rare minnow Gobiocypris rarus

Energy Technology Data Exchange (ETDEWEB)

Zhang, Yingying; Cheng, Mengqian; Wu, Lang; Zhang, Guo; Wang, Zaizhao, E-mail: zzwang@nwsuaf.edu.cn

2016-10-15

Highlights: • Adult male G. rarus were exposed to 225 μg/L BPA for 7, 21 and 63 days. • BPA could induce spermatocyte apoptosis in rare minnow testis. • The mitochondrial apoptotic pathway participated in the germ cell apoptosis. • The spermatocyte apoptosis was likely initiated by BPA induced meiosis arrest. - Abstract: Bisphenol A (BPA) is an endocrine disruptor, and could induce germ cells apoptosis in the testis of mammals. But whether it could affect fish in the same mechanism has not’ been studied till now. In the present study, to investigate the influence of BPA on testis germ cells in fish, adult male rare minnow Gobiocypris rarus were exposed to 225 μg L{sup −1} (0.99 μM) BPA for 1, 3 and 9 weeks. Through TdT-mediated dUTP nick end labeling (TUNEL) and transmission electron microscope (TEM) analysis, we found that the amount of apoptotic spermatocytes significantly increased in a time dependent manner following BPA exposure. Western Blot results showed that the ratio of Bcl2/Bax, the important apoptosis regulators in intrinsic mitochondrial apoptotic pathway, was significantly decreased. qPCR showed that mRNA expression of several genes in mitochondrial apoptotic pathway including bcl2, bax, casp9, cytc and mcl1b were significantly changed following BPA exposure. In addition, mRNA expression of meiosis regulation genes (kpna7 and wee2), and genes involved in both apoptosis and meiosis (birc5, ccna1, and gsa1a) were also affected by BPA. Taken together, the present study demonstrated that BPA could induce spermatocytes apoptosis in rare minnow testis, and the apoptosis was probably under regulation of intrinsic mitochondrial apoptotic pathway. Moreover, the spermatocyte apoptosis was likely initiated by BPA induced meiosis arrest.

Quercetin-6-C-β-D-glucopyranoside, natural analog of quercetin exhibits anti-prostate cancer activity by inhibiting Akt-mTOR pathway via aryl hydrocarbon receptor.

Science.gov (United States)

Hamidullah; Kumar, Rajeev; Saini, Karan Singh; Kumar, Amit; Kumar, Sudhir; Ramakrishna, E; Maurya, Rakesh; Konwar, Rituraj; Chattopadhyay, Naibedya

2015-12-01

Pre-clinical studies suggest mitigating effect of dietary flavonoid quercetin against cancer and other diseases. However, quercetin suffers from poor metabolic stability, which appears to offset its pharmacological efficacy. Recently, we isolated quercetin-6-C-β-D-glucopyranoside (QCG) from Ulmus wallichiana planchon that has greater stability profile over quercetin. In the present study, the cytotoxic and apoptotic effects of QCG on prostate cancer cells were assessed. QCG inhibited prostate cancer cell proliferation by arresting cells at G0/G1 phase of cell cycle and induces apoptosis as evident from cytochrome c release, cleavage of caspase 3 and poly (ADP-ribose) polymerase. Mechanistic studies revealed that QCG inhibited reactive oxygen species (ROS) generation and Akt/mTOR cell survival pathways. Aryl hydrocarbon receptor (AhR) was a critical mediator of QCG action as knockdown of AhR attenuated QCG-induced cell cycle arrest, apoptosis and inhibition of Akt/mTOR pathway in prostate cancer cells. Taken together, our results suggest that QCG exhibits anti-cancer activity against prostate cancer cells via AhR-mediated down regulation of Akt/mTOR pathway in PC-3 cells. Copyright © 2015 Elsevier B.V. and Société Française de Biochimie et Biologie Moléculaire (SFBBM). All rights reserved.

Manuscripts and Rare Books in an Undergraduate Library.

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Mortimer, Ruth

1983-01-01

Description of Smith College Library's Rare Book Room notes printed book and manuscript collections including the Sylvia Plath collection, Ernest Hemingway collection, and collection of modern press books. Related undergraduate courses in typography and the history of printing, student exhibitions, and student publications are highlighted. (EJS)

Arabidopsis scaffold protein RACK1A modulates rare sugar D-allose regulated gibberellin signaling.

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Fennell, Herman; Olawin, Abdulquadri; Mizanur, Rahman M; Izumori, Ken; Chen, Jin-Gui; Ullah, Hemayet

2012-11-01

As energy sources and structural components, sugars are the central regulators of plant growth and development. In addition to the abundant natural sugars in plants, more than 50 different kinds of rare sugars exist in nature, several of which show distinct roles in plant growth and development. Recently, one of the rare sugars, D-allose, an epimer of D-glucose at C3, is found to suppress plant hormone gibberellin (GA) signaling in rice. Scaffold protein RACK1A in the model plant Arabidopsis is implicated in the GA pathway as rack1a knockout mutants show insensitivity to GA in GA-induced seed germination. Using genetic knockout lines and a reporter gene, the functional role of RACK1A in the D-allose pathway was investigated. It was found that the rack1a knockout seeds showed hypersensitivity to D-allose-induced inhibition of seed germination, implicating a role for RACK1A in the D-allose mediated suppression of seed germination. On the other hand, a functional RACK1A in the background of the double knockout mutations in the other two RACK1 isoforms, rack1b/rack1c, showed significant resistance to the D-allose induced inhibition of seed germination. The collective results implicate the RACK1A in the D-allose mediated seed germination inhibition pathway. Elucidation of the rare sugar signaling mechanism will help to advance understanding of this less studied but important cellular signaling pathway.

Ubiquitylation and the Fanconi Anemia Pathway

Science.gov (United States)

Garner, Elizabeth; Smogorzewska, Agata

2012-01-01

The Fanconi anemia (FA) pathway maintains genome stability through co-ordination of DNA repair of interstrand crosslinks (ICLs). Disruption of the FA pathway yields hypersensitivity to interstrand crosslinking agents, bone marrow failure and cancer predisposition. Early steps in DNA damage dependent activation of the pathway are governed by monoubiquitylation of FANCD2 and FANCI by the intrinsic FA E3 ubiquitin ligase, FANCL. Downstream FA pathway components and associated factors such as FAN1 and SLX4 exhibit ubiquitin-binding motifs that are important for their DNA repair function, underscoring the importance of ubiquitylation in FA pathway mediated repair. Importantly, ubiquitylation provides the foundations for cross-talk between repair pathways, which in concert with the FA pathway, resolve interstrand crosslink damage and maintain genomic stability. PMID:21605559

Witnessing Each Other: An Intersubjective Stance for Exhibitions Relating to Substance Use and Abuse.

Science.gov (United States)

Hennes, Tom

2015-01-01

Most exhibitions are conceived to convey information the experts making the exhibition believe other people need or want. But the notion that the intended exhibition public will cooperate with the exhibition organizers. intent disregards the reasons people come to exhibitions and the way they use them. While the author contends that an exhibition cannot use facts to convince someone to abstain from substances they crave, exhibitions can nonetheless make a difference in lives complicated by substance use by providing representation for voices that are rarely heard and building empathy between witness and witnessed. The purpose of such an endeavor is not to change attitudes or behaviors toward a pre-determined outcome, but to facilitate a witnessing of others. The uniquely intersubjective medium of exhibition can thus succeed in this field by opening the potential of mutual, humanizing recognition among people with varied life experience of substance use and abuse.

Chemical synthesis and characterization of nano-sized rare-earth ...

Indian Academy of Sciences (India)

2017-12-06

Dec 6, 2017 ... exhibited the average particle size in the range of 36.4–73.8nm. The data on the ... For example, some of the rare-earth ruthenates Ln2Ru2O7 ..... From qualitative band-model consideration of the type discussed by ...

Extreme Quantum Memory Advantage for Rare-Event Sampling

Science.gov (United States)

Aghamohammadi, Cina; Loomis, Samuel P.; Mahoney, John R.; Crutchfield, James P.

2018-02-01

We introduce a quantum algorithm for memory-efficient biased sampling of rare events generated by classical memoryful stochastic processes. Two efficiency metrics are used to compare quantum and classical resources for rare-event sampling. For a fixed stochastic process, the first is the classical-to-quantum ratio of required memory. We show for two example processes that there exists an infinite number of rare-event classes for which the memory ratio for sampling is larger than r , for any large real number r . Then, for a sequence of processes each labeled by an integer size N , we compare how the classical and quantum required memories scale with N . In this setting, since both memories can diverge as N →∞ , the efficiency metric tracks how fast they diverge. An extreme quantum memory advantage exists when the classical memory diverges in the limit N →∞ , but the quantum memory has a finite bound. We then show that finite-state Markov processes and spin chains exhibit memory advantage for sampling of almost all of their rare-event classes.

Extreme Quantum Memory Advantage for Rare-Event Sampling

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Cina Aghamohammadi

2018-02-01

Full Text Available We introduce a quantum algorithm for memory-efficient biased sampling of rare events generated by classical memoryful stochastic processes. Two efficiency metrics are used to compare quantum and classical resources for rare-event sampling. For a fixed stochastic process, the first is the classical-to-quantum ratio of required memory. We show for two example processes that there exists an infinite number of rare-event classes for which the memory ratio for sampling is larger than r, for any large real number r. Then, for a sequence of processes each labeled by an integer size N, we compare how the classical and quantum required memories scale with N. In this setting, since both memories can diverge as N→∞, the efficiency metric tracks how fast they diverge. An extreme quantum memory advantage exists when the classical memory diverges in the limit N→∞, but the quantum memory has a finite bound. We then show that finite-state Markov processes and spin chains exhibit memory advantage for sampling of almost all of their rare-event classes.

Rare diseases in clinical endocrinology: a taxonomic classification system.

Science.gov (United States)

Marcucci, G; Cianferotti, L; Beck-Peccoz, P; Capezzone, M; Cetani, F; Colao, A; Davì, M V; degli Uberti, E; Del Prato, S; Elisei, R; Faggiano, A; Ferone, D; Foresta, C; Fugazzola, L; Ghigo, E; Giacchetti, G; Giorgino, F; Lenzi, A; Malandrino, P; Mannelli, M; Marcocci, C; Masi, L; Pacini, F; Opocher, G; Radicioni, A; Tonacchera, M; Vigneri, R; Zatelli, M C; Brandi, M L

2015-02-01

Rare endocrine-metabolic diseases (REMD) represent an important area in the field of medicine and pharmacology. The rare diseases of interest to endocrinologists involve all fields of endocrinology, including rare diseases of the pituitary, thyroid and adrenal glands, paraganglia, ovary and testis, disorders of bone and mineral metabolism, energy and lipid metabolism, water metabolism, and syndromes with possible involvement of multiple endocrine glands, and neuroendocrine tumors. Taking advantage of the constitution of a study group on REMD within the Italian Society of Endocrinology, consisting of basic and clinical scientists, a document on the taxonomy of REMD has been produced. This document has been designed to include mainly REMD manifesting or persisting into adulthood. The taxonomy of REMD of the adult comprises a total of 166 main disorders, 338 including all variants and subtypes, described into 11 tables. This report provides a complete taxonomy to classify REMD of the adult. In the future, the creation of registries of rare endocrine diseases to collect data on cohorts of patients and the development of common and standardized diagnostic and therapeutic pathways for each rare endocrine disease is advisable. This will help planning and performing intervention studies in larger groups of patients to prove the efficacy, effectiveness, and safety of a specific treatment.

Chondroblastoma and chondromyxoid fibroma : disentangling the neoplastic chondrogenesis of two rare cartilaginous tumours

NARCIS (Netherlands)

Romeo, Salvatore

2010-01-01

The scope of this study was to disentangle neoplastic chondrogenesis in two rare cartilaginous tumours: chondroblastoma and chondromyxoid fibroma. It was addressed: 1 The spectrum of phenotypic differentiation in chondroblastoma and chondromyxoid fibroma, 2 The signalling pathways driving

Hiccup: An Extremely Rare Presentation of Thyrotoxicosis of Graves’ Disease

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Irshad Parray

2011-03-01

Full Text Available Persistent hiccup is a rare but potentially severe condition that can be symptomatic of a variety of diseases or idiopathic. Most episodes last only a few minutes and are self-limited, but hiccup can get persistent and become a real problem for physician and patient alike. The center of hiccup may be activated by a great variety of stimuli travelling along different nerve pathways and bring different effecter responses. We report a case of persistent hiccup as a presentation of impending thyroid storm of Graves’ disease. Though the condition is rare, clinicians should remain alert to the possibility of this diagnosis.

Rare earth oxide-doped titania nanocomposites with enhanced photocatalytic activity towards the degradation of partially hydrolysis polyacrylamide

International Nuclear Information System (INIS)

Li Jinhuan; Yang Xia; Yu Xiaodan; Xu, Leilei; Kang Wanli; Yan Wenhua; Gao Hongfeng; Liu Zhonghe; Guo Yihang

2009-01-01

Rare-earth oxide-doped titania nanocomposites (RE 3+ /TiO 2 , where RE = Eu 3+ , Pr 3+ , Gd 3+ , Nd 3+ , and Y 3+ ) were prepared by a one-step sol-gel-solvothermal method. The products exhibited anatase phase structure, mesoporosity, and interesting surface compositions with three oxygen species and two titanium species. The products were used as the photocatalysts to degrade a partially hydrolysis polyacrylamide (HPAM) under UV-light irradiation, a very useful polymer in oil recovery. For comparison, Degussa P25 and as-prepared pure TiO 2 were also tested under the same conditions. The enhanced photocatalytic activity was obtained on as-prepared Eu 3+ (Gd 3+ , Pr 3+ )/TiO 2 composites, and the reasons were explained. Finally, the degradation pathway of HPAM over the RE 3+ /TiO 2 composite was put forward based on the intermediates produced during the photocatalysis procedure.

Block of the Mevalonate Pathway Triggers Oxidative and Inflammatory Molecular Mechanisms Modulated by Exogenous Isoprenoid Compounds

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Paola Maura Tricarico

2014-04-01

Full Text Available Deregulation of the mevalonate pathway is known to be involved in a number of diseases that exhibit a systemic inflammatory phenotype and often neurological involvements, as seen in patients suffering from a rare disease called mevalonate kinase deficiency (MKD. One of the molecular mechanisms underlying this pathology could depend on the shortage of isoprenoid compounds and the subsequent mitochondrial damage, leading to oxidative stress and pro-inflammatory cytokines’ release. Moreover, it has been demonstrated that cellular death results from the balance between apoptosis and pyroptosis, both driven by mitochondrial damage and the molecular platform inflammasome. In order to rescue the deregulated pathway and decrease inflammatory markers, exogenous isoprenoid compounds were administered to a biochemical model of MKD obtained treating a murine monocytic cell line with a compound able to block the mevalonate pathway, plus an inflammatory stimulus. Our results show that isoprenoids acted in different ways, mainly increasing the expression of the evaluated markers [apoptosis, mitochondrial dysfunction, nucleotide-binding oligomerization-domain protein-like receptors 3 (NALP3, cytokines and nitric oxide (NO]. Our findings confirm the hypothesis that inflammation is triggered, at least partially, by the shortage of isoprenoids. Moreover, although further studies are necessary, the achieved results suggest a possible role for exogenous isoprenoids in the treatment of MKD.

Rare cancers are not so rare: The rare cancer burden in Europe

NARCIS (Netherlands)

Gatta, Gemma; van der Zwan, Jan Maarten; Casali, Paolo G.; Siesling, Sabine; Dei Tos, Angelo Paolo; Kunkler, Ian; Otter, Renee; Licitra, Lisa

2011-01-01

Purpose: Epidemiologic information on rare cancers is scarce. The project Surveillance of Rare Cancers in Europe (RARECARE) provides estimates of the incidence, prevalence and survival of rare cancers in Europe based on a new and comprehensive list of these diseases. Materials and methods: RARECARE

The role of APC in WNT pathway activation in serrated neoplasia.

Science.gov (United States)

Borowsky, Jennifer; Dumenil, Troy; Bettington, Mark; Pearson, Sally-Ann; Bond, Catherine; Fennell, Lochlan; Liu, Cheng; McKeone, Diane; Rosty, Christophe; Brown, Ian; Walker, Neal; Leggett, Barbara; Whitehall, Vicki

2018-03-01

Conventional adenomas are initiated by APC gene mutation that activates the WNT signal. Serrated neoplasia is commonly initiated by BRAF or KRAS mutation. WNT pathway activation may also occur, however, to what extent this is owing to APC mutation is unknown. We examined aberrant nuclear β-catenin immunolocalization as a surrogate for WNT pathway activation and analyzed the entire APC gene coding sequence in serrated and conventional pathway polyps and cancers. WNT pathway activation was a common event in conventional pathway lesions with aberrant nuclear immunolocalization of β-catenin and truncating APC mutations in 90% and 89% of conventional adenomas and 82% and 70% of BRAF wild-type cancers, respectively. WNT pathway activation was seen to a lesser extent in serrated pathway lesions. It occurred at the transition to dysplasia in serrated polyps with a significant increase in nuclear β-catenin labeling from sessile serrated adenomas (10%) to sessile serrated adenomas with dysplasia (55%) and traditional serrated adenomas (9%) to traditional serrated adenomas with dysplasia (39%) (P=0.0001). However, unlike the conventional pathway, truncating APC mutations were rare in the serrated pathway lesions especially sessile serrated adenomas even when dysplastic (15%) and in the BRAF mutant cancers with microsatellite instability that arise from them (8%). In contrast, APC missense mutations that were rare in conventional pathway adenomas and cancers (3% in BRAF wild-type cancers) were more frequent in BRAF mutant cancers with microsatellite instability (32%). We conclude that increased WNT signaling is important in the transition to malignancy in the serrated pathway but that APC mutation is less common and the spectrum of mutations is different than in conventional colorectal carcinogenesis. Moderate impact APC mutations and non-APC-related causes of increased WNT signaling may have a more important role in serrated neoplasia than the truncating APC mutations

Drosophila interspecific hybrids phenocopy piRNA-pathway mutants.

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Erin S Kelleher

Full Text Available The Piwi-interacting RNA (piRNA pathway defends the germline of animals from the deleterious activity of selfish transposable elements (TEs through small-RNA mediated silencing. Adaptation to novel invasive TEs is proposed to occur by incorporating their sequences into the piRNA pool that females produce and deposit into their eggs, which then propagates immunity against specific TEs to future generations. In support of this model, the F1 offspring of crosses between strains of the same Drosophila species sometimes suffer from germline derepression of paternally inherited TE families, caused by a failure of the maternal strain to produce the piRNAs necessary for their regulation. However, many protein components of the Drosophila piRNA pathway exhibit signatures of positive selection, suggesting that they also contribute to the evolution of host genome defense. Here we investigate piRNA pathway function and TE regulation in the F1 hybrids of interspecific crosses between D. melanogaster and D. simulans and compare them with intraspecific control crosses of D. melanogaster. We confirm previous reports showing that intraspecific crosses are characterized by derepression of paternally inherited TE families that are rare or absent from the maternal genome and piRNA pool, consistent with the role of maternally deposited piRNAs in shaping TE silencing. In contrast to the intraspecific cross, we discover that interspecific hybrids are characterized by widespread derepression of both maternally and paternally inherited TE families. Furthermore, the pattern of derepression of TE families in interspecific hybrids cannot be attributed to their paucity or absence from the piRNA pool of the maternal species. Rather, we demonstrate that interspecific hybrids closely resemble piRNA effector-protein mutants in both TE misregulation and aberrant piRNA production. We suggest that TE derepression in interspecific hybrids largely reflects adaptive divergence of pi

Impacts of visitor number on Kangaroos housed in free-range exhibits.

Science.gov (United States)

Sherwen, Sally L; Hemsworth, Paul H; Butler, Kym L; Fanson, Kerry V; Magrath, Michael J L

2015-01-01

Free range exhibits are becoming increasingly popular in zoos as a means to enhance interaction between visitors and animals. However very little research exists on the impacts of visitors on animal behaviour and stress in free range exhibits. We investigated the effects of visitor number on the behaviour and stress physiology of Kangaroo Island (KI) Kangaroos, Macropus fuliginosus fuliginosus, and Red Kangaroos, Macropus rufus, housed in two free range exhibits in Australian zoos. Behavioural observations were conducted on individual kangaroos at each site using instantaneous scan sampling to record activity (e.g., vigilance, foraging, resting) and distance from the visitor pathway. Individually identifiable faecal samples were collected at the end of each study day and analysed for faecal glucocorticoid metabolite (FGM) concentration. When visitor number increased, both KI Kangaroos and Red Kangaroos increased the time spent engaged in visitor-directed vigilance and KI Kangaroos also increased the time spent engaged in locomotion and decreased the time spent resting. There was no effect of visitor number on the distance kangaroos positioned themselves from the visitor pathway or FGM concentration in either species. While there are limitations in interpreting these results in terms of fear of visitors, there was no evidence of adverse effects animal welfare in these study groups based on avoidance behaviour or stress physiology under the range of visitor numbers that we studied. © 2015 Wiley Periodicals, Inc.

Fabrication of Superhydrophobic and Luminescent Rare Earth/Polymer complex Films.

Science.gov (United States)

Wang, Zefeng; Ye, Weiwei; Luo, Xinran; Wang, Zhonggang

2016-04-18

The motivation of this work is to create luminescent rare earth/polymer films with outstanding water-resistance and superhydrophobicity. Specifically, the emulsion polymerization of styrene leads to core particles. Then core-shell-structured polymer nanoparticles are synthesized by copolymerization of styrene and acrylic acid on the core surface. The coordination reaction between carboxylic groups and rare earth ions (Eu(3+) and Tb(3+)) generates uniform spherical rare earth/polymer nanoparticles, which are subsequently complexed with PTFE microparticles to obtain micro-/nano-scaled PTFE/rare earth films with hierarchical rough morphology. The films exhibit large water contact angle up to 161° and sliding angle of about 6°, and can emit strong red and green fluorescence under UV excitation. More surprisingly, it is found that the films maintain high fluorescence intensity after submersed in water and even in aqueous salt solution for two days because of the excellent water repellent ability of surfaces.

Direct current electroluminescence in rare-earth-doped zinc sulphide

International Nuclear Information System (INIS)

Bryant, F.J.; Krier, A.

1984-01-01

Some of the properties and characteristics of rare-earth-doped zinc sulphide DCEL devices are reported. Two types of devices are discussed, co-evaporated ZnS:RE thin films and ion implanted ZnS:RE single crystal diodes. The thin film devices exhibit bright DCEL of various colours at low applied voltages (typically approximately 12 V). A study of the spectral intensities and lifetimes of the Er 3+ ion in ZnS:Er 3+ thin films is consistent with a Boltzmann energy distribution amongst the conduction electrons present in these devices. The ZnS:RE single crystal diodes fabricated in this laboratory by ion implantation are also capable of various colour DCEL. By comparing the EL emission obtained from the different rare earth dopants, erbium and neodymium are identified as the most efficient luminescence centres. Further consideration of the EL emission spectra gives evidence for the presence of inter-conduction band hot electron transitions in those devices containing rare earth dopants which are inefficent electroluminescence centres. These findings can be explained in terms of Auger processes occurring in rare earth complexes. (author)

Molecular, Phenotypic Aspects and Therapeutic Horizons of Rare Genetic Bone Disorders

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Taha Faruqi

2014-01-01

Full Text Available A rare disease afflicts less than 200,000 individuals, according to the National Organization for Rare Diseases (NORD of the United States. Over 6,000 rare disorders affect approximately 1 in 10 Americans. Rare genetic bone disorders remain the major causes of disability in US patients. These rare bone disorders also represent a therapeutic challenge for clinicians, due to lack of understanding of underlying mechanisms. This systematic review explored current literature on therapeutic directions for the following rare genetic bone disorders: fibrous dysplasia, Gorham-Stout syndrome, fibrodysplasia ossificans progressiva, melorheostosis, multiple hereditary exostosis, osteogenesis imperfecta, craniometaphyseal dysplasia, achondroplasia, and hypophosphatasia. The disease mechanisms of Gorham-Stout disease, melorheostosis, and multiple hereditary exostosis are not fully elucidated. Inhibitors of the ACVR1/ALK2 pathway may serve as possible therapeutic intervention for FOP. The use of bisphosphonates and IL-6 inhibitors has been explored to be useful in the treatment of fibrous dysplasia, but more research is warranted. Cell therapy, bisphosphonate polytherapy, and human growth hormone may avert the pathology in osteogenesis imperfecta, but further studies are needed. There are still no current effective treatments for these bone disorders; however, significant promising advances in therapeutic modalities were developed that will limit patient suffering and treat their skeletal disabilities.

Survey of absence. The use of photographic data for the survey of temporary art exhibitions.

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Matteo Ballarin

2013-10-01

Full Text Available Modern Art Exhibitions succeeded in conveying long-lasting ideas and meanings despite their ephemeral nature. Art history and Museum studies recently started to focus on the Exhibition as a medium, requiring precise graphic reconstructions of exhibit designs for critical and theoretical purposes. Exhibitions were rarely designed through graphic devices such as the project; they were usually a result of improvisation processes. In this sense, photographs are the only documents for graphic reconstructions of temporary art spaces. Nevertheless, the photographic datum has been over-edited in its span of life for more than one reason: it requires a critical approach to be used as a source. The use of digital tools for photography can lead to satisfactory results when applied to humanities.

The electrorheological properties of nano-sized SiO2 particle materials doped with rare earths

International Nuclear Information System (INIS)

Liu Yang; Liao Fuhui; Li Junran; Zhang Shaohua; Chen Shumei; Wei Chenguan; Gao Song

2006-01-01

Electrorheological (ER) materials of pure SiO 2 and SiO 2 doped with rare earths (RE = Ce, Gd, Y) (non-metallic glasses (silicates)) were prepared using Na 2 SiO 3 and RECl 3 as starting materials. The electrorheological properties are not enhanced by all rare earth additions. The material doped with Ce exhibits the best ER performance

Sampling rare events in nonequilibrium and nonstationary systems.

Science.gov (United States)

Berryman, Joshua T; Schilling, Tanja

2010-12-28

Although many computational methods for rare event sampling exist, this type of calculation is not usually practical for general nonequilibrium conditions, with macroscopically irreversible dynamics and away from both stationary and metastable states. A novel method for calculating the time-series of the probability of a rare event is presented which is designed for these conditions. The method is validated for the cases of the Glauber-Ising model under time-varying shear flow, the Kawasaki-Ising model after a quench into the region between nucleation dominated and spinodal decomposition dominated phase change dynamics, and the parallel open asymmetric exclusion process. The method requires a subdivision of the phase space of the system: it is benchmarked and found to scale well for increasingly fine subdivisions, meaning that it can be applied without detailed foreknowledge of the physically important reaction pathways.

Synthesis and luminescence studies of novel rare earth activated lanthanum pentaborate

International Nuclear Information System (INIS)

Nagpure, P.A.; Bajaj, N.S.; Omanwar, S.K.; Sonekar, R.P.

2011-01-01

The lanthanum pentaborate (LaB 5 O 9 ) is a novel material which exhibits excellent luminescence when doped with rare earth ions. It was prepared by a novel technique which is a slight variation of solution combustion synthesis. The synthesis is based on the exothermic reaction between the fuel (urea) and oxidizer (ammonium nitrate). The structure of the prepared material was confirmed by powder XRD technique. The photoluminescence of rare earth ions (Ce 3+ , Eu 3+ ) and sensitized luminescence of Gd 3+ (Pr 3+ -Gd 3+ and Bi 3+ -Gd 3+ ) in LaB 5 O 9 have been studied. LaB 5 O 9 :Ce 3+ shows broad band UV emission at 317 nm and LaB 5 O 9 :Eu 3+ shows orange red emission. LaB 5 O 9 : Pr 3+ -Gd 3+ and LaB 5 O 9 : Bi 3+ -Gd 3+ exhibit efficient luminescence of Gd 3+ in narrow UVB region at 310 nm. The material (La 0.5 Pr 0.4 )B 5 O 9 :Gd 0.1 exhibits intense narrow band UVB emission at 310 nm and could be a potential candidate for UVB phosphors used in phototherapy lamps. (author)

Sporulation properties and antimicrobial susceptibility in endemic and rare Clostridium difficile PCR ribotypes.

Science.gov (United States)

Zidaric, Valerija; Rupnik, Maja

2016-06-01

Increased sporulation and antibiotic resistance have been proposed to be associated with certain Clostridium difficile epidemic strains such as PCR ribotype 027. In this study we examined these properties in another widespread PCR ribotype, 014/020, in comparison to prevalent PCR ribotype 002 and a group of rarely represented PCR ribotypes. Highest sporulation was observed in 014/020 strains at 24 h, while after 72 h PCR ribotype 002 and rare PCR ribotypes formed higher total number of spores. PCR ribotype 014/020 strains exhibited slightly higher resistance to tested antimicrobials, followed by group of rare PCR ribotypes and less common PCR ribotype 002. Neither sporulation properties nor antibiotic resistance clearly differed in endemic and rare strains. Copyright © 2016 Elsevier Ltd. All rights reserved.

Antibacterial, Anticancer and Neuroprotective Activities of Rare Actinobacteria from Mangrove Forest Soils.

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Azman, Adzzie-Shazleen; Othman, Iekhsan; Fang, Chee-Mun; Chan, Kok-Gan; Goh, Bey-Hing; Lee, Learn-Han

2017-06-01

Mangrove is a complex ecosystem that contains diverse microbial communities, including rare actinobacteria with great potential to produce bioactive compounds. To date, bioactive compounds extracted from mangrove rare actinobacteria have demonstrated diverse biological activities. The discovery of three novel rare actinobacteria by polyphasic approach, namely Microbacterium mangrovi MUSC 115 T , Sinomonas humi MUSC 117 T and Monashia flava MUSC 78 T from mangrove soils at Tanjung Lumpur, Peninsular Malaysia have led to the screening on antibacterial, anticancer and neuroprotective activities. A total of ten different panels of bacteria such as Methicillin-resistant Staphylococcus aureus (MRSA) ATCC 43300, ATCC 70069, Pseudomonas aeruginosa NRBC 112582 and others were selected for antibacterial screening. Three different neuroprotective models (hypoxia, oxidative stress, dementia) were done using SHSY5Y neuronal cells while two human cancer cells lines, namely human colon cancer cell lines (HT-29) and human cervical carcinoma cell lines (Ca Ski) were utilized for anticancer activity. The result revealed that all extracts exhibited bacteriostatic effects on the bacteria tested. On the other hand, the neuroprotective studies demonstrated M. mangrovi MUSC 115 T extract exhibited significant neuroprotective properties in oxidative stress and dementia model while the extract of strain M. flava MUSC 78 T was able to protect the SHSY5Y neuronal cells in hypoxia model. Furthermore, the extracts of M. mangrovi MUSC 115 T and M. flava MUSC 78 T exhibited anticancer effect against Ca Ski cell line. The chemical analysis of the extracts through GC-MS revealed that the majority of the compounds present in all extracts are heterocyclic organic compound that could explain for the observed bioactivities. Therefore, the results obtained in this study suggested that rare actinobacteria discovered from mangrove environment could be potential sources of antibacterial, anticancer and

A scan statistic to extract causal gene clusters from case-control genome-wide rare CNV data

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Scherer Stephen W

2011-05-01

Full Text Available Abstract Background Several statistical tests have been developed for analyzing genome-wide association data by incorporating gene pathway information in terms of gene sets. Using these methods, hundreds of gene sets are typically tested, and the tested gene sets often overlap. This overlapping greatly increases the probability of generating false positives, and the results obtained are difficult to interpret, particularly when many gene sets show statistical significance. Results We propose a flexible statistical framework to circumvent these problems. Inspired by spatial scan statistics for detecting clustering of disease occurrence in the field of epidemiology, we developed a scan statistic to extract disease-associated gene clusters from a whole gene pathway. Extracting one or a few significant gene clusters from a global pathway limits the overall false positive probability, which results in increased statistical power, and facilitates the interpretation of test results. In the present study, we applied our method to genome-wide association data for rare copy-number variations, which have been strongly implicated in common diseases. Application of our method to a simulated dataset demonstrated the high accuracy of this method in detecting disease-associated gene clusters in a whole gene pathway. Conclusions The scan statistic approach proposed here shows a high level of accuracy in detecting gene clusters in a whole gene pathway. This study has provided a sound statistical framework for analyzing genome-wide rare CNV data by incorporating topological information on the gene pathway.

Genetic Factors of the Disease Course After Sepsis: Rare Deleterious Variants Are Predictive

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Stefan Taudien

2016-10-01

Sepsis patients with favorable disease course after sepsis, even in the case of unfavorable preconditions, seem to be affected more often by rare deleterious SNVs in cell signaling and innate immunity related pathways, suggesting a protective role of impairments in these processes against a poor disease course.

[Rare diseases and their patient organization: the Hungarian Federation of People with Rare and Congenital Diseases].

Science.gov (United States)

Pogány, Gábor

2014-03-02

The aim of the author is to discuss special issues of rare diseases, with emphasis on circumstances present in Hungary, including those leading to the foundation of the non-governmental organization, the Hungarian Federation of People with Rare and Congenital Diseases. The author briefly reviews the most important findings of current international surveys which have been performed with or without the involvement of member associations of the Hungarian Federation of People with Rare and Congenital Diseases. At the level of medical and social services in Hungary, it is still "incidental" to get to the appropriate expert or centre providing the diagnosis or treatment. It is difficult to find the still very few existing services due to the lack of suitable "pathways" and referrals. There are long delays in obtaining the first appointment, resulting in vulnerability and inequality along the regions. The overall consequence is the insufficiency or lack of access to medical and social services. There are also difficulties related to the supply of orphan medication and the long duration of hospitalization. At the level of patient organizations financial scarcity and uncertainty are typical, combined with inappropriate infrastructural background and human resources. The poor quality of organization of patient bodies along with insufficient cooperation among them are characteristic as well. The author concludes that a National Plan or Strategy is needed to improve the current fragmentation of services which would enable patients and health, social and educational professionals to provide and use the best care in the practice. This would ensure all patients with rare diseases to be diagnosed within a possible shortest time allowing access to the care and support needed in time resulting in a decrease in burden of families and society.

Rare earth metals, rare earth hydrides, and rare earth oxides as thin films

International Nuclear Information System (INIS)

Gasgnier, M.

1980-01-01

The review deals with pure rare earth materials such as rare earth metals, rare earth hydrides, and rare earth oxides as thin films. Several preparation techniques, control methods, and nature of possible contaminations of thin films are described. These films can now be produced in an extremely well-known state concerning chemical composition, structure and texture. Structural, electric, magnetic, and optical properties of thin films are studied and discussed in comparison with the bulk state. The greatest contamination of metallic rare earth thin films is caused by reaction with hydrogen or with water vapour. The compound with an f.c.c. structure is the dihydride LnH 2 (Ln = lanthanides). The oxygen contamination takes place after annealing at higher temperatures. Then there appears a compound with a b.c.c. structure which is the C-type sesquioxide C-Ln 2 O 3 . At room atmosphere dihydride light rare earth thin films are converted to hydroxide Ln(OH) 3 . For heavy rare earth thin films the oxinitride LnNsub(x)Osub(y) is observed. The LnO-type compound was never seen. The present review tries to set the stage anew for the investigations to be undertaken in the future especially through the new generations of electron microscopes

Rare variants in RTEL1 are associated with familial interstitial pneumonia.

Science.gov (United States)

Cogan, Joy D; Kropski, Jonathan A; Zhao, Min; Mitchell, Daphne B; Rives, Lynette; Markin, Cheryl; Garnett, Errine T; Montgomery, Keri H; Mason, Wendi R; McKean, David F; Powers, Julia; Murphy, Elissa; Olson, Lana M; Choi, Leena; Cheng, Dong-Sheng; Blue, Elizabeth Marchani; Young, Lisa R; Lancaster, Lisa H; Steele, Mark P; Brown, Kevin K; Schwarz, Marvin I; Fingerlin, Tasha E; Schwartz, David A; Lawson, William E; Loyd, James E; Zhao, Zhongming; Phillips, John A; Blackwell, Timothy S

2015-03-15

Up to 20% of cases of idiopathic interstitial pneumonia cluster in families, comprising the syndrome of familial interstitial pneumonia (FIP); however, the genetic basis of FIP remains uncertain in most families. To determine if new disease-causing rare genetic variants could be identified using whole-exome sequencing of affected members from FIP families, providing additional insights into disease pathogenesis. Affected subjects from 25 kindreds were selected from an ongoing FIP registry for whole-exome sequencing from genomic DNA. Candidate rare variants were confirmed by Sanger sequencing, and cosegregation analysis was performed in families, followed by additional sequencing of affected individuals from another 163 kindreds. We identified a potentially damaging rare variant in the gene encoding for regulator of telomere elongation helicase 1 (RTEL1) that segregated with disease and was associated with very short telomeres in peripheral blood mononuclear cells in 1 of 25 families in our original whole-exome sequencing cohort. Evaluation of affected individuals in 163 additional kindreds revealed another eight families (4.7%) with heterozygous rare variants in RTEL1 that segregated with clinical FIP. Probands and unaffected carriers of these rare variants had short telomeres (RTEL1 function. Rare loss-of-function variants in RTEL1 represent a newly defined genetic predisposition for FIP, supporting the importance of telomere-related pathways in pulmonary fibrosis.

Structurally triggered metal-insulator transition in rare-earth nickelates.

Science.gov (United States)

Mercy, Alain; Bieder, Jordan; Íñiguez, Jorge; Ghosez, Philippe

2017-11-22

Rare-earth nickelates form an intriguing series of correlated perovskite oxides. Apart from LaNiO 3 , they exhibit on cooling a sharp metal-insulator electronic phase transition, a concurrent structural phase transition, and a magnetic phase transition toward an unusual antiferromagnetic spin order. Appealing for various applications, full exploitation of these compounds is still hampered by the lack of global understanding of the interplay between their electronic, structural, and magnetic properties. Here we show from first-principles calculations that the metal-insulator transition of nickelates arises from the softening of an oxygen-breathing distortion, structurally triggered by oxygen-octahedra rotation motions. The origin of such a rare triggered mechanism is traced back in their electronic and magnetic properties, providing a united picture. We further develop a Landau model accounting for the metal-insulator transition evolution in terms of the rare-earth cations and rationalizing how to tune this transition by acting on oxygen rotation motions.

Solid state chemistry of rare earth oxides. Final report, September 1, 1950--July 31, 1977

International Nuclear Information System (INIS)

Eyring, L.

1977-07-01

Work under Contract E(11-1)-1109 and its antecedents has been primarily for the purpose of obtaining detailed thermodynamic, kinetic and structural information on the complex rare earth oxides of praseodymium and terbium. These systems exhibit homologous series of ordered phases, order-disorder transformations, wide-range nonstoichiometric phases, chemical hysteresis in two-phase regions and many other solid state reaction phenomena. Fluorite-related materials of importance to ERDA occur as nuclear fuels, radiation power sources, insulators and solid electrolytes. The rare earth oxides serve directly as model systems for such similar materials and, in a more general sense, they serve as models of solids in general since they exhibit nearly the full range of solid state properties

Immersive Exhibitions

DEFF Research Database (Denmark)

Achiam, Marianne

2015-01-01

The immersive exhibition is a specialized exhibition genre in museums, which creates the illusion of time and place by representing key characteristics of a reference world and by integrating the visitor in this three-dimensionally reconstructed world (Mortensen 2010). A successful representation...... of the reference world depends on three criteria: whether the exhibition is staged as a coherent whole with all the displayed objects supporting the representation, whether the visitor is integrated as a component of the exhibition, and whether the content and message of the exhibition become dramatized...

Rare earth industry in India

International Nuclear Information System (INIS)

Singh, D.S.

2016-01-01

Rare Earths (RE) comprises of 17 elements i.e. elements from atomic No. 57-71 (lanthanide series) along with yttrium (atomic No. 39) and scandium (atomic No. 21). They exhibit special electronic, magnetic, optical and catalytic properties. The first 7 elements in the lanthanide series from atomic Nos. 57 to 63 (La to Eu) are called Light Rare Earths (LRE), while the remaining elements from atomic Nos. 64 to 71 (Gd to Lu) are grouped as Heavy Rare Earths (HRE). Scandium and Yttrium have properties similar to HRE. The concentration of the REs in the earth's crust is as high as some other elements including that of copper. The only difference is that REs do not occur as separate minerals amenable for easy exploration and mining and are widely distributed across the earth's surface, hence they are called as REs. Resources In India, monazite has been the principal source of RE. It occurs in association with other heavy minerals, such as ilmenite, rutile, zircon etc. in the beach sands and inland placer deposits. The monazite content in this assemblage varies from negligible quantity to as high as 5%. As per AMD resource estimation, the reported resource of monazite in India is about 11.93 million tons which corresponds with about 6.9 million tons of RE oxides. Although India possesses large deposits of monazite, the heavier RE are not present in sufficient quantities in this mineral. (author)

Monitoring the orientation of rare-earth-doped nanorods for flow shear tomography

NARCIS (Netherlands)

Kim, J.; Michelin, S.; Hilbers, M.; Martinelli, L.; Chaudan, E.; Amselem, G.; Fradet, E.; Boilot, J.-P.; Brouwer, A.M.; Baroud, C.N.; Peretti, J.; Gacoin, T.

Rare-earth phosphors exhibit unique luminescence polarization features originating from the anisotropic symmetry of the emitter ion's chemical environment. However, to take advantage of this peculiar property, it is necessary to control and measure the ensemble orientation of the host particles with

Gene regulatory and signaling networks exhibit distinct topological distributions of motifs

Science.gov (United States)

Ferreira, Gustavo Rodrigues; Nakaya, Helder Imoto; Costa, Luciano da Fontoura

2018-04-01

The biological processes of cellular decision making and differentiation involve a plethora of signaling pathways and gene regulatory circuits. These networks in turn exhibit a multitude of motifs playing crucial parts in regulating network activity. Here we compare the topological placement of motifs in gene regulatory and signaling networks and observe that it suggests different evolutionary strategies in motif distribution for distinct cellular subnetworks.

Fish and phytoplankton exhibit contrasting temporal species abundance patterns in a dynamic north temperate lake.

Directory of Open Access Journals (Sweden)

Gretchen J A Hansen

Full Text Available Temporal patterns of species abundance, although less well-studied than spatial patterns, provide valuable insight to the processes governing community assembly. We compared temporal abundance distributions of two communities, phytoplankton and fish, in a north temperate lake. We used both 17 years of observed relative abundance data as well as resampled data from Monte Carlo simulations to account for the possible effects of non-detection of rare species. Similar to what has been found in other communities, phytoplankton and fish species that appeared more frequently were generally more abundant than rare species. However, neither community exhibited two distinct groups of "core" (common occurrence and high abundance and "occasional" (rare occurrence and low abundance species. Both observed and resampled data show that the phytoplankton community was dominated by occasional species appearing in only one year that exhibited large variation in their abundances, while the fish community was dominated by core species occurring in all 17 years at high abundances. We hypothesize that the life-history traits that enable phytoplankton to persist in highly dynamic environments may result in communities dominated by occasional species capable of reaching high abundances when conditions allow. Conversely, longer turnover times and broad environmental tolerances of fish may result in communities dominated by core species structured primarily by competitive interactions.

Fish and Phytoplankton Exhibit Contrasting Temporal Species Abundance Patterns in a Dynamic North Temperate Lake

Science.gov (United States)

Hansen, Gretchen J. A.; Carey, Cayelan C.

2015-01-01

Temporal patterns of species abundance, although less well-studied than spatial patterns, provide valuable insight to the processes governing community assembly. We compared temporal abundance distributions of two communities, phytoplankton and fish, in a north temperate lake. We used both 17 years of observed relative abundance data as well as resampled data from Monte Carlo simulations to account for the possible effects of non-detection of rare species. Similar to what has been found in other communities, phytoplankton and fish species that appeared more frequently were generally more abundant than rare species. However, neither community exhibited two distinct groups of “core” (common occurrence and high abundance) and “occasional” (rare occurrence and low abundance) species. Both observed and resampled data show that the phytoplankton community was dominated by occasional species appearing in only one year that exhibited large variation in their abundances, while the fish community was dominated by core species occurring in all 17 years at high abundances. We hypothesize that the life-history traits that enable phytoplankton to persist in highly dynamic environments may result in communities dominated by occasional species capable of reaching high abundances when conditions allow. Conversely, longer turnover times and broad environmental tolerances of fish may result in communities dominated by core species structured primarily by competitive interactions. PMID:25651399

Rare-Earth Tantalates and Niobates Single Crystals: Promising Scintillators and Laser Materials

Directory of Open Access Journals (Sweden)

Renqin Dou

2018-01-01

Full Text Available Rare-earth tantalates, with high density and monoclinic structure, and niobates with monoclinic structure have been paid great attention as potential optical materials. In the last decade, we focused on the crystal growth technology of rare-earth tantalates and niobates and studied their luminescence and physical properties. A series of rare-earth tantalates and niobates crystals have been grown by the Czochralski method successfully. In this work, we summarize the research results on the crystal growth, scintillation, and laser properties of them, including the absorption and emission spectra, spectral parameters, energy levels structure, and so on. Most of the tantalates and niobates exhibit excellent luminescent properties, rich physical properties, and good chemical stability, indicating that they are potential outstanding scintillators and laser materials.

Rare Earth Garnet Selective Emitter

Science.gov (United States)

Lowe, Roland A.; Chubb, Donald L.; Farmer, Serene C.; Good, Brian S.

1994-01-01

Thin film Ho-YAG and Er-YAG emitters with a platinum substrate exhibit high spectral emittance in the emission band (epsilon(sub lambda) approx. = 0.75, sup 4)|(sub 15/2) - (sup 4)|(sub 13/2),for Er-YAG and epsilon(sub lambda) approx. = 0.65, (sup 5)|(sub 7) - (sup 5)|(sub 8) for Ho-YAG) at 1500 K. In addition, low out-of-band spectral emittance, epsilon(sub lambda) less than 0.2, suggest these materials would be excellent candidates for high efficiency selective emitters in thermophotovoltaic (TPV) systems operating at moderate temperatures (1200-1500 K). Spectral emittance measurements of the thin films were made (1.2 less than lambda less than 3.0 microns) and compared to the theoretical emittances calculated using measured values of the spectral extinction coefficient. In this paper we present the results for a new class of rare earth ion selective emitters. These emitters are thin sections (less than 1 mm) of yttrium aluminum garnet (YAG) single crystal with a rare earth substitutional impurity. Selective emitters in the near IR are of special interest for thermophotovoltaic (TPV) energy conversion. The most promising solid selective emitters for use in a TPV system are rare earth oxides. Early spectral emittance work on rare earth oxides showed strong emission bands in the infrared (0.9 - 3 microns). However, the emittance outside the emission band was also significant and the efficiency of these emitters was low. Recent improvements in efficiency have been made with emitters fabricated from fine (5 - 10 microns) rare earth oxide fibers similar to the Welsbach mantle used in gas lanterns. However, the rare earth garnet emitters are more rugged than the mantle type emitters. A thin film selective emitter on a low emissivity substrate such as gold, platinum etc., is rugged and easily adapted to a wide variety of thermal sources. The garnet structure and its many subgroups have been successfully used as hosts for rare earth ions, introduced as substitutional

Successful catheter ablation of a left anterior accessory pathway from the non-coronary cusp of the aortic valve.

Science.gov (United States)

Laranjo, Sérgio; Oliveira, Mário; Trigo, Conceição

2015-08-01

Left anterior accessory pathways are considered to be rare findings. Catheter ablation of accessory pathways in this location remains a challenging target, and few reports about successful ablation of these accessory pathways are available. We describe our experience regarding a case of a manifest left anterior accessory pathway ablation using radiofrequency energy at the junction of the left coronary cusp with the non-coronary cusp.

Origin of planetary primordial rare gas - The possible role of adsorption.

Science.gov (United States)

Fanale, F. P.; Cannon, W. A.

1972-01-01

The degree of physical adsorption of Ne, Ar, Kr, and Xe on pulverized samples of the Allende meteorite at 113 K has been measured. The observed pattern of equilibrium enrichment of heavy rare gases over light on the pulverized meteorite surfaces relative to the gas phase is similar to the enrichment pattern exhibited by planetary primordial rare gas when compared with the composition of solar rare gas. Results indicate that, at 113 K, a total nebular pressure of from .01 to .001 atm would be required to explain the Ar, Kr, and Xe abundances in carbonaceous chondrites with an adsorption mechanism. This pressure estimate is compatible with the range of possible nebular pressures suggested by astrophysical arguments. However, the subsequent mechanism by which initially adsorbed gas might have been transferred into the interiors of grains cannot be identified at present.

A journey through the lectin pathway of complement-MBL and beyond

DEFF Research Database (Denmark)

Garred, Peter; Genster, Ninette; Pilely, Katrine

2016-01-01

, and Carnevale) embryonic development syndrome originates from rare mutations affecting either collectin-11 or MASP-3, indicating a broader functionality of the complement system than previously anticipated. This review summarizes the characteristics of the molecules in the lectin pathway....

Exhibit Engineering

DEFF Research Database (Denmark)

Mortensen, Marianne Foss

Science museums define the objectives of their exhibitions in terms of visitor learning outcomes. Yet, exhibit designers lack theoretical and empirical research findings on which to base the creation of such educational environments. Here, this shortcoming is addressed through the development...... of tools and processes to guide the design of educational science exhibits. The guiding paradigm for this development is design-based research, which is characterised by an iterative cycle of design, enactment, and analysis. In the design phase, an educational intervention is planned and carried out based...... on the generation of theoretical ideas for exhibit design is offered in a fourth and parallel research undertaking, namely the application of the notion of cultural border-crossing to a hypothetical case of exhibit design....

Trans-ancestry meta-analyses identify rare and common variants associated with blood pressure and hypertension

NARCIS (Netherlands)

Surendran, P. (Praveen); F. Drenos (Fotios); R. Young (Robin); H. Warren (Helen); Cook, J.P. (James P.); A.K. Manning (Alisa); N. Grarup (Niels); X. Sim (Xueling); D. Barnes (Daniel); H.E. Witkowska (Ewa); J.R. Staley (James R.); V. Tragante (Vinicius); T. Tukiainen (Taru); H. Yaghootkar (Hanieh); Masca, N. (Nicholas); C.M. Freitag (Christine); T. Ferreira (Teresa); O. Giannakopoulou (Olga); Tinker, A. (Andrew); M. Harakalova (Magdalena); E. Mihailov (Evelin); Liu, C. (Chunyu); A. Kraja (Aldi); S.F. Nielsen (Sune); A. Rasheed (Asif); M. Samuel (Maria); W. Zhao (Wei); L.L. Bonnycastle (Lori); A.U. Jackson (Anne); N. Narisu (Narisu); A.J. Swift (Amy); L. Southam (Lorraine); J. Marten (Jonathan); J.R. Huyghe (Jeroen R.); A. Stancáková (Alena); C. Fava (Cristiano); Ohlsson, T. (Therese); A. Matchan (Angela); K. Stirrups (Kathy); J. Bork-Jensen (Jette); A.P. Gjesing (Anette); Kontto, J. (Jukka); M. Perola (Markus); S. Shaw-Hawkins (Sue); A.S. Havulinna (Aki); Zhang, H. (He); L.A. Donnelly (Louise); C.J. Groves (Christopher); N.W. Rayner (Nigel William); M.J. Neville (Matthew); N.R. Robertson (Neil); Yiorkas, A.M. (Andrianos M.); K.H. Herzig; E. Kajantie (Eero); W. Zhang (Weihua); S.M. Willems (Sara); L. Lannfelt (Lars); G. Malerba (Giovanni); N. Soranzo (Nicole); E. Trabetti (Elisabetta); N. Verweij (Niek); E. Evangelou (Evangelos); A. Moayyeri (Alireza); Vergnaud, A.-C. (Anne-Claire); C.P. Nelson (Christopher P.); Poveda, A. (Alaitz); T.V. Varga (Tibor V.); M. Caslake (Muriel); A.J.M. De Craen (Anton J. M.); S. Trompet (Stella); J. Luan (Jian'An); R.A. Scott (Robert); S.E. Harris (Sarah); D.C. Liewald (David C.); R.E. Marioni (Riccardo); C. Menni (Cristina); A.-E. Farmaki (Aliki-Eleni); G. Hallmans (Göran); F. Renström (Frida); J.E. Huffman (Jennifer); Hassinen, M. (Maija); S. Burgess (Stephen); Vasan, R.S. (Ramachandran S.); J.F. Felix (Janine); Uria-Nickelsen, M. (Maria); A. Mälarstig (Anders); Reilly, D.F. (Dermot F.); Hoek, M. (Maarten); Vogt, T.F. (Thomas F.); H. Lin (Honghuang); W. Lieb (Wolfgang); M. Traylor (Matthew); H.S. Markus (Hugh); H. Highland (Heather); A.E. Justice (Anne); E. Marouli (Eirini); J. Lindström (Jaana); M. Uusitupa (Matti); P. Komulainen (Pirjo); T.A. Lakka (Timo); R. Rauramaa (Rainer); O. Polasek (Ozren); I. Rudan (Igor); Rolandsson, O. (Olov); P.W. Franks (Paul); G.V. Dedoussis (George); T.D. Spector (Timothy); P. Jousilahti (Pekka); S. Männistö (Satu); I.J. Deary (Ian J.); J.M. Starr (John); C. Langenberg (Claudia); N.J. Wareham (Nick); M.J. Brown (Morris); A. Dominiczak (Anna); Connell, J.M. (John M.); J.W. Jukema (Jan Wouter); N. Sattar (Naveed); I. Ford (Ian); Packard, C.J. (Chris J.); T. Esko (Tõnu); R. Mägi (Reedik); A. Metspalu (Andres); R.A. de Boer (Rudolf); Van Der Meer, P. (Peter); P. van der Harst (Pim); G. Gambaro (Giovanni); Ingelsson, E. (Erik); W.H.L. Kao (Wen); P.I.W. de Bakker (Paul); M.E. Numans (Mattijs); I. Brandslund (Ivan); Christensen, C. (Cramer); Petersen, E.R.B. (Eva R. B.); E. Korpi-Hyövälti (Eeva); H. Oksa (Heikki); J.C. Chambers (John); J.S. Kooner (Jaspal S.); A.I.F. Blakemore (Alexandra); S. Franks (Steve); M.-R. Jarvelin (Marjo-Riitta); L.L.N. Husemoen (Lise Lotte); Linneberg, A. (Allan); T. Skaaby (Tea); Thuesen, B. (Betina); F. Karpe (Fredrik); J. Tuomilehto (Jaakko); A.S.F. Doney (Alex); A.D. Morris (Andrew); C.N.A. Palmer (Colin); O.L. Holmen (Oddgeir); K. Hveem (Kristian); C.J. Willer (Cristen); T. Tuomi (Tiinamaija); L. Groop (Leif); Käräjämäki, A. (Annemari); A. Palotie (Aarno); S. Ripatti (Samuli); V. Salomaa (Veikko); D.S. Alam (Dewan S.); Majumder, A.A.S. (Abdulla Al Shafi); E. di Angelantonio (Emanuele); R. Chowdhury (Rajiv); M.I. McCarthy (Mark); N.R. Poulter (Neil); A. Stanton (Alice); P. Sever (Peter); P. Amouyel (Philippe); D. Arveiler (Dominique); Blankenberg, S. (Stefan); J. Ferrieres (Jean); F. Kee (Frank); K. Kuulasmaa (Kari); M. Müller-Nurasyid (Martina); G. Veronesi (Giovanni); J. Virtamo (Jarmo); P. Deloukas (Panagiotis); P. Elliott (Paul); E. Zeggini (Eleftheria); S. Kathiresan (Sekar); O. Melander (Olle); J. Kuusisto (Johanna); M. Laakso (Markku); S. Padmanabhan (Sandosh); D. Porteous (David); C. Hayward (Caroline); G. Scotland (Generation); F.S. Collins (Francis); K.L. Mohlke (Karen); T. Hansen (T.); O. Pedersen (Oluf); M. Boehnke (Michael); H.M. Stringham (Heather); R. Frossard; C. Newton-Cheh (Christopher); M.D. Tobin (Martin); B.G. Nordestgaard (Børge); M. Caulfield (Mark); A. Mahajan (Anubha); A.P. Morris (Andrew); Tomaszewski, M. (Maciej); N.J. Samani (Nilesh); Saleheen, D. (Danish); F.W. Asselbergs (Folkert); C.M. Lindgren (Cecilia M.); J. Danesh (John); Wain, L.V. (Louise V.); A.S. Butterworth (Adam); Howson, J.M.M. (Joanna M. M.); P. Munroe (Patricia)

2016-01-01

textabstractHigh blood pressure is a major risk factor for cardiovascular disease and premature death. However, there is limited knowledge on specific causal genes and pathways. To better understand the genetics of blood pressure, we genotyped 242,296 rare, low-frequency and common genetic variants

Preparation and characterization of rare-earth bulks with controllable nanostructures

International Nuclear Information System (INIS)

Song Xiaoyan; Zhang Jiuxing; Li Erdong; Lu Nianduan; Yin Fuxing

2006-01-01

The preparation and characterization of pure rare-earth-metal bulks with controllable nanostructures are reported in this paper. A novel 'oxygen-free' in situ synthesis technique that combines inert-gas condensation with spark plasma sintering (SPS) technology is proposed. Taking into account the special mechanisms of SPS consolidation and the scale effects of nanoparticles, we introduced practical procedures for preparing rare-earth bulks of amorphous, mixed amorphous and nanocrystals, and nanocrystalline microstructures, respectively. Compared with the conventional polycrystalline bulk, these nanostructured bulks exhibit substantially improved physical and mechanical properties. This technique enables comprehensive studies on the microstructures and properties of a large variety of nanostructured metallic materials that are highly reactive in the air

Analysis of genes involved in the PI3K/Akt pathway in radiation- and MNU-induced rat mammary carcinomas.

Science.gov (United States)

Showler, Kaye; Nishimura, Mayumi; Daino, Kazuhiro; Imaoka, Tatsuhiko; Nishimura, Yukiko; Morioka, Takamitsu; Blyth, Benjamin J; Kokubo, Toshiaki; Takabatake, Masaru; Fukuda, Maki; Moriyama, Hitomi; Kakinuma, Shizuko; Fukushi, Masahiro; Shimada, Yoshiya

2017-03-01

The PI3K/AKT pathway is one of the most important signaling networks in human breast cancer, and since it was potentially implicated in our preliminary investigations of radiation-induced rat mammary carcinomas, our aim here was to verify its role. We included mammary carcinomas induced by the chemical carcinogen 1-methyl-1-nitrosourea to determine whether any changes were radiation-specific. Most carcinomas from both groups showed activation of the PI3K/AKT pathway, but phosphorylation of AKT1 was often heterogeneous and only present in a minority of carcinoma cells. The negative pathway regulator Inpp4b was significantly downregulated in both groups, compared with in normal mammary tissue, and radiation-induced carcinomas also showed a significant decrease in Pten expression, while the chemically induced carcinomas showed a decrease in Pik3r1 and Pdk1. Significant upregulation of the positive regulators Erbb2 and Pik3ca was observed only in chemically induced carcinomas. However, no genes showed clear correlations with AKT phosphorylation levels, except in individual carcinomas. Only rare carcinomas showed mutations in PI3K/AKT pathway genes, yet these carcinomas did not exhibit stronger AKT phosphorylation. Thus, while AKT phosphorylation is a common feature of rat mammary carcinomas induced by radiation or a canonical chemical carcinogen, the mutation of key genes in the pathways or permanent changes to gene expression of particular signaling proteins do not explain the pathway activation in the advanced cancers. Although AKT signaling likely facilitates cancer development and growth in rat mammary carcinomas, it is unlikely that permanent disruption of the PI3K/AKT pathway genes is a major causal event in radiation carcinogenesis. © The Author 2016. Published by Oxford University Press on behalf of The Japan Radiation Research Society and Japanese Society for Radiation Oncology.

Integrated Transitions of Care for Patients With Rare Pulmonary Diseases.

Science.gov (United States)

Moreo, Kathleen; Lattimer, Cheri; Lett, James E; Heggen-Peay, Cherilyn L; Simone, Laura

Many continuing education (CE) resources are available to support case management professionals in developing competencies in transitions of care (TOC) that apply generally across disease areas. However, CE programs and tools are lacking for advanced TOC competencies in specific disease areas. This article describes 2 projects in which leading TOC, case management, and CE organizations collaborated to develop CE-accredited interdisciplinary pathways for promoting safe and effective TOC for patients with rare pulmonary diseases, including pulmonary arterial hypertension (PAH) and idiopathic pulmonary fibrosis (IPF). The interdisciplinary pathways apply to PAH and IPF case management practice and TOC across settings that include community-based primary care and specialty care, PAH or IPF centers of expertise, acute care and post-acute settings, long-term care, rehabilitation and skilled nursing facilities, and patients' homes. Both PAH and IPF are chronic, progressive respiratory diseases that are associated with severe morbidity and mortality, along with high health care costs. Because they are relatively rare diseases with nonspecific symptoms and many comorbidities, PAH and IPF are difficult to diagnose. Early diagnosis, referral to centers of expertise, and aggressive treatment initiation are essential for slowing disease progression and maintaining quality of life and function. Both the rarity and complexity of PAH and IPF pose unique challenges to ensuring effective and safe TOC. Expert consensus and evidence-based approaches to meeting these challenges, and thereby improving PAH and IPF patient outcomes, are presented in the 2 interdisciplinary TOC pathways that are described in this article. In coordinating care for patients with complex pulmonary diseases such as PAH and IPF, case managers across practice settings can play key roles in improving workflow processes and communication, transition planning, coordinating TOC with centers of expertise

RAS signalling in energy metabolism and rare human diseases.

Science.gov (United States)

Dard, L; Bellance, N; Lacombe, D; Rossignol, R

2018-05-08

The RAS pathway is a highly conserved cascade of protein-protein interactions and phosphorylation that is at the heart of signalling networks that govern proliferation, differentiation and cell survival. Recent findings indicate that the RAS pathway plays a role in the regulation of energy metabolism via the control of mitochondrial form and function but little is known on the participation of this effect in RAS-related rare human genetic diseases. Germline mutations that hyperactivate the RAS pathway have been discovered and linked to human developmental disorders that are known as RASopathies. Individuals with RASopathies, which are estimated to affect approximately 1/1000 human birth, share many overlapping characteristics, including cardiac malformations, short stature, neurocognitive impairment, craniofacial dysmorphy, cutaneous, musculoskeletal, and ocular abnormalities, hypotonia and a predisposition to developing cancer. Since the identification of the first RASopathy, type 1 neurofibromatosis (NF1), which is caused by the inactivation of neurofibromin 1, several other syndromes have been associated with mutations in the core components of the RAS-MAPK pathway. These syndromes include Noonan syndrome (NS), Noonan syndrome with multiple lentigines (NSML), which was formerly called LEOPARD syndrome, Costello syndrome (CS), cardio-facio-cutaneous syndrome (CFC), Legius syndrome (LS) and capillary malformation-arteriovenous malformation syndrome (CM-AVM). Here, we review current knowledge about the bioenergetics of the RASopathies and discuss the molecular control of energy homeostasis and mitochondrial physiology by the RAS pathway. Copyright © 2018 Elsevier B.V. All rights reserved.

Stone formation in peach fruit exhibits spatial coordination of the lignin and flavonoid pathways and similarity to Arabidopsis dehiscence

Directory of Open Access Journals (Sweden)

Piagnani M Claudia

2010-02-01

Full Text Available Abstract Background Lignification of the fruit endocarp layer occurs in many angiosperms and plays a critical role in seed protection and dispersal. This process has been extensively studied with relationship to pod shatter or dehiscence in Arabidopsis. Dehiscence is controlled by a set of transcription factors that define the fruit tissue layers and whether or not they lignify. In contrast, relatively little is known about similar processes in other plants such as stone fruits which contain an extremely hard lignified endocarp or stone surrounding a single seed. Results Here we show that lignin deposition in peach initiates near the blossom end within the endocarp layer and proceeds in a distinct spatial-temporal pattern. Microarray studies using a developmental series from young fruits identified a sharp and transient induction of phenylpropanoid, lignin and flavonoid pathway genes concurrent with lignification and subsequent stone hardening. Quantitative polymerase chain reaction studies revealed that specific phenylpropanoid (phenylalanine ammonia-lyase and cinnamate 4-hydroxylase and lignin (caffeoyl-CoA O-methyltransferase, peroxidase and laccase pathway genes were induced in the endocarp layer over a 10 day time period, while two lignin genes (p-coumarate 3-hydroxylase and cinnamoyl CoA reductase were co-regulated with flavonoid pathway genes (chalcone synthase, dihydroflavanol 4-reductase, leucoanthocyanidin dioxygen-ase and flavanone-3-hydrosylase which were mesocarp and exocarp specific. Analysis of other fruit development expression studies revealed that flavonoid pathway induction is conserved in the related Rosaceae species apple while lignin pathway induction is not. The transcription factor expression of peach genes homologous to known endocarp determinant genes in Arabidopsis including SHATTERPROOF, SEEDSTCK and NAC SECONDARY WALL THICENING PROMOTING FACTOR 1 were found to be specifically expressed in the endocarp while the

Stress and DNA repair biology of the Fanconi anemia pathway

Science.gov (United States)

Longerich, Simonne; Li, Jian; Xiong, Yong; Sung, Patrick

2014-01-01

Fanconi anemia (FA) represents a paradigm of rare genetic diseases, where the quest for cause and cure has led to seminal discoveries in cancer biology. Although a total of 16 FA genes have been identified thus far, the biochemical function of many of the FA proteins remains to be elucidated. FA is rare, yet the fact that 5 FA genes are in fact familial breast cancer genes and FA gene mutations are found frequently in sporadic cancers suggest wider applicability in hematopoiesis and oncology. Establishing the interaction network involving the FA proteins and their associated partners has revealed an intersection of FA with several DNA repair pathways, including homologous recombination, DNA mismatch repair, nucleotide excision repair, and translesion DNA synthesis. Importantly, recent studies have shown a major involvement of the FA pathway in the tolerance of reactive aldehydes. Moreover, despite improved outcomes in stem cell transplantation in the treatment of FA, many challenges remain in patient care. PMID:25237197

Trans-ancestry meta-analyses identify rare and common variants associated with blood pressure and hypertension

NARCIS (Netherlands)

Surendran, Praveen; Drenos, Fotios; Young, Robin; Warren, Helen; Cook, James P; Manning, Alisa K; Grarup, Niels; Sim, Xueling; Barnes, Daniel R; Witkowska, Kate; Staley, James R; Tragante, Vinicius; Tukiainen, Taru; Yaghootkar, Hanieh; Masca, Nicholas; Freitag, Daniel F; Ferreira, Teresa; Giannakopoulou, Olga; Tinker, Andrew; Harakalova, Magdalena; Mihailov, Evelin; Liu, Chunyu; Kraja, Aldi T; Nielsen, Sune Fallgaard; Rasheed, Asif; Samuel, Maria; Zhao, Wei; Bonnycastle, Lori L; Jackson, Anne U; Narisu, Narisu; Swift, Amy J; Southam, Lorraine; Marten, Jonathan; Huyghe, Jeroen R; Stančáková, Alena; Fava, Cristiano; Ohlsson, Therese; Matchan, Angela; Stirrups, Kathleen E; Bork-Jensen, Jette; Gjesing, Anette P; Kontto, Jukka; Perola, Markus; Shaw-Hawkins, Susan; Havulinna, Aki S; Zhang, He; Donnelly, Louise A; Groves, Christopher J; Rayner, N William; Neville, Matt J; Robertson, Neil R; Yiorkas, Andrianos M; Herzig, Karl-Heinz; Kajantie, Eero; Zhang, Weihua; Willems, Sara M; Lannfelt, Lars; Malerba, Giovanni; Soranzo, Nicole; Trabetti, Elisabetta; Verweij, Niek; Evangelou, Evangelos; Moayyeri, Alireza; Vergnaud, Anne-Claire; Nelson, Christopher P; Poveda, Alaitz; Varga, Tibor V; Caslake, Muriel; de Craen, Anton J M; Trompet, Stella; Luan, Jian'an; Scott, Robert A; Harris, Sarah E; Liewald, David C M; Marioni, Riccardo; Menni, Cristina; Farmaki, Aliki-Eleni; Hallmans, Göran; Renström, Frida; Huffman, Jennifer E; Hassinen, Maija; Burgess, Stephen; Vasan, Ramachandran S; Felix, Janine F; Uria-Nickelsen, Maria; Malarstig, Anders; Reilly, Dermot F; Hoek, Maarten; Vogt, Thomas F; Lin, Honghuang; Lieb, Wolfgang; Traylor, Matthew; Markus, Hugh S; Highland, Heather M; Justice, Anne E; Marouli, Eirini; Lindström, Jaana; Uusitupa, Matti; Komulainen, Pirjo; Lakka, Timo A; Rauramaa, Rainer; Polasek, Ozren; Rudan, Igor; Rolandsson, Olov; Franks, Paul W; Dedoussis, George; Spector, Timothy D; Jousilahti, Pekka; Männistö, Satu; Deary, Ian J; Starr, John M; Langenberg, Claudia; Wareham, Nick J; Brown, Morris J; Dominiczak, Anna F; Connell, John M; Jukema, J Wouter; Sattar, Naveed; Ford, Ian; Packard, Chris J; Esko, Tõnu; Mägi, Reedik; Metspalu, Andres; de Boer, Rudolf A; van der Meer, Peter; van der Harst, Pim; Gambaro, Giovanni; Ingelsson, Erik; Lind, Lars; de Bakker, Paul I W; Numans, Mattijs E; Brandslund, Ivan; Christensen, Cramer; Petersen, Eva R B; Korpi-Hyövälti, Eeva; Oksa, Heikki; Chambers, John C; Kooner, Jaspal S; Blakemore, Alexandra I F; Franks, Steve; Jarvelin, Marjo-Riitta; Husemoen, Lise L; Linneberg, Allan; Skaaby, Tea; Thuesen, Betina; Karpe, Fredrik; Tuomilehto, Jaakko; Doney, Alex S F; Morris, Andrew D; Palmer, Colin N A; Holmen, Oddgeir Lingaas; Hveem, Kristian; Willer, Cristen J; Tuomi, Tiinamaija; Groop, Leif; Käräjämäki, AnneMari; Palotie, Aarno; Ripatti, Samuli; Salomaa, Veikko; Alam, Dewan S; Majumder, Abdulla Al Shafi; Di Angelantonio, Emanuele; Chowdhury, Rajiv; McCarthy, Mark I; Poulter, Neil; Stanton, Alice V; Sever, Peter; Amouyel, Philippe; Arveiler, Dominique; Blankenberg, Stefan; Ferrières, Jean; Kee, Frank; Kuulasmaa, Kari; Müller-Nurasyid, Martina; Veronesi, Giovanni; Virtamo, Jarmo; Deloukas, Panos; Elliott, Paul; Zeggini, Eleftheria; Kathiresan, Sekar; Melander, Olle; Kuusisto, Johanna; Laakso, Markku; Padmanabhan, Sandosh; Porteous, David J; Hayward, Caroline; Scotland, Generation; Collins, Francis S; Mohlke, Karen L; Hansen, Torben; Pedersen, Oluf; Boehnke, Michael; Stringham, Heather M; Frossard, Philippe; Newton-Cheh, Christopher; Tobin, Martin D; Nordestgaard, Børge Grønne; Caulfield, Mark J; Mahajan, Anubha; Morris, Andrew P; Tomaszewski, Maciej; Samani, Nilesh J; Saleheen, Danish; Asselbergs, Folkert W; Lindgren, Cecilia M; Danesh, John; Wain, Louise V; Butterworth, Adam S; Howson, Joanna M M; Munroe, Patricia B

2016-01-01

High blood pressure is a major risk factor for cardiovascular disease and premature death. However, there is limited knowledge on specific causal genes and pathways. To better understand the genetics of blood pressure, we genotyped 242,296 rare, low-frequency and common genetic variants in up to

Trans-ancestry meta-analyses identify rare and common variants associated with blood pressure and hypertension

DEFF Research Database (Denmark)

Surendran, Praveen; Drenos, Fotios; Young, Robin

2016-01-01

High blood pressure is a major risk factor for cardiovascular disease and premature death. However, there is limited knowledge on specific causal genes and pathways. To better understand the genetics of blood pressure, we genotyped 242,296 rare, low-frequency and common genetic variants in up to ...

Trans-ancestry meta-analyses identify rare and common variants associated with blood pressure and hypertension

NARCIS (Netherlands)

Surendran, Praveen; Drenos, Fotios; Young, Robin; Warren, Helen; Cook, James P.; Manning, Alisa K.; Grarup, Niels; Sim, Xueling; Barnes, Daniel R.; Witkowska, Kate; Staley, James R.; Tragante, Vinicius; Tukiainen, Taru; Yaghootkar, Hanieh; Masca, Nicholas; Freitag, Daniel F.; Ferreira, Teresa; Giannakopoulou, Olga; Tinker, Andrew; Harakalova, Magdalena; Mihailov, Evelin; Liu, Chunyu; Kraja, Aldi T.; Nielsen, Sune Fallgaard; Rasheed, Asif; Samue, Maria; Zhao, Wei; Bonnycastle, Lori L.; Jackson, Anne U.; Narisu, Narisu; Swift, Amy J.; Southam, Lorraine; Marten, Jonathan; Huyghe, Jeroen R.; Stancakova, Alena; Fava, Cristiano; Ohlsson, Therese; Matchan, Angela; Stirrups, Kathleen E.; Bork-Jensen, Jette; Gjesing, Anette P.; Kontto, Jukka; Perola, Markus; Shaw-Hawkins, Susan; Havulinna, Aki S.; Verweij, Niek; de Boer, Rudolf A.; van der Meer, Peter; van der Harst, Pim; Asselbergs, Folkert W.

2016-01-01

High blood pressure is a major risk factor for cardiovascular disease and premature death. However, there is limited knowledge on specific causal genes and pathways. To better understand the genetics of blood pressure, we genotyped 242,296 rare, low frequency and common genetic variants in up to

Rare earths

Energy Technology Data Exchange (ETDEWEB)

Cranstone, D A

1979-01-01

Rare earth elements are commonly extracted from the minerals monazite, bastnaesite, and xenotine. New uses for these elements are constantly developing; they have found applications in glass polishing, television tube phosphors, high-strength low-alloy steels, magnets, catalysts, refractory ceramics, and hydrogen sponge alloys. In Canada, rare earths have been produced as byproducts of the uranium mining industry, but there was no production of rare earths in 1978 or 1979. The world sources of and markets for the rare earth elements are discussed.

Charge-order driven multiferroic and magneto-dielectric properties of rare earth manganates

International Nuclear Information System (INIS)

Serrao, Claudy Rayan; Sahu, Jyoti Ranjan; Ghosh, Anirban

2010-01-01

Charge-order driven magnetic ferroelectricity is shown to occur in several rare earth manganates of the general formula, Ln 1-x A x MnO 3 (Ln rare earth, A = alkaline earth). Charge-ordered manganates exhibit dielectric constant anomalies around the charge-ordering or the antiferromagnetic transition temperature. Magnetic fields have a marked effect on the dielectric properties of these compounds, indicating the presence of coupling between the magnetic and electrical order parameters. Magneto-dielectric properties are retained in small particles of the manganates. The observation of magneto-ferroelectricity in these manganates is in accordance with theoretical predictions. (author)

Technology Exhibition

Energy Technology Data Exchange (ETDEWEB)

Anon.

1979-09-15

Linked to the 25th Anniversary celebrations, an exhibition of some of CERN's technological achievements was opened on 22 June. Set up in a new 600 m{sup 2} Exhibition Hall on the CERN site, the exhibition is divided into eight technology areas — magnets, vacuum, computers and data handling, survey and alignment, radiation protection, beam monitoring and handling, detectors, and workshop techniques.

Macrolactone Nuiapolide, Isolated from a Hawaiian Marine Cyanobacterium, Exhibits Anti-Chemotactic Activity.

Science.gov (United States)

Mori, Shogo; Williams, Howard; Cagle, Davey; Karanovich, Kristopher; Horgen, F David; Smith, Roger; Watanabe, Coran M H

2015-10-09

A new bioactive macrolactone, nuiapolide (1) was identified from a marine cyanobacterium collected off the coast of Niihau, near Lehua Rock. The natural product exhibits anti-chemotactic activity at concentrations as low as 1.3 μM against Jurkat cells, cancerous T lymphocytes, and induces a G2/M phase cell cycle shift. Structural characterization of the natural product revealed the compound to be a 40-membered macrolactone with nine hydroxyl functional groups and a rare tert-butyl carbinol residue.

Macrolactone Nuiapolide, Isolated from a Hawaiian Marine Cyanobacterium, Exhibits Anti-Chemotactic Activity

OpenAIRE

Mori, Shogo; Williams, Howard; Cagle, Davey; Karanovich, Kristopher; Horgen, F. David; Smith, Roger; Watanabe, Coran M. H.

2015-01-01

A new bioactive macrolactone, nuiapolide (1) was identified from a marine cyanobacterium collected off the coast of Niihau, near Lehua Rock. The natural product exhibits anti-chemotactic activity at concentrations as low as 1.3 μM against Jurkat cells, cancerous T lymphocytes, and induces a G2/M phase cell cycle shift. Structural characterization of the natural product revealed the compound to be a 40-membered macrolactone with nine hydroxyl functional groups and a rare tert-butyl carbinol re...

Macrolactone Nuiapolide, Isolated from a Hawaiian Marine Cyanobacterium, Exhibits Anti-Chemotactic Activity

Directory of Open Access Journals (Sweden)

Shogo Mori

2015-10-01

Full Text Available A new bioactive macrolactone, nuiapolide (1 was identified from a marine cyanobacterium collected off the coast of Niihau, near Lehua Rock. The natural product exhibits anti-chemotactic activity at concentrations as low as 1.3 μM against Jurkat cells, cancerous T lymphocytes, and induces a G2/M phase cell cycle shift. Structural characterization of the natural product revealed the compound to be a 40-membered macrolactone with nine hydroxyl functional groups and a rare tert-butyl carbinol residue.

Novel and rare functional genomic variants in multiple autoimmune syndrome and Sjögren's syndrome.

Science.gov (United States)

Johar, Angad S; Mastronardi, Claudio; Rojas-Villarraga, Adriana; Patel, Hardip R; Chuah, Aaron; Peng, Kaiman; Higgins, Angela; Milburn, Peter; Palmer, Stephanie; Silva-Lara, Maria Fernanda; Velez, Jorge I; Andrews, Dan; Field, Matthew; Huttley, Gavin; Goodnow, Chris; Anaya, Juan-Manuel; Arcos-Burgos, Mauricio

2015-06-02

Multiple autoimmune syndrome (MAS), an extreme phenotype of autoimmune disorders, is a very well suited trait to tackle genomic variants of these conditions. Whole exome sequencing (WES) is a widely used strategy for detection of protein coding and splicing variants associated with inherited diseases. The DNA of eight patients affected by MAS [all of whom presenting with Sjögren's syndrome (SS)], four patients affected by SS alone and 38 unaffected individuals, were subject to WES. Filters to identify novel and rare functional (pathogenic-deleterious) homozygous and/or compound heterozygous variants in these patients and controls were applied. Bioinformatics tools such as the Human gene connectome as well as pathway and network analysis were applied to test overrepresentation of genes harbouring these variants in critical pathways and networks involved in autoimmunity. Eleven novel and rare functional variants were identified in cases but not in controls, harboured in: MACF1, KIAA0754, DUSP12, ICA1, CELA1, LRP1/STAT6, GRIN3B, ANKLE1, TMEM161A, and FKRP. These were subsequently subject to network analysis and their functional relatedness to genes already associated with autoimmunity was evaluated. Notably, the LRP1/STAT6 novel mutation was homozygous in one MAS affected patient and heterozygous in another. LRP1/STAT6 disclosed the strongest plausibility for autoimmunity. LRP1/STAT6 are involved in extracellular and intracellular anti-inflammatory pathways that play key roles in maintaining the homeostasis of the immune system. Further; networks, pathways, and interaction analyses showed that LRP1 is functionally related to the HLA-B and IL10 genes and it has a substantial impact within immunological pathways and/or reaction to bacterial and other foreign proteins (phagocytosis, regulation of phospholipase A2 activity, negative regulation of apoptosis and response to lipopolysaccharides). Further, ICA1 and STAT6 were also closely related to AIRE and IRF5, two very

The volatile profiles of a rare apple (Malus domestica Borkh.) honey: shikimic acid-pathway derivatives, terpenes, and others.

Science.gov (United States)

Kuś, Piotr Marek; Jerković, Igor; Tuberoso, Carlo Ignazio Giovanni; Šarolić, Mladenka

2013-09-01

The volatile profiles of rare Malus domestica Borkh. honey were investigated for the first time. Two representative samples from Poland (sample I) and Spain (sample II) were selected by pollen analysis (44-45% of Malus spp. pollen) and investigated by GC/FID/MS after headspace solid-phase microextraction (HS-SPME) and ultrasonic solvent extraction (USE). The apple honey is characterized by high percentage of shikimic acid-pathway derivatives, as well as terpenes, norisoprenoids, and some other compounds such as coumaran and methyl 1H-indole-3-acetate. The main compounds of the honey headspace were (sample I; sample II): benzaldehyde (9.4%; 32.1%), benzyl alcohol (0.3%; 14.4%), hotrienol (26.0%, 6.2%), and lilac aldehyde isomers (26.3%; 1.7%), but only Spanish sample contained car-2-en-4-one (10.2%). CH2 Cl2 and pentane/Et2 O 1 : 2 (v/v) were used for USE. The most relevant compounds identified in the extracts were: benzaldehyde (0.9-3.9%), benzoic acid (2.0-11.2%), terpendiol I (0.3-7.4%), coumaran (0.0-2.8%), 2-phenylacetic acid (2.0-26.4%), methyl syringate (3.9-13.1%), vomifoliol (5.0-31.8%), and methyl 1H-indole-3-acetate (1.9-10.2%). Apple honey contained also benzyl alcohol, 2-phenylethanol, (E)-cinnamaldehyde, (E)-cinnamyl alcohol, eugenol, vanillin, and linalool that have been found previously in apple flowers, thus disclosing similarity of both volatile profiles. Copyright © 2013 Verlag Helvetica Chimica Acta AG, Zürich.

Robinow Syndrome: A Rare Case Report and Review of Literature

OpenAIRE

Soman, Cristalle; Lingappa, Ashok

2015-01-01

ABSTRACT Robinow syndrome is an extremely rare genetic disorder. Short-limbed dwarfism, abnormalities in the head, face, and external genitalia, as well as vertebral defects comprise its distinct features. This disorder exists in dominant and recessive patterns. Patients with the dominant pattern exhibit moderate symptoms. More physical characteristics and skeletal abnormalities characterize the recessive group. The syndrome is also known as Robinow-Silverman-Smith syndrome, Robinow dwarfism,...

Mice lacking inositol 1,4,5-trisphosphate receptors exhibit dry eye.

Directory of Open Access Journals (Sweden)

Takaaki Inaba

Full Text Available Tear secretion is important as it supplies water to the ocular surface and keeps eyes moist. Both the parasympathetic and sympathetic pathways contribute to tear secretion. Although intracellular Ca2+ elevation in the acinar cells of lacrimal glands is a crucial event for tear secretion in both the pathways, the Ca2+ channel, which is responsible for the Ca2+ elevation in the sympathetic pathway, has not been sufficiently analyzed. In this study, we examined tear secretion in mice lacking the inositol 1,4,5-trisphosphate receptor (IP3R types 2 and 3 (Itpr2-/-;Itpr3-/-double-knockout mice. We found that tear secretion in both the parasympathetic and sympathetic pathways was abolished in Itpr2-/-;Itpr3-/- mice. Intracellular Ca2+ elevation in lacrimal acinar cells after acetylcholine and epinephrine stimulation was abolished in Itpr2-/-;Itpr3-/- mice. Consequently, Itpr2-/-;Itpr3-/- mice exhibited keratoconjunctival alteration and corneal epithelial barrier disruption. Inflammatory cell infiltration into the lacrimal glands and elevation of serum autoantibodies, a representative marker for Sjögren's syndrome (SS in humans, were also detected in older Itpr2-/-;Itpr3-/- mice. These results suggested that IP3Rs are essential for tear secretion in both parasympathetic and sympathetic pathways and that Itpr2-/-;Itpr3-/- mice could be a new dry eye mouse model with symptoms that mimic those of SS.

Rare species support vulnerable functions in high-diversity ecosystems.

Science.gov (United States)

Mouillot, David; Bellwood, David R; Baraloto, Christopher; Chave, Jerome; Galzin, Rene; Harmelin-Vivien, Mireille; Kulbicki, Michel; Lavergne, Sebastien; Lavorel, Sandra; Mouquet, Nicolas; Paine, C E Timothy; Renaud, Julien; Thuiller, Wilfried

2013-01-01

Around the world, the human-induced collapses of populations and species have triggered a sixth mass extinction crisis, with rare species often being the first to disappear. Although the role of species diversity in the maintenance of ecosystem processes has been widely investigated, the role of rare species remains controversial. A critical issue is whether common species insure against the loss of functions supported by rare species. This issue is even more critical in species-rich ecosystems where high functional redundancy among species is likely and where it is thus often assumed that ecosystem functioning is buffered against species loss. Here, using extensive datasets of species occurrences and functional traits from three highly diverse ecosystems (846 coral reef fishes, 2,979 alpine plants, and 662 tropical trees), we demonstrate that the most distinct combinations of traits are supported predominantly by rare species both in terms of local abundance and regional occupancy. Moreover, species that have low functional redundancy and are likely to support the most vulnerable functions, with no other species carrying similar combinations of traits, are rarer than expected by chance in all three ecosystems. For instance, 63% and 98% of fish species that are likely to support highly vulnerable functions in coral reef ecosystems are locally and regionally rare, respectively. For alpine plants, 32% and 89% of such species are locally and regionally rare, respectively. Remarkably, 47% of fish species and 55% of tropical tree species that are likely to support highly vulnerable functions have only one individual per sample on average. Our results emphasize the importance of rare species conservation, even in highly diverse ecosystems, which are thought to exhibit high functional redundancy. Rare species offer more than aesthetic, cultural, or taxonomic diversity value; they disproportionately increase the potential breadth of functions provided by ecosystems across

Emerging functions of the Fanconi anemia pathway at a glance.

Science.gov (United States)

Sumpter, Rhea; Levine, Beth

2017-08-15

Fanconi anemia (FA) is a rare disease, in which homozygous or compound heterozygous inactivating mutations in any of 21 genes lead to genomic instability, early-onset bone marrow failure and increased cancer risk. The FA pathway is essential for DNA damage response (DDR) to DNA interstrand crosslinks. However, proteins of the FA pathway have additional cytoprotective functions that may be independent of DDR. We have shown that many FA proteins participate in the selective autophagy pathway that is required for the destruction of unwanted intracellular constituents. In this Cell Science at a Glance and the accompanying poster, we briefly review the role of the FA pathway in DDR and recent findings that link proteins of the FA pathway to selective autophagy of viruses and mitochondria. Finally, we discuss how perturbations in FA protein-mediated selective autophagy may contribute to inflammatory as well as genotoxic stress. © 2017. Published by The Company of Biologists Ltd.

Skin & bones: an artistic repair of a science exhibition by a mobile app

Directory of Open Access Journals (Sweden)

Diana Marques

2015-12-01

Full Text Available Due to the costs involved with renovating exhibitions at natural history museums, some permanent exhibits stay on display unchanged for decades. The Bone Hall at the Smithsonian’s National Museum of Natural History has remained intact for 51 years. Here we discuss this exhibition as a stark exemplification of a science-only, art-free approach to communicating ideas and how creative reimaginings of the visitor experience have made it more accessible for the majority of visitors. Within the Bone Hall, mounted skeletons are displayed in static poses without any hint of movement and interpretation of their behaviors and text labels describe, in esoteric language, skeletal details. In a rare opportunity to redesign the visitor experience for an existing exhibition, we produced a mobile app. The app is guided by concepts in the natural sciences, yet inspired by artistic ideas applied to audio, video and 3D animation, which created a multisensory visitor experience. Indispensable to the approach was a production team comprised of individuals rooted in the arts/humanities and sciences. They used their crafts to make science more accessible to non-specialized visitors through audio/visual creations. Interviews and surveys with visitors confirmed the value of producing artistic interpretations of science as a more effective method of communication in the exhibit.

Rare earth germanates

International Nuclear Information System (INIS)

Bondar', I.A.; Vinogradova, N.V.; Dem'yanets, L.N.

1983-01-01

Rare earth germanates attract close attention both as an independent class of compounds and analogues of a widely spread class of natural and synthetic minerals. The methods of rare earth germanate synthesis (solid-phase, hydrothermal) are considered. Systems on the basis of germanium and rare earth oxides, phase diagrams, phase transformations are studied. Using different chemical analysese the processes of rare earth germanate formation are investigated. IR spectra of alkali and rare earth metal germanates are presented, their comparative analysis being carried out. Crystal structures of the compounds, lattice parameters are studied. Fields of possible application of rare earth germanates are shown

Identification of N-[3-(3,4-Dihydroxyphenyl)-1-oxo-2-propenyl]-2-hydroxybenzamide (CGK-101) as a Small Molecule Inhibitor of the Wnt/β-catenin Pathway

Energy Technology Data Exchange (ETDEWEB)

Park, Seoyoung; Oh, Sangtaek [Kookmin Univ., Seoul (Korea, Republic of); Lee, Jeehyun; Lee, Jung Sook; Song, Gyuyong [Chungnam National Univ., Daejeon (Korea, Republic of)

2013-04-15

We identified CGK-101 as an antiproliferative agent that acts against HCT116 colon cancer cells. CGK-101 induced the degradation of intracellular β-catenin, thereby suppressing the Wnt/β-catenin pathway. Therefore, CGK-101 can be developed as a preventive or therapeutic agent against various cancers that involve abnormal β-catenin accumulation. Colorectal cancer is the most prevalent type of cancer and the second leading cause of cancer-related mortalities in Western countries. Current therapies for colorectal cancer rely on surgical resection, which is rarely curative in advanced disease, and traditional cytotoxic agents exhibit limited effects. Therefore, it is crucial to develop new therapeutic strategies that are based on defined molecular lesions.

Elastic, thermal and high pressure structural properties of heavy rare earth antimonides

International Nuclear Information System (INIS)

Soni, P.; Pagare, G.; Sanyal, S.P.

2009-01-01

Pressure induced structural phase transition of two heavy rare earth antimonides (RESb; RE=Ho, Er) have been studied theoretically by using an inter-ionic potential theory. This method has been found quite satisfactory in the case of pnictides of rare earth and describes the crystal properties in the framework of rigid-ion modal. The long-range Coulomb interaction, short-range repulsive interaction and van der Waals (vdW) interactions are properly incorporated in this theory. These compounds exhibit first order crystallographic phase transition from their NaCl-type structure to CsCl-type structure at 27 GPa and 33.2 GPa, respectively. The bulk moduli of RESb compounds are compared with the experimental values of elastic constants. We have also calculated the Debye temperature by incorporating the elastic constants for both the rare earth antimonides. (author)

Stepwise radical cation Diels-Alder reaction via multiple pathways.

Science.gov (United States)

Shimizu, Ryo; Okada, Yohei; Chiba, Kazuhiro

2018-01-01

Herein we disclose the radical cation Diels-Alder reaction of aryl vinyl ethers by electrocatalysis, which is triggered by an oxidative SET process. The reaction clearly proceeds in a stepwise fashion, which is a rare mechanism in this class. We also found that two distinctive pathways, including "direct" and "indirect", are possible to construct the Diels-Alder adduct.

A case of typical atrioventricular nodal (AVN) reentrant tachycardia confined to the compact AV node, showing a variety of rare electrophysiological findings, including eccentric AVN echoes

OpenAIRE

Tanaka, Kazushi; Shiotani, Shinji; Fukuda, Keisuke; Fujihara, Masahiko; Higashimori, Akihiro; Morioka, Nobuyuki; Yokoi, Yoshiaki; Fujimura, Osamu

2014-01-01

Herein, we report the case of a 49-year-old woman with typical atrioventricular nodal (AVN) reentrant tachycardia, confined to the compact atrioventricular node, showing numerous rare electrophysiological findings such as unique AVN reentrant echoes, double ventricular responses, latent retrograde dual AVN pathways, antegrade triple AVN pathways, and longitudinal dissociation within the lower final common pathway.

A case of typical atrioventricular nodal (AVN reentrant tachycardia confined to the compact AV node, showing a variety of rare electrophysiological findings, including eccentric AVN echoes

Directory of Open Access Journals (Sweden)

Kazushi Tanaka, M.D., Ph.D.

2014-12-01

Full Text Available Herein, we report the case of a 49-year-old woman with typical atrioventricular nodal (AVN reentrant tachycardia, confined to the compact atrioventricular node, showing numerous rare electrophysiological findings such as unique AVN reentrant echoes, double ventricular responses, latent retrograde dual AVN pathways, antegrade triple AVN pathways, and longitudinal dissociation within the lower final common pathway.

A new continuous two-step molecular precursor route to rare-earth oxysulfides Ln2O2S

International Nuclear Information System (INIS)

De Crom, N.; Devillers, M.

2012-01-01

A continuous two-step molecular precursor pathway is designed for the preparation of rare-earth oxysulfides Ln 2 O 2 S (Ln=Y, La, Pr, Nd, Sm–Lu). This new route involves a first oxidation step leading to the rare-earth oxysulfate Ln 2 O 2 SO 4 which is subsequently reduced to the rare-earth oxysulfide Ln 2 O 2 S by switching to a H 2 –Ar atmosphere. The whole process occurs at a temperature significantly lower than usual solid state synthesis (T≤650 °C) and avoids the use of dangerous sulfur-based gases, providing a convenient route to the synthesis of the entire series of Ln 2 O 2 S. The molecular precursors consist in heteroleptic dithiocarbamate complexes [Ln(Et 2 dtc) 3 (phen)] and [Ln(Et 2 dtc) 3 (bipy)] (Et 2 dtc=N,N-diethyldithiocarbamate; phen=1,10-phenanthroline; bipy=2,2′-bipyridine) and were synthesized by a new high yield and high purity synthesis route. The nature of the molecular precursor determines the minimum synthesis temperature and influences therefore the purity of the final Ln 2 O 2 S crystalline phase. - Graphical abstract: A continuous two-step molecular precursor pathway was designed for the preparation of rare-earth oxysulfides Ln 2 O 2 S (Ln=Y, La, Pr, Nd, Sm–Lu), starting from heteroleptic dithiocarbamate complexes. The influence of the nature of the molecular precursor on the minimum synthesis temperature and on the purity of the final Ln 2 O 2 S crystalline phase is discussed. Highlights: ► A new high yield and high purity synthesis route of rare earth dithiocarbamates is described. ► These compounds are used as precursors in a continuous process leading to rare-earth oxysulfides. ► The oxysulfides are obtained under much more moderate conditions than previously described.

SMARCE1, a rare cause of Coffin-Siris Syndrome: Clinical description of three additional cases.

Science.gov (United States)

Zarate, Yuri A; Bhoj, Elizabeth; Kaylor, Julie; Li, Dong; Tsurusaki, Yoshinori; Miyake, Noriko; Matsumoto, Naomichi; Phadke, Shubha; Escobar, Luis; Irani, Afifa; Hakonarson, Hakon; Schrier Vergano, Samantha A

2016-08-01

Coffin-Siris syndrome (CSS, MIM 135900), is a well-described, multiple congenital anomaly syndrome characterized by coarse facial features, hypertrichosis, sparse scalp hair, and hypo/aplastic digital nails and phalanges, typically of the 5th digits. Mutations in the BAF (SWI/SNF)-complex subunits (SMARCA4, SMARCE1, SMARCB1, SMARCA2, ARID1B, and ARID1A) have been shown to cause not only CSS, but also related disorders including Nicolaides-Baraitser (MIM 601358) syndrome and ARID1B-intellectual disability syndrome (MIM 614562). At least 200 individuals with CSS have been found to have a mutation in the BAF pathway. However, to date, only three individuals with CSS have been reported to have pathogenic variants in SMARCE1. We report here three additional individuals with clinical features consistent with CSS and alterations in SMARCE1, one of which is novel. The probands all exhibited dysmorphic facial features, moderate developmental and cognitive delay, poor growth, and hypoplastic digital nails/phalanges, including digits not typically affected in the other genes associated with CSS. Two of the three probands had a variety of different organ system anomalies, including cardiac disease, genitourinary abnormalities, feeding difficulties, and vision abnormalities. The 3rd proband has not had further investigative studies. Although an increasing number of individuals are being diagnosed with disorders in the BAF pathway, SMARCE1 is the least common of these genes. This report doubles the number of probands with these mutations, and allows for better phenotypic information of this rare syndrome. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

SMARCE1, a Rare Cause of Coffin–Siris Syndrome: Clinical Description of Three Additional Cases

Science.gov (United States)

Zarate, Yuri A.; Bhoj, Elizabeth; Kaylor, Julie; Li, Dong; Tsurusaki, Yoshinori; Miyake, Noriko; Matsumoto, Naomichi; Phadke, Shubha; Escobar, Luis; Irani, Afifa; Hakonarson, Hakon; Schrier Vergano, Samantha A.

2018-01-01

Coffin–Siris syndrome (CSS, MIM 135900), is a well-described, multiple congenital anomaly syndrome characterized by coarse facial features, hypertrichosis, sparse scalp hair, and hypo/aplastic digital nails and phalanges, typically of the 5th digits. Mutations in the BAF (SWI/SNF)-complex subunits (SMARCA4, SMARCE1, SMARCB1, SMARCA2, ARID1B, and ARID1A) have been shown to cause not only CSS, but also related disorders including Nicolaides–Baraitser (MIM 601358) syndrome and ARID1B-intellectual disability syndrome (MIM 614562).At least 200 individuals with CSS have been found to have a mutation in the BAF pathway. However, to date, only three individuals with CSS have been reported to have pathogenic variants in SMARCE1. We report here three additional individuals with clinical features consistent with CSS and alterations in SMARCE1, one of which is novel. The probands all exhibited dysmorphic facial features, moderate developmental and cognitive delay, poor growth, and hypoplastic digital nails/phalanges, including digits not typically affected in the other genes associated with CSS. Two of the three probands had a variety of different organ system anomalies, including cardiac disease, genitourinary abnormalities, feeding difficulties, and vision abnormalities. The 3rd proband has not had further investigative studies. Although an increasing number of individuals are being diagnosed with disorders in the BAF pathway, SMARCE1 is the least common of these genes. This report doubles the number of probands with these mutations, and allows for better phenotypic information of this rare syndrome. PMID:27264197

Effects of Rare Earth Elements on Properties of Ni-Base Superalloy Powders and Coatings

Directory of Open Access Journals (Sweden)

Chunlian Hu

2017-02-01

Full Text Available NiCrMoY alloy powders were prepared using inert gas atomization by incorporation of rare earth elements, such as Mo, Nb, and Y into Ni60A powders, the coatings were sprayed by oxy-acetylene flame spray and then remelted with high-frequency induction. The morphologies, hollow particle ratio, particle-size distribution, apparent density, flowability, and the oxygen content of the NiCrMoY alloy powders were investigated, and the microstructure and hardness of the coatings were evaluated by optical microscopy (OM. Due to incorporation of the rare earth elements of Mo, Nb, or Y, the majority of the NiCrMoY alloy particles are near-spherical, the minority of which have small satellites, the surface of the particles is smoother and hollow particles are fewer, the particles exhibit larger apparent density and lower flowability than those of particles without incorporation, i.e., Ni60A powders, and particle-size distribution exhibits a single peak and fits normal distribution. The microstructure of the NiCrMoY alloy coatings exhibits finer structure and Rockwell hardness HRC of 60–63 in which the bulk- and needle-like hard phases are formed.

Wolff-Parkinson-White syndrome with cleft lip and palate: A rare, previously unreported association

Directory of Open Access Journals (Sweden)

K Kannan

2008-01-01

Full Text Available Wolff-Parkinson-White syndrome, also called Pre Excitation Syndrome, is characterized by an extra pathway that conducts the electrical impulses from the atria to the ventricles without the normal delay. We are reporting a case of WPW syndrome with a cleft lip and palate, which is a rare association and previously unreported in literature.

Tetracyanidoborates with triply charged rare earth metal cations and their optical properties

International Nuclear Information System (INIS)

Hackbarth, Liisa

2015-01-01

exhibit rare earth cation specific luminescence properties which are apparently improved by the tetracyanidoborate anion. Therefore, the tetracyanidoborates with trivalent rare earth metal cations could have possible applications in phosphors.

Rare earth element lithogeochemistry of granitoid mineral deposits

Energy Technology Data Exchange (ETDEWEB)

Taylor, R.P.; Fryer, B.J. (Memorial Univ. of Newfoundland, St. John' s (Canada). Dept. of Earth Sciences)

1983-12-01

As a monitor of the processes involved in the formation of granitoid mineral deposits the coherent group behaviour of the rare earth elements (REE) actively reflects changing fluid characteristics. For example, in the porphyry environment, magmatic-hydrothermal fluids produce potassic alteration with strong enrichment in the light rare earth elements, reflecting their high pH, low fluid/rock ratios, and the dominant role of Cl/sup -/ complexing in metal (i.e. Cu, Au) transport. With increasing fluid/rock ratios and decreasing pH accompanying the progressive involvement of meteoric fluids (and the production of propylitic, argillic, and phyllic alteration) anionic species such as F/sup -/ and CO/sub 3//sup 2 -/ become important in metal (e.g. Mo, W) transport through complexing, and their activity in the hydrothermal fluids is illustrated by mobilization of the heavy rare earth elements. The relative involvement of Cl/sup -/ versus F/sup -/ versus CO/sub 3//sup 2 -/ in metal transport in other granite-related systems can also be monitored through REE behaviour. Hence granitoid tin-tungsten mineralization and associated greisenization typically exhibit heavy rare earth enrichment and evidence the importance of F/sup -/ and CO/sub 3//sup 2 -/ in metal transport. Similarly, heavy rare earth element enrichment in hydrothermal uranium deposits can be related to the transport of uranium as carbonate complexes. REE are widely accepted as powerful tools in the study of rock petrogenesis, but their use has been neglected in the investigation of mineral deposits. The recognition of the systematic variation of REE distributions in granitoid mineral deposits suggests that the application of REE geochemistry, particularly when integrated with fluid inclusion and isotope studies, can provide l) an effective method for identifying the physiochemical controls of metal transport and 2) a useful criterion of elucidating metal distribution.

Rare earth element lithogeochemistry of granitoid mineral deposits

International Nuclear Information System (INIS)

Taylor, R.P.; Fryer, B.J.

1983-01-01

As a monitor of the processes involved in the formation of granitoid mineral deposits the coherent group behaviour of the rare earth elements (REE) actively reflects changing fluid characteristics. For example, in the porphyry environment, magmatic-hydrothermal fluids produce potassic alteration with strong enrichment in the light rare earth elements, reflecting their high pH, low fluid/rock ratios, and the dominant role of Cl - complexing in metal (i.e. Cu, Au) transport. With increasing fluid/rock ratios and decreasing pH accompanying the progressive involvement of meteoric fluids (and the production of propylitic, argillic, and phyllic alteration) anionic species such as F - and CO 3 2- become important in metal (e.g. Mo, W) transport through complexing, and their activity in the hydrothermal fluids is illustrated by mobilization of the heavy rare earth elements. The relative involvement of Cl - versus F - versus CO 3 2- in metal transport in other granite-related systems can also be monitored through REE behaviour. Hence granitoid tin-tungsten mineralization and associated greisenization typically exhibit heavy rare earth enrichment and evidence the importance of F - and CO 3 2- in metal transport. Similarly, heavy rare earth element enrichment in hydrothermal uranium deposits can be related to the transport of uranium as carbonate complexes. REE are widely accepted as powerful tools in the study of rock petrogenesis, but their use has been neglected in the investigation of mineral deposits. The recognition of the systematic variation of REE distributions in granitoid mineral deposits suggests that the application of REE geochemistry, particularly when integrated with fluid inclusion and isotope studies, can provide l) an effective method for identifying the physiochemical controls of metal transport and 2) a useful criterion of elucidating metal distribution

The Fanconi anemia DNA damage repair pathway in the spotlight for germline predisposition to colorectal cancer.

Science.gov (United States)

Esteban-Jurado, Clara; Franch-Expósito, Sebastià; Muñoz, Jenifer; Ocaña, Teresa; Carballal, Sabela; López-Cerón, Maria; Cuatrecasas, Miriam; Vila-Casadesús, Maria; Lozano, Juan José; Serra, Enric; Beltran, Sergi; Brea-Fernández, Alejandro; Ruiz-Ponte, Clara; Castells, Antoni; Bujanda, Luis; Garre, Pilar; Caldés, Trinidad; Cubiella, Joaquín; Balaguer, Francesc; Castellví-Bel, Sergi

2016-10-01

Colorectal cancer (CRC) is one of the most common neoplasms in the world. Fanconi anemia (FA) is a very rare genetic disease causing bone marrow failure, congenital growth abnormalities and cancer predisposition. The comprehensive FA DNA damage repair pathway requires the collaboration of 53 proteins and it is necessary to restore genome integrity by efficiently repairing damaged DNA. A link between FA genes in breast and ovarian cancer germline predisposition has been previously suggested. We selected 74 CRC patients from 40 unrelated Spanish families with strong CRC aggregation compatible with an autosomal dominant pattern of inheritance and without mutations in known hereditary CRC genes and performed germline DNA whole-exome sequencing with the aim of finding new candidate germline predisposition variants. After sequencing and data analysis, variant prioritization selected only those very rare alterations, producing a putative loss of function and located in genes with a role compatible with cancer. We detected an enrichment for variants in FA DNA damage repair pathway genes in our familial CRC cohort as 6 families carried heterozygous, rare, potentially pathogenic variants located in BRCA2/FANCD1, BRIP1/FANCJ, FANCC, FANCE and REV3L/POLZ. In conclusion, the FA DNA damage repair pathway may play an important role in the inherited predisposition to CRC.

Research and development of tungsten electrodes added with rare earth oxides

International Nuclear Information System (INIS)

Zuoren Nie; Ying Chen; Meiling Zhou; Tieyong Zuo

2001-01-01

The recent research and development of tungsten electrodes used in TIG and Plasma technologies are introduced, and the tungsten materials as well as the effects of rare earth oxides are specially discussed. in W-La 2 O 3 , W-CeO 2 , W-Y 2 O 3 and W-ThO 2 electrode materials, the W-2.2mass%La 2 O 3 electrode exhibited the best properties when the current is of little or middle volume, and when the electrodes are used in large current, the W-Y 2 O 3 electrode is the best. By a comparative study between the tungsten electrodes activated with single metal oxides, as above-mentioned, and those containing two or three rare earth oxides, namely La 2 O 3 , CeO 2 and Y 2 O 3 , it was indicated that the welding arc properties of the tungsten electrodes activated with combined rare earth oxides additions is superior than that of the electrodes containing single oxides as above mentioned. It was also shown that the operating properties of tungsten electrodes depend intensively on the rare earth oxides contained in the electrodes, and the actions of rare earth oxides during arcing are the most important factors to the electrodes' operating properties, temperature, work function as well as the arc stability. (author)

Port Pirie rare earths plant stage 3

International Nuclear Information System (INIS)

1990-08-01

SX Holdings Limited intends to establish a rare earths plant at Port Pirie, South Australia. The proposal involves three stages of development, Stage 3 being to develop a monazite cracking plant and associated rare earths separation facility with the capacity to process up to 8,000 t/a of monazite-type ores. The proposed initial capacity is 4,000 t/a. This Draft Environmental Impact Statement relates to Stage 3 and is based on a monazite processing capacity of 8,000 t/a. The justification of the project is given in terms of use and the market for rare earths, the economic and environmental benefits of the proposal, the site selection process, site rehabilitation, and the consequences of not proceeding. A detailed description of the project is given, including the treatment process, site development and facilities, the supply of raw materials, product and waste handling, transport and storage, plant commissioning, operation and decommissioning, construction and staffing. The environmental issues entailed in the proposed development are discussed and include social effects, land use and infrasturcture considerations, risk management and transport. Occupational and environmental radiation issues, including assessments of exposure pathways and doses, management and monitoring, disposal of monosite residue are also discussed. It is estimated that the effects of disposal of 2,330 t/year of radioactive slurry in the sub-aerial tailing disposal system at Olympic Dam will be negligible. Moreover, the gamma dose increases would not result in any significant increase in occupational exposures. 38 refs., tabs., ills

Rare earth sulfates

International Nuclear Information System (INIS)

Komissarova, L.N.; Shatskij, V.M.; Pokrovskij, A.N.; Chizhov, S.M.; Bal'kina, T.I.; Suponitskij, Yu.L.

1986-01-01

Results of experimental works on the study of synthesis conditions, structure and physico-chemical properties of rare earth, scandium and yttrium sulfates, have been generalized. Phase diagrams of solubility and fusibility, thermodynamic and crystallochemical characteristics, thermal stability of hydrates and anhydrous sulfates of rare earths, including normal, double (with cations of alkali and alkaline-earth metals), ternary and anion-mixed sulfates of rare earths, as well as their adducts, are considered. The state of ions of rare earths, scandium and yttrium in aqueous sulfuric acid solutions is discussed. Data on the use of rare earth sulfates are given

Isomerization of butene-1 on rare earth oxides. [Rare earths: La, Nd, Dy

Energy Technology Data Exchange (ETDEWEB)

Khodakov, Yu S; Nesterov, V K; Minachev, Kh M [AN SSSR, Moscow. Inst. Organicheskoj Khimii

1975-09-01

A study has been made into the isomerization of butene-1 on oxides of rare-earth elements. The dependence of the reaction rate at 20/sup 0/C on the baking temperature of La and Nd oxides have the maximum at 700/sup 0/C. A decrease in the activity of these oxides after bakinq at 800/sup 0/C is observed, as well as during experiments at -30 deq C. In the case of Dy/sub 2/O/sub 3/, the activity at 20/sup 0/C increases gradually with Tsub(bak)=500 to 800/sup 0/C Zeolite 0.57LaNaY exhibits maximum activity at Tsub(bak)=500/sup 0/C Similar data as to the effect of the baking temperature on the catalyst activity were obtained earlier for hydrogenation of ethylene. According to their maximum activity, oxides of rare-earth elements, in the isomerization as well as hydrogenation reactions, can be arranged as follows: La/sub 2/O/sub 3/>Nd/sub 2/O/sub 3/

Exhibition

CERN Document Server

Staff Association

2017-01-01

A Look of Hope Islam Mahmoud Sweity From 19 to 30 June 2017 CERN Meyrin, Main Building Islam Mahmoud Sweity Islam Mahmoud Sweity was born in 1997 at Beit Awwa, Palestine. She is currently following a course to get an Art diploma of Painting at the college of Fine Arts at An-Najah National University under the supervision of Esmat Al As'aad. Her portraits, landscapes and still life paintings are full of life and shining colours. Charged of emotional empathy they catch the attention of the viewer and are reminding us that life is beautiful and worth living in spite of all difficulties we have to go through. She participated in many exhibitions and has exposed her drawings in 2015 at CERN and in France in the framework of the exhibition "The Origin“, and in 2017 in the Former Yugoslav Republic of Macedonia, Palestina and Jordan. In this exhibition the oil paintings made in the past year will be presented. For more information : staff.association@cern.ch | T&eacu...

Rare species support vulnerable functions in high-diversity ecosystems.

Directory of Open Access Journals (Sweden)

David Mouillot

Full Text Available Around the world, the human-induced collapses of populations and species have triggered a sixth mass extinction crisis, with rare species often being the first to disappear. Although the role of species diversity in the maintenance of ecosystem processes has been widely investigated, the role of rare species remains controversial. A critical issue is whether common species insure against the loss of functions supported by rare species. This issue is even more critical in species-rich ecosystems where high functional redundancy among species is likely and where it is thus often assumed that ecosystem functioning is buffered against species loss. Here, using extensive datasets of species occurrences and functional traits from three highly diverse ecosystems (846 coral reef fishes, 2,979 alpine plants, and 662 tropical trees, we demonstrate that the most distinct combinations of traits are supported predominantly by rare species both in terms of local abundance and regional occupancy. Moreover, species that have low functional redundancy and are likely to support the most vulnerable functions, with no other species carrying similar combinations of traits, are rarer than expected by chance in all three ecosystems. For instance, 63% and 98% of fish species that are likely to support highly vulnerable functions in coral reef ecosystems are locally and regionally rare, respectively. For alpine plants, 32% and 89% of such species are locally and regionally rare, respectively. Remarkably, 47% of fish species and 55% of tropical tree species that are likely to support highly vulnerable functions have only one individual per sample on average. Our results emphasize the importance of rare species conservation, even in highly diverse ecosystems, which are thought to exhibit high functional redundancy. Rare species offer more than aesthetic, cultural, or taxonomic diversity value; they disproportionately increase the potential breadth of functions provided by

Rare lung cancers

International Nuclear Information System (INIS)

Berzinec, P.

2013-01-01

The RARECARE Project (Rare Cancers in the Europe) supported by the European Union defined the rare cancers by the incidence rate of less than 6/100 000. There are several variants of lung cancer which are rare according to this definition. From the clinical point of view the most interesting are the rare adenocarcinomas and large cell neuroendocrine carcinoma. There are important differences in the diagnostic probability of EGFR and ALK mutations in the mutinous and non-mucin ous adenocarcinomas, in the signet ring cell adenocarcinomas, and large cell carcinomas. The optimal chemotherapy for neuroendocrine large cell carcinomas remains undefined. There is only very limited number of clinical trials aimed on the rare lung cancers and actually none phase III trial. Rare lung cancers continue to be a challenge both for the laboratory and the clinical research. (author)

The phenotypic manifestations of rare CNVs in schizophrenia.

Science.gov (United States)

Merikangas, Alison K; Segurado, Ricardo; Cormican, Paul; Heron, Elizabeth A; Anney, Richard J L; Moore, Susan; Kelleher, Eric; Hargreaves, April; Anderson-Schmidt, Heike; Gill, Michael; Gallagher, Louise; Corvin, Aiden

2014-09-01

There is compelling evidence for the role of copy number variants (CNVs) in schizophrenia susceptibility, and it has been estimated that up to 2-3% of schizophrenia cases may carry rare CNVs. Despite evidence that these events are associated with an increased risk across categorical neurodevelopmental disorders, there is limited understanding of the impact of CNVs on the core features of disorders like schizophrenia. Our objective was to evaluate associations between rare CNVs in differentially brain expressed (BE) genes and the core features and clinical correlates of schizophrenia. The sample included 386 cases of Irish ancestry with a diagnosis of schizophrenia, at least one rare CNV impacting any gene, and a core set of phenotypic measures. Statistically significant associations between deletions in differentially BE genes were found for family history of mental illness (decreased prevalence of all CNVs and deletions, unadjusted and adjusted) and for paternal age (increase in deletions only, unadjusted, among those with later ages at birth of patient). The strong effect of a lack of a family history on BE genes suggests that CNVs may comprise one pathway to schizophrenia, whereas a positive family history could index other genetic mechanisms that increase schizophrenia vulnerability. To our knowledge, this is the first investigation of the association between genome-wide CNVs and risk factors and sub-phenotypic features of schizophrenia beyond cognitive function. Copyright © 2014 Elsevier B.V. All rights reserved.

Exhibiting Epistemic Objects

DEFF Research Database (Denmark)

Tybjerg, Karin

2017-01-01

of exhibiting epistemic objects that utilize their knowledge-generating potential and allow them to continue to stimulate curiosity and generate knowledge in the exhibition. The epistemic potential of the objects can then be made to work together with the function of the exhibition as a knowledge-generating set...

Trichloroethylene degradation by two independent aromatic-degrading pathways in Alcaligenes eutrophus JMP134.

OpenAIRE

Harker, A R; Kim, Y

1990-01-01

The bacterium Alcaligenes eutrophus JMP134(pJP4) degrades trichloroethylene (TCE) by a chromosomal phenol-dependent pathway and by the plasmid-encoded 2,4-dichlorophenoxyacetic acid pathway. The two pathways were independent and exhibited different rates of removal and capacities for quantity of TCE removed. The phenol-dependent pathway was more rapid (0.2 versus 0.06 nmol of TCE removed per min per mg of protein) and consumed all detectable TCE. The 2,4-dichlorophenoxyacetic acid-dependent p...

Rare metal and rare earth pegmatites of Western India

International Nuclear Information System (INIS)

Maithani, P.B.; Nagar, R.K.

1999-01-01

Rajasthan Mica Belt in western India is one of the three major mica-producing Proterozoic pegmatite belts of India, the others being in Bihar and Andhra Pradesh. The pegmatites of these mica belts, in general, are associated with the rare metal (RM) and rare earth element (REE)-bearing minerals like columbite-tantalite, beryl, lepidolite and other multiple oxides. RM-REE pegmatites of Gujarat are devoid of commercially workable mica. These pegmatites are geologically characterised in this paper, based on their association with granite plutons geochemistry, and RM and REE potential. In addition to RM and RE-bearing pegmatites, granites of the Umedpur area, Gujarat also show anomalous concentration (0.97 wt%) of rare metals (6431 ppm Nb, 1266 ppm Ta, 454 ppm Sn, 173 ppm W), (1098 ppm Ce 1.36% Y 2 O 3 ) rare earths, and uranium (0.40% eU 3 O 8 ). Eluvial concentrations in the soil and panned concentrate (0.04-0.28 wt%) analysed up to 7.4%Nb 2 O 5 , 836 ppm Ta, and 1.31% Y. Discrete columbite-tantalite and betafite have been identified in these concentrates in addition to other minerals like zircon, rutile, sphene and xenotime. This area with discrete RM R EE mineral phases could be significant as a non-pegmatite source for rare metal and rare earths. (author)

Spectrofluorimetric characterization and study for the determination of rare earth carbonate complexes

International Nuclear Information System (INIS)

Dantas, E.S.K.; Abrao, A.

1982-01-01

Some rare earths exhibit fluorescence in acid solution (HC1, H 2 SO 4 or HC10 4 ) when irradiated with ultraviolet radiation; however, analytical application of this property has not been proposed due to the lack of sensitivity resulted from the weakness of the fluorescence transitions. The rare earths are soluble in alkali carbonate solutions, forming anionic carbonate complexes (TR(CO 3 ) sup(n-) sub(x)); some of them have stronger absorption bands than those obtained in acid media. Using this property, the characterization and determination of the six rare earths which fluoresce in carbonate solution has been studied. The excitation and emission wavelengths are (in nm): Sm (406, 596); Eu (280,612); Gd (272,312); Tb (240,542); Dy (350, 576) and Tm (360, 450). Although Ce-III is highly fluorescent in acid media, when in carbonate solution it is easily oxidized to Ce-IV which does not fluoresce neither in acid nor in carbonate solutions. The other rare earths, although soluble in carbonate solution, do not fluoresce in this medium. A comparison is made, between the fluorescence of the fluorescing lanthanides, in acid and carbonate media. The precision and accuracy of this method, the detection limit of all rare earths studied and the mutual interference of some of them is also discussed. The method will be applied to the analytical control of the separation and purification of individual rare earths, now under development. (Author) [pt

Collateral pathways in lumbar epidural venography

International Nuclear Information System (INIS)

Thijssen, H.O.M.; Ike, B.W.; Chevrot, A.; Bijlsma, R.

1982-01-01

Opacification of collateral pathways other than the central channels is very rare in lumbar epidural venography. Two cases of opacification of the inferior mesenteric vein following extravasation of contrast medium at the tip of the lateral sacral vein catheter are reported. One case is presented in which filling of normal parametrial venous plexuses and the left ovarian vein occurred as a consequence of incompetent or absent valves in the internal iliac vein. The literature containing comparable collateral flow patterns in disease is reviewed. The significance of the phlebographic features in our cases is discussed. (orig.)

Tetracyanidoborates with triply charged rare earth metal cations and their optical properties

Energy Technology Data Exchange (ETDEWEB)

Hackbarth, Liisa

2015-11-24

measurements indicate that the tetracyanidoborates with rare earth metal cations exhibit rare earth cation specific luminescence properties which are apparently improved by the tetracyanidoborate anion. Therefore, the tetracyanidoborates with trivalent rare earth metal cations could have possible applications in phosphors.

Rare earths

International Nuclear Information System (INIS)

1984-01-01

The conference was held from September 12 to 13, 1984 in Jetrichovice, Czechoslovakia. The participants heard 16 papers of which 4 were inputted in INIS. These papers dealt with industrial separation processes of rare earths, the use of chemical methods of separation from the concentrate of apatite and bastnesite, the effect of the relative permittivity of solvents in the elution of rare earth elements from a cation exchanger, and the determination of the content of different rare earth elements using X-ray fluorescence analysis and atomic absorption spectroscopy. (E.S.)

Anomalous magnetoresistance in antiferromagnetic polycrystalline materials R2Ni3Si5 (R=rare earth)

International Nuclear Information System (INIS)

Mazumdar, C.; Nigam, A.K.; Nagarajan, R.; Gupta, L.C.; Chandra, G.; Padalia, B.D.; Godart, C.; Vijayaraghaven, R.

1997-01-01

Magnetoresistance (MR) studies on polycrystalline R 2 Ni 3 Si 5 , (R=Y, rare earth) which order antiferromagnetically at low temperatures, are reported here. MR of the Nd, Sm, and Tb members of the series exhibit positive giant magnetoresistance, largest among polycrystalline materials (85%, 75%, and 58% for Tb 2 Ni 3 Si 5 , Sm 2 Ni 3 Si 5 , and Nd 2 Ni 3 Si 5 , respectively, at 4.4 K in a field of 45 kG). These materials have, to the best of our knowledge, the largest positive GMR reported ever for any bulk polycrystalline compounds. The magnitude of MR does not correlate with the rare earth magnetic moments. We believe that the structure of these materials, which can be considered as a naturally occurring multilayer of wavy planes of rare earth atoms separated by Ni endash Si network, plays a role. The isothermal MR of other members of this series (R=Pr,Dy,Ho) exhibits a maximum and a minimum, below their respective T N close-quote s. We interpret these in terms of a metamagnetic transition and short-range ferromagnetic correlations. The short-range ferromagnetic correlations seem to be dominant in the temperature region just above T N . copyright 1997 American Institute of Physics

Modelling and Analysis of Biochemical Signalling Pathway Cross-talk

Directory of Open Access Journals (Sweden)

Robin Donaldson

2010-02-01

Full Text Available Signalling pathways are abstractions that help life scientists structure the coordination of cellular activity. Cross-talk between pathways accounts for many of the complex behaviours exhibited by signalling pathways and is often critical in producing the correct signal-response relationship. Formal models of signalling pathways and cross-talk in particular can aid understanding and drive experimentation. We define an approach to modelling based on the concept that a pathway is the (synchronising parallel composition of instances of generic modules (with internal and external labels. Pathways are then composed by (synchronising parallel composition and renaming; different types of cross-talk result from different combinations of synchronisation and renaming. We define a number of generic modules in PRISM and five types of cross-talk: signal flow, substrate availability, receptor function, gene expression and intracellular communication. We show that Continuous Stochastic Logic properties can both detect and distinguish the types of cross-talk. The approach is illustrated with small examples and an analysis of the cross-talk between the TGF-b/BMP, WNT and MAPK pathways.

Fluorescence of europium in oxyhalides of rare earths

International Nuclear Information System (INIS)

Hoelsae, Jorma; Niinistoe, Lauri

1980-01-01

Fluorescence spectra of the Eu 3+ ion embedded in rare earth oxyhalides LnOX (Ln=Y, La, Gd; X=Cl, Br) have been obtained at 300, 77 and 4.2 K. The number of lines observed for each transition is compatible to the one allowed by the Csub(4v) point site symmetry predicted by crystallography. Positions of Stark levels have been analyzed in terms of nephelauxetic effect and strength of the crystal field parameters, versus host cation and anion. Moreover, the so-called 'forbidden' transition 5 D 0 → 7 F 0 exhibits a strong intensity, also varying versus the matrix [fr

Multiferroic nature of charge-ordered rare earth manganites

International Nuclear Information System (INIS)

Serrao, Claudy Rayan; Sundaresan, A; Rao, C N R

2007-01-01

Charge-ordered rare earth manganites Nd 0.5 Ca 0.5 MnO 3 ,La 0.25 Nd 0.25 Ca 0.5 MnO 3 , Pr 0.7 Ca 0.3 MnO 3 and Pr 0.6 Ca 0.4 MnO 3 are found to exhibit dielectric constant anomalies around the charge-ordering or the antiferromagnetic transition temperatures. Magnetic fields have a marked effect on the dielectric properties, indicating the presence of coupling between the magnetic and electrical order parameters. The observation of magnetoferroelectricity in these manganites is in accord with the recent theoretical predictions of Khomskii and co-workers

Genotypic variations in the accumulation of Cd, Cu, Pb and Zn exhibited by six commonly grown vegetables

International Nuclear Information System (INIS)

Alexander, P.D.; Alloway, B.J.; Dourado, A.M.

2006-01-01

Metal contaminants in garden and allotment soils could possibly affect human health through a variety of pathways. This study focused on the potential pathway of consumption of vegetables grown on contaminated soil. Five cultivars each of six common vegetables were grown in a control and in a soil spiked with Cd, Cu, Pb and Zn. Highly significant differences in metal content were evident between cultivars of a number of vegetables for several of the contaminants. Carrot and pea cultivars exhibited significant differences in accumulated concentrations of Cd and Cu with carrot cultivars also exhibiting significant differences in Zn. Distinctive differences were also identified when comparing one vegetable to another, legumes (Leguminosae) tending to be low accumulators, root vegetables (Umbelliferae and Liliaceae) tending to be moderate accumulators and leafy vegetables (Compositae and Chenopodiaceae) being high accumulators. - Genotypic differences between cultivars of vegetable species can be important in determining the extent of accumulation of metals from contaminated soil

cDNA Clones with Rare and Recurrent Mutations Found in Cancers | Office of Cancer Genomics

Science.gov (United States)

The CTD2 Center at UT- MD Anderson Cancer Center has developed High-Throughput Mutagenesis and Molecular Barcoding (HiTMMoB)1,2 pipeline to construct mutant alleles open reading frame expression clones that are either recurrent or rare in cancers. These barcoded genes can be used for context-specific functional validation, detection of novel biomarkers (pathway activation) and targets (drug sensitivity).

InP/ZnS QDs exposure induces developmental toxicity in rare minnow (Gobiocypris rarus) embryos.

Science.gov (United States)

Chen, Yao; Yang, Yang; Ou, Fang; Liu, Li; Liu, Xiao-Hong; Wang, Zhi-Jian; Jin, Li

2018-04-05

We investigated the in vivo toxicity of InP/ZnS quantum dots (QDs) in Chinese rare minnow (Gobiocypris rarus) embryos. The 72 h post-fertilization (hpf) LC 50 (median lethal concentration) was 1678.007 nmol/L. Rare minnows exposed to InP/ZnS QDs exhibited decreased spontaneous movement, decreased survival and hatchability rates, and an increased malformation rate. Pericardial edema, spinal curvature, bent tails and vitelline cysts were observed. Embryonic Wnt8a and Mstn mRNA levels were significantly up-regulated after InP/ZnS QDs treatment at 48 hpf (200 nmol/L) (p InP/ZnS QDs treatments did not significantly change the Olive tail moments (p > 0.05). Thus, InP/ZnS QDs caused teratogenic effects and death during the development of Chinese rare minnow embryos, but InP/ZnS QDs did not cause significant genetic toxicity during Chinese rare minnow development. Copyright © 2018 Elsevier B.V. All rights reserved.

Level density and thermal properties in rare earth nuclei

International Nuclear Information System (INIS)

Siem, S.; Schiller, A.; Guttormsen, M.; Hjorth-Jensen, M.; Melby, E.; Rekstad, J.

2000-01-01

The level density at low spin has been extracted for several nuclei in the rare earth region using the ( 3 He,α) reaction. Within the framework of the microcanonical ensemble, the entropy and the temperature of the nuclei are derived. The temperature curve shows bumps which are associated with the break up of Cooper pairs. The entropies of the even-even and even-odd nuclei have been compared. The nuclear heat capacity is deduced within the framework of the canonical ensemble and exhibits an S-formed shape as function of temperature. (author)

Disruption of the FA/BRCA pathway in bladder cancer.

Science.gov (United States)

Neveling, K; Kalb, R; Florl, A R; Herterich, S; Friedl, R; Hoehn, H; Hader, C; Hartmann, F H; Nanda, I; Steinlein, C; Schmid, M; Tonnies, H; Hurst, C D; Knowles, M A; Hanenberg, H; Schulz, W A; Schindler, D

2007-01-01

Bladder carcinomas frequently show extensive deletions of chromosomes 9p and/or 9q, potentially including the loci of the Fanconi anemia (FA) genes FANCC and FANCG. FA is a rare recessive disease due to defects in anyone of 13 FANC genes manifesting with genetic instability and increased risk of neoplasia. FA cells are hypersensitive towards DNA crosslinking agents such as mitomycin C and cisplatin that are commonly employed in the chemotherapy of bladder cancers. These observations suggest the possibility of disruption of the FA/BRCA DNA repair pathway in bladder tumors. However, mutations in FANCC or FANCG could not be detected in any of 23 bladder carcinoma cell lines and ten surgical tumor specimens by LOH analysis or by FANCD2 immunoblotting assessing proficiency of the pathway. Only a single cell line, BFTC909, proved defective for FANCD2 monoubiquitination and was highly sensitive towards mitomycin C. This increased sensitivity was restored specifically by transfer of the FANCF gene. Sequencing of FANCF in BFTC909 failed to identify mutations, but methylation of cytosine residues in the FANCF promoter region was demonstrated by methylation-specific PCR, HpaII restriction and bisulfite DNA sequencing. Methylation-specific PCR uncovered only a single instance of FANCF promoter hypermethylation in surgical specimens of further 41 bladder carcinomas. These low proportions suggest that in contrast to other types of tumors silencing of FANCF is a rare event in bladder cancer and that an intact FA/BRCA pathway might be advantageous for tumor progression. Copyright (c) 2007 S. Karger AG, Basel.

Rare variants analysis of cutaneous malignant melanoma genes in Parkinson's disease.

Science.gov (United States)

Lubbe, S J; Escott-Price, V; Brice, A; Gasser, T; Pittman, A M; Bras, J; Hardy, J; Heutink, P; Wood, N M; Singleton, A B; Grosset, D G; Carroll, C B; Law, M H; Demenais, F; Iles, M M; Bishop, D T; Newton-Bishop, J; Williams, N M; Morris, H R

2016-12-01

A shared genetic susceptibility between cutaneous malignant melanoma (CMM) and Parkinson's disease (PD) has been suggested. We investigated this by assessing the contribution of rare variants in genes involved in CMM to PD risk. We studied rare variation across 29 CMM risk genes using high-quality genotype data in 6875 PD cases and 6065 controls and sought to replicate findings using whole-exome sequencing data from a second independent cohort totaling 1255 PD cases and 473 controls. No statistically significant enrichment of rare variants across all genes, per gene, or for any individual variant was detected in either cohort. There were nonsignificant trends toward different carrier frequencies between PD cases and controls, under different inheritance models, in the following CMM risk genes: BAP1, DCC, ERBB4, KIT, MAPK2, MITF, PTEN, and TP53. The very rare TYR p.V275F variant, which is a pathogenic allele for recessive albinism, was more common in PD cases than controls in 3 independent cohorts. Tyrosinase, encoded by TYR, is the rate-limiting enzyme for the production of neuromelanin, and has a role in the production of dopamine. These results suggest a possible role for another gene in the dopamine-biosynthetic pathway in susceptibility to neurodegenerative Parkinsonism, but further studies in larger PD cohorts are needed to accurately determine the role of these genes/variants in disease pathogenesis. Copyright © 2016 The Author(s). Published by Elsevier Inc. All rights reserved.

Council Chamber exhibition

CERN Multimedia

CERN Bulletin

2010-01-01

To complete the revamp of CERN’s Council Chamber, a new exhibition is being installed just in time for the June Council meetings.   Panels will showcase highlights of CERN’s history, using some of the content prepared for the exhibitions marking 50 years of the PS, which were displayed in the main building last November. The previous photo exhibition in the Council Chamber stopped at the 1970s. To avoid the new panels becoming quickly out of date, photos are grouped together around specific infrastructures, rather than following a classic time-line. “We have put the focus on the accelerators – the world-class facilities that CERN has been offering researchers over the years, from the well-known large colliders to the lesser-known smaller facilities,” says Emma Sanders, who worked on the content. The new exhibition will be featured in a future issue of the Bulletin with photos and an interview with Fabienne Marcastel, designer of the exhibit...

Extreme magnetoresistance in magnetic rare-earth monopnictides

Science.gov (United States)

Ye, Linda; Suzuki, Takehito; Wicker, Christina R.; Checkelsky, Joseph G.

2018-02-01

The acute sensitivity of the electrical resistance of certain systems to magnetic fields known as extreme magnetoresistance (XMR) has recently been explored in a new materials context with topological semimetals. Exemplified by WTe2 and rare-earth monopnictide La(Sb,Bi), these systems tend to be nonmagnetic, nearly compensated semimetals and represent a platform for large magnetoresistance driven by intrinsic electronic structure. Here we explore electronic transport in magnetic members of the latter family of semimetals and find that XMR is strongly modulated by magnetic order. In particular, CeSb exhibits XMR in excess of 1.6 ×106% at fields of 9 T whereas the magnetoresistance itself is nonmonotonic across the various magnetic phases and shows a transition from negative magnetoresistance to XMR with fields above magnetic ordering temperature TN. The magnitude of the XMR is larger than in other rare-earth monopnictides including the nonmagnetic members and follows a nonsaturating power law to fields above 30 T. We show that the overall response can be understood as the modulation of conductivity by the Ce orbital state and for intermediate temperatures can be characterized by an effective medium model. Comparison to the orbitally quenched compound GdBi supports the correlation of XMR with the onset of magnetic ordering and compensation and highlights the unique combination of orbital inversion and type-I magnetic ordering in CeSb in determining its large response. These findings suggest a paradigm for magneto-orbital control of XMR and are relevant to the understanding of rare-earth-based correlated topological materials.

Extraction of rare earths from iron-rich rare earth deposits

OpenAIRE

Bisaka, K.; Thobadi, I.C.; Pawlik, C.

2017-01-01

Rare earth metals are classified as critical metals by the United Nations, as they have found wide application in the fabrication of magnets, particularly those used in green energy technologies which mitigate global warming. Processing of ores containing rare earth elements is complex, and differs according to the nature of each ore. In the conventional process, run of mine (ROM) ores are processed in a physical separation plant to produce a concentrate from which rare earth elements are ext...

Lost in a random forest: Using Big Data to study rare events

Directory of Open Access Journals (Sweden)

Christopher A Bail

2015-12-01

Full Text Available Sudden, broad-scale shifts in public opinion about social problems are relatively rare. Until recently, social scientists were forced to conduct post-hoc case studies of such unusual events that ignore the broader universe of possible shifts in public opinion that do not materialize. The vast amount of data that has recently become available via social media sites such as Facebook and Twitter—as well as the mass-digitization of qualitative archives provide an unprecedented opportunity for scholars to avoid such selection on the dependent variable. Yet the sheer scale of these new data creates a new set of methodological challenges. Conventional linear models, for example, minimize the influence of rare events as “outliers”—especially within analyses of large samples. While more advanced regression models exist to analyze outliers, they suffer from an even more daunting challenge: equifinality, or the likelihood that rare events may occur via different causal pathways. I discuss a variety of possible solutions to these problems—including recent advances in fuzzy set theory and machine learning—but ultimately advocate an ecumenical approach that combines multiple techniques in iterative fashion.

Resources, challenges and way forward in rare mitochondrial diseases research [v1; ref status: indexed, http://f1000r.es/54x

Directory of Open Access Journals (Sweden)

Anshu Bhardwaj

2015-03-01

Full Text Available Over 300 million people are affected by about 7000 rare diseases globally. There are tremendous resource limitations and challenges in driving research and drug development for rare diseases. Hence, innovative approaches are needed to identify potential solutions. This review focuses on the resources developed over the past years for analysis of genome data towards understanding disease biology especially in the context of mitochondrial diseases, given that mitochondria are central to major cellular pathways and their dysfunction leads to a broad spectrum of diseases. Platforms for collaboration of research groups, clinicians and patients and the advantages of community collaborative efforts in addressing rare diseases are also discussed. The review also describes crowdsourcing and crowdfunding efforts in rare diseases research and how the upcoming initiatives for understanding disease biology including analyses of large number of genomes are also applicable to rare diseases.

EXHIBITION: Accelerated Particles

CERN Multimedia

2004-01-01

An exhibition of plastic arts and two evenings of performances by sound and visual artists as part of CERN's 50th anniversary celebrations. Fifty candles for CERN, an international laboratory renowned for fundamental research, is a cause for celebration. Since March this year, Geneva and neighbouring parts of France have been the venues for a wealth of small and large-scale events, which will continue until November. Given CERN's location in the commune of Meyrin, the ForuMeyrin is hosting exhibitions of plastic arts and performances entitled: Accelerated Particles. Several works will be exhibited and performed in two 'salons'. Salon des matières: An exhibition of plastic arts From Tues 12 October to Wed 3 November 2004 Tuesdays to Fridays: 16:00 to 19:00 Saturdays: 14:00 to 18:00 Exhibition open late on performance nights, entrance free Salon des particules: Musical and visual performances Tues 12 and Mon 25 October from 20:00 to 23:00 Preview evening for both events: Tues 12 October from 18:...

Rare Case of a Chondrosarcoma of the Mandible in a Child

Directory of Open Access Journals (Sweden)

Hendryk Vieweg

2013-01-01

Full Text Available Chondrosarcoma of the mandible is rare, especially in children. The available literature consists mostly of a few case reports which are partly integrated in small studies. Growing this small pool of literature is helpful in solidifying knowledge about this disease and facilitating appropriate treatment for children. Therefore, we present such a case in a 12-year-old boy, exhibit comprehensive and relevant information concerning this entity, and discuss our findings in the context of other publications.

China's rare-earth industry

Science.gov (United States)

Tse, Pui-Kwan

2011-01-01

Introduction China's dominant position as the producer of over 95 percent of the world output of rare-earth minerals and rapid increases in the consumption of rare earths owing to the emergence of new clean-energy and defense-related technologies, combined with China's decisions to restrict exports of rare earths, have resulted in heightened concerns about the future availability of rare earths. As a result, industrial countries such as Japan, the United States, and countries of the European Union face tighter supplies and higher prices for rare earths. This paper briefly reviews China's rare-earth production, consumption, and reserves and the important policies and regulations regarding the production and trade of rare earths, including recently announced export quotas. The 15 lanthanide elements-lanthanum, cerium, praseodymium, neodymium, promethium, samarium, europium, gadolinium, terbium, dysprosium, holmium, erbium, thulium, ytterbium, and lutetium (atomic numbers 57-71)-were originally known as the rare earths from their occurrence in oxides mixtures. Recently, some researchers have included two other elements-scandium and yttrium-in their discussion of rare earths. Yttrium (atomic number 39), which lies above lanthanum in transition group III of the periodic table and has a similar 3+ ion with a noble gas core, has both atomic and ionic radii similar in size to those of terbium and dysprosium and is generally found in nature with lanthanides. Scandium (atomic number 21) has a smaller ionic radius than yttrium and the lanthanides, and its chemical behavior is intermediate between that of aluminum and the lanthanides. It is found in nature with the lanthanides and yttrium. Rare earths are used widely in high-technology and clean-energy products because they impart special properties of magnetism, luminescence, and strength. Rare earths are also used in weapon systems to obtain the same properties.

Three-dimensional MR imaging of the cerebrospinal system with the RARE technique

International Nuclear Information System (INIS)

Hennig, J.; Ott, D.; Ylayasski, J.

1987-01-01

Three-dimensional RARE myelography is a fast technique for high-resolution imaging of the cerebrospinal fluid. A data set with 1 x 1 x 1-mm resolution can be generated with a 12-minute acquisition time. Sophisticated three-dimensional display algorithms allow reconstruction of planes at arbitrary angles and full three-dimensional displays, which yield extremely useful information for neurosurgical planning. Additionally, the injection of contrast agent can be simulated on the computer and communication pathways between structures of interest can be found noninvasively

Mining with Rare Cases

Science.gov (United States)

Weiss, Gary M.

Rare cases are often the most interesting cases. For example, in medical diagnosis one is typically interested in identifying relatively rare diseases, such as cancer, rather than more frequently occurring ones, such as the common cold. In this chapter we discuss the role of rare cases in Data Mining. Specific problems associated with mining rare cases are discussed, followed by a description of methods for addressing these problems.

Resources, challenges and way forward in rare mitochondrial diseases research [v2; ref status: indexed, http://f1000r.es/5r6

Directory of Open Access Journals (Sweden)

Neeraj Kumar Rajput

2015-08-01

Full Text Available Over 300 million people are affected by about 7000 rare diseases globally. There are tremendous resource limitations and challenges in driving research and drug development for rare diseases. Hence, innovative approaches are needed to identify potential solutions. This review focuses on the resources developed over the past years for analysis of genome data towards understanding disease biology especially in the context of mitochondrial diseases, given that mitochondria are central to major cellular pathways and their dysfunction leads to a broad spectrum of diseases. Platforms for collaboration of research groups, clinicians and patients and the advantages of community collaborative efforts in addressing rare diseases are also discussed. The review also describes crowdsourcing and crowdfunding efforts in rare diseases research and how the upcoming initiatives for understanding disease biology including analyses of large number of genomes are also applicable to rare diseases.

Britain exhibition at CERN

CERN Multimedia

Bertin; CERN PhotoLab

1969-01-01

The United Kingdom inaugurated the Industrial Exhibitions in 1968, and it wasn't till 1971 that other countries staged exhibitions at CERN. This photo was taken in 1969, at the second British exhibition, where 16 companies were present.

Charge ordering in the rare earth manganates: the experimental situation

International Nuclear Information System (INIS)

Rao, C.N.R.; Cheetham, A.K.; Raveau, Bernard

2000-01-01

Charge-ordered phases of rare earth manganates are novel manifestations arising from interactions between the charge carriers and phonons, giving rise to the localization of carriers at specific sites in the lattice below a certain temperature. Accompanying this phenomenon, the Mn 3+ (e g ) orbitals and the associated lattice distortions also exhibit long range ordering (orbital ordering). What makes the manganates even more interesting is the occurrence of complex spin ordering related to anisotropic magnetic interactions. In this article, we discuss the emerging scenario of charge-ordered rare earth manganates in the light of specific case studies and highlight some of the new experimental findings related to spin, orbital and charge ordering. We also examine features such as the charge stripes and phase separation found experimentally in these materials, and discuss the factors that affect charge-ordering such as the size of A-site cations and magnetic and electric fields, as well as isotopic and chemical substitutions. (author)

Opportunities to change development pathways toward lower greenhouse gas emissions through energy efficiency

Energy Technology Data Exchange (ETDEWEB)

Alterra, Swart; Masanet, Eric; Lecocq, Franck; Najam, Adil; Schaeffer, Robert; Winkler, Harald; Sathaye, Jayant

2008-07-04

There is a multiplicity of development pathways in which low energy sector emissions are not necessarily associated with low economic growth. However, changes in development pathways can rarely be imposed from the top. On this basis, examples of energy efficiency opportunities to change development pathways toward lower emissions are presented in this paper. We review opportunities at the sectoral and macro level. The potential for action on nonclimate policies that influence energy use and emissions are presented. Examples are drawn from policies already adopted and implemented in the energy sector. The paper discusses relationships between energy efficiency policies and their synergies and tradeoffs with sustainable development and greenhouse gas emissions. It points to ways that energy efficiency could be mainstreamed into devel?opment choices.

Digital collections and exhibits

CERN Document Server

Denzer, Juan

2015-01-01

Today's libraries are taking advantage of cutting-edge technologies such as flat panel displays using touch, sound, and hands-free motions to design amazing exhibits using everything from simple computer hardware to advanced technologies such as the Microsoft Kinect. Libraries of all types are striving to add new interactive experiences for their patrons through exciting digital exhibits, both online and off. Digital Collections and Exhibits takes away the mystery of designing stunning digital exhibits to spotlight library trea

The complement system in age-related macular degeneration: A review of rare genetic variants and implications for personalized treatment

NARCIS (Netherlands)

Geerlings, M.J.; Jong, E.K.; Hollander, A.I. den

2017-01-01

Age-related macular degeneration (AMD) is a progressive retinal disease and the major cause of irreversible vision loss in the elderly. Numerous studies have found both common and rare genetic variants in the complement pathway to play a role in the pathogenesis of AMD. In this review we provide an

Rare earth octacyanomolybdates(4)

International Nuclear Information System (INIS)

Zubritskaya, D.I.; Sergeeva, A.N.; Pisak, Yu.V.

1980-01-01

Optimal conditions for synthesis of rare-earth octacyanomolybdates(4) of the Ln 4 [Mo(CN) 8 ] 3 xnH 2 O composition (where Ln is a rare-earth element, other than Pr, Pm, Lu, Tb) have been worked out. The synthesis has been accomplished by neutralization with octacianomolybdic acid with rare-earth carbonates. The composition and structure of the compounds synthesized have been studied by infrared-spectroscopy. It has been established that rare-earth octacyanomolybdates(4) form three isostructural groups

Pathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathway.

Science.gov (United States)

Fineran, Paul; Lloyd-Evans, Emyr; Lack, Nathan A; Platt, Nick; Davis, Lianne C; Morgan, Anthony J; Höglinger, Doris; Tatituri, Raju Venkata V; Clark, Simon; Williams, Ian M; Tynan, Patricia; Al Eisa, Nada; Nazarova, Evgeniya; Williams, Ann; Galione, Antony; Ory, Daniel S; Besra, Gurdyal S; Russell, David G; Brenner, Michael B; Sim, Edith; Platt, Frances M

2016-11-18

Tuberculosis remains a major global health concern. The ability to prevent phagosome-lysosome fusion is a key mechanism by which intracellular mycobacteria, including Mycobacterium tuberculosis , achieve long-term persistence within host cells. The mechanisms underpinning this key intracellular pro-survival strategy remain incompletely understood. Host macrophages infected with persistent mycobacteria share phenotypic similarities with cells taken from patients suffering from Niemann-Pick Disease Type C (NPC), a rare lysosomal storage disease in which endocytic trafficking defects and lipid accumulation within the lysosome lead to cell dysfunction and cell death. We investigated whether these shared phenotypes reflected an underlying mechanistic connection between mycobacterial intracellular persistence and the host cell pathway dysfunctional in NPC. The induction of NPC phenotypes in macrophages from wild-type mice or obtained from healthy human donors was assessed via infection with mycobacteria and subsequent measurement of lipid levels and intracellular calcium homeostasis. The effect of NPC therapeutics on intracellular mycobacterial load was also assessed. Macrophages infected with persistent intracellular mycobacteria phenocopied NPC cells, exhibiting accumulation of multiple lipid types, reduced lysosomal Ca 2+ levels, and defects in intracellular trafficking. These NPC phenotypes could also be induced using only lipids/glycomycolates from the mycobacterial cell wall. These data suggest that persistent intracellular mycobacteria inhibit the NPC pathway, likely via inhibition of the NPC1 protein, and subsequently induce altered acidic store Ca 2+ homeostasis. Reduced lysosomal calcium levels may provide a mechanistic explanation for the reduced levels of phagosome-lysosome fusion in mycobacterial infection. Treatments capable of correcting defects in NPC mutant cells via modulation of host cell calcium were of benefit in promoting clearance of mycobacteria

Two-Electron Transfer Pathways.

Science.gov (United States)

Lin, Jiaxing; Balamurugan, D; Zhang, Peng; Skourtis, Spiros S; Beratan, David N

2015-06-18

The frontiers of electron-transfer chemistry demand that we develop theoretical frameworks to describe the delivery of multiple electrons, atoms, and ions in molecular systems. When electrons move over long distances through high barriers, where the probability for thermal population of oxidized or reduced bridge-localized states is very small, the electrons will tunnel from the donor (D) to acceptor (A), facilitated by bridge-mediated superexchange interactions. If the stable donor and acceptor redox states on D and A differ by two electrons, it is possible that the electrons will propagate coherently from D to A. While structure-function relations for single-electron superexchange in molecules are well established, strategies to manipulate the coherent flow of multiple electrons are largely unknown. In contrast to one-electron superexchange, two-electron superexchange involves both one- and two-electron virtual intermediate states, the number of virtual intermediates increases very rapidly with system size, and multiple classes of pathways interfere with one another. In the study described here, we developed simple superexchange models for two-electron transfer. We explored how the bridge structure and energetics influence multielectron superexchange, and we compared two-electron superexchange interactions to single-electron superexchange. Multielectron superexchange introduces interference between singly and doubly oxidized (or reduced) bridge virtual states, so that even simple linear donor-bridge-acceptor systems have pathway topologies that resemble those seen for one-electron superexchange through bridges with multiple parallel pathways. The simple model systems studied here exhibit a richness that is amenable to experimental exploration by manipulating the multiple pathways, pathway crosstalk, and changes in the number of donor and acceptor species. The features that emerge from these studies may assist in developing new strategies to deliver multiple

Evolutionary rate patterns of the Gibberellin pathway genes

Directory of Open Access Journals (Sweden)

Zhang Fu-min

2009-08-01

Full Text Available Abstract Background Analysis of molecular evolutionary patterns of different genes within metabolic pathways allows us to determine whether these genes are subject to equivalent evolutionary forces and how natural selection shapes the evolution of proteins in an interacting system. Although previous studies found that upstream genes in the pathway evolved more slowly than downstream genes, the correlation between evolutionary rate and position of the genes in metabolic pathways as well as its implications in molecular evolution are still less understood. Results We sequenced and characterized 7 core structural genes of the gibberellin biosynthetic pathway from 8 representative species of the rice tribe (Oryzeae to address alternative hypotheses regarding evolutionary rates and patterns of metabolic pathway genes. We have detected significant rate heterogeneity among 7 GA pathway genes for both synonymous and nonsynonymous sites. Such rate variation is mostly likely attributed to differences of selection intensity rather than differential mutation pressures on the genes. Unlike previous argument that downstream genes in metabolic pathways would evolve more slowly than upstream genes, the downstream genes in the GA pathway did not exhibited the elevated substitution rate and instead, the genes that encode either the enzyme at the branch point (GA20ox or enzymes catalyzing multiple steps (KO, KAO and GA3ox in the pathway had the lowest evolutionary rates due to strong purifying selection. Our branch and codon models failed to detect signature of positive selection for any lineage and codon of the GA pathway genes. Conclusion This study suggests that significant heterogeneity of evolutionary rate of the GA pathway genes is mainly ascribed to differential constraint relaxation rather than the positive selection and supports the pathway flux theory that predicts that natural selection primarily targets enzymes that have the greatest control on fluxes.

Research on refractory, reactive and rare metals in BARC

International Nuclear Information System (INIS)

Banerjee, Srikumar

2016-01-01

Material processing activities were given a due thrust in Atomic Energy Programme right from the beginning. Initially research was primarily focused on metals such as uranium, thorium and zirconium which are of direct relevance to the nuclear programme. Having attained the success in processing these metals from the indigenous resources, the scope and range of material processing activities were enhanced and broadened considerably in the subsequent years. Having mastered the Kroll process for zirconium extraction, the same process was adopted in laboratory and in pilot scales for the extraction of titanium. With the experience gained in processing and handling reactive metals, flow sheet development of group V-A and VI-A metals was taken up. This presentation will summarise how different unit operations for the processing of Mo, V, Ta, W and Nb were developed and optimized. These elements have the same crystal structure (bcc), high melting points, similar chemical interactions with other elements, generally exhibiting high ductile to brittle transition temperatures and have similar alloying behavior. The general approach of processing and purification of these metals for improving the strength and ductability are discussed. The principles for the development of protective coatings on these reactive metals are also covered. The manufacturing process for the multi-filamentary superconducting cable made out of Niobium is described to illustrate the success story of the development of entire chain of operations leading to the final deployable product meeting the exacting specifications. The challenges faced in the development of beryllium, another reactive and toxic metal is briefly discussed. In order to exploit the sizeable resources of rare earth elements in India, research on processing of rare earths was initiated quite early. In the recent past there have been efforts on separation of individual rare earth elements and in preparing rare earth compounds and inter

Rare B decays, rare τ decays, and grand unification

International Nuclear Information System (INIS)

Sher, M.; Yuan, Y.

1991-01-01

In multi-Higgs-boson extensions of the standard model, tree-level flavor-changing neutral currents exist naturally, unless suppressed by some symmetry. For a given rate, the exchanged scalar or pseudoscalar mass is very sensitive to the flavor-changing coupling between the first two generations. Since the Yukawa couplings of the first two generations are unknown and certainly very small, bounds which rely on some assumed value of this flavor-changing coupling are quite dubious. One might expect the size (and reliability) of the Yukawa couplings involving the third generation to be greater. In this paper, we consider processes involving τ's and B's, and determine the bounds on the flavor-changing couplings which involve third-generation fields. The strongest bound in the quark sector comes from B-bar B mixing and in the lepton sector, surprisingly, from μ→eγ. It is then noted that the flavor-changing couplings in the quark sector are related to those in the lepton sector in many grand unified theories, and one can ask whether an analysis of rare τ decays or rare B decays will provide the strongest constraints. We show that rare B decays provide the strongest bounds, and that no useful information can be obtained from rare τ decays. It is also noted that the most promising decay modes are B→Kμτ and B s →μτ, and we urge experimenters to look for rare decay modes of the B in which a τ is in the final state

Rare particles

International Nuclear Information System (INIS)

Kutschera, W.

1984-01-01

The use of Accelerator Mass Spectrometry (AMS) to search for hypothetical particles and known particles of rare processes is discussed. The hypothetical particles considered include fractionally charged particles, anomalously heavy isotopes, and superheavy elements. The known particles produced in rare processes discussed include doubly-charged negative ions, counting neutrino-produced atoms in detectors for solar neutrino detection, and the spontaneous emission of 14 C from 223 Ra. 35 references

Spontaneously immortalised bovine mammary epithelial cells exhibit a distinct gene expression pattern from the breast cancer cells

Directory of Open Access Journals (Sweden)

Li Qianqian

2010-10-01

Full Text Available Abstract Background Spontaneous immortalisation of cultured mammary epithelial cells (MECs is an extremely rare event, and the molecular mechanism behind spontaneous immortalisation of MECs is unclear. Here, we report the establishment of a spontaneously immortalised bovine mammary epithelial cell line (BME65Cs and the changes in gene expression associated with BME65Cs cells. Results BME65Cs cells maintain the general characteristics of normal mammary epithelial cells in morphology, karyotype and immunohistochemistry, and are accompanied by the activation of endogenous bTERT (bovine Telomerase Reverse Transcriptase and stabilisation of the telomere. Currently, BME65Cs cells have been passed for more than 220 generations, and these cells exhibit non-malignant transformation. The expression of multiple genes was investigated in BME65Cs cells, senescent BMECs (bovine MECs cells, early passage BMECs cells and MCF-7 cells (a human breast cancer cell line. In comparison with early passage BMECs cells, the expression of senescence-relevant apoptosis-related gene were significantly changed in BME65Cs cells. P16INK4a was downregulated, p53 was low expressed and Bax/Bcl-2 ratio was reversed. Moreover, a slight upregulation of the oncogene c-Myc, along with an undetectable level of breast tumor-related gene Bag-1 and TRPS-1, was observed in BME65Cs cells while these genes are all highly expressed in MCF-7. In addition, DNMT1 is upregulated in BME65Cs. These results suggest that the inhibition of both senescence and mitochondrial apoptosis signalling pathways contribute to the immortality of BME65Cs cells. The expression of p53 and p16INK4a in BME65Cs was altered in the pattern of down-regulation but not "loss", suggesting that this spontaneous immortalization is possibly initiated by other mechanism rather than gene mutation of p53 or p16INK4a. Conclusions Spontaneously immortalised BME65Cs cells maintain many characteristics of normal BMEC cells and

Rare earths as a future resource

International Nuclear Information System (INIS)

Cornell, D.H.

1988-01-01

The fourteen rare earth or lanthanide elements have recently emerged as an important natural resource because of the rapidly growing demand in the electronic, chemical and metallurgical industries. The Symposium on rare earth elements as a future resource presented a multidisciplinary review of rare earth chemistry, geology, beneficiation, industrial applications and marketing. Papers by experts in many fields were presented on the following topics: chemical properties of the rare earth elements; the analysis of rare earth elements and minerals; beneficiation and extraction of rare earth elements; economic geochemistry and mineralogy of rare earths; present industrial uses of rare earth elements; the role of rare earth elements in high-temperature superconductors; the technical application of high-temperature superconductors; supply and demand for rare earth products - now and in the future, and the geology of rare earth deposits

Multiferroic nature of charge-ordered rare earth manganites

Energy Technology Data Exchange (ETDEWEB)

Serrao, Claudy Rayan [Chemistry and Physics of Materials Unit, Jawaharlal Nehru Centre for Advanced Scientific Research, Jakkur PO, Bangalore-560064 (India); Sundaresan, A [Chemistry and Physics of Materials Unit, Jawaharlal Nehru Centre for Advanced Scientific Research, Jakkur PO, Bangalore-560064 (India); Rao, C N R [Chemistry and Physics of Materials Unit, Jawaharlal Nehru Centre for Advanced Scientific Research, Jakkur PO, Bangalore-560064 (India)

2007-12-12

Charge-ordered rare earth manganites Nd{sub 0.5}Ca{sub 0.5}MnO{sub 3},La{sub 0.25}Nd{sub 0.25}Ca{sub 0.5}MnO{sub 3}, Pr{sub 0.7}Ca{sub 0.3}MnO{sub 3} and Pr{sub 0.6}Ca{sub 0.4}MnO{sub 3} are found to exhibit dielectric constant anomalies around the charge-ordering or the antiferromagnetic transition temperatures. Magnetic fields have a marked effect on the dielectric properties, indicating the presence of coupling between the magnetic and electrical order parameters. The observation of magnetoferroelectricity in these manganites is in accord with the recent theoretical predictions of Khomskii and co-workers.

Discrimination? - Exhibition of posters

OpenAIRE

Jakimovska, Jana

2017-01-01

Participation in the exhibition with the students form the Art Academy. The exhibition consisted of 15 posters tackling the subjects of hate speech and discrimination. The exhibition happened thanks to the invitation of the Faculty of Law at UGD, and it was a part of a larger event of launching books on the aforementioned subjects.

Deciphering chemotaxis pathways using cross species comparisons

Directory of Open Access Journals (Sweden)

Armitage Judith P

2010-01-01

Full Text Available Abstract Background Chemotaxis is the process by which motile bacteria sense their chemical environment and move towards more favourable conditions. Escherichia coli utilises a single sensory pathway, but little is known about signalling pathways in species with more complex systems. Results To investigate whether chemotaxis pathways in other bacteria follow the E. coli paradigm, we analysed 206 species encoding at least 1 homologue of each of the 5 core chemotaxis proteins (CheA, CheB, CheR, CheW and CheY. 61 species encode more than one of all of these 5 proteins, suggesting they have multiple chemotaxis pathways. Operon information is not available for most bacteria, so we developed a novel statistical approach to cluster che genes into putative operons. Using operon-based models, we reconstructed putative chemotaxis pathways for all 206 species. We show that cheA-cheW and cheR-cheB have strong preferences to occur in the same operon as two-gene blocks, which may reflect a functional requirement for co-transcription. However, other che genes, most notably cheY, are more dispersed on the genome. Comparison of our operons with shuffled equivalents demonstrates that specific patterns of genomic location may be a determining factor for the observed in vivo chemotaxis pathways. We then examined the chemotaxis pathways of Rhodobacter sphaeroides. Here, the PpfA protein is known to be critical for correct partitioning of proteins in the cytoplasmically-localised pathway. We found ppfA in che operons of many species, suggesting that partitioning of cytoplasmic Che protein clusters is common. We also examined the apparently non-typical chemotaxis components, CheA3, CheA4 and CheY6. We found that though variants of CheA proteins are rare, the CheY6 variant may be a common type of CheY, with a significantly disordered C-terminal region which may be functionally significant. Conclusions We find that many bacterial species potentially have multiple

A rare metastasis from a rare brain tumour

DEFF Research Database (Denmark)

Aabenhus, Kristine; Hahn, Christoffer Holst

2014-01-01

This case report presents the story of a patient with an oligodendroglioma metastasizing to the bone marrow and to lymph nodes of the neck. The patient had undergone primary brain surgery 13 years prior to the discovery of metastases and radiotherapy directed at the brain tumour two months prior........ Oligodendroglioma are rare primary brain tumours of which extraneural metastasis is even more rare. The incidence of cases like this may be increasing because of better treatment and thus longer survival of patients with oligodendroglioma....

Rare Neural Correlations Implement Robotic Conditioning with Delayed Rewards and Disturbances

Science.gov (United States)

Soltoggio, Andrea; Lemme, Andre; Reinhart, Felix; Steil, Jochen J.

2013-01-01

Neural conditioning associates cues and actions with following rewards. The environments in which robots operate, however, are pervaded by a variety of disturbing stimuli and uncertain timing. In particular, variable reward delays make it difficult to reconstruct which previous actions are responsible for following rewards. Such an uncertainty is handled by biological neural networks, but represents a challenge for computational models, suggesting the lack of a satisfactory theory for robotic neural conditioning. The present study demonstrates the use of rare neural correlations in making correct associations between rewards and previous cues or actions. Rare correlations are functional in selecting sparse synapses to be eligible for later weight updates if a reward occurs. The repetition of this process singles out the associating and reward-triggering pathways, and thereby copes with distal rewards. The neural network displays macro-level classical and operant conditioning, which is demonstrated in an interactive real-life human-robot interaction. The proposed mechanism models realistic conditioning in humans and animals and implements similar behaviors in neuro-robotic platforms. PMID:23565092

Pathway Distiller - multisource biological pathway consolidation.

Science.gov (United States)

Doderer, Mark S; Anguiano, Zachry; Suresh, Uthra; Dashnamoorthy, Ravi; Bishop, Alexander J R; Chen, Yidong

2012-01-01

One method to understand and evaluate an experiment that produces a large set of genes, such as a gene expression microarray analysis, is to identify overrepresentation or enrichment for biological pathways. Because pathways are able to functionally describe the set of genes, much effort has been made to collect curated biological pathways into publicly accessible databases. When combining disparate databases, highly related or redundant pathways exist, making their consolidation into pathway concepts essential. This will facilitate unbiased, comprehensive yet streamlined analysis of experiments that result in large gene sets. After gene set enrichment finds representative pathways for large gene sets, pathways are consolidated into representative pathway concepts. Three complementary, but different methods of pathway consolidation are explored. Enrichment Consolidation combines the set of the pathways enriched for the signature gene list through iterative combining of enriched pathways with other pathways with similar signature gene sets; Weighted Consolidation utilizes a Protein-Protein Interaction network based gene-weighting approach that finds clusters of both enriched and non-enriched pathways limited to the experiments' resultant gene list; and finally the de novo Consolidation method uses several measurements of pathway similarity, that finds static pathway clusters independent of any given experiment. We demonstrate that the three consolidation methods provide unified yet different functional insights of a resultant gene set derived from a genome-wide profiling experiment. Results from the methods are presented, demonstrating their applications in biological studies and comparing with a pathway web-based framework that also combines several pathway databases. Additionally a web-based consolidation framework that encompasses all three methods discussed in this paper, Pathway Distiller (http://cbbiweb.uthscsa.edu/PathwayDistiller), is established to allow

EXHIBITION: Accelerated Particles

CERN Multimedia

2004-01-01

http://www.cern.ch/cern50/ An exhibition of plastic arts and two evenings of performances by sound and visual artists as part of CERN's fiftieth anniversary celebrations. The fiftieth anniversary of a world famous organization like CERN, an international laboratory specializing in fundamental research, is a cause for celebration. Since March this year, Geneva and neighbouring parts of France have been the venues for a wealth of small and large-scale events, which will continue until November. Given CERN's location in the commune of Meyrin, the ForuMeyrin is hosting two "salons" consisting of an exhibition of plastic arts and evenings of music and visual arts performances with the collective title of "Accelerated Particles". Several works will be exhibited and performed. Salon des matières: An exhibition of plastic arts Until Wednesday 3 November 2004. Tuesdays to Fridays: 4.00 p.m. to 7.00 p.m. Saturdays: 2.00 p.m. to 6.00 p.m. Doors open late on the evening of the performances. Salon des ...

Exhibiting the Human/Exhibiting the Cyborg: “Who Am I?”

Directory of Open Access Journals (Sweden)

Sophia C. Vackimes

2013-08-01

Full Text Available The role of the museum in shaping our relationship to science and technology, particularly cyborgization, is illuminated by a close examination of the Who Am I permanent exhibition in the Wellcome Wing of the Science Museum of London. This innovative exhibition raises real questions both about the human-technology-science relationship but also about museography. In the context of the history and current practices of museums engaging contemporary technological developments the evidence suggest that even as the Who am I? exhibit did break somewhat from previous approaches, especially the didactic presentation of the socially useful, it has not changed the feld as a whole.

Oxygen diffusion pathways in a cofactor-independent dioxygenase

Science.gov (United States)

Di Russo, Natali V.; Condurso, Heather L.; Li, Kunhua; Bruner, Steven D.; Roitberg, Adrian E.

2015-01-01

Molecular oxygen plays an important role in a wide variety of enzymatic reactions. Through recent research efforts combining computational and experimental methods a new view of O2 diffusion is emerging, where specific channels guide O2 to the active site. The focus of this work is DpgC, a cofactor-independent oxygenase. Molecular dynamics simulations, together with mutagenesis experiments and xenon-binding data, reveal that O2 reaches the active site of this enzyme using three main pathways and four different access points. These pathways connect a series of dynamic hydrophobic pockets, concentrating O2 at a specific face of the enzyme substrate. Extensive molecular dynamics simulations provide information about which pathways are more frequently used. This data is consistent with the results of kinetic measurements on mutants and is difficult to obtain using computational cavity-location methods. Taken together, our results reveal that although DpgC is rare in its ability of activating O2 in the absence of cofactors or metals, the way O2 reaches the active site is similar to that reported for other O2-using proteins: multiple access channels are available, and the architecture of the pathway network can provide regio- and stereoselectivity. Our results point to the existence of common themes in O2 access that are conserved among very different types of proteins. PMID:26508997

Co-existence of Endometriotic Cyst of the Ovary and Arias-Stella Reaction in a Non-Pregnant Woman: Report of a Rare Case.

Science.gov (United States)

Harikrishnan, Volga; Esaki, Muthuvel; Srinivasan, Chitra; Arockiasamy, Parimala; Ethirajan, Shanthi

2016-03-01

Endometriosis is defined as presence of endometrial tissue outside the uterus. It can occur anywhere in the ovary. In the ovary it is usually presented as cyst, termed as endometriotic cyst or Chocolate cyst. Arias-Stella reaction is usually seen in gestational endometrium or in ectopic gestation site and rarely in non-pregnant uterus with hormonal intake. Co-existence of endometriosis and Arias-Stella reaction is very rare. We present a very rare case of endometriotic cyst of the ovary exhibiting Arias -Stella reaction which was seen in of non pregnant patient without any history of hormonal intake.

Rare earth germanates

International Nuclear Information System (INIS)

Bondar', I.A.; Vinogradova, N.V.; Dem'yanets, L.N.

1983-01-01

From the viewpoint of structural chemistry and general regularities controlling formation reactions of compounds and phases in melts, solid and gaseous states, recent achievements in the chemistry of rare earth germanates are generalized. Methods of synthesizing germanates, systems on the base of germanium oxides and rare earths are considered. The data on crystallochemical characteristics are tabulated. Individual compounds of scandium germanate are also characterized. Processes of germanate formation using the data of IR-spectroscopy, X-ray phase analysis are studied. The structure and morphotropic series of rare earth germanates and silicates are determined. Fields of their present and possible future application are considered

Application of an imaging system to a museum exhibition for developing interactive exhibitions

Science.gov (United States)

Miyata, Kimiyoshi; Inoue, Yuka; Takiguchi, Takahiro; Tsumura, Norimichi; Nakaguchi, Toshiya; Miyake, Yoichi

2009-10-01

In the National Museum of Japanese History, 215,759 artifacts are stored and used for research and exhibitions. In museums, due to the limitation of space in the galleries, a guidance system is required to satisfy visitors' needs and to enhance their understanding of the artifacts. We introduce one exhibition using imaging technology to improve visitors' understanding of a kimono (traditional Japanese clothing) exhibition. In the imaging technology introduced, one data projector, one display with touch panel interface, and magnifiers were used as exhibition tools together with a real kimono. The validity of this exhibition method was confirmed by results from a visitors' interview survey. Second, to further develop the interactive guidance system, an augmented reality system that consisted of cooperation between the projector and a digital video camera was also examined. A white paper board in the observer's hand was used as a projection screen and also as an interface to control the images projected on the board. The basic performance of the proposed system was confirmed; however continuous development was necessary for applying the system to actual exhibitions.

A fast dynamic mode in rare earth based glasses

Energy Technology Data Exchange (ETDEWEB)

Zhao, L. Z.; Xue, R. J.; Zhu, Z. G.; Wang, W. H.; Bai, H. Y., E-mail: hybai@iphy.ac.cn [Institute of Physics, Chinese Academy of Sciences, Beijing 100190 (China); Ngai, K. L. [Dipartimento di Fisica, Università di Pisa, Largo B. Pontecorvo 3, I-56127 Pisa (Italy)

2016-05-28

Metallic glasses (MGs) usually exhibit only slow β-relaxation peak, and the signature of the fast dynamic is challenging to be observed experimentally in MGs. We report a general and unusual fast dynamic mode in a series of rare earth based MGs manifested as a distinct fast β′-relaxation peak in addition to slow β-relaxation and α-relaxation peaks. We show that the activation energy of the fast β′-relaxation is about 12RT{sub g} and is equivalent to the activation of localized flow event. The coupling of these dynamic processes as well as their relationship with glass transition and structural heterogeneity is discussed.

Exhibition; Image display agency

International Nuclear Information System (INIS)

Normazlin Ismail

2008-01-01

This article touches on the role of Malaysian Nuclear Agency as nuclear research institutions to promote, develop and encourage the peaceful uses of nuclear technology in its agricultural, medical, manufacturing, industrial, health and environment for the development of the country running successfully. Maturity of Malaysian Nuclear Agency in dealing with nuclear technology that are very competitive and globalization cannot be denied. On this basis Malaysian Nuclear Agency was given the responsibility to strengthen the nuclear technology in Malaysia. One way is through an exhibition featuring the research, discoveries and new technology products of the nuclear technology. Through this exhibition is to promote the nuclear technology and introduce the image of the agency in the public eye. This article also states a number of exhibits entered by the Malaysian Nuclear Agency and achievements during the last exhibition. Authors hope that the exhibition can be intensified further in the future.

Rare earths 1998 market update

International Nuclear Information System (INIS)

Tourre, J.M.

1998-01-01

The rare earth industry has always been a world of rapid change with the emergence of new markets, new ores and new players, as well as the disappearance of old applications. Rare earth based products are used in a great diversity of applications such as hard disk drives, CD drives, batteries, capacitors, pigments, ceramics, polishing powders, fuel cells, flints, catalyst converter, fluid cracking catalysts, etc. South East Asia holds the largest share of the known reserve of rare earth ores and is one of the major markets for rare earth compounds; in the last ten years, China has become the largest producer of rare earth intermediates as well as an important exporter of separated rare earth elements. Today, China has approximately 150 factories producing rare earth compounds, most of which are experiencing financial difficulties due to the lack of knowledge of true market needs, lack of control of their distribution channels and production over-capacity. Recently the Chinese rare earth producers have recognized the situation and efforts are underway to rationalize rare earth production. Japan has dominated many of the major application markets, and is by far the largest market for metal and alloy products. This will remain the case for the next five years; however, new countries are emerging as significant users of rare earth products such as Korea, Taiwan and Malaysia. During the last ten years rare earth producers adjusted to several radical changes that affected the raw materials, the application mix and the price structure. New producers have emerged, especially from China; some have subsequently stopped their activities while others have focused their efforts in a specific market segment

Rare earths: occurrence, production and applications

International Nuclear Information System (INIS)

Murthy, T.K.S.; Mukherjee, T.K.

2002-01-01

The mining and processing of rare earth minerals, particularly of monazite, began in a modest way in 1880s for commercialized production of mantle for gas lighting. For all major applications up to mid-twentieth century- production of lighter flints, misch metal as a metallurgical alloying agent, colouring, decolourizing and polishing agents for glass, petroleum cracking catalysts and arc-carbons, unseparated or partially separated rare earths were adequate. These applications continue till today. With the development and industrial application of powerful techniques like ion exchange and solvent extraction for the separation of rare earths, the decades after 1960 saw increasing utilization of the specific properties of the individual rare earths. Some of these advanced technological applications include: special glass for optical systems including camera lenses, phosphors for colour television, cathode ray tubes and fluorescent lighting, X-ray intensification screens, high intensity permanent magnets, electro optical devices, lasers, hydrogen storage materials, hydride rechargeable batteries, photomagnetic data storage systems, autoexhaust catalysts, special ceramics of unusual toughness, artificial diamonds and nonpoisonous plastic colorants. The topics covered in the book include rare earths: their story identity, rare earth resources, processing of ores and recovery of mixed rare earths products, separation and purification of rare earths, nonmetallic applications of rare earths, rare earth metals: production and applications, rare earth alloys and their applications, analysis of rare earth, processing of rare earth resources in India by Indian Rare Earth Ltd. and availability and market conditions

Protein-altering variants associated with body mass index implicate pathways that control energy intake and expenditure underpinning obesity

Science.gov (United States)

Turcot, Valérie; Lu, Yingchang; Highland, Heather M; Schurmann, Claudia; Justice, Anne E; Fine, Rebecca S; Bradfield, Jonathan P; Esko, Tõnu; Giri, Ayush; Graff, Mariaelisa; Guo, Xiuqing; Hendricks, Audrey E; Karaderi, Tugce; Lempradl, Adelheid; Locke, Adam E; Mahajan, Anubha; Marouli, Eirini; Sivapalaratnam, Suthesh; Young, Kristin L; Alfred, Tamuno; Feitosa, Mary F; Masca, Nicholas GD; Manning, Alisa K; Medina-Gomez, Carolina; Mudgal, Poorva; Ng, Maggie CY; Reiner, Alex P; Vedantam, Sailaja; Willems, Sara M; Winkler, Thomas W; Abecasis, Goncalo; Aben, Katja K; Alam, Dewan S; Alharthi, Sameer E; Allison, Matthew; Amouyel, Philippe; Asselbergs, Folkert W; Auer, Paul L; Balkau, Beverley; Bang, Lia E; Barroso, Inês; Bastarache, Lisa; Benn, Marianne; Bergmann, Sven; Bielak, Lawrence F; Blüher, Matthias; Boehnke, Michael; Boeing, Heiner; Boerwinkle, Eric; Böger, Carsten A; Bork-Jensen, Jette; Bots, Michiel L; Bottinger, Erwin P; Bowden, Donald W; Brandslund, Ivan; Breen, Gerome; Brilliant, Murray H; Broer, Linda; Brumat, Marco; Burt, Amber A; Butterworth, Adam S; Campbell, Peter T; Cappellani, Stefania; Carey, David J; Catamo, Eulalia; Caulfield, Mark J; Chambers, John C; Chasman, Daniel I; Chen, Yii-Der Ida; Chowdhury, Rajiv; Christensen, Cramer; Chu, Audrey Y; Cocca, Massimiliano; Collins, Francis S; Cook, James P; Corley, Janie; Galbany, Jordi Corominas; Cox, Amanda J; Crosslin, David S; Cuellar-Partida, Gabriel; D'Eustacchio, Angela; Danesh, John; Davies, Gail; de Bakker, Paul IW; de Groot, Mark CH; de Mutsert, Renée; Deary, Ian J; Dedoussis, George; Demerath, Ellen W; den Heijer, Martin; den Hollander, Anneke I; den Ruijter, Hester M; Dennis, Joe G; Denny, Josh C; Di Angelantonio, Emanuele; Drenos, Fotios; Du, Mengmeng; Dubé, Marie-Pierre; Dunning, Alison M; Easton, Douglas F; Edwards, Todd L; Ellinghaus, David; Ellinor, Patrick T; Elliott, Paul; Evangelou, Evangelos; Farmaki, Aliki-Eleni; Farooqi, I. Sadaf; Faul, Jessica D; Fauser, Sascha; Feng, Shuang; Ferrannini, Ele; Ferrieres, Jean; Florez, Jose C; Ford, Ian; Fornage, Myriam; Franco, Oscar H; Franke, Andre; Franks, Paul W; Friedrich, Nele; Frikke-Schmidt, Ruth; Galesloot, Tessel E.; Gan, Wei; Gandin, Ilaria; Gasparini, Paolo; Gibson, Jane; Giedraitis, Vilmantas; Gjesing, Anette P; Gordon-Larsen, Penny; Gorski, Mathias; Grabe, Hans-Jörgen; Grant, Struan FA; Grarup, Niels; Griffiths, Helen L; Grove, Megan L; Gudnason, Vilmundur; Gustafsson, Stefan; Haessler, Jeff; Hakonarson, Hakon; Hammerschlag, Anke R; Hansen, Torben; Harris, Kathleen Mullan; Harris, Tamara B; Hattersley, Andrew T; Have, Christian T; Hayward, Caroline; He, Liang; Heard-Costa, Nancy L; Heath, Andrew C; Heid, Iris M; Helgeland, Øyvind; Hernesniemi, Jussi; Hewitt, Alex W; Holmen, Oddgeir L; Hovingh, G Kees; Howson, Joanna MM; Hu, Yao; Huang, Paul L; Huffman, Jennifer E; Ikram, M Arfan; Ingelsson, Erik; Jackson, Anne U; Jansson, Jan-Håkan; Jarvik, Gail P; Jensen, Gorm B; Jia, Yucheng; Johansson, Stefan; Jørgensen, Marit E; Jørgensen, Torben; Jukema, J Wouter; Kahali, Bratati; Kahn, René S; Kähönen, Mika; Kamstrup, Pia R; Kanoni, Stavroula; Kaprio, Jaakko; Karaleftheri, Maria; Kardia, Sharon LR; Karpe, Fredrik; Kathiresan, Sekar; Kee, Frank; Kiemeney, Lambertus A; Kim, Eric; Kitajima, Hidetoshi; Komulainen, Pirjo; Kooner, Jaspal S; Kooperberg, Charles; Korhonen, Tellervo; Kovacs, Peter; Kuivaniemi, Helena; Kutalik, Zoltán; Kuulasmaa, Kari; Kuusisto, Johanna; Laakso, Markku; Lakka, Timo A; Lamparter, David; Lange, Ethan M; Lange, Leslie A; Langenberg, Claudia; Larson, Eric B; Lee, Nanette R; Lehtimäki, Terho; Lewis, Cora E; Li, Huaixing; Li, Jin; Li-Gao, Ruifang; Lin, Honghuang; Lin, Keng-Hung; Lin, Li-An; Lin, Xu; Lind, Lars; Lindström, Jaana; Linneberg, Allan; Liu, Ching-Ti; Liu, Dajiang J; Liu, Yongmei; Lo, Ken Sin; Lophatananon, Artitaya; Lotery, Andrew J; Loukola, Anu; Luan, Jian'an; Lubitz, Steven A; Lyytikäinen, Leo-Pekka; Männistö, Satu; Marenne, Gaëlle; Mazul, Angela L; McCarthy, Mark I; McKean-Cowdin, Roberta; Medland, Sarah E; Meidtner, Karina; Milani, Lili; Mistry, Vanisha; Mitchell, Paul; Mohlke, Karen L; Moilanen, Leena; Moitry, Marie; Montgomery, Grant W; Mook-Kanamori, Dennis O; Moore, Carmel; Mori, Trevor A; Morris, Andrew D; Morris, Andrew P; Müller-Nurasyid, Martina; Munroe, Patricia B; Nalls, Mike A; Narisu, Narisu; Nelson, Christopher P; Neville, Matt; Nielsen, Sune F; Nikus, Kjell; Njølstad, Pål R; Nordestgaard, Børge G; Nyholt, Dale R; O'Connel, Jeffrey R; O’Donoghue, Michelle L.; Olde Loohuis, Loes M; Ophoff, Roel A; Owen, Katharine R; Packard, Chris J; Padmanabhan, Sandosh; Palmer, Colin NA; Palmer, Nicholette D; Pasterkamp, Gerard; Patel, Aniruddh P; Pattie, Alison; Pedersen, Oluf; Peissig, Peggy L; Peloso, Gina M; Pennell, Craig E; Perola, Markus; Perry, James A; Perry, John RB; Pers, Tune H; Person, Thomas N; Peters, Annette; Petersen, Eva RB; Peyser, Patricia A; Pirie, Ailith; Polasek, Ozren; Polderman, Tinca J; Puolijoki, Hannu; Raitakari, Olli T; Rasheed, Asif; Rauramaa, Rainer; Reilly, Dermot F; Renström, Frida; Rheinberger, Myriam; Ridker, Paul M; Rioux, John D; Rivas, Manuel A; Roberts, David J; Robertson, Neil R; Robino, Antonietta; Rolandsson, Olov; Rudan, Igor; Ruth, Katherine S; Saleheen, Danish; Salomaa, Veikko; Samani, Nilesh J; Sapkota, Yadav; Sattar, Naveed; Schoen, Robert E; Schreiner, Pamela J; Schulze, Matthias B; Scott, Robert A; Segura-Lepe, Marcelo P; Shah, Svati H; Sheu, Wayne H-H; Sim, Xueling; Slater, Andrew J; Small, Kerrin S; Smith, Albert Vernon; Southam, Lorraine; Spector, Timothy D; Speliotes, Elizabeth K; Starr, John M; Stefansson, Kari; Steinthorsdottir, Valgerdur; Stirrups, Kathleen E; Strauch, Konstantin; Stringham, Heather M; Stumvoll, Michael; Sun, Liang; Surendran, Praveen; Swift, Amy J; Tada, Hayato; Tansey, Katherine E; Tardif, Jean-Claude; Taylor, Kent D; Teumer, Alexander; Thompson, Deborah J; Thorleifsson, Gudmar; Thorsteinsdottir, Unnur; Thuesen, Betina H; Tönjes, Anke; Tromp, Gerard; Trompet, Stella; Tsafantakis, Emmanouil; Tuomilehto, Jaakko; Tybjaerg-Hansen, Anne; Tyrer, Jonathan P; Uher, Rudolf; Uitterlinden, André G; Uusitupa, Matti; van der Laan, Sander W; van Duijn, Cornelia M; van Leeuwen, Nienke; van Setten, Jessica; Vanhala, Mauno; Varbo, Anette; Varga, Tibor V; Varma, Rohit; Velez Edwards, Digna R; Vermeulen, Sita H; Veronesi, Giovanni; Vestergaard, Henrik; Vitart, Veronique; Vogt, Thomas F; Völker, Uwe; Vuckovic, Dragana; Wagenknecht, Lynne E; Walker, Mark; Wallentin, Lars; Wang, Feijie; Wang, Carol A; Wang, Shuai; Wang, Yiqin; Ware, Erin B; Wareham, Nicholas J; Warren, Helen R; Waterworth, Dawn M; Wessel, Jennifer; White, Harvey D; Willer, Cristen J; Wilson, James G; Witte, Daniel R; Wood, Andrew R; Wu, Ying; Yaghootkar, Hanieh; Yao, Jie; Yao, Pang; Yerges-Armstrong, Laura M; Young, Robin; Zeggini, Eleftheria; Zhan, Xiaowei; Zhang, Weihua; Zhao, Jing Hua; Zhao, Wei; Zhao, Wei; Zhou, Wei; Zondervan, Krina T; Rotter, Jerome I; Pospisilik, John A; Rivadeneira, Fernando; Borecki, Ingrid B; Deloukas, Panos; Frayling, Timothy M; Lettre, Guillaume; North, Kari E; Lindgren, Cecilia M; Hirschhorn, Joel N; Loos, Ruth JF

2018-01-01

Genome-wide association studies (GWAS) have identified >250 loci for body mass index (BMI), implicating pathways related to neuronal biology. Most GWAS loci represent clusters of common, non-coding variants from which pinpointing causal genes remains challenging. Here, we combined data from 718,734 individuals to discover rare and low-frequency (MAFobesity, two (MC4R, KSR2) previously observed in extreme obesity, and two variants in GIPR. Effect sizes of rare variants are ~10 times larger than of common variants, with the largest effect observed in carriers of an MC4R stop-codon (p.Tyr35Ter, MAF=0.01%), weighing ~7kg more than non-carriers. Pathway analyses confirmed enrichment of neuronal genes and provide new evidence for adipocyte and energy expenditure biology, widening the potential of genetically-supported therapeutic targets to treat obesity. PMID:29273807

RARE DISEASES AND GENETIC DISCRIMINATION

Directory of Open Access Journals (Sweden)

Mariela Yaneva – Deliverska

2011-04-01

Full Text Available Rare diseases are characterised by their low prevalence (less than 1/2,000 and their heterogeneity. They affect both children and adults anywhere in the world. From the medical perspective, rare diseases are characterised by the large number and broad diversity of disorders and symptoms that vary not only from disease to disease, but also within the same disease.Main characteristics of rare diseases include:· Rare diseases are often chronic, progressive, degenerative, and often life-threatening· Rare diseases are disabling: the quality of life of patients is often compromised by the lack or loss of autonomy· High level of pain and suffering for the patient and his/ her family · No existing effective cure· There are between 6000 and 8000 rare diseases· 75% of rare diseases affect children· 30% of rare disease patients die before the age of 5· 80% of rare diseases have identified genetic origins. Other rare diseases are the result of infections (bacterial or viral, allergies and environmental causes, or are degenerative and proliferative.Beyond the diversity of the diseases, rare disease patients and their families are confronted with the same wide range of difficulties arising directly from the rarity of these pathologies. The period between the emergence of the first symptoms and the appropriate diagnosis involves unacceptable and highly risky delays, as well as wrong diagnosis leading to inaccurate treatments. Living with a rare disease has implications in all areas of life, whether school, choice of future work, leisure time with friends, or affective life. It may lead to stigmatisation, isolation, exclusion from social community, discrimination for insurance subscription (health insurance, travel insurance, mortgage, and often reduced professional opportunities.Innovative treatments are often unevenly available in the EU because of delays in price determination and/or reimbursement decision, lack of experience of the treating

The Chinese Society of Rare Earth is Studying The Feasibility of Marketing Rare Earth Futures

Institute of Scientific and Technical Information of China (English)

2015-01-01

Lin Donglu,secretary-general of the Chinese Society of Rare Earth recently said,the Chinese Society of Rare Earth undertook the research on subject of the National Social Science Fund Foundation on the reform of Chinese rare earth trading pricing mechanism on promoting RMB globalization,and is focusing on studying the feasibility of marketing rare earth futures variety.

PDGFB partial deletion: a new, rare mechanism causing brain calcification with leukoencephalopathy.

Science.gov (United States)

Nicolas, Gaël; Rovelet-Lecrux, Anne; Pottier, Cyril; Martinaud, Olivier; Wallon, David; Vernier, Louis; Landemore, Gérard; Chapon, Françoise; Prieto-Morin, Carol; Tournier-Lasserve, Elisabeth; Frébourg, Thierry; Campion, Dominique; Hannequin, Didier

2014-06-01

Idiopathic basal ganglia calcification (IBGC) is a progressive cerebral disorder with diverse motor, cognitive, and psychiatric expression. It is inherited as an autosomal dominant trait. Three IBGC-causing genes have been identified in the past 2 years: SLC20A2, PDGFRB, and PDGFB. Biological and genetic evidence showed that loss of function of either SLC20A2 or the PDGFB/PDGFRB pathway was the mechanism underlying calcification in patients with a mutation. Recently, in a study focusing on SLC20A2, a large deletion at this locus was reported. No study has systematically searched for copy number variants (CNV) involving these three genes. We designed a quantitative PCR assay of multiple short fluorescent fragments (QMPSF) to detect CNVs involving one of these three genes in a single assay. Among the 27 unrelated patients from our IBGC case series with no mutation in SLC20A2, PDGFRB, and PDGFB, we identified in one patient a heterozygous partial deletion involving exons 2 to 5 of PDGFB. This patient exhibited both strio-pallido-dentate calcification and white matter hyperintensity of presumed vascular origin, associated with mood disorder, subtle cognitive decline, and gait disorder. We confirmed by RT-PCR experiments that the allele carrying the deletion was transcribed. The resulting cDNA lacks sequence for several critical functional domains of the protein. Intragenic deletion of PDGFB is a new and rare mechanism causing IBGC. CNVs involving the three IBGC-causing genes should be investigated in patients with no point mutation.

A Rare Presentation of a Rare Disease: Pulmonary Lymphomatoid Granulomatosis

Directory of Open Access Journals (Sweden)

Ghulam Rehman Mohyuddin

2012-01-01

Full Text Available A 70-year-old female presented with a 4-week history of dry cough and wheezing. Chest radiograph showed a 10.5 cm mass-like density in the anterior mediastinum which had not been previously visualized. Computed tomography scan (CT of the chest showed a right hilar mass encasing and narrowing right upper lobe bronchus and right mainstem bronchus and secondary atelectatic changes. Biopsy was consistent with a diagnosis of lymphomatoid granulomatosis Grade 3. She responded well clinically and radiologically to therapy. Lymphomatoid granulomatosis is a rare EBV-associated disorder which is considered a lymphoproliferative disease. The most common radiographic feature is multiple lung nodules. An isolated hilar mass is an exceptionally rare presentation of this rare disease.

Targeting Hodgkin and Reed–Sternberg Cells with an Inhibitor of Heat-Shock Protein 90: Molecular Pathways of Response and Potential Mechanisms of Resistance

Directory of Open Access Journals (Sweden)

Priscilla Segges

2018-03-01

Full Text Available Classical Hodgkin lymphoma (cHL cells overexpress heat-shock protein 90 (HSP90, an important intracellular signaling hub regulating cell survival, which is emerging as a promising therapeutic target. Here, we report the antitumor effect of celastrol, an anti-inflammatory compound and a recognized HSP90 inhibitor, in Hodgkin and Reed–Sternberg cell lines. Two disparate responses were recorded. In KM-H2 cells, celastrol inhibited cell proliferation, induced G0/G1 arrest, and triggered apoptosis through the activation of caspase-3/7. Conversely, L428 cells exhibited resistance to the compound. A proteomic screening identified a total of 262 differentially expressed proteins in sensitive KM-H2 cells and revealed that celastrol’s toxicity involved the suppression of the MAPK/ERK (extracellular signal regulated kinase/mitogen activated protein kinase pathway. The apoptotic effects were preceded by a decrease in RAS (proto-oncogene protein Ras, p-ERK1/2 (phospho-extracellular signal-regulated Kinase-1/2, and c-Fos (proto-oncogene protein c-Fos protein levels, as validated by immunoblot analysis. The L428 resistant cells exhibited a marked induction of HSP27 mRNA and protein after celastrol treatment. Our results provide the first evidence that celastrol has antitumor effects in cHL cells through the suppression of the MAPK/ERK pathway. Resistance to celastrol has rarely been described, and our results suggest that in cHL it may be mediated by the upregulation of HSP27. The antitumor properties of celastrol against cHL and whether the disparate responses observed in vitro have clinical correlates deserve further research.

Walk, Look, Remember: The Influence of the Gallery's Spatial Layout on Human Memory for an Art Exhibition.

Science.gov (United States)

Krukar, Jakub

2014-09-01

The spatial organisation of museums and its influence on the visitor experience has been the subject of numerous studies. Previous research, despite reporting some actual behavioural correlates, rarely had the possibility to investigate the cognitive processes of the art viewers. In the museum context, where spatial layout is one of the most powerful curatorial tools available, attention and memory can be measured as a means of establishing whether or not the gallery fulfils its function as a space for contemplating art. In this exploratory experiment, 32 participants split into two groups explored an experimental, non-public exhibition and completed two unanticipated memory tests afterwards. The results show that some spatial characteristics of an exhibition can inhibit the recall of pictures and shift the focus to perceptual salience of the artworks.

Rare earths and actinides

International Nuclear Information System (INIS)

Coqblin, B.

1982-01-01

This paper reviews the different properties of rare-earths and actinides, either as pure metals or as in alloys or compounds. Three different cases are considered: (i) First, in the case of 'normal' rare-earths which are characterized by a valence of 3, we discuss essentially the magnetic ordering, the coexistence between superconductivity and magnetism and the properties of amorphous rare-earth systems. (ii) Second, in the case of 'anomalous' rare-earths, we distinguish between either 'intermediate-valence' systems or 'Kondo' systems. Special emphasis is given to the problems of the 'Kondo lattice' (for compounds such as CeAl 2 ,CeAl 3 or CeB 6 ) or the 'Anderson lattice' (for compounds such as TmSe). The problem of neutron diffraction in these systems is also discussed. (iii) Third, in the case of actinides, we can separate between the d-f hybridized and almost magnetic metals at the beginning of the series and the rare-earth like the metals after americium. (orig.)

The burden of rare cancers in Italy: the surveillance of rare cancers in Italy (RITA) project.

Science.gov (United States)

Trama, Annalisa; Mallone, Sandra; Ferretti, Stefano; Meduri, Francesca; Capocaccia, Riccardo; Gatta, Gemma

2012-01-01

The project Surveillance of rare cancers in Italy (RITA) provides, for the first time, estimates of the burden of rare cancers in Italy based on the list of rare cancers proposed in collaboration with the European project Surveillance of Rare Cancers in Europe (RARECARE). RITA analyzed data from Italian population-based cancer registries (CR). The period of diagnosis was 1988 to 2002, and vital status information was available up to December 31, 2003. Incidence rates were estimated for the period 1995-2002, survival for the years 2000-2002 (with the period method of Brenner), and complete prevalence at January 1, 2003. Rare cancers are those with an incidence <6/100,000/year. In Italy, every year there are 60,000 new diagnoses of rare cancers corresponding to 15% of all new cancer diagnoses. Five-year relative survival was on the average worse for rare cancers (53%) than for common cancers (73%). A total of 770,000 patients were living in Italy in 2008 with a diagnosis of a rare cancer, 22% of the total cancer prevalence. Our estimates constitute a useful base for further research and support the idea that rare cancers are a public health problem that deserves attention. Centers of expertise for rare cancers that pool cases, expertise and resources could ensure an adequate clinical management for these diseases. Our data also showed that cancer registries are suitable sources of data to estimate incidence, prevalence and survival for rare cancers and should continue to monitoring rare cancers in Italy.

Rare-earth metal transition metal borocarbide and nitridoborate superconductors

Energy Technology Data Exchange (ETDEWEB)

Niewa, Rainer; Shlyk, Larysa; Blaschkowski, Bjoern [Stuttgart Univ. (Germany). Inst. fuer Anorganische Chemie

2011-07-01

Few years after the discovery of superconductivity in high-T{sub c} cuprates, borocarbides and shortly after nitridoborates with reasonably high T{sub c}s up to about 23 K attracted considerable attention. Particularly for the rare-earth metal series with composition RNi{sub 2}[B{sub 2}C] it turned out, that several members exhibit superconductivity next to magnetic order with both T{sub c} above or below the magnetic ordering temperature. Therefore, these compounds have been regarded as ideal materials to study the interplay and coexistence of superconductivity and long range magnetic order, due to their comparably high ordering temperatures and similar magnetic and superconducting condensation energies. This review gathers information on the series RNi{sub 2}[B{sub 2}C] and isostructural compounds with different transition metals substituting Ni as well as related series like RM[BC], RM[BN], AM[BN] and R{sub 3}M{sub 2}[BN]{sub 2}N (all with R = rare-earth metal, A = alkaline-earth metal, M = transition metal) with special focus on synthesis, crystal structures and structural trends in correspondence to physical properties. (orig.)

GAS6/TAM Pathway Signaling in Hemostasis and Thrombosis.

Science.gov (United States)

Law, Luke A; Graham, Douglas K; Di Paola, Jorge; Branchford, Brian R

2018-01-01

The GAS6/TYRO3-AXL-MERTK (TAM) signaling pathway is essential for full and sustained platelet activation, as well as thrombus stabilization. Inhibition of this pathway decreases platelet aggregation, shape change, clot retraction, aggregate formation under flow conditions, and surface expression of activation markers. Transgenic mice deficient in GAS6, or any of the TAM family of receptors that engage this ligand, exhibit in vivo protection against arterial and venous thrombosis but do not demonstrate either spontaneous or prolonged bleeding compared to their wild-type counterparts. Comparable results are observed in wild-type mice treated with pharmacological inhibitors of the GAS6-TAM pathway. Thus, GAS6/TAM inhibition offers an attractive novel therapeutic option that may allow for a moderate reduction in platelet activation and decreased thrombosis while still permitting the primary hemostatic function of platelet plug formation.

Porphyromonas gingivalis and Treponema denticola exhibit metabolic symbioses.

Directory of Open Access Journals (Sweden)

Kheng H Tan

2014-03-01

Full Text Available Porphyromonas gingivalis and Treponema denticola are strongly associated with chronic periodontitis. These bacteria have been co-localized in subgingival plaque and demonstrated to exhibit symbiosis in growth in vitro and synergistic virulence upon co-infection in animal models of disease. Here we show that during continuous co-culture a P. gingivalis:T. denticola cell ratio of 6∶1 was maintained with a respective increase of 54% and 30% in cell numbers when compared with mono-culture. Co-culture caused significant changes in global gene expression in both species with altered expression of 184 T. denticola and 134 P. gingivalis genes. P. gingivalis genes encoding a predicted thiamine biosynthesis pathway were up-regulated whilst genes involved in fatty acid biosynthesis were down-regulated. T. denticola genes encoding virulence factors including dentilisin and glycine catabolic pathways were significantly up-regulated during co-culture. Metabolic labeling using 13C-glycine showed that T. denticola rapidly metabolized this amino acid resulting in the production of acetate and lactate. P. gingivalis may be an important source of free glycine for T. denticola as mono-cultures of P. gingivalis and T. denticola were found to produce and consume free glycine, respectively; free glycine production by P. gingivalis was stimulated by T. denticola conditioned medium and glycine supplementation of T. denticola medium increased final cell density 1.7-fold. Collectively these data show P. gingivalis and T. denticola respond metabolically to the presence of each other with T. denticola displaying responses that help explain enhanced virulence of co-infections.

Consumer preference for seeds and seedlings of rare species impacts tree diversity at multiple scales.

Science.gov (United States)

Young, Hillary S; McCauley, Douglas J; Guevara, Roger; Dirzo, Rodolfo

2013-07-01

Positive density-dependent seed and seedling predation, where herbivores selectively eat seeds or seedlings of common species, is thought to play a major role in creating and maintaining plant community diversity. However, many herbivores and seed predators are known to exhibit preferences for rare foods, which could lead to negative density-dependent predation. In this study, we first demonstrate the occurrence of increased predation of locally rare tree species by a widespread group of insular seed and seedling predators, land crabs. We then build computer simulations based on these empirical data to examine the effects of such predation on diversity patterns. Simulations show that herbivore preferences for locally rare species are likely to drive scale-dependent effects on plant community diversity: at small scales these foraging patterns decrease plant community diversity via the selective consumption of rare plant species, while at the landscape level they should increase diversity, at least for short periods, by promoting clustered local dominance of a variety of species. Finally, we compared observed patterns of plant diversity at the site to those obtained via computer simulations, and found that diversity patterns generated under simulations were highly consistent with observed diversity patterns. We posit that preference for rare species by herbivores may be prevalent in low- or moderate-diversity systems, and that these effects may help explain diversity patterns across different spatial scales in such ecosystems.

Evidence of Parallel Pathways: Gender Similarity in the Impact of Social Support on Adolescent Depression and Delinquency

Science.gov (United States)

Meadows, Sarah O.

2007-01-01

Despite an apparent connection, depression and delinquency have rarely been examined simultaneously. Instead, research has examined each topic separately and emphasized gender differences--rather than similarities--in outcomes. Using the National Longitudinal Study of Adolescent Health, this paper examines possible parallel pathways between social…

Safety aspects in rare earths recovery

International Nuclear Information System (INIS)

Bhattacharya, R.

2014-01-01

Recovery of rare earths involves mining of beach sands, mineral separation to obtain monazite and its chemical processing to obtain rare earth composites. The composites are then subjected to further chemical treatment to obtain individual rare earths. Although the separated out rare earths are not radioactive, the process for recovery of rare earths involve both radiological as well as conventional hazards. This paper highlights the safety aspects in the mining, mineral separation and chemical processing of monazite to obtain rare earths

Distribution Patterns of Ohio Stoneflies, with an Emphasis on Rare and Uncommon Species

Science.gov (United States)

Grubbs, Scott A.; Pessimo, Massimo; DeWalt, R. Edward

2013-01-01

Presently, 102 stonefly species (Plecoptera) have been reported from Ohio. All 9 Nearctic families are represented. Over 90% of the fauna exhibit a combination of broad Nearctic-widespread, eastern Nearctic-widespread, Appalachian, and eastern Nearctic-unglaciated distributions. In contrast, only 2 species display a central Nearctic-Prairie distribution. Seven species of Perlidae are likely no longer present (Acroneuria evoluta Klapálek, A. perplexa Frison, Attaneuria ruralis (Hagen), and Neoperla mainensis Banks) or have experienced marked range reductions (Acroneuria abnormis (Newman), A. frisoni Stark and Brown, and A. filicis Frison). Another nearly 31% of the fauna (32 species) are rare, uncommon, or have highly-limited distributions within the state. Twelve of these species have Appalachian distributions, and an additional 8 have eastern Nearctic-unglaciated distributions. The distributional status for each of the 32 rare/uncommon species is discussed. PMID:24219390

Rare B decays at LHCb

CERN Document Server

Puig Navarro, Albert

2017-01-01

Rare decays are flavour changing neutral current processes that allow sensitive searches for phenomena beyond the Standard Model (SM). In the SM, rare decays are loop-suppressed and new particles in SM extensions can give significant contributions. The very rare decay $B^0_s\\to\\mu^+\\mu^-$ in addition helicity suppressed and constitutes a powerful probe for new (pseudo) scalar particles. Of particular interest are furthermore tests of lepton universality in rare $b\\to s\\ell^+\\ell^-$ decays. The LHCb experiment is designed for the study of b-hadron decays and ideally suited for the analysis of rare decays due to its high trigger efficiency, as well as excellent tracking and particle identification performance. Recent results from the LHCb experiment in the area of rare decays are presented, including tests of lepton universality and searches for lepton flavour violation.

Rare earths and rare earth alloys electrolytic preparation process and device for this process

International Nuclear Information System (INIS)

Seon, F.; Barthole, G.

1986-01-01

Electrolysis of a molten salt of rare earth or rare earth alloy for preparation of the metal or alloy is described. The molten salt bath comprises at least a rare earth chloride, at least an alkaline or alkaline earth chloride and at least an alkaline or alkaline earth fluoride [fr

Diversity and impact of rare variants in genes encoding the platelet G protein-coupled receptors.

Science.gov (United States)

Jones, Matthew L; Norman, Jane E; Morgan, Neil V; Mundell, Stuart J; Lordkipanidzé, Marie; Lowe, Gillian C; Daly, Martina E; Simpson, Michael A; Drake, Sian; Watson, Steve P; Mumford, Andrew D

2015-04-01

Platelet responses to activating agonists are influenced by common population variants within or near G protein-coupled receptor (GPCR) genes that affect receptor activity. However, the impact of rare GPCR gene variants is unknown. We describe the rare single nucleotide variants (SNVs) in the coding and splice regions of 18 GPCR genes in 7,595 exomes from the 1,000-genomes and Exome Sequencing Project databases and in 31 cases with inherited platelet function disorders (IPFDs). In the population databases, the GPCR gene target regions contained 740 SNVs (318 synonymous, 410 missense, 7 stop gain and 6 splice region) of which 70 % had global minor allele frequency (MAF) < 0.05 %. Functional annotation using six computational algorithms, experimental evidence and structural data identified 156/740 (21 %) SNVs as potentially damaging to GPCR function, most commonly in regions encoding the transmembrane and C-terminal intracellular receptor domains. In 31 index cases with IPFDs (Gi-pathway defect n=15; secretion defect n=11; thromboxane pathway defect n=3 and complex defect n=2) there were 256 SNVs in the target regions of 15 stimulatory platelet GPCRs (34 unique; 12 with MAF< 1 % and 22 with MAF≥ 1 %). These included rare variants predicting R122H, P258T and V207A substitutions in the P2Y12 receptor that were annotated as potentially damaging, but only partially explained the platelet function defects in each case. Our data highlight that potentially damaging variants in platelet GPCR genes have low individual frequencies, but are collectively abundant in the population. Potentially damaging variants are also present in pedigrees with IPFDs and may contribute to complex laboratory phenotypes.

Towards Rare Itemset Mining

OpenAIRE

Szathmary , Laszlo; Napoli , Amedeo; Valtchev , Petko

2007-01-01

site de la conférence : http://ictai07.ceid.upatras.gr/; International audience; We describe here a general approach for rare itemset mining. While mining literature has been almost exclusively focused on frequent itemsets, in many practical situations rare ones are of higher interest (e.g., in medical databases, rare combinations of symptoms might provide useful insights for the physicians). Based on an examination of the relevant substructures of the mining space, our approach splits the ra...

An integrated analysis of genes and pathways exhibiting metabolic differences between estrogen receptor positive breast cancer cells

International Nuclear Information System (INIS)

Mandal, Soma; Davie, James R

2007-01-01

The sex hormone estrogen (E2) is pivotal to normal mammary gland growth and differentiation and in breast carcinogenesis. In this in silico study, we examined metabolic differences between ER(+)ve breast cancer cells during E2 deprivation. Public repositories of SAGE and MA gene expression data generated from E2 deprived ER(+)ve breast cancer cell lines, MCF-7 and ZR75-1 were compared with normal breast tissue. We analyzed gene ontology (GO), enrichment, clustering, chromosome localization, and pathway profiles and performed multiple comparisons with cell lines and tumors with different ER status. In all GO terms, biological process (BP), molecular function (MF), and cellular component (CC), MCF-7 had higher gene utilization than ZR75-1. Various analyses showed a down-regulated immune function, an up-regulated protein (ZR75-1) and glucose metabolism (MCF-7). A greater percentage of 77 common genes localized to the q arm of all chromosomes, but in ZR75-1 chromosomes 11, 16, and 19 harbored more overexpressed genes. Despite differences in gene utilization (electron transport, proteasome, glycolysis/gluconeogenesis) and expression (ribosome) in both cells, there was an overall similarity of ZR75-1 with ER(-)ve cell lines and ER(+)ve/ER(-)ve breast tumors. This study demonstrates integral metabolic differences may exist within the same cell subtype (luminal A) in representative ER(+)ve cell line models. Selectivity of gene and pathway usage for strategies such as energy requirement minimization, sugar utilization by ZR75-1 contrasted with MCF-7 cells, expressing genes whose protein products require ATP utilization. Such characteristics may impart aggressiveness to ZR75-1 and may be prognostic determinants of ER(+)ve breast tumors

Refined potentials for rare gas atom adsorption on rare gas and alkali-halide surfaces

Science.gov (United States)

Wilson, J. W.; Heinbockel, J. H.; Outlaw, R. A.

1985-01-01

The utilization of models of interatomic potential for physical interaction to estimate the long range attractive potential for rare gases and ions is discussed. The long range attractive force is calculated in terms of the atomic dispersion properties. A data base of atomic dispersion parameters for rare gas atoms, alkali ion, and halogen ions is applied to the study of the repulsive core; the procedure for evaluating the repulsive core of ion interactions is described. The interaction of rare gas atoms on ideal rare gas solid and alkali-halide surfaces is analyzed; zero coverage absorption potentials are derived.

Genetic/metabolic effect of iron metabolism and rare anemias

Directory of Open Access Journals (Sweden)

Clara Camaschella

2013-03-01

Full Text Available Advances in iron metabolism have allowed a novel classification of iron disorders and to identify previously unknown diseases. These disorders include genetic iron overload (hemochromatosis and inherited iron-related anemias, in some cases accompanied by iron overload. Rare inherited anemias may affect the hepcidin pathway, iron absorption, transport, utilization and recycling. Among the genetic iron-related anemias the most common form is likely the iron-refractory iron-deficiency anemia (IRIDA, due to mutations of the hepcidin inhibitor TMPRSS6 encoding the serine protease matriptase-2. IRIDA is characterized by hepcidin up-regulation, decrease iron absorption and macrophage recycling and by microcytic- hypochromic anemia, unresponsive to oral iron. High serum hepcidin levels may suggest the diagnosis, which requires demonstrating the causal TMPRSS6 mutations by gene sequencing. Other rare microcytic hypochromic anemias associated with defects of iron transport-uptake are the rare hypotransferrinemia, and DMT1 and STEAP3 mutations. The degree of anemia is variable and accompanied by secondary iron overload even in the absence of blood transfusions. This is due to the iron-deficient or expanded erythropoiesis that inhibits hepcidin transcription, increases iron absorption, through the erythroid regulator, as in untransfused beta-thalassemia. Sideroblastic anemias are due to decreased mitochondrial iron utilization for heme or sulfur cluster synthesis. Their diagnosis requires demonstrating ringed sideroblasts by Perl’s staining of the bone marrow smears. The commonest X-linked form is due to deltaamino- levulinic-synthase-2-acid (ALAS2 mutations. The recessive, more severe form, affects SLC25A38, which encodes a potential mitochondrial importer of glycine, an amino acid essential for ALA synthesis and thus results in heme deficiency. Two disorders affect iron/sulfur cluster biogenesis: deficiency of the ATP-binding cassette B7 (ABCB7 causes X

Rare Disease Video Portal

OpenAIRE

Sánchez Bocanegra, Carlos Luis

2011-01-01

Rare Disease Video Portal (RD Video) is a portal web where contains videos from Youtube including all details from 12 channels of Youtube. Rare Disease Video Portal (RD Video) es un portal web que contiene los vídeos de Youtube incluyendo todos los detalles de 12 canales de Youtube. Rare Disease Video Portal (RD Video) és un portal web que conté els vídeos de Youtube i que inclou tots els detalls de 12 Canals de Youtube.

Arginine deiminase pathway enzymes: evolutionary history in metamonads and other eukaryotes.

Science.gov (United States)

Novák, Lukáš; Zubáčová, Zuzana; Karnkowska, Anna; Kolisko, Martin; Hroudová, Miluše; Stairs, Courtney W; Simpson, Alastair G B; Keeling, Patrick J; Roger, Andrew J; Čepička, Ivan; Hampl, Vladimír

2016-10-06

Multiple prokaryotic lineages use the arginine deiminase (ADI) pathway for anaerobic energy production by arginine degradation. The distribution of this pathway among eukaryotes has been thought to be very limited, with only two specialized groups living in low oxygen environments (Parabasalia and Diplomonadida) known to possess the complete set of all three enzymes. We have performed an extensive survey of available sequence data in order to map the distribution of these enzymes among eukaryotes and to reconstruct their phylogenies. We have found genes for the complete pathway in almost all examined representatives of Metamonada, the anaerobic protist group that includes parabasalids and diplomonads. Phylogenetic analyses indicate the presence of the complete pathway in the last common ancestor of metamonads and heterologous transformation experiments suggest its cytosolic localization in the metamonad ancestor. Outside Metamonada, the complete pathway occurs rarely, nevertheless, it was found in representatives of most major eukaryotic clades. Phylogenetic relationships of complete pathways are consistent with the presence of the Archaea-derived ADI pathway in the last common ancestor of all eukaryotes, although other evolutionary scenarios remain possible. The presence of the incomplete set of enzymes is relatively common among eukaryotes and it may be related to the fact that these enzymes are involved in other cellular processes, such as the ornithine-urea cycle. Single protein phylogenies suggest that the evolutionary history of all three enzymes has been shaped by frequent gene losses and horizontal transfers, which may sometimes be connected with their diverse roles in cellular metabolism.

Synthesis and sorption properties of new synthesized rare-earth-doped sodium titanate

International Nuclear Information System (INIS)

Ali, I.M.

2010-01-01

A series of rare-earth-doped sodium titanates with the chemical formula R x H y Na 4-(x+y) TiO 4 ·nH 2 O (where R = Ce 3+ , Nd 3+ and Sm 3+ ) were grown employing solid-state fusion reaction technique. The physico-chemical investigations indicated that the new materials were self engineered into large particles enough to be used in sorption process and having crystalline structures containing localized Na + ions. Equilibrium studies revealed that an enhancement in sorption efficiency of sodium titanate after rare-earth doping. The neodymium-rich sodium titanate exhibited a better exchange affinity for Cs + compared to the other studied series. Data on the kinetics of cesium exchange fit well to pseudo-second order and intra-particle diffusion models. In a separate experiment, it was reported that the R-HNaTi series showed responsible sorption affinity toward Ce, Nd and Sm ions in their solution mixture with insignificant selectivity trend which reflects the high stability of titanate matrices. (author)

Structural and magentic characterization of rare earth and transition metal films grown on epitaxial buffer films on semiconductor substrates

International Nuclear Information System (INIS)

Farrow, R.F.C.; Parkin, S.S.P.; Speriosu, V.S.; Bezinge, A.; Segmuller, A.P.

1989-01-01

Structural and magnetic data are presented and discussed for epitaxial films of rare earth metals (Dy, Ho, Er) on LaF 3 films on the GaAs(TTT) surface and Fe on Ag films on the GaAs(001) surface. Both systems exhibit unusual structural characteristics which influence the magnetic properties of the metal films. In the case of rare earth epitaxy on LaF 3 the authors present evidence for epitaxy across an incommensurate or discommensurate interface. Coherency strain is not transmitted into the metal which behaves much like bulk crystals of the rare earths. In the case of Fe films, tilted epitaxy and long-range coherency strain are confirmed by X- ray diffractometry. Methods of controlling some of these structural effects by modifying the epitaxial structures are presented

Exhibition at the AAA library

DEFF Research Database (Denmark)

2013-01-01

Sonnesgade 11 The exhibition at the AAA library presents selected work produced by students prior to the exhibition of installations in project and praxis constructing an archive at Sonnesgade 11. The exhibition at Sonnesgade 11 was the culmination of collaboration with SLETH architects and studio...

Walk, Look, Remember: The Influence of the Gallery’s Spatial Layout on Human Memory for an Art Exhibition

Directory of Open Access Journals (Sweden)

Jakub Krukar

2014-07-01

Full Text Available The spatial organisation of museums and its influence on the visitor experience has been the subject of numerous studies. Previous research, despite reporting some actual behavioural correlates, rarely had the possibility to investigate the cognitive processes of the art viewers. In the museum context, where spatial layout is one of the most powerful curatorial tools available, attention and memory can be measured as a means of establishing whether or not the gallery fulfils its function as a space for contemplating art. In this exploratory experiment, 32 participants split into two groups explored an experimental, non-public exhibition and completed two unanticipated memory tests afterwards. The results show that some spatial characteristics of an exhibition can inhibit the recall of pictures and shift the focus to perceptual salience of the artworks.

Upcycling CERN Exhibitions

CERN Multimedia

Katarina Anthony

2015-01-01

Summer is coming - and with it, a new Microcosm exhibition showcasing CERN (see here). But while the new exhibit is preparing to enchant visitors, many have been asking about the site's former content. Will it simply be out with the old and in with the new? Not as such!   The plasma ball from Microcosm is now on display at the LHCb site. As Microcosm's new content is moving in, its old content is moving up. From LHCb to IdeaSquare, former Microcosm displays and objects are being installed across the CERN site. "Microcosm featured many elements that were well suited to life outside of the exhibition," says Emma Sanders, Microcosm project leader in the EDU group. "We didn't want this popular content to go to waste, and so set out to find them new homes across CERN." The LHCb experiment has received a number of Microcosm favourites, including the Rutherford experiment, the cosmic ray display and the Thomson experiment. "We&...

Globe exhibit wins international acclaim

CERN Multimedia

Katarina Anthony

2011-01-01

The Globe’s “Universe of Particles” exhibition has recently received four prestigious awards for its avant-garde design. This external praise is great encouragement for the CERN exhibitions currently on the drawing board.   The Universe of Particles exhibition has won 4 awards for its avant-garde design. Back in 2008, the design company Atelier Brückner was presented with a challenge: to design the layout of a new permanent exhibition for CERN, one that would epitomize both the Organization and its research. The brief was concise but complex: the exhibit had to be symbolic of the Organization, use modern technology, engage and immerse visitors, and, preferably, use touch-screen technology. With the help of IArt, an interactive technology firm, and based on the content provided by CERN’s Education Group, Atelier Brückner developed the “Universe of Particles” exhibit as it is today. Its principal concept centred on the s...

Malignant Hidradenocarcinoma in the Lower Extremity: A Case Report of a Rare Tumor.

Science.gov (United States)

Kane, Brendan; Adler, Evan; Bhandari, Tarun; Rose, Michael; DiGuglielmo, Nicola; Sun, Xiu

Malignant hidradenocarcinomas are rare soft tissue tumors of sweat gland origin. We present the case of a soft tissue, fungating tumor of 15 years' duration of the medial ankle in an 85-year-old male that exhibited malignant features clinically and radiographically. Subsequent punch biopsy revealed a diagnosis of malignant hidradenocarcinoma. Given the risk of recurrence and the poor radiation and chemotherapy options, the patient initially decided to leave the lesion untreated. However, he soon developed lower extremity cellulitis from the exposed lesion and decided to have the tumor excised, eliminating the source of the infection. In the present case study, we discuss the etiology, clinical and radiographic characteristics, and treatment options for this rare lesion. At the 18-month follow-up visit, he had had no recurrence of the lesion. Copyright © 2017 The American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

Ethacrynic acid exhibits selective toxicity to chronic lymphocytic leukemia cells by inhibition of the Wnt/beta-catenin pathway.

Directory of Open Access Journals (Sweden)

Desheng Lu

Full Text Available BACKGROUND: Aberrant activation of Wnt/beta-catenin signaling promotes the development of several cancers. It has been demonstrated that the Wnt signaling pathway is activated in chronic lymphocytic leukemia (CLL cells, and that uncontrolled Wnt/beta-catenin signaling may contribute to the defect in apoptosis that characterizes this malignancy. Thus, the Wnt signaling pathway is an attractive candidate for developing targeted therapies for CLL. METHODOLOGY/PRINCIPAL FINDINGS: The diuretic agent ethacrynic acid (EA was identified as a Wnt inhibitor using a cell-based Wnt reporter assay. In vitro assays further confirmed the inhibitory effect of EA on Wnt/beta-catenin signaling. Cell viability assays showed that EA selectively induced cell death in primary CLL cells. Exposure of CLL cells to EA decreased the expression of Wnt/beta-catenin target genes, including LEF-1, cyclin D1 and fibronectin. Immune co-precipitation experiments demonstrated that EA could directly bind to LEF-1 protein and destabilize the LEF-1/beta-catenin complex. N-acetyl-L-cysteine (NAC, which can react with the alpha, beta-unsaturated ketone in EA, but not other anti-oxidants, prevented the drug's inhibition of Wnt/beta-catenin activation and its ability to induce apoptosis in CLL cells. CONCLUSIONS/SIGNIFICANCE: Our studies indicate that EA selectively suppresses CLL survival due to inhibition of Wnt/beta-catenin signaling. Antagonizing Wnt signaling in CLL with EA or related drugs may represent an effective treatment of this disease.

Gaps and pseudogaps in perovskite rare earth nickelates

Directory of Open Access Journals (Sweden)

S. James Allen

2015-06-01

Full Text Available We report on tunneling measurements that reveal the evolution of the quasiparticle state density in two rare earth perovskite nickelates, NdNiO3 and LaNiO3, that are close to a bandwidth controlled metal to insulator transition. We measure the opening of a sharp gap of ∼30 meV in NdNiO3 in its insulating ground state. LaNiO3, which remains a correlated metal at all practical temperatures, exhibits a pseudogap of the same order. The results point to both types of gaps arising from a common origin, namely, a quantum critical point associated with the T = 0 K metal-insulator transition. The results support theoretical models of the quantum phase transition in terms of spin and charge instabilities of an itinerant Fermi surface.

Why some plant species are rare.

Science.gov (United States)

Wieger Wamelink, G W; Wamelink, G W Weiger; Goedhart, Paul W; Frissel, Joep; Frissel, Josep Y

2014-01-01

Biodiversity, including plant species diversity, is threatened worldwide as a result of anthropogenic pressures such as an increase of pollutants and climate change. Rare species in particular are on the verge of becoming extinct. It is still unclear as to why some plant species are rare and others are not. Are they rare due to: intrinsic reasons, dispersal capacity, the effects of management or abiotic circumstances? Habitat preference of rare plant species may play an important role in determining why some species are rare. Based on an extensive data set of soil parameters we investigated if rarity is due to a narrow habitat preference for abiotic soil parameters. For 23 different abiotic soil parameters, of which the most influential were groundwater-table, soil-pH and nutrient-contents, we estimated species responses for common and rare species. Based on the responses per species we calculated the range of occurrence, the range between the 5 and 95 percentile of the response curve giving the habitat preference. Subsequently, we calculated the average response range for common and rare species. In addition, we designed a new graphic in order to provide a better means for presentation of the results. The habitat preferences of rare species for abiotic soil conditions are significantly narrower than for common species. Twenty of the twenty-three abiotic parameters showed on average significantly narrower habitat preferences for rare species than for common species; none of the abiotic parameters showed on average a narrower habitat preference for common species. The results have major implications for the conservation of rare plant species; accordingly management and nature development should be focussed on the maintenance and creation of a broad range of environmental conditions, so that the requirements of rare species are met. The conservation of (abiotic) gradients within ecosystems is particularly important for preserving rare species.

Raman scattering of rare earth hexaborides

International Nuclear Information System (INIS)

Ogita, Norio; Hasegawa, Takumi; Udagawa, Masayuki; Iga, Fumitoshi; Kunii, Satoru

2009-01-01

Raman scattering spectra were measured for the rare-earth hexaborides RB 6 (R = Ce, Gd, or Dy). All Raman-active phonons due to B 6 vibrations were observed in the range 600 - 1400 cm -1 . Anomalous peaks were detected below 200 cm -1 , which correspond to vibrations of rare-earth ion excited by second-order Raman scattering process. The intensity and energy of the rare-earth mode decrease with decreasing temperature. This suggests that the rare-earth ion vibrates in a shallow and anharmonic potential due to the boron cage. Using the reported values of mean square displacement of rare-earth ion, we estimated the anharmonic contribution for the rare-earth vibrations.

Revealing Pathway Dynamics in Heart Diseases by Analyzing Multiple Differential Networks.

Directory of Open Access Journals (Sweden)

Xiaoke Ma

2015-06-01

Full Text Available Development of heart diseases is driven by dynamic changes in both the activity and connectivity of gene pathways. Understanding these dynamic events is critical for understanding pathogenic mechanisms and development of effective treatment. Currently, there is a lack of computational methods that enable analysis of multiple gene networks, each of which exhibits differential activity compared to the network of the baseline/healthy condition. We describe the iMDM algorithm to identify both unique and shared gene modules across multiple differential co-expression networks, termed M-DMs (multiple differential modules. We applied iMDM to a time-course RNA-Seq dataset generated using a murine heart failure model generated on two genotypes. We showed that iMDM achieves higher accuracy in inferring gene modules compared to using single or multiple co-expression networks. We found that condition-specific M-DMs exhibit differential activities, mediate different biological processes, and are enriched for genes with known cardiovascular phenotypes. By analyzing M-DMs that are present in multiple conditions, we revealed dynamic changes in pathway activity and connectivity across heart failure conditions. We further showed that module dynamics were correlated with the dynamics of disease phenotypes during the development of heart failure. Thus, pathway dynamics is a powerful measure for understanding pathogenesis. iMDM provides a principled way to dissect the dynamics of gene pathways and its relationship to the dynamics of disease phenotype. With the exponential growth of omics data, our method can aid in generating systems-level insights into disease progression.

Cubic rare-earth compounds: variants of the three-state Potts model

International Nuclear Information System (INIS)

Kim, D.; Levy, P.M.; Uffer, L.F.

1975-01-01

In appropriate cubic fields, rare-earth ions have sixfold degenerate ground states. When the angular momentum of the rare earth is large, the six levels are characterized by states that are directed along the cube edges. Within these states the angular momentum operators J/sub x/, J/sub y/, and J/sub z/ have particularly simple matrix representations. The projection of an isotropic pair coupling between the rare earths onto these sixfold degenerate states leads to an interaction Hamiltonian H = -I Σ/sub (ij)/ sigma/sub i/sigma/sub j/delta/sub l/sub i/sub l/sub j//, where sigma takes on the values +-1 and l the values x, y, and z. This interaction is a variant of the three-state Potts model. Magnetic and quadrupolar anisotropy field terms are added to the Hamiltonian and the symmetry properties of the phase diagram associated with this model are determined. For nonzero quadrupolar anisotropy fields, the model is shown to have the thermodynamic behavior of an Ising model. However, for zero fields a new symmetry appears and in the mean-field approximation the model has tricritical-like exponents. This simple model is able to account for the large specific-heat critical exponent α' = 1 / 2 which has been observed for holmium antimonide in zero external fields. To the extent that the mean-field approximation is an accurate guide, we predict there are many cubic rare-earth compounds which exhibit tricritical-like behavior in zero field. In addition, for pure quadrupole coupling between rare earths in the sixfold degenerate states, the interaction Hamiltonian is exactly the three-state Potts model. In the mean-field approximation this system has a first-order phase transition. However, a small quadrupolar anisotropy field is sufficient to drive the system to a wing critical point. The specific heat has a critical exponent of α = 2 / 3 or 1 depending on the path taken to approach this critical point. (auth)

PathwayAccess: CellDesigner plugins for pathway databases.

Science.gov (United States)

Van Hemert, John L; Dickerson, Julie A

2010-09-15

CellDesigner provides a user-friendly interface for graphical biochemical pathway description. Many pathway databases are not directly exportable to CellDesigner models. PathwayAccess is an extensible suite of CellDesigner plugins, which connect CellDesigner directly to pathway databases using respective Java application programming interfaces. The process is streamlined for creating new PathwayAccess plugins for specific pathway databases. Three PathwayAccess plugins, MetNetAccess, BioCycAccess and ReactomeAccess, directly connect CellDesigner to the pathway databases MetNetDB, BioCyc and Reactome. PathwayAccess plugins enable CellDesigner users to expose pathway data to analytical CellDesigner functions, curate their pathway databases and visually integrate pathway data from different databases using standard Systems Biology Markup Language and Systems Biology Graphical Notation. Implemented in Java, PathwayAccess plugins run with CellDesigner version 4.0.1 and were tested on Ubuntu Linux, Windows XP and 7, and MacOSX. Source code, binaries, documentation and video walkthroughs are freely available at http://vrac.iastate.edu/~jlv.

Rare Genome-Wide Copy Number Variation and Expression of Schizophrenia in 22q11.2 Deletion Syndrome.

Science.gov (United States)

Bassett, Anne S; Lowther, Chelsea; Merico, Daniele; Costain, Gregory; Chow, Eva W C; van Amelsvoort, Therese; McDonald-McGinn, Donna; Gur, Raquel E; Swillen, Ann; Van den Bree, Marianne; Murphy, Kieran; Gothelf, Doron; Bearden, Carrie E; Eliez, Stephan; Kates, Wendy; Philip, Nicole; Sashi, Vandana; Campbell, Linda; Vorstman, Jacob; Cubells, Joseph; Repetto, Gabriela M; Simon, Tony; Boot, Erik; Heung, Tracy; Evers, Rens; Vingerhoets, Claudia; van Duin, Esther; Zackai, Elaine; Vergaelen, Elfi; Devriendt, Koen; Vermeesch, Joris R; Owen, Michael; Murphy, Clodagh; Michaelovosky, Elena; Kushan, Leila; Schneider, Maude; Fremont, Wanda; Busa, Tiffany; Hooper, Stephen; McCabe, Kathryn; Duijff, Sasja; Isaev, Karin; Pellecchia, Giovanna; Wei, John; Gazzellone, Matthew J; Scherer, Stephen W; Emanuel, Beverly S; Guo, Tingwei; Morrow, Bernice E; Marshall, Christian R

2017-11-01

Chromosome 22q11.2 deletion syndrome (22q11.2DS) is associated with a more than 20-fold increased risk for developing schizophrenia. The aim of this study was to identify additional genetic factors (i.e., "second hits") that may contribute to schizophrenia expression. Through an international consortium, the authors obtained DNA samples from 329 psychiatrically phenotyped subjects with 22q11.2DS. Using a high-resolution microarray platform and established methods to assess copy number variation (CNV), the authors compared the genome-wide burden of rare autosomal CNV, outside of the 22q11.2 deletion region, between two groups: a schizophrenia group and those with no psychotic disorder at age ≥25 years. The authors assessed whether genes overlapped by rare CNVs were overrepresented in functional pathways relevant to schizophrenia. Rare CNVs overlapping one or more protein-coding genes revealed significant between-group differences. For rare exonic duplications, six of 19 gene sets tested were enriched in the schizophrenia group; genes associated with abnormal nervous system phenotypes remained significant in a stepwise logistic regression model and showed significant interactions with 22q11.2 deletion region genes in a connectivity analysis. For rare exonic deletions, the schizophrenia group had, on average, more genes overlapped. The additional rare CNVs implicated known (e.g., GRM7, 15q13.3, 16p12.2) and novel schizophrenia risk genes and loci. The results suggest that additional rare CNVs overlapping genes outside of the 22q11.2 deletion region contribute to schizophrenia risk in 22q11.2DS, supporting a multigenic hypothesis for schizophrenia. The findings have implications for understanding expression of psychotic illness and herald the importance of whole-genome sequencing to appreciate the overall genomic architecture of schizophrenia.

A neonate with intestinal volvulus without malrotation exhibiting early jaundice with a suspected fetal onset.

Science.gov (United States)

Hara, Kaori; Kinoshita, Mari; Kin, Takane; Arimitsu, Takeshi; Matsuzaki, Yohei; Ikeda, Kazushige; Tomita, Hiroshi; Fujino, Akihiro; Kuroda, Tatsuo

2015-01-01

Intestinal volvulus without malrotation is a rare disease that causes volvulus of the small intestine despite normal intestinal rotation and fixation. We encountered a neonate with this disease who developed early jaundice and was suspected to have a fetal onset. This patient was characterized by early jaundice complicating intestinal volvulus without malrotation and is considered to have exhibited reduced fetal movement and early jaundice as a result of volvulus, necrosis, and hemorrhage of the small intestine in the fetal period. If abdominal distention accompanied by early jaundice is noted in a neonate, intestinal volvulus without malrotation and associated intraabdominal hemorrhage should be suspected and promptly treated.

IceCube Polar Virtual Reality exhibit: immersive learning for learners of all ages

Science.gov (United States)

Madsen, J.; Bravo Gallart, S.; Chase, A.; Dougherty, P.; Gagnon, D.; Pronto, K.; Rush, M.; Tredinnick, R.

2017-12-01

The IceCube Polar Virtual Reality project is an innovative, interactive exhibit that explains the operation and science of a flagship experiment in polar research, the IceCube Neutrino Observatory. The exhibit allows users to travel from the South Pole, where the detector is located, to the furthest reaches of the universe, learning how the detection of high-energy neutrinos has opened a new view to the universe. This novel exhibit combines a multitouch tabletop display system and commercially available virtual reality (VR) head-mounted displays to enable informal STEM learning of polar research. The exhibit, launched in early November 2017 during the Wisconsin Science Festival in Madison, WI, will study how immersive VR can enhance informal STEM learning. The foundation of this project is built upon a strong collaborative effort between the Living Environments Laboratory (LEL), the Wisconsin IceCube Particle Astrophysics Center (WIPAC), and the Field Day Laboratory groups from the University of Wisconsin-Madison campus. The project is funded through an NSF Advancing Informal STEM Learning (AISL) grant, under a special call for engaging students and the public in polar research. This exploratory pathways project seeks to build expertise to allow future extensions. The plan is to submit a subsequent AISL Broad Implementation proposal to add more 3D environments for other Antarctic research topics and locations in the future. We will describe the current implementation of the project and discuss the challenges and opportunities of working with an interdisciplinary team of scientists and technology and education researchers. We will also present preliminary assessment results, which seek to answer questions such as: Did users gain a better understanding of IceCube research from interacting with the exhibit? Do both technologies (touch table and VR headset) provide the same level of engagement? Is one technology better suited for specific learning outcomes?

Rare earth industries: Downstream business

International Nuclear Information System (INIS)

2011-01-01

The value chain of the rare earths business involves mining, extraction, processing, refining and the manufacture of an extensive range of downstream products which find wide applications in such industries including aerospace, consumer electronics, medical, military, automotive, renewable wind and solar energy and telecommunications. In fact the entire gamut of the high-tech industries depends on a sustainable supply of rare earths elements. The explosive demand in mobile phones is an excellent illustration of the massive potential that the rare earths business offers. In a matter of less than 20 years, the number of cell phones worldwide has reached a staggering 5 billion. Soon, going by the report of their growth in sales, the world demand for cell phones may even exceed the global population. Admittedly, the rare earths business does pose certain risks. Top among the risks are the health and safety risks. The mining, extraction and refining of rare earths produce residues and wastes which carry health and safety risks. The residues from the extraction and refining are radioactive, while their effluent waste streams do pose pollution risks to the receiving rivers and waterways. But, as clearly elaborated in a recent report by IAEA experts, there are technologies and systems available to efficiently mitigate such risks. The risks are Rare Earth manageable. However, it is crucial that the risk and waste management procedures are strictly followed and adhered to. This is where effective monitoring and surveillance throughout the life of all such rare earths facilities is crucial. Fortunately, Malaysia's regulatory standards on rare earths follow international standards. In some areas, Malaysia's regulatory regime is even more stringent than the international guidelines. (author)

Rare events: a state of the art

International Nuclear Information System (INIS)

Uppuluri, V.R.R.

1980-12-01

The study of rare events has become increasingly important in the context of nuclear safety. Some philosophical considerations, such as the framework for the definition of a rare event, rare events and science, rare events and trans-science, and rare events and public perception, are discussed. The technical work of the Task Force on problems of Rare Events in the Reliability Analysis of Nuclear Plants (1976-1978), sponsored by OECD, is reviewed. Some recent technical considerations are discussed, and conclusions are drawn. The appendix contains an essay written by Anne E. Beachey, under the title: A Study of Rare Events - Problems and Promises

Coordinated Ramping of Dorsal Striatal Pathways preceding Food Approach and Consumption.

Science.gov (United States)

London, Tanisha D; Licholai, Julia A; Szczot, Ilona; Ali, Mohamed A; LeBlanc, Kimberly H; Fobbs, Wambura C; Kravitz, Alexxai V

2018-04-04

The striatum controls food-related actions and consumption and is linked to feeding disorders, including obesity and anorexia nervosa. Two populations of neurons project from the striatum: direct pathway medium spiny neurons and indirect pathway medium spiny neurons. The selective contribution of direct pathway medium spiny neurons and indirect pathway medium spiny neurons to food-related actions and consumption remains unknown. Here, we used in vivo electrophysiology and fiber photometry in mice (of both sexes) to record both spiking activity and pathway-specific calcium activity of dorsal striatal neurons during approach to and consumption of food pellets. While electrophysiology revealed complex task-related dynamics across neurons, population calcium was enhanced during approach and inhibited during consumption in both pathways. We also observed ramping changes in activity that preceded both pellet-directed actions and spontaneous movements. These signals were heterogeneous in the spiking units, with neurons exhibiting either increasing or decreasing ramps. In contrast, the population calcium signals were homogeneous, with both pathways having increasing ramps of activity for several seconds before actions were initiated. An analysis comparing population firing rates to population calcium signals also revealed stronger ramping dynamics in the calcium signals than in the spiking data. In a second experiment, we trained the mice to perform an action sequence to evaluate when the ramping signals terminated. We found that the ramping signals terminated at the beginning of the action sequence, suggesting they may reflect upcoming actions and not preconsumption activity. Plasticity of such mechanisms may underlie disorders that alter action selection, such as drug addiction or obesity. SIGNIFICANCE STATEMENT Alterations in striatal function have been linked to pathological consumption in disorders, such as obesity and drug addiction. We recorded spiking and

Metallothermic reduction of rare earth oxides

International Nuclear Information System (INIS)

Sharma, R.A.

1986-01-01

Rare earth oxides can be reduced to rare earth metals by a novel, high yield, metallothermic process. The oxides are dispersed in a suitable, molten, calcium chloride bath along with sodium metal. The sodium reacts with the calcium chloride to produce calcium metal which reduces the rare earth oxides to rare earth metals. The metals are collected in a discrete layer in the reaction vessel

Rare earth industries: Strategies for Malaysia

International Nuclear Information System (INIS)

2011-01-01

Evidently, many reports cite Malaysia as having reasonably substantial amounts of rare earths elements. In fact, based on the rare earths found in the residual tin deposits alone, Malaysia has about 30,000 tonnes. This does not take into account unmapped deposits which experts believe may offer more tonnages of rare earths. Brazil which is reported to have about 48,000 tonnes has announced plans to invest aggressively in the rare earths business. China has on record the largest reserves with about 36 million tonnes. This explains why China has invested heavily in the entire value chain of the rare earths business. Chinas committed investment in rare earths started many years ago when the country's foremost leaders proclaimed the strategic position of rare earths in the world economy. That forecast is now a reality where the rise in the green high-tech economy is seen driving global demand for rare earths in a big way. Malaysia needs to discover and venture into new economic growth areas. This will help fuel the country's drive to achieve a high income status by 2020 as articulated in the New Economic Model (NEM) and the many supporting Economic Transformation Plans that the Government has recently launched. Rare earths may be the new growth area for Malaysia. However, the business opportunities should not just be confined to the mining, extraction and production of rare earths elements alone if Malaysia is to maximise benefits from this industry. The industry's gold mine is in the downstream products. This is also the sector that China wants to expand. Japan which now controls about 50 % of the global market for downstream rare earths-based high-tech components is desperately looking for partners to grow their stake in the business. Malaysia needs to embark on the right strategies in order to build the rare earths industry in the country. What are the strategies? (author)

Greenhouse Earth: A Traveling Exhibition

International Nuclear Information System (INIS)

Booth, W.H.; Caesar, S.

1992-09-01

The Franklin Institute Science Museum provided an exhibit entitled the Greenhouse Earth: A Traveling Exhibition. This 3500 square-foot exhibit on global climate change was developed in collaboration with the Association of Science-Technology Centers. The exhibit opened at The Franklin Institute on February 14, 1992, welcoming 291,000 visitors over its three-month stay. During its three-year tour, Greenhouse Earth will travel to ten US cities, reaching two million visitors. Greenhouse Earth aims to deepen public understanding of the scientific issues of global warming and the conservation measures that can be taken to slow its effects. The exhibit features hands-on exhibitry, interactive computer programs and videos, a theater production, a ''demonstration cart,'' guided tours, and lectures. supplemental educational programs at the Institute included a teachers preview, a symposium on climate change, and a ''satellite field trip.'' The development of Greenhouse Earth included front-end and formative evaluation procedures. Evaluation includes interviews with visitors, prototypes, and summative surveys for participating museums. During its stay in Philadelphia, Greenhouse Earth was covered by the local and national press, with reviews in print and broadcast media. Greenhouse Earth is the first large-scale museum exhibit to address global climate change

Rare earth element and rare metal inventory of central Asia

Science.gov (United States)

Mihalasky, Mark J.; Tucker, Robert D.; Renaud, Karine; Verstraeten, Ingrid M.

2018-03-06

Rare earth elements (REE), with their unique physical and chemical properties, are an essential part of modern living. REE have enabled development and manufacture of high-performance materials, processes, and electronic technologies commonly used today in computing and communications, clean energy and transportation, medical treatment and health care, glass and ceramics, aerospace and defense, and metallurgy and chemical refining. Central Asia is an emerging REE and rare metals (RM) producing region. A newly compiled inventory of REE-RM-bearing mineral occurrences and delineation of areas-of-interest indicate this region may have considerable undiscovered resources.

Rare earth-iron-boron premanent magnets

International Nuclear Information System (INIS)

Ghendehari, M.H.

1988-01-01

This patent describes a method for producing rare earth-iron-boron permanent magnets containing added rare earth oxide, comprising the steps of: (a) mixing a particulate alloy containing at least one rare earth metal, iron, and boron with at least one particulate rare earth oxide; (b) aligning magnetic domains of the mixture in a magnetic field; (c) compacting the aligned mixture to form a shape; and (d) sintering the compacted shape

Developing Breast Cancer Program at Xavier; Genomic and Proteomic Analysis of Signaling Pathways Involved in Xenohormone and MEK5 Regulation of Breast Cancer

Science.gov (United States)

2010-05-01

Tagatose are rare. In this study we have determined the effect of these rare ketohexoses on breast cancer cell proliferation and estrogen signalling...Cancer Center (TCC) will build a core of human talent that will address scientific problems such as drug resistance and the effect of environmental agents...pathways for ER(+) (MCF-7) and ER(-) (MCF-7-MEK5) as potentially more effective therapeutic targets. 11 Abstract of RCMI proposal submitted to

Chemical analysis of rare earth elements

International Nuclear Information System (INIS)

Tsukahara, Ryoichi; Sakoh, Takefumi; Nagai, Iwao

1994-01-01

Recently attention has been paid to ICP-AES or ICP-MS, and the reports on the analysis of rare earth elements by utilizing these methods continue to increase. These reports have become to take about 30% of the reports on rare earth analysis, and this is because these methods are highly sensitive to rare earth elements, and also these methods have spread widely. In ICP-AES and ICP-MS, mostly solution samples are measured, therefore, solids must be made into solution. At the time of quantitatively determining the rare earth elements of low concentration, separation and concentration are necessary. Referring to the literatures reported partially in 1990 and from 1991 to 1993, the progress of ICP-AES and ICP-MS is reported. Rare earth oxides and the alloys containing rare earth elements are easily decomposed with acids, but the decomposition of rocks is difficult, and its method is discussed. The separation of the rare earth elements from others in geochemical samples, cation exchange process is frequently utilized. Also solvent extraction process has been studied. For the separation of rare earth elements mutually, chromatography is used. The spectral interference in spectral analysis was studied. The comparison of these methods with other methods is reported. (K.I)

Exhibition

CERN Multimedia

Staff Association

2016-01-01

Encounters Hanne Blitz From February 1st to 12th 2016 CERN Meyrin, Main Building What is our reaction to a first encounter with a tourist attraction? Contemporary Dutch painter Hanne Blitz captures visitors' responses to art and architecture, sweeping vistas and symbolic memorials. Encounters, a series of oil paintings curated specially for this CERN exhibition, depicts tourists visiting cultural highlights around the world. A thought-provoking journey not to be missed, and a tip of the hat to CERN's large Hadron Collider.

Activation of the MAPK pathway is a common event in uveal melanomas although it rarely occurs through mutation of BRAF or RAS.

Science.gov (United States)

Zuidervaart, W; van Nieuwpoort, F; Stark, M; Dijkman, R; Packer, L; Borgstein, A-M; Pavey, S; van der Velden, P; Out, C; Jager, M J; Hayward, N K; Gruis, N A

2005-06-06

In contrast to cutaneous melanoma, there is no evidence that BRAF mutations are involved in the activation of the mitogen-activated protein kinase (MAPK) pathway in uveal melanoma, although there is increasing evidence that this pathway is activated frequently in the latter tumours. In this study, we performed mutation analysis of the RAS and BRAF genes in a panel of 11 uveal melanoma cell lines and 19 primary uveal melanoma tumours. In addition, Western blot and immunohistochemical analyses were performed on downstream members of the MAPK pathway in order to assess the contribution of each of these components. No mutations were found in any of the three RAS gene family members and only one cell line carried a BRAF mutation (V599E). Despite this, mitogen-activated protein kinase/extracellular signal-regulated kinase kinase (MEK), ERK and ELK were constitutively activated in all samples. These data suggest that activation of the MAPK pathway is commonly involved in the development of uveal melanoma, but occurs through a mechanism different to that of cutaneous melanoma.

Integrated Genomic Analysis of the Ubiquitin Pathway across Cancer Types

Directory of Open Access Journals (Sweden)

Zhongqi Ge

2018-04-01

Full Text Available Summary: Protein ubiquitination is a dynamic and reversible process of adding single ubiquitin molecules or various ubiquitin chains to target proteins. Here, using multidimensional omic data of 9,125 tumor samples across 33 cancer types from The Cancer Genome Atlas, we perform comprehensive molecular characterization of 929 ubiquitin-related genes and 95 deubiquitinase genes. Among them, we systematically identify top somatic driver candidates, including mutated FBXW7 with cancer-type-specific patterns and amplified MDM2 showing a mutually exclusive pattern with BRAF mutations. Ubiquitin pathway genes tend to be upregulated in cancer mediated by diverse mechanisms. By integrating pan-cancer multiomic data, we identify a group of tumor samples that exhibit worse prognosis. These samples are consistently associated with the upregulation of cell-cycle and DNA repair pathways, characterized by mutated TP53, MYC/TERT amplification, and APC/PTEN deletion. Our analysis highlights the importance of the ubiquitin pathway in cancer development and lays a foundation for developing relevant therapeutic strategies. : Ge et al. analyze a cohort of 9,125 TCGA samples across 33 cancer types to provide a comprehensive characterization of the ubiquitin pathway. They detect somatic driver candidates in the ubiquitin pathway and identify a cluster of patients with poor survival, highlighting the importance of this pathway in cancer development. Keywords: ubiquitin pathway, pan-cancer analysis, The Cancer Genome Atlas, tumor subtype, cancer prognosis, therapeutic targets, biomarker, FBXW7

SYMPOSIUM: Rare decays

Energy Technology Data Exchange (ETDEWEB)

Anon.

1989-04-15

Late last year, a symposium entitled 'Rare Decays' attracted 115 participants to a hotel in Vancouver, Canada. These participants were particle physicists interested in checking conventional selection rules to look for clues of possible new behaviour outside today's accepted 'Standard Model'. For physicists, 'rare decays' include processes that have so far not been seen, explicitly forbidden by the rules of the Standard Model, or processes highly suppressed because the decay is dominated by an easier route, or includes processes resulting from multiple transitions.

Magnetic properties of 3d-transition metal and rare earth fluoride glasses

International Nuclear Information System (INIS)

Renard, J.P.; Dupas, C.; Velu, E.; Jacobini, C.; Fonteneau, G.; Lucas, J.

1981-01-01

The ac susceptibility of fluoride glasses in the ternary systems PbF 2 -MnF 2 -FeF 3 , ThF 4 -BaF 2 -MnF 2 , ZnF 2 -BaF 2 -RF 3 (R = Dy-Ho) has been studied down to 0.3 K. The susceptibility of rare earth glasses exhibits a broad maximum strongly dependent on the measuring frequency ν while a spin glass transition with a sharp susceptibility cusp nearly independent on ν is observed in 3d-transition metal glasses. Magnetic after effects are observed below the spin freezing temperature. (orig.)

Non-Hodgkin's lymphoma of the maxilla: A rare case report and review

Directory of Open Access Journals (Sweden)

Rajarshi Banerjee

2017-01-01

Full Text Available Non-Hodgkin's lymphomas (NHLs embody a diverse group of malignancies that originate from the lymphoid system. NHL often exhibit in an extranodal pattern, pertaining to the head and neck region. Intraoral sites are much less frequent, accounting for approximately 3.5% of all oral malignancies. Although the exact cause of NHL still remains inconspicuous, however, research has focused on some factors that may contribute to the development of lymphoma, including genetic factors, impaired immune system and viruses, such as HIV or EBV. Clinically, the bony lesion may present as localized or diffuse swelling, with low-grade pain, sweating, unexplained weight loss, fever, etc. Radiographically, these lesions resemble osteomyelitis or other malignancies creating a diagnostic dilemma. Microscopically, diffused lymphomas consist of large tumor cells with large nuclei that are more than twice the size of lymphocytes which may either exhibit centroblastic or immunoblastic features. Here, we report a rare case of NHL affecting the jaws of a 60-year-old male patient.

The industry of metallic rare earths (R.E.)

International Nuclear Information System (INIS)

Poirier, P.

1979-01-01

The following subjects are discussed: rare earths resources (rare earths abondance and world reserves, main ores). Rare earths separation and purification (ionic exchange, solvent extraction). Metallic rare earths and their mixtures, metallothermic reduction of oxides or fluorides (Ca, Mg, Al, Si or rare earth metals), Co-reduction process for intermetallic compounds (SmCo 5 ). Industrial applications of metallic rare earths (traditional applications such as flints, nodular cast iron, steel refining, magnesium industrie, applications under development such as rare earths/cobalt magnets, LaNi 5 for hydrogen storage, special alloys (automotive post combustion), magnetostrictive alloys). Economical problems: rare earth are elements relatively abundant and often at easily accessible prices. However, this group of 15 elements are liable to certain economical restraints. It is difficult to crack ore for only one rare earth. Availability of one given rare earth must be associated with the other corresponding rare earths to absorb all the other rare earths in other applications. Rare-earth industry has a strong expanding rate. 20% per year average for 6 years with Rhone-Poulenc. Thanks to their exceptional, specific characteristics rare earths have a bright future particularly for their metals

Carcinoma ex basal cell adenoma of the parotid gland: A report of an extremely rare case.

Science.gov (United States)

Kusafuka, Kimihide; Kawasaki, Takuya; Nakajima, Takashi; Sugino, Takashi

2017-07-01

Malignant non-basaloid tumors that arise from basal cell adenoma (BCA) are extremely rare. The patient was a 72-year-old Japanese male, who had noticed swelling of the left parotid region 21 years ago. A superficial lobectomy was performed. About 60% of the tumor was made up of cribriform and trabecular tissue composed of basaloid cells, which exhibited mild atypia and nuclear expression of β-catenin. This portion of the tumor was considered to be a BCA. In the other part of the tumor, the proliferation of large eosinophilic atypical cells, most of which formed intraductal structures, was observed. These tumor cells displayed cellular atypia, and some of them formed Roman bridge structures or contributed to intracapsular invasion. Immunohistochemically, these cells were positive for cytokeratin 7, gross cystic disease fluid proten-15 (GCDFP-15), androgen receptor (AR), and mammaglobin (MMG) and exhibited a high Ki-67 labeling index. So, this portion of the tumor was considered to be a salivary duct carcinoma (SDC). The tumor's final diagnosis was SDC ex BCA (intracapsular type), which is extremely rare. GCDFP-15, AR, MMG, and Ki-67 are useful immunohistochemical markers for diagnosing SDC ex BCA. © 2017 The Authors. Pathology International Published by Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.

Rare earth elements: end use and recyclability

Science.gov (United States)

Goonan, Thomas G.

2011-01-01

Rare earth elements are used in mature markets (such as catalysts, glassmaking, lighting, and metallurgy), which account for 59 percent of the total worldwide consumption of rare earth elements, and in newer, high-growth markets (such as battery alloys, ceramics, and permanent magnets), which account for 41 percent of the total worldwide consumption of rare earth elements. In mature market segments, lanthanum and cerium constitute about 80 percent of rare earth elements used, and in new market segments, dysprosium, neodymium, and praseodymium account for about 85 percent of rare earth elements used. Regardless of the end use, rare earth elements are not recycled in large quantities, but could be if recycling became mandated or very high prices of rare earth elements made recycling feasible.

Radioresistance of chordoma cells is associated with the ATM/ATR pathway, in which RAD51 serves as an important downstream effector.

Science.gov (United States)

Zhang, Chao; Wang, Bing; Li, Lei; Li, Yawei; Li, Pengzhi; Lv, Guohua

2017-09-01

Surgery followed by radiotherapy is the standard treatment for chordomas, which are a rare but low-grade type of bone cancer arising from remnants of the embryonic notochord. However, disease recurrence following radiotherapy is common, most likely due to endogenous DNA repair mechanisms that promote cell survival upon radiation strikes. The ataxia telangiectasia mutated/ataxia telangiectasia mutated and Rad3 related (ATM/ATR)-mediated pathway has a critical role in DNA repair mechanisms; however, it has rarely been investigated in chordomas. In the present study, the expression of signal molecules related to the ATM/ATR pathway in chordoma tissues and adjacent normal tissues were initially examined using immunohistochemistry and western blot analysis. Chordoma U-CH1 and U-CH2 cells were subsequently used to investigate cell responses to ionizing radiation and the potential protective actions mediated by the ATM/ATR pathway. Phosphorylated (p)-ATM, p-ATR, γ-H2A histone family, member X (H2AX) and RAD51 were significantly upregulated in chordoma tissues relative to adjacent normal tissues (PATM, γ-H2AX and RAD51 expression in U-CH1 cells (PATM, p-ATR and RAD51 levels in U-CH2 cells (PATM/ATR pathway, in which RAD51 serves as an important downstream effector. Thus, RAD51 presents a promising therapeutic target for improving the outcome of radiotherapy treatment in chordomas.

Organizational Learning in Rare Events

DEFF Research Database (Denmark)

Andersen, Kristina Vaarst; Tyler, Beverly; Beukel, Karin

When organizations encounter rare events they often find it challenging to extract learning from the experience. We analyze opportunities for organizational learning in one such rare event, namely Intellectual Property (IP) litigation, i.e., when organizations take disputes regarding their intell......When organizations encounter rare events they often find it challenging to extract learning from the experience. We analyze opportunities for organizational learning in one such rare event, namely Intellectual Property (IP) litigation, i.e., when organizations take disputes regarding...... the organization little discretion to utilize any learning from past litigation success. Thus, learning appears be to most beneficial in infringement cases. Based on statistical analysis of 10,211 litigation court cases in China, we find support for our hypotheses. Our findings suggest that organizations can learn...

Investigating Design Research Landscapes through Exhibition

DEFF Research Database (Denmark)

Jönsson, Li; Hansen, Flemming Tvede; Mäkelä, Maarit

2013-01-01

What characterizes a design research exhibition compared to a traditional design and art exhibition? How do you show the very materialities of the design experiments as a means for communicating knowledge of research and of practice? How do you present, review and utilize such an exhibition......? With those questions in mind, the intention and challenge for the Nordes 2013 Design Research Exhibition was to expand on current notions of staging research enquires in design research conference contexts. Artefacts, installations, performances, and other materialities that relate to the theme...... of the conference - Experiments in Design Research – were displayed as tools to express and communicate different design research enquires. Through this paper we will describe the Nordes exhibition as a specific case that renders questions visible in relation to how to utilize a design research exhibition...

Influence of strain and polycrystalline ordering on magnetic properties of high moment rare earth metals and alloys

International Nuclear Information System (INIS)

Scheunert, G; Ward, C; Hendren, W R; Bowman, R M; Lapicki, A A; Hardeman, R; Mooney, M; Gubbins, M

2014-01-01

Despite being the most suitable candidates for solenoid pole pieces in state-of-the-art superconductor-based electromagnets, the intrinsic magnetic properties of heavy rare earth metals and their alloys have gained comparatively little attention. With the potential of integration in micro and nanoscale devices, thin films of Gd, Dy, Tb, DyGd and DyTb were plasma-sputtered and investigated for their in-plane magnetic properties, with an emphasis on magnetization versus temperature profiles. Based on crystal structure analysis of the polycrystalline rare earth films, which consist of a low magnetic moment fcc layer at the seed interface topped with a higher moment hcp layer, an experimental protocol is introduced which allows the direct magnetic analysis of the individual layers. In line with the general trend of heavy lanthanides, the saturation magnetization was found to drop with increasing unit cell size. In situ annealed rare earth films exceeded the saturation magnetization of a high-moment Fe 65 Co 35 reference film in the cryogenic temperature regime, proving their potential for pole piece applications; however as-deposited rare earth films were found completely unsuitable. In agreement with theoretical predictions, sufficiently strained crystal phases of Tb and Dy did not exhibit an incommensurate magnetic order, unlike their single-crystal counterparts which have a helical phase. DyGd and DyTb alloys followed the trends of the elemental rare earth metals in terms of crystal structure and magnetic properties. Inter-rare-earth alloys hence present a desirable blend of saturation magnetization and operating temperature. (paper)

Designing and Implementing an Assay for the Detection of Rare and Divergent NRPS and PKS Clones in European, Antarctic and Cuban Soils.

Directory of Open Access Journals (Sweden)

Gregory C A Amos

Full Text Available The ever increasing microbial resistome means there is an urgent need for new antibiotics. Metagenomics is an underexploited tool in the field of drug discovery. In this study we aimed to produce a new updated assay for the discovery of biosynthetic gene clusters encoding bioactive secondary metabolites. PCR assays targeting the polyketide synthases (PKS and non-ribosomal peptide synthetases (NRPS were developed. A range of European soils were tested for their biosynthetic potential using clone libraries developed from metagenomic DNA. Results revealed a surprising number of NRPS and PKS clones with similarity to rare Actinomycetes. Many of the clones tested were phylogenetically divergent suggesting they were fragments from novel NRPS and PKS gene clusters. Soils did not appear to cluster by location but did represent NRPS and PKS clones of diverse taxonomic origin. Fosmid libraries were constructed from Cuban and Antarctic soil samples; 17 fosmids were positive for NRPS domains suggesting a hit rate of less than 1 in 10 genomes. NRPS hits had low similarities to both rare Actinobacteria and Proteobacteria; they also clustered with known antibiotic producers suggesting they may encode for pathways producing novel bioactive compounds. In conclusion we designed an assay capable of detecting divergent NRPS and PKS gene clusters from the rare biosphere; when tested on soil samples results suggest the majority of NRPS and PKS pathways and hence bioactive metabolites are yet to be discovered.

Rare B decays at LEP

CERN Document Server

Kluit, P M

2001-01-01

The results of the LEP experiments for rare B decays will be reviewed, covering hadronic final states, radiative and other rare decays and results for the inclusive charmless branching ratio. (8 refs).

Protein-altering variants associated with body mass index implicate pathways that control energy intake and expenditure in obesity.

OpenAIRE

Turcot, Valérie; Lu, Yingchang; Highland, Heather M; Schurmann, Claudia; Justice, Anne E; Fine, Rebecca S; Bradfield, Jonathan P; Esko, Tõnu; Giri, Ayush; Graff, Mariaelisa; Guo, Xiuqing; Hendricks, Audrey E; Karaderi, Tugce; Lempradl, Adelheid; Locke, Adam E

2018-01-01

Genome-wide association studies (GWAS) have identified >250 loci for body mass index (BMI), implicating pathways related to neuronal biology. Most GWAS loci represent clusters of common, non-coding variants from which pinpointing causal genes remains challenging. Here, we combined data from 718,734 individuals to discover rare and low-frequency (MAF

Antituberculotic activity of actinobacteria isolated from the rare habitats.

Science.gov (United States)

Hussain, A; Rather, M A; Shah, A M; Bhat, Z S; Shah, A; Ahmad, Z; Parvaiz Hassan, Q

2017-09-01

A distinctive screening procedure resulted in the isolation and identification of antituberculotic actinobacteria. In this course, a total of 125 actinobacteria were isolated from various soil samples from untapped areas in Northwestern Himalayas, India. The antibacterial screening showed that 26 isolates inhibited the growth of at least one of the tested bacterial pathogens including Staphylococcus aureus (ATCC 25923), Staphylococcus epidermidis (ATCC 12228), Bacillus subtilis (ATCC 11774), Micrococcus luteus (ATCC 10240), Escherichia coli (10536), Pseudomonas aeruginosa (ATCC 10145) and Klebsiella pneumonia (ATCC BAA-2146). The production media was optimized for the active strains by estimation of their extract value by the quantification of the ethyl acetate extract. The screening of fermentation products from the selected 26 bioactive isolates revealed that 10 strains have metabolites antagonistic against the standard H37Rv strain of Mycobacterium tuberculosis. The characterization by 16S rRNA gene sequencing and phylogenetic analysis demonstrated the diverse nature of these antituberculosis strains. The secondary metabolites of potent, rare strain, Lentzea violacea AS08 exhibited promising antituberculosis activity with minimal inhibitory concentration (MIC) of 3·9 μg ml -1 . The metabolites identified by gas chromatography-mass spectrometry (GC-MS) included, Phenol, 2,5-bis (1, 1-dimethylethyl), n-Hexadecanoic acid, Hexadecanoic acid methyl-ester, Hexadecanoic acid ethyl-ester and, 9,12-Octadecadienoyl chloride(Z,Z) are biologically significant molecules. The study presents the isolation of rare actinobacteria from untapped sites in the Northwestern Himalayas and their in vitro potential against Mycobacterium tuberculosis for their metabolites. The study revealed that exploring the untapped natural sources as one of the resourceful approaches for the discovery of new natural products. This study also provided strong evidence for the ability of rare and

Coarsely resolved topography along protein folding pathways

Science.gov (United States)

Fernández, Ariel; Kostov, Konstantin S.; Berry, R. Stephen

2000-03-01

The kinetic data from the coarse representation of polypeptide torsional dynamics described in the preceding paper [Fernandez and Berry, J. Chem. Phys. 112, 5212 (2000), preceding paper] is inverted by using detailed balance to obtain a topographic description of the potential-energy surface (PES) along the dominant folding pathway of the bovine pancreatic trypsin inhibitor (BPTI). The topography is represented as a sequence of minima and effective saddle points. The dominant folding pathway displays an overall monotonic decrease in energy with a large number of staircaselike steps, a clear signature of a good structure-seeker. The diversity and availability of alternative folding pathways is analyzed in terms of the Shannon entropy σ(t) associated with the time-dependent probability distribution over the kinetic ensemble of contact patterns. Several stages in the folding process are evident. Initially misfolded states form and dismantle revealing no definite pattern in the topography and exhibiting high Shannon entropy. Passage down a sequence of staircase steps then leads to the formation of a nativelike intermediate, for which σ(t) is much lower and fairly constant. Finally, the structure of the intermediate is refined to produce the native state of BPTI. We also examine how different levels of tolerance to mismatches of side chain contacts influence the folding kinetics, the topography of the dominant folding pathway, and the Shannon entropy. This analysis yields upper and lower bounds of the frustration tolerance required for the expeditious and robust folding of BPTI.

Angle-resolved photoemission spectroscopy of rare earth LaSb{sub 2}

Energy Technology Data Exchange (ETDEWEB)

Michiardi, Matteo; Arnold, Fabian; Faerch Fisher, Karl Frederik; Svane, Axel; Bianchi, Marco; Brummerstedt Iversen, Bo; Hofmann, Philip [Aarhus University (Denmark); Shwetha, G.; Kanchana, V. [IIT-Hyderabad (India); Ganapathy, Vaitheeswaran [University of Hyderabad (India)

2016-07-01

Several rare earth diantimonides have been found to exhibit intriguing electronic properties such as anisotropic linear and non-saturating magnetoresistance. Among these materials, LaSb{sub 2} is not only considered for application in magnetoresistive devices but it is also found to be superconducting at low temperatures and it is investigated as candidate material to host charge density wave phases. Despite the several studies on its transport properties, the electronic structure of LaSb{sub 2} is still largely unknown. Here we present an angle-resolved photoemission spectroscopy and ab-initio calculation study of LaSb{sub 2}(001). The observed band structure is found to be in good agreement with theoretical predictions. Our results reveal that LaSb{sub 2} is a semimetal with a strongly nested two-dimensional Fermi surface. The low energy spectrum is characterized by four massive hole pockets and by four shallow, strongly directional, electron pockets that exhibit Dirac-like dispersion. We speculate on the possibility that this peculiar electronic structure drives the magnetoresistance to its quantum limit, explaining its unconventional behavior.

Bayesian analysis of rare events

Energy Technology Data Exchange (ETDEWEB)

Straub, Daniel, E-mail: straub@tum.de; Papaioannou, Iason; Betz, Wolfgang

2016-06-01

In many areas of engineering and science there is an interest in predicting the probability of rare events, in particular in applications related to safety and security. Increasingly, such predictions are made through computer models of physical systems in an uncertainty quantification framework. Additionally, with advances in IT, monitoring and sensor technology, an increasing amount of data on the performance of the systems is collected. This data can be used to reduce uncertainty, improve the probability estimates and consequently enhance the management of rare events and associated risks. Bayesian analysis is the ideal method to include the data into the probabilistic model. It ensures a consistent probabilistic treatment of uncertainty, which is central in the prediction of rare events, where extrapolation from the domain of observation is common. We present a framework for performing Bayesian updating of rare event probabilities, termed BUS. It is based on a reinterpretation of the classical rejection-sampling approach to Bayesian analysis, which enables the use of established methods for estimating probabilities of rare events. By drawing upon these methods, the framework makes use of their computational efficiency. These methods include the First-Order Reliability Method (FORM), tailored importance sampling (IS) methods and Subset Simulation (SuS). In this contribution, we briefly review these methods in the context of the BUS framework and investigate their applicability to Bayesian analysis of rare events in different settings. We find that, for some applications, FORM can be highly efficient and is surprisingly accurate, enabling Bayesian analysis of rare events with just a few model evaluations. In a general setting, BUS implemented through IS and SuS is more robust and flexible.

A mini-exhibition with maximum content

CERN Multimedia

Laëtitia Pedroso

2011-01-01

The University of Budapest has been hosting a CERN mini-exhibition since 8 May. While smaller than the main travelling exhibition it has a number of major advantages: its compact design alleviates transport difficulties and makes it easier to find suitable venues in the Member States. Its content can be updated almost instantaneously and it will become even more interactive and high-tech as time goes by.   The exhibition on display in Budapest. The purpose of CERN's new mini-exhibition is to be more interactive and easier to install. Due to its size, the main travelling exhibition cannot be moved around quickly, which is why it stays in the same country for 4 to 6 months. But this means a long waiting list for the other Member States. To solve this problem, the Education Group has designed a new exhibition, which is smaller and thus easier to install. Smaller maybe, but no less rich in content, as the new exhibition conveys exactly the same messages as its larger counterpart. However, in the slimm...

Reverse (posterior) cloaca with congenital rectal stenosis and pseudohermaphrodism: a rare entity with rare association

Science.gov (United States)

Ahmed, Intezar; Kureel, Shiv Narain; Chandra, Naveen

2010-01-01

Reverse (posterior) cloaca is itself a rare entity and association of this defect with other congenital syndromes or defects is extremely rare to find. To diagnose this unique anomaly one should be aware about this very infrequently encountered clinical entity. Reverse cloaca is a very rare variant of anorectal malformation in which urethra and vagina fused to form a urogenital sinus that diverts posteriorly and opens in the anterior rectal wall at the anus or immediately anterior to it. We report a neonate with the rare clinical entity of reverse cloaca type C, pseudophallus with rudimentary urethra as a component of female pseudohermaphrodism and congenital rectal stenosis. Surprisingly, there was no associated urological abnormality on ultrasonography and laparotomy. PMID:22797480

SYMPOSIUM: Rare decays

International Nuclear Information System (INIS)

Anon.

1989-01-01

Late last year, a symposium entitled 'Rare Decays' attracted 115 participants to a hotel in Vancouver, Canada. These participants were particle physicists interested in checking conventional selection rules to look for clues of possible new behaviour outside today's accepted 'Standard Model'. For physicists, 'rare decays' include processes that have so far not been seen, explicitly forbidden by the rules of the Standard Model, or processes highly suppressed because the decay is dominated by an easier route, or includes processes resulting from multiple transitions

Elite suppressor-derived HIV-1 envelope glycoproteins exhibit reduced entry efficiency and kinetics.

Directory of Open Access Journals (Sweden)

Kara G Lassen

2009-04-01

Full Text Available Elite suppressors (ES are a rare subset of HIV-1-infected individuals who are able to maintain HIV-1 viral loads below the limit of detection by ultra-sensitive clinical assays in the absence of antiretroviral therapy. Mechanism(s responsible for this elite control are poorly understood but likely involve both host and viral factors. This study assesses ES plasma-derived envelope glycoprotein (env fitness as a function of entry efficiency as a possible contributor to viral suppression. Fitness of virus entry was first evaluated using a novel inducible cell line with controlled surface expression levels of CD4 (receptor and CCR5 (co-receptor. In the context of physiologic CCR5 and CD4 surface densities, ES envs exhibited significantly decreased entry efficiency relative to chronically infected viremic progressors. ES envs also demonstrated slow entry kinetics indicating the presence of virus with reduced entry fitness. Overall, ES env clones were less efficient at mediating entry than chronic progressor envs. Interestingly, acute infection envs exhibited an intermediate phenotypic pattern not distinctly different from ES or chronic progressor envs. These results imply that lower env fitness may be established early and may directly contribute to viral suppression in ES individuals.

Rare variants in MYD88, IRAK4 and IKBKG and susceptibility to invasive pneumococcal disease: a population-based case-control study.

Directory of Open Access Journals (Sweden)

Magda K Ellis

Full Text Available Although rare variants within the Toll-like receptor signalling pathway genes have been found to underlie human primary immunodeficiencies associated with selective predisposition to invasive pneumococcal disease (IPD, the contribution of variants in these genes to IPD susceptibility at the population level remains unknown. Complete re-sequencing of IRAK4, MYD88 and IKBKG genes was undertaken in 164 IPD cases from the UK and 164 geographically-matched population-based controls. 233 single-nucleotide variants (SNVs were identified, of which ten were in coding regions. Four rare coding variants were predicted to be deleterious, two variants in MYD88 and two in IRAK4. The predicted deleterious variants in MYD88 were observed as two heterozygote cases but not seen in controls. Frequencies of predicted deleterious IRAK4 SNVs were the same in cases and controls. Our findings suggest that rare, functional variants in MYD88, IRAK4 or IKBKG do not significantly contribute to IPD susceptibility in adults at the population level.

Recovering heavy rare earth metals from magnet scrap

Science.gov (United States)

Ott, Ryan T.; McCallum, Ralph W.; Jones, Lawrence L.

2017-08-08

A method of treating rare earth metal-bearing permanent magnet scrap, waste or other material in a manner to recover the heavy rare earth metal content separately from the light rare earth metal content. The heavy rare earth metal content can be recovered either as a heavy rare earth metal-enriched iron based alloy or as a heavy rare earth metal based alloy.

Pathway Relevance Ranking for Tumor Samples through Network-Based Data Integration.

Directory of Open Access Journals (Sweden)

Lieven P C Verbeke

Full Text Available The study of cancer, a highly heterogeneous disease with different causes and clinical outcomes, requires a multi-angle approach and the collection of large multi-omics datasets that, ideally, should be analyzed simultaneously. We present a new pathway relevance ranking method that is able to prioritize pathways according to the information contained in any combination of tumor related omics datasets. Key to the method is the conversion of all available data into a single comprehensive network representation containing not only genes but also individual patient samples. Additionally, all data are linked through a network of previously identified molecular interactions. We demonstrate the performance of the new method by applying it to breast and ovarian cancer datasets from The Cancer Genome Atlas. By integrating gene expression, copy number, mutation and methylation data, the method's potential to identify key pathways involved in breast cancer development shared by different molecular subtypes is illustrated. Interestingly, certain pathways were ranked equally important for different subtypes, even when the underlying (epi-genetic disturbances were diverse. Next to prioritizing universally high-scoring pathways, the pathway ranking method was able to identify subtype-specific pathways. Often the score of a pathway could not be motivated by a single mutation, copy number or methylation alteration, but rather by a combination of genetic and epi-genetic disturbances, stressing the need for a network-based data integration approach. The analysis of ovarian tumors, as a function of survival-based subtypes, demonstrated the method's ability to correctly identify key pathways, irrespective of tumor subtype. A differential analysis of survival-based subtypes revealed several pathways with higher importance for the bad-outcome patient group than for the good-outcome patient group. Many of the pathways exhibiting higher importance for the bad

Twenty-First Century Diseases: Commonly Rare and Rarely Common?

Science.gov (United States)

Daunert, Sylvia; Sittampalam, Gurusingham Sitta; Goldschmidt-Clermont, Pascal J

2017-09-20

Alzheimer's drugs are failing at a rate of 99.6%, and success rate for drugs designed to help patients with this form of dementia is 47 times less than for drugs designed to help patients with cancers ( www.scientificamerican.com/article/why-alzheimer-s-drugs-keep-failing/2014 ). How can it be so difficult to produce a valuable drug for Alzheimer's disease? Each human has a unique genetic and epigenetic makeup, thus endowing individuals with a highly unique complement of genes, polymorphisms, mutations, RNAs, proteins, lipids, and complex sugars, resulting in distinct genome, proteome, metabolome, and also microbiome identity. This editorial is taking into account the uniqueness of each individual and surrounding environment, and stresses the point that a more accurate definition of a "common" disorder could be simply the amalgamation of a myriad of "rare" diseases. These rare diseases are being grouped together because they share a rather constant complement of common features and, indeed, generally respond to empirically developed treatments, leading to a positive outcome consistently. We make the case that it is highly unlikely that such treatments, despite their statistical success measured with large cohorts using standardized clinical research, will be effective on all patients until we increase the depth and fidelity of our understanding of the individual "rare" diseases that are grouped together in the "buckets" of common illnesses. Antioxid. Redox Signal. 27, 511-516.

Human cardiac stem cells exhibit mesenchymal features and are maintained through Akt/GSK-3β signaling

International Nuclear Information System (INIS)

Tateishi, Kento; Ashihara, Eishi; Honsho, Shoken; Takehara, Naofumi; Nomura, Tetsuya; Takahashi, Tomosaburo; Ueyama, Tomomi; Yamagishi, Masaaki; Yaku, Hitoshi; Matsubara, Hiroaki; Oh, Hidemasa

2007-01-01

Recent evidence suggested that human cardiac stem cells (hCSCs) may have the clinical application for cardiac repair; however, their characteristics and the regulatory mechanisms of their growth have not been fully investigated. Here, we show the novel property of hCSCs with respect to their origin and tissue distribution in human heart, and demonstrate the signaling pathway that regulates their growth and survival. Telomerase-active hCSCs were predominantly present in the right atrium and outflow tract of the heart (infant > adult) and had a mesenchymal cell-like phenotype. These hCSCs expressed the embryonic stem cell markers and differentiated into cardiomyocytes to support cardiac function when transplanted them into ischemic myocardium. Inhibition of Akt pathway impaired the hCSC proliferation and induced apoptosis, whereas inhibition of glycogen synthase kinase-3 (GSK-3) enhanced their growth and survival. We conclude that hCSCs exhibit mesenchymal features and that Akt/GSK-3β may be crucial modulators for hCSC maintenance in human heart

Afganistan and rare earths

Directory of Open Access Journals (Sweden)

Emilian M. Dobrescu

2013-05-01

Full Text Available On our planet, over a quarter of new technologies for the economic production of industrial goods, are using rare earths, which are also called critical minerals and industries that rely on these precious items being worth of an estimated nearly five trillion dollars, or 5 percent of world gross domestic product. In the near future, competition will increase for the control of rare earth minerals embedded in high-tech products. Rare minerals are in the twenty-first century what oil accounted for in the twentieth century and coal in the nineteenth century: the engine of a new industrial revolution. Future energy will be produced increasingly by more sophisticated technological equipment based not just on steel and concrete, but incorporating significant quantities of metals and rare earths. Widespread application of these technologies will result in an exponential increase in demand for such minerals, and what is worrying is that minerals of this type are almost nowhere to be found in Europe and in other industrialized countries in the world, such as U.S. and Japan, but only in some Asian countries, like China and Afghanistan.

Dosimetric and kinetic parameters of lithium cadmium borate glasses doped with rare earth ions

Directory of Open Access Journals (Sweden)

J. Anjaiah

2014-10-01

Full Text Available Thermoluminescence (TL characteristics of X-ray irradiated pure and doped with four different rare earth ions (viz., Pr3+, Nd3+, Sm3+ and Eu3+ Li2O–Cdo–B2O3 glasses have been studied in the temperature range 303–573 K; the pure glass has exhibited single TL peak at 466 K. When this glass is doped with different rare earth ions no additional peaks are observed but the glow peak temperature of the existing glow peak shifted gradually towards higher temperatures with gain in intensity of TL light output. The area under the glow curve is found to be maximum for Eu3+ doped glasses. The trap depth parameters associated with the observed TL peaks have been evaluated using Chen's formulae. The possible use of these glasses in radiation dosimetry has been described. The result clearly showed that europium doped cadmium borate glass has a potential to be considered as the thermoluminescence dosimeter.

The Genetic Architecture Underlying the Evolution of a Rare Piscivorous Life History Form in Brown Trout after Secondary Contact and Strong Introgression

Directory of Open Access Journals (Sweden)

Arne Jacobs

2018-05-01

Full Text Available Identifying the genetic basis underlying phenotypic divergence and reproductive isolation is a longstanding problem in evolutionary biology. Genetic signals of adaptation and reproductive isolation are often confounded by a wide range of factors, such as variation in demographic history or genomic features. Brown trout (Salmo trutta in the Loch Maree catchment, Scotland, exhibit reproductively isolated divergent life history morphs, including a rare piscivorous (ferox life history form displaying larger body size, greater longevity and delayed maturation compared to sympatric benthivorous brown trout. Using a dataset of 16,066 SNPs, we analyzed the evolutionary history and genetic architecture underlying this divergence. We found that ferox trout and benthivorous brown trout most likely evolved after recent secondary contact of two distinct glacial lineages, and identified 33 genomic outlier windows across the genome, of which several have most likely formed through selection. We further identified twelve candidate genes and biological pathways related to growth, development and immune response potentially underpinning the observed phenotypic differences. The identification of clear genomic signals divergent between life history phenotypes and potentially linked to reproductive isolation, through size assortative mating, as well as the identification of the underlying demographic history, highlights the power of genomic studies of young species pairs for understanding the factors shaping genetic differentiation.

Emergence of differentially regulated pathways associated with the development of regional specificity in chicken skin.

Science.gov (United States)

Chang, Kai-Wei; Huang, Nancy A; Liu, I-Hsuan; Wang, Yi-Hui; Wu, Ping; Tseng, Yen-Tzu; Hughes, Michael W; Jiang, Ting Xin; Tsai, Mong-Hsun; Chen, Chien-Yu; Oyang, Yen-Jen; Lin, En-Chung; Chuong, Cheng-Ming; Lin, Shau-Ping

2015-01-23

Regional specificity allows different skin regions to exhibit different characteristics, enabling complementary functions to make effective use of the integumentary surface. Chickens exhibit a high degree of regional specificity in the skin and can serve as a good model for when and how these regional differences begin to emerge. We used developing feather and scale regions in embryonic chickens as a model to gauge the differences in their molecular pathways. We employed cosine similarity analysis to identify the differentially regulated and co-regulated genes. We applied low cell techniques for expression validation and chromatin immunoprecipitation (ChIP)-based enhancer identification to overcome limited cell availabilities from embryonic chicken skin. We identified a specific set of genes demonstrating a high correlation as being differentially expressed during feather and scale development and maturation. Some members of the WNT, TGF-beta/BMP, and Notch family known to be involved in feathering skin differentiation were found to be differentially regulated. Interestingly, we also found genes along calcium channel pathways that are differentially regulated. From the analysis of differentially regulated pathways, we used calcium signaling pathways as an example for further verification. Some voltage-gated calcium channel subunits, particularly CACNA1D, are expressed spatio-temporally in the skin epithelium. These calcium signaling pathway members may be involved in developmental decisions, morphogenesis, or epithelial maturation. We further characterized enhancers associated with histone modifications, including H3K4me1, H3K27ac, and H3K27me3, near calcium channel-related genes and identified signature intensive hotspots that may be correlated with certain voltage-gated calcium channel genes. We demonstrated the applicability of cosine similarity analysis for identifying novel regulatory pathways that are differentially regulated during development. Our study

Sintered cobalt-rare earth intermetallic product

International Nuclear Information System (INIS)

Benz, M.C.

1975-01-01

A process is described for preparing novel sintered cobalt--rare earth intermetallic products which can be magnetized to form permanent magnets having stable improved magnetic properties. A cobalt--rare earth metal alloy is formed having a composition which at sintering temperature falls outside the composition covered by the single Co 5 R intermetallic phase on the rare earth richer side. The alloy contains a major amount of the Co 5 R intermetallic phase and a second solid CoR phase which is richer in rare earth metal content than the Co 5 R phase. The specific cobalt and rare earth metal content of the alloy is substantially the same as that desired in the sintered product. The alloy, in particulate form, is pressed into compacts and sintered to the desired density. The sintered product is comprised of a major amount of the Co 5 R solid intermetallic phase and up to about 35 percent of the product of the second solid CoR intermetallic phase which is richer in rare earth metal content than the Co 5 R phase

HfO2 - rare earth oxide systems in the region with high content of rare earth oxide

International Nuclear Information System (INIS)

Shevchenko, A.V.; Lopato, L.M.

1982-01-01

Using the methods of annealing and hardenings (10 2 -10 4 deg/s cooling rate) and differential thermal analysis elements of state diagrams of HfO 2 - rare earth oxide (rare earths-La, Pr, Nd, Sm, Gd, Tb, Dy, Y, Er, Yb, Lu, Sc) systems from 1800 deg C up to melting in the range of 60-100 mol% rare earth oxide concentration were constructed. Regularities of HfQ 2 addition effect on high-temperature polymorphic transformations of rare earth oxides were studied. Results of investigation were discussed from viewpoint of crystal chemistry

Management of optic pathway and chiasmatic-hypothalamic gliomas in children with radiation therapy

International Nuclear Information System (INIS)

Erkal, Haldun Suekrue; Serin, Meltem; Cakmak, Ahmet

1997-01-01

Background and purpose: Optic pathway and chiasmatic-hypothalamic gliomas are rare childhood tumors. This study presents the experience in management of these tumors with radiation therapy. Materials and methods: Thirty-three children with the diagnosis of optic pathway and chiasmatic-hypothalamic gliomas were treated with radiation therapy from 1973 through 1994 in the Department of Radiation Oncology at Ankara University Faculty of Medicine. Twenty-four children had optic pathway gliomas and nine had chiasmatic-hypothalamic gliomas. Evidence of neurofibromatosis was present in six children. Subtotal resection was performed in 22 children and a biopsy in seven. The most common prescription for total tumor dose was 50 Gy, delivered in 2 Gy daily fractions. Follow-up ranged from 0.5 to 16.1 years (mean, 13.6 years). Results: Overall, progression-free and cause-specific survival probabilities for the entire group were 93%, 82% and 93%, respectively, at 5 years and 79%, 77% and 88%, respectively, at 10 years. Differences in overall, progression-free and cause-specific survival probabilities between optic pathway and chiasmatic-hypothalamic gliomas were not statistically significant. Absence of evidence of neurofibromatosis correlated with significantly better progression-free and cause-specific survival probabilities. Conclusion: Radiation therapy is effective in stabilization or improvement of vision and prevention of tumor progression in both optic pathway and chiasmatic-hypothalamic gliomas

Economic aspects of rare diseases.

Science.gov (United States)

Borski, Krzysztof

2015-01-01

Economic problems related to the prevention, diagnosis and treatment of rare diseases are presented paying particular attention to the costs of financing treatment, including the issue of its refund, which is a fundamental and difficult to solve economic problem of the health care system. Rare diseases, despite the low frequency of occurrence, together cover a large group of diseases being a serious medical, social and economic problem. The adoption of Polish National Plan for Rare Diseases resulting from the recommendations of the Council of the European Union, the extension of institutional activities related to the area of public health and social initiatives seeking innovative solutions to create a model of social support for patients and their families, with very high complexity of the issues regarding rare diseases, results in the need for a coherent, comprehensive, system operations and adoption of comprehensive solutions.

Highly fractionated rare-earth elements in ferromagnesian chondrules from the Felix (CO3) meteorite

International Nuclear Information System (INIS)

Misawa, Keiji; Nakamura, Noboru

1988-01-01

Here we describe two ferromagnesian chondrules from the Felix (Ornans-subtype) carbonaceous chondrite which carry a marker signature of REE (rare earth element) fractionation in the nebula. Both show positive Ce and Yb anomalies and one exhibits a light/heavy REE fractionation. On the basis of the REE characteristics of these chondrules, as well as those of the authors' work on Allende (CV) [N Geochim. Cosmochim. Acta. in press], we suggest that one of the precursor materials of chondrules in CO-CV carbonaceous chondrites is a high-temperature condensate from the nebular gas. (author)

Skeletal muscle-specific HMG-CoA reductase knockout mice exhibit rhabdomyolysis: A model for statin-induced myopathy.

Science.gov (United States)

Osaki, Yoshinori; Nakagawa, Yoshimi; Miyahara, Shoko; Iwasaki, Hitoshi; Ishii, Akiko; Matsuzaka, Takashi; Kobayashi, Kazuto; Yatoh, Shigeru; Takahashi, Akimitsu; Yahagi, Naoya; Suzuki, Hiroaki; Sone, Hirohito; Ohashi, Ken; Ishibashi, Shun; Yamada, Nobuhiro; Shimano, Hitoshi

2015-10-23

HMG-CoA reductase (HMGCR) catalyzes the conversion of HMG-CoA to mevalonic acid (MVA); this is the rate-limiting enzyme of the mevalonate pathway that synthesizes cholesterol. Statins, HMGCR inhibitors, are widely used as cholesterol-reducing drugs. However, statin-induced myopathy is the most adverse side effect of statins. To eludicate the mechanisms underlying statin the myotoxicity and HMGCR function in the skeletal muscle, we developed the skeletal muscle-specific HMGCR knockout mice. Knockout mice exhibited postnatal myopathy with elevated serum creatine kinase levels and necrosis. Myopathy in knockout mice was completely rescued by the oral administration of MVA. These results suggest that skeletal muscle toxicity caused by statins is dependent on the deficiencies of HMGCR enzyme activity and downstream metabolites of the mevalonate pathway in skeletal muscles rather than the liver or other organs. Copyright © 2015 Elsevier Inc. All rights reserved.

The genus Nonomuraea: A review of a rare actinomycete taxon for novel metabolites.

Science.gov (United States)

Sungthong, Rungroch; Nakaew, Nareeluk

2015-05-01

The genus Nonomuraea is a rare actinomycete taxon with a long taxonomic history, while its generic description was recently emended. The genus is less known among the rare actinomycete genera as its taxonomic position was revised several times. It can be found in diverse ecological niches, while most of its member species were isolated from soil samples. However, new trends to discover the genus in other habitats are increasing. Generic abundance of the genus was found to be dependent on geographical changes. Novel sources together with selective and invented isolation techniques might increase a chance to explore the genus and its novel candidates. Interestingly, some of its members have been revealed as a valuable source of novel metabolites for medical and industrial purposes. Broad-range of potent bioactive compounds including antimicrobial, anticancer, and antipsychotic substances, broad-spectrum antibiotics and biocatalysts can be synthesized by the genus. In order to investigate biosynthetic pathways of the bioactive compounds and self-resistant mechanisms to these compounds, the links from genes to metabolites have yet been needed for further discovery and biotechnological development of the genus Nonomuraea. © 2014 WILEY-VCH Verlag GmbH & Co. KGaA, Weinheim.

Production of rare earth-silicon-iron alloys

International Nuclear Information System (INIS)

Mehra, O.K.; Bose, D.K.; Gupta, C.K.

1987-01-01

At Metallurgy Division, BARC, improved procedures for producing rare earth-silicon alloys have been investigated. In these methods, reduction of mixed rare earth oxide by a ferro-silicon and aluminium mixture in combination with CaO-MgO flux/CaO-CaF 2 flux have been tried to prepare an alloy product with a higher rare earth recovery at a higher rare earth content than the present commercial production method. The rare earth recovery using CaO-CaF 2 was 85 per cent while in the case of CaO-MgO flux it was 76 per cent. The corresponding rare earth contents in the alloy correspond to 40 per cent and 55 per cent by weight respectively. (author)

The Rare Earth Magnet Industry and Rare Earth Price in China

Directory of Open Access Journals (Sweden)

Ding Kaihong

2014-07-01

Full Text Available In the past four years, the price of rare earth metal fluctuates sharply for many reasons. Currently, it has become more stable and more reasonable. This presentation is focused on the effect about the rare earth metal price. Some motor manufacturers have shifted from rare earth permanent magnet to ferrite magnet. Many motor manufacturers changed the design for the motor cooling system to make the motor function at a lower temperature. Thus the consumption of Dy can be markedly reduced. As for manufacturer of NdFeB magnet, we are also trying to optimize our process to reduce to dependence of HREE such as Dy and Tb. HS process have been introduced to solve the problem. With more and more people focusing and engaging on the REE industry, the price of REE will be more transparent without too many fluctuations. China is considering the problems of balancing the environment, energy sources, and labor sources. The application field about NdFeB such as wind turbine generator, HEV/EV, FA /OA is flourishing.

The Rare Earth Magnet Industry and Rare Earth Price in China

Science.gov (United States)

Ding, Kaihong

2014-07-01

In the past four years, the price of rare earth metal fluctuates sharply for many reasons. Currently, it has become more stable and more reasonable. This presentation is focused on the effect about the rare earth metal price. Some motor manufacturers have shifted from rare earth permanent magnet to ferrite magnet. Many motor manufacturers changed the design for the motor cooling system to make the motor function at a lower temperature. Thus the consumption of Dy can be markedly reduced. As for manufacturer of NdFeB magnet, we are also trying to optimize our process to reduce to dependence of HREE such as Dy and Tb. HS process have been introduced to solve the problem. With more and more people focusing and engaging on the REE industry, the price of REE will be more transparent without too many fluctuations. China is considering the problems of balancing the environment, energy sources, and labor sources. The application field about NdFeB such as wind turbine generator, HEV/EV, FA /OA is flourishing.

Screening key candidate genes and pathways involved in insulinoma by microarray analysis.

Science.gov (United States)

Zhou, Wuhua; Gong, Li; Li, Xuefeng; Wan, Yunyan; Wang, Xiangfei; Li, Huili; Jiang, Bin

2018-06-01

Insulinoma is a rare type tumor and its genetic features remain largely unknown. This study aimed to search for potential key genes and relevant enriched pathways of insulinoma.The gene expression data from GSE73338 were downloaded from Gene Expression Omnibus database. Differentially expressed genes (DEGs) were identified between insulinoma tissues and normal pancreas tissues, followed by pathway enrichment analysis, protein-protein interaction (PPI) network construction, and module analysis. The expressions of candidate key genes were validated by quantitative real-time polymerase chain reaction (RT-PCR) in insulinoma tissues.A total of 1632 DEGs were obtained, including 1117 upregulated genes and 514 downregulated genes. Pathway enrichment results showed that upregulated DEGs were significantly implicated in insulin secretion, and downregulated DEGs were mainly enriched in pancreatic secretion. PPI network analysis revealed 7 hub genes with degrees more than 10, including GCG (glucagon), GCGR (glucagon receptor), PLCB1 (phospholipase C, beta 1), CASR (calcium sensing receptor), F2R (coagulation factor II thrombin receptor), GRM1 (glutamate metabotropic receptor 1), and GRM5 (glutamate metabotropic receptor 5). DEGs involved in the significant modules were enriched in calcium signaling pathway, protein ubiquitination, and platelet degranulation. Quantitative RT-PCR data confirmed that the expression trends of these hub genes were similar to the results of bioinformatic analysis.The present study demonstrated that candidate DEGs and enriched pathways were the potential critical molecule events involved in the development of insulinoma, and these findings were useful for better understanding of insulinoma genesis.

Science Fiction Exhibits as STEM Gateways

Science.gov (United States)

Robie, Samantha

Women continue to hold less than a quarter of all STEM jobs in the United States, prompting many museums to develop programs and exhibits with the express goal of interesting young girls in scientific fields. At the same time, a number of recent museum exhibits have harnessed the popularity of pop culture and science fiction in order to interest general audiences in STEM subject matter, as well as using the exhibits as springboards to expand or shift mission goals and focus. Because science fiction appears to be successful at raising interest in STEM fields, it may be an effective way to garner the interest of young girls in STEM in particular. This research seeks to describe the ways in which museums are currently using science fiction exhibits to interest young girls in STEM fields and careers. Research focused on four institutions across the country hosting three separate exhibits, and included staff interviews and content analysis of exhibit descriptions, promotional materials, a summative evaluation and supplementary exhibit productions. In some ways, science fiction exhibits do serve young girls, primarily through the inclusion of female role models, staff awareness, and prototype testing to ensure interactives are attractive to girls as well as to boys. However, STEM appears to be underutilized, which may be partly due to a concern within the field that the outcome of targeting a specific gender could be construed as "stereotyping".

Novel and ultra-rare damaging variants in neuropeptide signaling are associated with disordered eating behaviors.

Directory of Open Access Journals (Sweden)

Michael Lutter

Full Text Available Eating disorders develop through a combination of genetic vulnerability and environmental stress, however the genetic basis of this risk is unknown.To understand the genetic basis of this risk, we performed whole exome sequencing on 93 unrelated individuals with eating disorders (38 restricted-eating and 55 binge-eating to identify novel damaging variants. Candidate genes with an excessive burden of predicted damaging variants were then prioritized based upon an unbiased, data-driven bioinformatic analysis. One top candidate pathway was empirically tested for therapeutic potential in a mouse model of binge-like eating.An excessive burden of novel damaging variants was identified in 186 genes in the restricted-eating group and 245 genes in the binge-eating group. This list is significantly enriched (OR = 4.6, p<0.0001 for genes involved in neuropeptide/neurotrophic pathways implicated in appetite regulation, including neurotensin-, glucagon-like peptide 1- and BDNF-signaling. Administration of the glucagon-like peptide 1 receptor agonist exendin-4 significantly reduced food intake in a mouse model of 'binge-like' eating.These findings implicate ultra-rare and novel damaging variants in neuropeptide/neurotropic factor signaling pathways in the development of eating disorder behaviors and identify glucagon-like peptide 1-receptor agonists as a potential treatment for binge eating.

Optimizing a magnetic resonance care pathway: A strategy for radiography managers

International Nuclear Information System (INIS)

Castillo, J.; Caruana, C.J.; Morgan, P.S.; Westbrook, C.

2015-01-01

Purpose: This study reports the optimization of a local MR care pathway. A search of the literature did not result in any studies regarding the optimization of MRI care pathways through a formal research process. Discussions with international MR radiographers indicated that such development is often carried out using informal methods that are highly dependent on local conditions, that are rarely reported in the public domain and the validities of which are therefore not open to scrutiny; in addition, care pathways need to be specific to local healthcare needs and culture. In this study, the authors propose a formal documented methodology for developing a local MRI care pathway based on the well-established nominal group technique. Methods and materials: A nominal group technique was conducted amongst a multi-professional panel. Results: 14 participants accepted the invitation to participate: an executive from the principal public general hospital, a manager from the national Ministry for Health, a service development manager from the allied healthcare professional sector, 2 senior physiotherapists, 3 nursing officers, 3 MRI radiographers, 2 medical physicists, 1 radiologist. Ten optimization related issues were identified and ranked in order of decreasing importance. Highest ranking scores were assigned to patient safety, education of referrers and use of quality criteria. The NGT method also brought forward novel themes in particular the need for a radiographer's technical report and the need for referrers to indicate pain levels of patients. Conclusion: The design of an MR care pathway was successfully optimized using a collaborative multi-stakeholder approach. - Highlights: • We optimized an MRI clinical pathway using a nominal group technique. • The NGT brought forward novel themes such as the introduction of radiographer technical report. • The MRI clinical pathway will help management to establish knowledge, skills and competences. �

On halide derivatives of rare-earth metal(III) oxidomolybdates(VI) and -tungstates(VI)

International Nuclear Information System (INIS)

Schleid, Thomas; Hartenbach, Ingo

2016-01-01

Halide derivatives of rare-earth metal(III) oxidomolybdates(VI) have been investigated comprehensively over the last decade comprising the halogens fluorine, chlorine, and bromine. Iodide-containing compounds are so far unknown. The simple composition REXMoO 4 (RE=rare-earth element, X=halogen) is realized for X=F almost throughout the complete lanthanide series as well as for yttrium. While ytterbium and lutetium do not form any fluoride derivative, for lanthanum, only a fluoride-deprived compound with the formula La 3 FMo 4 O 16 is realized. Moreover, molybdenum-rich compounds with the formula REXMo 2 O 7 are also known for yttrium and the smaller lanthanoids. For X=Cl the composition REClMoO 4 is known for yttrium and the whole lanthanide series, although, four different structure types were identified. Almost the same holds for X=Br, however, only two different structure types are realized in this class of compounds. In the case of halide derivatives of rare-earth metal(III) oxidotungstates(VI) the composition REXWO 4 is found for chlorides and bromides only, so far. Due to the similar size of Mo 6+ and W 6+ cations, the structures found for the tungstates are basically the same as for the molybdates. With the larger lanthanides, the representatives for both chloride and bromide derivates exhibit similar structural motifs as seen in the molybdates, however, the crystal structure cannot be determined reliably. In case of the smaller lanthanoids, the chloride derivatives are isostructural with the respective molybdates, although the existence ranges differ slightly. The same is true for rare-earth metal(III) bromide oxidotungstates(VI).

Comparative TEA for Indirect Liquefaction Pathways to Distillate-Range Fuels via Oxygenated Intermediates

Energy Technology Data Exchange (ETDEWEB)

Tan, Eric; Snowden-Swan, Lesley J.; Talmadge, Michael; Dutta, Abhijit; Jones, Susanne; Ramasamy, Karthikeyan; Gray, Michael; Dagle, Robert; Padmaperuma, Asanga; Gerber, Mark; Sahir, Asad; Tao, Ling; Zhang, Yanan

2017-03-03

This paper presents a comparative techno-economic analysis of five conversion pathways from biomass to gasoline-, jet-, and diesel-range hydrocarbons via indirect liquefaction with specific focus on pathways utilizing oxygenated intermediates (derived either via thermochemical or biochemical conversion steps). The four emerging pathways of interest are compared with one conventional pathway (Fischer-Tropsch) for the production of the hydrocarbon blendstocks. The processing steps of the four emerging pathways include: biomass-to-syngas via indirect gasification, gas cleanup, conversion of syngas to alcohols/oxygenates, followed by conversion of alcohols/oxygenates to hydrocarbon blendstocks via dehydration, oligomerization, and hydrogenation. We show that the emerging pathways via oxygenated intermediates have the potential to be cost competitive with the conventional Fischer-Tropsch process. The evaluated pathways and the benchmark process generally exhibit similar fuel yields and carbon conversion efficiencies. The resulting minimum fuel selling prices are comparable to the benchmark at approximately $3.60 per gallon-gasoline equivalent, with potential for two new pathways to be more economically competitive. Additionally, the coproduct values can play an important role in the economics of the processes with oxygenated intermediates derived via syngas fermentation. Major cost drivers for the integrated processes are tied to achievable fuel yields and conversion efficiency of the intermediate steps, i.e., the production of oxygenates/alcohols from syngas and the conversion of oxygenates/alcohols to hydrocarbon fuels.

Rare and semi-rare decays at ATLAS

CERN Document Server

AUTHOR|(INSPIRE)INSPIRE-00213194; The ATLAS collaboration

2016-01-01

The measurements of the rare $B^0$-mesons decays processes performed by the ATLAS experiment at LHC are reviewed. Particular attention will be given to the measurement of the branching ratio of the $B^0_s$ and $B^0_d$ mesons decays into a pair of muons with the full Run 1 dataset corresponding to an integrated luminosity of 25 $\\rm{fb^{-1}}$.

Epigenetic telomere protection by Drosophila DNA damage response pathways.

Science.gov (United States)

Oikemus, Sarah R; Queiroz-Machado, Joana; Lai, KuanJu; McGinnis, Nadine; Sunkel, Claudio; Brodsky, Michael H

2006-05-01

Analysis of terminal deletion chromosomes indicates that a sequence-independent mechanism regulates protection of Drosophila telomeres. Mutations in Drosophila DNA damage response genes such as atm/tefu, mre11, or rad50 disrupt telomere protection and localization of the telomere-associated proteins HP1 and HOAP, suggesting that recognition of chromosome ends contributes to telomere protection. However, the partial telomere protection phenotype of these mutations limits the ability to test if they act in the epigenetic telomere protection mechanism. We examined the roles of the Drosophila atm and atr-atrip DNA damage response pathways and the nbs homolog in DNA damage responses and telomere protection. As in other organisms, the atm and atr-atrip pathways act in parallel to promote telomere protection. Cells lacking both pathways exhibit severe defects in telomere protection and fail to localize the protection protein HOAP to telomeres. Drosophila nbs is required for both atm- and atr-dependent DNA damage responses and acts in these pathways during DNA repair. The telomere fusion phenotype of nbs is consistent with defects in each of these activities. Cells defective in both the atm and atr pathways were used to examine if DNA damage response pathways regulate telomere protection without affecting telomere specific sequences. In these cells, chromosome fusion sites retain telomere-specific sequences, demonstrating that loss of these sequences is not responsible for loss of protection. Furthermore, terminally deleted chromosomes also fuse in these cells, directly implicating DNA damage response pathways in the epigenetic protection of telomeres. We propose that recognition of chromosome ends and recruitment of HP1 and HOAP by DNA damage response proteins is essential for the epigenetic protection of Drosophila telomeres. Given the conserved roles of DNA damage response proteins in telomere function, related mechanisms may act at the telomeres of other organisms.

Rare earth mobility in hydrothermal ore-forming systems

International Nuclear Information System (INIS)

Cornell, D.H.; Schade, J.; Scheepers, R.; Watkeys, M.K.

1988-01-01

Rocks and ores which form by magmatic processes display a range of chondrite-normalised rare earth profiles. One REE (rare earth elements) profile feature which seems unrelated to magmatic processes is the birdwing profile, in which both heavy and light rare earths are enriched relative to the middle rare earths. Birdwing rare earth profiles are an easily identified geochemical anomaly. It is proposed that rare earth geochemistry could be applied in geochemical prospecting for ore formed by hydrothermal processes. 5 figs

Pooled Sequencing of 531 Genes in Inflammatory Bowel Disease Identifies an Associated Rare Variant in BTNL2 and Implicates Other Immune Related Genes

Science.gov (United States)

Prescott, Natalie J.; Lehne, Benjamin; Stone, Kristina; Lee, James C.; Taylor, Kirstin; Knight, Jo; Papouli, Efterpi; Mirza, Muddassar M.; Simpson, Michael A.; Spain, Sarah L.; Lu, Grace; Fraternali, Franca; Bumpstead, Suzannah J.; Gray, Emma; Amar, Ariella; Bye, Hannah; Green, Peter; Chung-Faye, Guy; Hayee, Bu’Hussain; Pollok, Richard; Satsangi, Jack; Parkes, Miles; Barrett, Jeffrey C.; Mansfield, John C.; Sanderson, Jeremy; Lewis, Cathryn M.; Weale, Michael E.; Schlitt, Thomas; Mathew, Christopher G.

2015-01-01

The contribution of rare coding sequence variants to genetic susceptibility in complex disorders is an important but unresolved question. Most studies thus far have investigated a limited number of genes from regions which contain common disease associated variants. Here we investigate this in inflammatory bowel disease by sequencing the exons and proximal promoters of 531 genes selected from both genome-wide association studies and pathway analysis in pooled DNA panels from 474 cases of Crohn’s disease and 480 controls. 80 variants with evidence of association in the sequencing experiment or with potential functional significance were selected for follow up genotyping in 6,507 IBD cases and 3,064 population controls. The top 5 disease associated variants were genotyped in an extension panel of 3,662 IBD cases and 3,639 controls, and tested for association in a combined analysis of 10,147 IBD cases and 7,008 controls. A rare coding variant p.G454C in the BTNL2 gene within the major histocompatibility complex was significantly associated with increased risk for IBD (p = 9.65x10−10, OR = 2.3[95% CI = 1.75–3.04]), but was independent of the known common associated CD and UC variants at this locus. Rare (T) or decreased risk (IL12B p.V298F, and NICN p.H191R) of IBD. These results provide additional insights into the involvement of the inhibition of T cell activation in the development of both sub-phenotypes of inflammatory bowel disease. We suggest that although rare coding variants may make a modest overall contribution to complex disease susceptibility, they can inform our understanding of the molecular pathways that contribute to pathogenesis. PMID:25671699

Photowalk Exhibition opens at Microcosm

CERN Document Server

Katarina Anthony

2011-01-01

The winning photographs from the 2010 Global Particle Physics Photowalk competition will go on display at Microcosm from 11 February to 2 April. The exhibition is part of a global photography event taking place over three continents, with Photowalk exhibitions opening simultaneously at Fermilab in the US, KEK in Japan and here at CERN.   DESY wire chamber - First place people's choice; second place global jury competition. Photographer: Hans-Peter Hildebrandt  If you were one of the 1,300 photography lovers who voted in last year’s Photowalk competition, this exhibition is your chance to see the winning entries in print. The exhibition will take place in the downstairs gallery of Microcosm, overlooking the garden. 15 photographs will be on display, with each of the laboratories that participated in Photowalk represented by their 3 winning entries. Among them will be the “people’s choice” sunburst photo of a particle detector at DESY (Photo 1), and...

Creating National Narrative: The Red Guard Art Exhibitions and the National Exhibitions in the Chinese Cultural Revolution 1966 - 1976

Directory of Open Access Journals (Sweden)

Winnie Tsang

2014-06-01

Full Text Available The artistic development in China experienced drastic changes during the Cultural Revolution from 1966 to 1976. Traditional Chinese art was denounced, whereas propaganda art became predominant in shaping the public’s loyalty towards the Communist Party and the country. Two major groups of art exhibitions emerged during the Revolution—the unofficial Red Guard art exhibitions organized by student activists in collaboration with local communes and art schools between 1966 and 1968, and the state-run national exhibitions from 1972 to 1975. These exhibitions were significant to this period because they were held frequently in the capital city Beijing and occasionally elsewhere, and through art they presented unique revolutionary beliefs to the Chinese people in a public setting. While the Red Guard art exhibitions and the national exhibitions certainly created different national narratives, I argue that the national exhibitions were in fact an attempt to revise the national narrative created by the Red Guard art exhibitions in order to re-establish a more utopian, consistent, and official national narrative. This paper unravels the intricate relationship between the two groups of exhibitions by comparing their exhibition venues, ideological focuses, work selection and quality editing. 

Microbial mineralization of ring-substituted anilines through an ortho-cleavage pathway.

Science.gov (United States)

Zeyer, J; Wasserfallen, A; Timmis, K N

1985-08-01

Moraxella sp. strain G is able to utilize as sole source of carbon and nitrogen aniline, 4-fluoroaniline, 2-chloroaniline, 3-chloroaniline, 4-chloroaniline (PCA), and 4-bromoaniline but not 4-iodoaniline, 4-methylaniline, 4-methoxyaniline, or 3,4-dichloroaniline. The generation time on PCA was 6 h. The pathway for the degradation of PCA was investigated by analysis of catabolic intermediates and enzyme activities. Mutants of strain G were isolated to enhance the accumulation of specific pathway intermediates. PCA was converted by an aniline oxygenase to 4-chlorocatechol, which in turn was degraded via a modified ortho-cleavage pathway. Synthesis of the aniline oxygenase was inducible by various anilines. This enzyme exhibited a broad substrate specificity. Its specific activity towards substituted anilines seemed to be correlated more with the size than with the electron-withdrawing effect of the substituent and was very low towards anilines having substituents larger than iodine or a methyl group. The initial enzyme of the modified ortho-cleavage pathway, catechol 1,2-dioxygenase, had similar characteristics to those of corresponding enzymes of pathways for the degradation of chlorobenzoic acid and chlorophenol, that is, a broad substrate specificity and high activity towards chlorinated and methylated catechols.

Community Assembly Processes of the Microbial Rare Biosphere.

Science.gov (United States)

Jia, Xiu; Dini-Andreote, Francisco; Falcão Salles, Joana

2018-03-14

Our planet teems with microorganisms that often present a skewed abundance distribution in a local community, with relatively few dominant species coexisting alongside a high number of rare species. Recent studies have demonstrated that these rare taxa serve as limitless reservoirs of genetic diversity, and perform disproportionate types of functions despite their low abundances. However, relatively little is known about the mechanisms controlling rarity and the processes promoting the development of the rare biosphere. Here, we propose the use of multivariate cut-offs to estimate rare species and phylogenetic null models applied to predefined rare taxa to disentangle the relative influences of ecoevolutionary processes mediating the assembly of the rare biosphere. Importantly, the identification of the factors controlling rare species assemblages is critical for understanding the types of rarity, how the rare biosphere is established, and how rare microorganisms fluctuate over spatiotemporal scales, thus enabling prospective predictions of ecosystem responses. Copyright © 2018 Elsevier Ltd. All rights reserved.

Improved Diagnosis and Care for Rare Diseases through Implementation of Precision Public Health Framework.

Science.gov (United States)

Baynam, Gareth; Bowman, Faye; Lister, Karla; Walker, Caroline E; Pachter, Nicholas; Goldblatt, Jack; Boycott, Kym M; Gahl, William A; Kosaki, Kenjiro; Adachi, Takeya; Ishii, Ken; Mahede, Trinity; McKenzie, Fiona; Townshend, Sharron; Slee, Jennie; Kiraly-Borri, Cathy; Vasudevan, Anand; Hawkins, Anne; Broley, Stephanie; Schofield, Lyn; Verhoef, Hedwig; Groza, Tudor; Zankl, Andreas; Robinson, Peter N; Haendel, Melissa; Brudno, Michael; Mattick, John S; Dinger, Marcel E; Roscioli, Tony; Cowley, Mark J; Olry, Annie; Hanauer, Marc; Alkuraya, Fowzan S; Taruscio, Domenica; Posada de la Paz, Manuel; Lochmüller, Hanns; Bushby, Kate; Thompson, Rachel; Hedley, Victoria; Lasko, Paul; Mina, Kym; Beilby, John; Tifft, Cynthia; Davis, Mark; Laing, Nigel G; Julkowska, Daria; Le Cam, Yann; Terry, Sharon F; Kaufmann, Petra; Eerola, Iiro; Norstedt, Irene; Rath, Ana; Suematsu, Makoto; Groft, Stephen C; Austin, Christopher P; Draghia-Akli, Ruxandra; Weeramanthri, Tarun S; Molster, Caron; Dawkins, Hugh J S

2017-01-01

Public health relies on technologies to produce and analyse data, as well as effectively develop and implement policies and practices. An example is the public health practice of epidemiology, which relies on computational technology to monitor the health status of populations, identify disadvantaged or at risk population groups and thereby inform health policy and priority setting. Critical to achieving health improvements for the underserved population of people living with rare diseases is early diagnosis and best care. In the rare diseases field, the vast majority of diseases are caused by destructive but previously difficult to identify protein-coding gene mutations. The reduction in cost of genetic testing and advances in the clinical use of genome sequencing, data science and imaging are converging to provide more precise understandings of the 'person-time-place' triad. That is: who is affected (people); when the disease is occurring (time); and where the disease is occurring (place). Consequently we are witnessing a paradigm shift in public health policy and practice towards 'precision public health'.Patient and stakeholder engagement has informed the need for a national public health policy framework for rare diseases. The engagement approach in different countries has produced highly comparable outcomes and objectives. Knowledge and experience sharing across the international rare diseases networks and partnerships has informed the development of the Western Australian Rare Diseases Strategic Framework 2015-2018 (RD Framework) and Australian government health briefings on the need for a National plan.The RD Framework is guiding the translation of genomic and other technologies into the Western Australian health system, leading to greater precision in diagnostic pathways and care, and is an example of how a precision public health framework can improve health outcomes for the rare diseases population.Five vignettes are used to illustrate how policy

Xeroderma pigmentosum complementation group F: A rare cause of cerebellar ataxia with chorea.

Science.gov (United States)

Carré, G; Marelli, C; Anheim, M; Geny, C; Renaud, M; Rezvani, H R; Koenig, M; Guissart, C; Tranchant, C

2017-05-15

The complementation group F of Xeroderma pigmentosum (XP-F) is rare in the Caucasian population, and usually devoid of neurological symptoms. We report two cases, both Caucasian, who exhibited progressive cerebellar ataxia, chorea, a mild subcortical frontal cognitive impairment, and in one case severe polyneuropathy. Brain MRI demonstrated cerebellar (2/2) and cortical (1/2) atrophy. Both patients had only mild sunburn sensitivity and no skin cancer. Mini-exome sequencing approach revealed in ERCC4, two heterozygous mutations, one of which was never described (c.580-584+1delCCAAGG, exon 3), in the first case, and an already reported homozygous mutation, in the second case. These cases emphasize that XP-F is a rare cause of recessive cerebellar ataxia and can in some cases clinically mimic Huntington's disease due to chorea and executive impairment. The association of ataxia, chorea, and sun hypersensitivity are major guidance for the diagnosis, which should not be missed, in order to prevent skin neoplastic complications. Copyright © 2017 Elsevier B.V. All rights reserved.

Poster "Maladies rares & sciences sociales"

OpenAIRE

Duysens, Fanny

2018-01-01

Le poster "Maladies rares & sciences sociales" visait à présenter la recherche doctorale de l'auteure au grand public dans le cadre de la Journée des Maladies Rares du Centre Hospitalier Universitaire de Liège dont le thème était "la recherche sur les maladies rares". Réalisé spécialement pour l'occasion, le poster explicitait de manière synthétique le sujet de recherche, la méthodologie, certains résultats, ainsi que les apports possibles des échanges entre chercheurs en sciences et sociales...

Designing immersion exhibits as border-crossing environments

DEFF Research Database (Denmark)

Mortensen, Marianne Foss

2010-01-01

be applied to achieve an understanding of the immersion exhibit form. The argument proceeds by demonstrating how the characteristics of immersion exhibits, and visitors to them, classify them as microcultures, and examining the implications of this for exhibit design using a hypothetical immersion exhibit...

Selective loss of bi-directional synaptic plasticity in the direct and indirect striatal output pathways accompanies generation of parkinsonism and l-DOPA induced dyskinesia in mouse models.

Science.gov (United States)

Thiele, Sherri L; Chen, Betty; Lo, Charlotte; Gertler, Tracey S; Warre, Ruth; Surmeier, James D; Brotchie, Jonathan M; Nash, Joanne E

2014-11-01

Parkinsonian symptoms arise due to over-activity of the indirect striatal output pathway, and under-activity of the direct striatal output pathway. l-DOPA-induced dyskinesia (LID) is caused when the opposite circuitry problems are established, with the indirect pathway becoming underactive, and the direct pathway becoming over-active. Here, we define synaptic plasticity abnormalities in these pathways associated with parkinsonism, symptomatic benefits of l-DOPA, and LID. We applied spike-timing dependent plasticity protocols to cortico-striatal synapses in slices from 6-OHDA-lesioned mouse models of parkinsonism and LID, generated in BAC transgenic mice with eGFP targeting the direct or indirect output pathways, with and without l-DOPA present. In naïve mice, bidirectional synaptic plasticity, i.e. LTP and LTD, was induced, resulting in an EPSP amplitude change of approximately 50% in each direction in both striatal output pathways, as shown previously. In parkinsonism and dyskinesia, both pathways exhibited unidirectional plasticity, irrespective of stimulation paradigm. In parkinsonian animals, the indirect pathway only exhibited LTP (LTP protocol: 143.5±14.6%; LTD protocol 177.7±22.3% of baseline), whereas the direct pathway only showed LTD (LTP protocol: 74.3±4.0% and LTD protocol: 63.3±8.7%). A symptomatic dose of l-DOPA restored bidirectional plasticity on both pathways to levels comparable to naïve animals (Indirect pathway: LTP protocol: 124.4±22.0% and LTD protocol: 52.1±18.5% of baseline. Direct pathway: LTP protocol: 140.7±7.3% and LTD protocol: 58.4±6.0% of baseline). In dyskinesia, in the presence of l-DOPA, the indirect pathway exhibited only LTD (LTP protocol: 68.9±21.3% and LTD protocol 52.0±14.2% of baseline), whereas in the direct pathway, only LTP could be induced (LTP protocol: 156.6±13.2% and LTD protocol 166.7±15.8% of baseline). We conclude that normal motor control requires bidirectional plasticity of both striatal outputs

Rare earth elements behavior in Peruibe black mud

International Nuclear Information System (INIS)

Torrecilha, Jefferson K.; Carvalho, Leandro P.; Gouvea, Paulo F.M.; Silva, Paulo S.C. da

2015-01-01

Rare earth elements in sediments have been used as powerful tools for environmental studies because of their behavior during geochemical processes and are also widely accepted as reliable provenance tracers because they are largely water-immobile and thus behave conservatively during sedimentary processes. The Peruibe Black Mud (PBM) is a sedimentary deposit originated from the interactions of marine sediments and organic matter in an estuarine environment that originates a peloid currently used for medicinal purposes. The objective of this study was to examine rare earth elements pattern distribution in the Peruibe black mud sedimentary deposit as a proxy for its geochemical development. Elemental ratios such as LaN/YbN, Th/U and La/Th were determined and a normalization of the mean rare earth elements concentrations in the samples related to NASC indicates that the light (La to Eu) rare earth elements present values close to the unity while the heavy (Tb to Lu) rare earth elements are depleted related to NASC. It can be observed that the light rare earth elements present enrichment values slightly enriched over the unity while the heavy rare earth elements present values generally below the unity reflecting the enrichment of the light rare earth elements over the heavy rare earth. Rare earth elements concentrations determined in Peruibe black mud samples showed a distribution similar to that found in the NASC for the light rare earth elements and depleted for the heavy rare earth elements. (author)

Rare earth elements behavior in Peruibe black mud

Energy Technology Data Exchange (ETDEWEB)

Torrecilha, Jefferson K.; Carvalho, Leandro P.; Gouvea, Paulo F.M.; Silva, Paulo S.C. da, E-mail: jeffkoy@usp.br [Instituto de Pesquisas Energeticas e Nucleares (IPEN/CNEN-SP), Sao Paulo, SP (Brazil)

2015-07-01

Rare earth elements in sediments have been used as powerful tools for environmental studies because of their behavior during geochemical processes and are also widely accepted as reliable provenance tracers because they are largely water-immobile and thus behave conservatively during sedimentary processes. The Peruibe Black Mud (PBM) is a sedimentary deposit originated from the interactions of marine sediments and organic matter in an estuarine environment that originates a peloid currently used for medicinal purposes. The objective of this study was to examine rare earth elements pattern distribution in the Peruibe black mud sedimentary deposit as a proxy for its geochemical development. Elemental ratios such as LaN/YbN, Th/U and La/Th were determined and a normalization of the mean rare earth elements concentrations in the samples related to NASC indicates that the light (La to Eu) rare earth elements present values close to the unity while the heavy (Tb to Lu) rare earth elements are depleted related to NASC. It can be observed that the light rare earth elements present enrichment values slightly enriched over the unity while the heavy rare earth elements present values generally below the unity reflecting the enrichment of the light rare earth elements over the heavy rare earth. Rare earth elements concentrations determined in Peruibe black mud samples showed a distribution similar to that found in the NASC for the light rare earth elements and depleted for the heavy rare earth elements. (author)

Structural elucidation and magnetic behavior evaluation of rare earth (La, Nd, Gd, Tb, Dy) doped BaCoNi-X hexagonal nano-sized ferrites

International Nuclear Information System (INIS)

Majeed, Abdul; Khan, Muhammad Azhar; Raheem, Faseeh ur; Hussain, Altaf; Iqbal, F.; Murtaza, Ghulam; Akhtar, Majid Niaz; Shakir, Imran; Warsi, Muhammad Farooq

2016-01-01

Rare-earth (RE=La 3+ , Nd 3+ , Gd 3+ , Tb 3+ , Dy 3+ ) doped Ba 2 NiCoRE x Fe 28−x O 46 (x=0.25) hexagonal ferrites were synthesized for the first time via micro-emulsion route, which is a fast chemistry route for obtaining nano-sized ferrite powders. These nanomaterials were investigated by X-ray diffraction (XRD), Fourier transform infrared spectroscopy (FTIR), as well as vibrating sample magnetometer (VSM). The XRD analysis exhibited that all the samples crystallized into single X-type hexagonal phase. The crystalline size calculated by Scherrer's formula was found in the range 7–19 nm. The variations in lattice parameters elucidated the incorporation of rare-earth cations in these nanomaterials. FTIR absorption spectra of these X-type ferrites were investigated in the wave number range 500–2400 cm −1. Each spectrum exhibited absorption bands in the low wave number range, thereby confirming the X-type hexagonal structure. The enhancement in the coercivity was observed with the doping of rare-earth cations. The saturation magnetization was lowered owing to the redistribution of rare-earth cations on the octahedral site (3b VI ). The higher values of coercivity (664–926 Oe) of these nanomaterials suggest their use in longitudinal recording media. - Graphical abstract: Nano-sized rare-earth (RE=La 3+ , Nd 3+ , Gd 3+ , Tb 3+ , Dy 3+ ) doped Ba 2 NiCoRE x Fe 28−x O 46 (x=0.25) hexagonal ferrites were synthesized for the first time via micro-emulsion route and the crystallite size was found in the range 7–19 nm. The enhancement in the coercivity was observed with the doping of rare-earth cations. The higher values of coercivity (664–926 Oe) of these nanomaterials suggest their use in longitudinal recording media. - Highlights: • Micro-emulsion route was used to synthesize Ba 2 NiCoRE x Fe 28−x O 46 ferrites. • The crystallite size was found in the range 7–19 nm. • The rare-earth incorporation enhanced the coercivity (664–926 Oe). �

Benchmarking pathway interaction network for colorectal cancer to identify dysregulated pathways

Directory of Open Access Journals (Sweden)

Q. Wang

Full Text Available Different pathways act synergistically to participate in many biological processes. Thus, the purpose of our study was to extract dysregulated pathways to investigate the pathogenesis of colorectal cancer (CRC based on the functional dependency among pathways. Protein-protein interaction (PPI information and pathway data were retrieved from STRING and Reactome databases, respectively. After genes were aligned to the pathways, each pathway activity was calculated using the principal component analysis (PCA method, and the seed pathway was discovered. Subsequently, we constructed the pathway interaction network (PIN, where each node represented a biological pathway based on gene expression profile, PPI data, as well as pathways. Dysregulated pathways were then selected from the PIN according to classification performance and seed pathway. A PIN including 11,960 interactions was constructed to identify dysregulated pathways. Interestingly, the interaction of mRNA splicing and mRNA splicing-major pathway had the highest score of 719.8167. Maximum change of the activity score between CRC and normal samples appeared in the pathway of DNA replication, which was selected as the seed pathway. Starting with this seed pathway, a pathway set containing 30 dysregulated pathways was obtained with an area under the curve score of 0.8598. The pathway of mRNA splicing, mRNA splicing-major pathway, and RNA polymerase I had the maximum genes of 107. Moreover, we found that these 30 pathways had crosstalks with each other. The results suggest that these dysregulated pathways might be used as biomarkers to diagnose CRC.

Specificity in Sociality: Mice and Prairie Voles Exhibit Different Patterns of Peer Affiliation

Science.gov (United States)

Beery, Annaliese K.; Christensen, Jennifer D; Lee, Nicole S.; Blandino, Katrina L.

2018-01-01

Social behavior is often described as a unified concept, but highly social (group-living) species exhibit distinct social structures and may make different social decisions. Prairie voles (Microtus ochrogaster) are socially monogamous rodents that often reside in extended family groups, and exhibit robust preferences for familiar social partners (same- and opposite-sex) during extended choice tests, although short-term preferences are not known. Mice (Mus musculus) are gregarious and colonial, but in brief laboratory tests of social preference they typically prefer social novelty. This preference for novel vs. familiar peers may represent a species-specific difference in social decision-making between mice and prairie voles. However, the tests used to measure preferences in each species differ markedly in duration and degree of contact, such that the behaviors cannot be directly compared. We assessed whether social preferences for novelty or familiarity differed between mice and prairie voles of both sexes when assessed with matching protocols: the sociability/social preference test (SPT) typically used in mice (short, no direct contact), and the partner preference test (PPT) used in voles (long, direct contact). A subset of voles also underwent a PPT using barriers (long, no direct contact). In the short SPT, behavior did not differ between species. In the longer test, pronounced partner preferences emerged in prairie voles, but mice exhibited no social preferences and rarely huddled. No sex differences were evident in either test. Direct physical contact was required for partner preferences in huddling time in voles, but preference for the partner chamber was evident with or without contact. Both prairie voles and mice are social, but they exhibit important differences in the specificity and extent of their social behavior. While mice are often used to study social approach and other behaviors, voles are a more suitable species for the study of selective social

Specificity in Sociality: Mice and Prairie Voles Exhibit Different Patterns of Peer Affiliation

Directory of Open Access Journals (Sweden)

Annaliese K. Beery

2018-03-01

Full Text Available Social behavior is often described as a unified concept, but highly social (group-living species exhibit distinct social structures and may make different social decisions. Prairie voles (Microtus ochrogaster are socially monogamous rodents that often reside in extended family groups, and exhibit robust preferences for familiar social partners (same- and opposite-sex during extended choice tests, although short-term preferences are not known. Mice (Mus musculus are gregarious and colonial, but in brief laboratory tests of social preference they typically prefer social novelty. This preference for novel vs. familiar peers may represent a species-specific difference in social decision-making between mice and prairie voles. However, the tests used to measure preferences in each species differ markedly in duration and degree of contact, such that the behaviors cannot be directly compared. We assessed whether social preferences for novelty or familiarity differed between mice and prairie voles of both sexes when assessed with matching protocols: the sociability/social preference test (SPT typically used in mice (short, no direct contact, and the partner preference test (PPT used in voles (long, direct contact. A subset of voles also underwent a PPT using barriers (long, no direct contact. In the short SPT, behavior did not differ between species. In the longer test, pronounced partner preferences emerged in prairie voles, but mice exhibited no social preferences and rarely huddled. No sex differences were evident in either test. Direct physical contact was required for partner preferences in huddling time in voles, but preference for the partner chamber was evident with or without contact. Both prairie voles and mice are social, but they exhibit important differences in the specificity and extent of their social behavior. While mice are often used to study social approach and other behaviors, voles are a more suitable species for the study of

SREBP-1c overactivates ROS-mediated hepatic NF-κB inflammatory pathway in dairy cows with fatty liver.

Science.gov (United States)

Li, Xinwei; Huang, Weikun; Gu, Jingmin; Du, Xiliang; Lei, Lin; Yuan, Xue; Sun, Guoquan; Wang, Zhe; Li, Xiaobing; Liu, Guowen

2015-10-01

Dairy cows with fatty liver are characterized by hepatic lipid accumulation and a severe inflammatory response. Sterol receptor element binding protein-1c (SREBP-1c) and nuclear factor κB (NF-κB) are components of the main pathways for controlling triglyceride (TG) accumulation and inflammatory levels, respectively. A previous study demonstrated that hepatic inflammatory levels are positively correlated with hepatic TG content. We therefore speculated that SREBP-1c might play an important role in the overactivation of the hepatic NF-κB inflammatory pathway in cows with fatty liver. Compared with healthy cows, cows with fatty liver exhibited severe hepatic injury and high blood concentrations of the inflammatory cytokines TNF-α, IL-6 and IL-1β. Hepatic SREBP-1c-mediated lipid synthesis and the NF-κB inflammatory pathway were both overinduced in cows with fatty liver. In vitro, treatment with non-esterified fatty acids (NEFA) further increased SREBP-1c expression and NF-κB pathway activation, which then promoted TG and inflammatory cytokine synthesis. SREBP-1c overexpression overactivated the NF-κB inflammatory pathway in hepatocytes by increasing ROS content and not through TLR4. Furthermore, SREBP-1c silencing decreased ROS content and further attenuated the activation of the NEFA-induced NF-κB pathway, thereby decreasing TNF-α, IL-6 and IL-1β synthesis. SREBP-1c-overexpressing mice exhibited hepatic steatosis and an overinduced hepatic NF-κB pathway. Taken together, these results indicate that SREBP-1c enhances the NEFA-induced overactivation of the NF-κB inflammatory pathway by increasing ROS in cow hepatocytes, thereby further increasing hepatic inflammatory injury in cows with fatty liver. Copyright © 2015. Published by Elsevier Inc.

Rare Earth Metals: Resourcefulness and Recovery

Science.gov (United States)

Wang, Shijie

2013-10-01

When we appreciate the digital revolution carried over from the twentieth century with mobile communication and the Internet, and when we enjoy our high-tech lifestyle filled with iDevices, hybrid cars, wind turbines, and solar cells in this new century, we should also appreciate that all of these advanced products depend on rare earth metals to function. Although there are only 136,000 tons of annual worldwide demand, (Cho, Rare Earth Metals, Will We Have Enough?)1 rare earth metals are becoming such hot commodities on international markets, due to not only to their increasing uses, including in most critical military hardware, but also to Chinese growth, which accounts for 95% of global rare earth metal production. Hence, the 2013 technical calendar topic, planned by the TMS/Hydrometallurgy and Electrometallurgy Committee, is particularly relevant, with four articles (including this commentary) contributed to the JOM October Issue discussing rare earth metals' resourcefulness and recovery.

Rare and semi-rare decays at ATLAS

CERN Document Server

AUTHOR|(INSPIRE)INSPIRE-00213194; The ATLAS collaboration

2016-01-01

The measurements of the rare $B^0$-meson-decay processes performed by the ATLAS experiment at the LHC are reviewed. Particular attention will be given to the measurement of the branching ratio of the $B^0_s$ and $B^0_d$ mesons decays into a pair of muons with the full Run 1 dataset corresponding to an integrated luminosity of 25 $\\rm{fb^{-1}}$.

Mohs micrographic surgery of rare cutaneous tumours

NARCIS (Netherlands)

Flohil, S.C.; Lee, C.B. van; Beisenherz, J.; Mureau, M.A.M.; Overbeek, L.I.H.; Nijsten, T.; Bos, R.R.

2017-01-01

BACKGROUND: Recurrence rates after Mohs micrographic surgery (MMS) for rare cutaneous tumours are poorly defined. OBJECTIVE: To investigate the recurrence rate after MMS for rare cutaneous tumours at a university centre. METHODS & MATERIALS: Retrospective review of all rare cutaneous tumours treated

A genomic pathway approach to a complex disease: axon guidance and Parkinson disease.

Directory of Open Access Journals (Sweden)

Timothy G Lesnick

2007-06-01

Full Text Available While major inroads have been made in identifying the genetic causes of rare Mendelian disorders, little progress has been made in the discovery of common gene variations that predispose to complex diseases. The single gene variants that have been shown to associate reproducibly with complex diseases typically have small effect sizes or attributable risks. However, the joint actions of common gene variants within pathways may play a major role in predisposing to complex diseases (the paradigm of complex genetics. The goal of this study was to determine whether polymorphism in a candidate pathway (axon guidance predisposed to a complex disease (Parkinson disease [PD]. We mined a whole-genome association dataset and identified single nucleotide polymorphisms (SNPs that were within axon-guidance pathway genes. We then constructed models of axon-guidance pathway SNPs that predicted three outcomes: PD susceptibility (odds ratio = 90.8, p = 4.64 x 10(-38, survival free of PD (hazards ratio = 19.0, p = 5.43 x 10(-48, and PD age at onset (R(2 = 0.68, p = 1.68 x 10(-51. By contrast, models constructed from thousands of random selections of genomic SNPs predicted the three PD outcomes poorly. Mining of a second whole-genome association dataset and mining of an expression profiling dataset also supported a role for many axon-guidance pathway genes in PD. These findings could have important implications regarding the pathogenesis of PD. This genomic pathway approach may also offer insights into other complex diseases such as Alzheimer disease, diabetes mellitus, nicotine and alcohol dependence, and several cancers.

Exhibition

CERN Multimedia

Staff Association

2017-01-01

Sintropie Flavio Pellegrini From 13 to 24 March 2017 CERN Meyrin, Main Building Energia imprigionata - Flavio Pellegrini. The exhibition is composed by eleven wood artworks with the expression of movement as theme. The artworks are the result of harmonics math applied to sculpture. The powerful black colour is dominated by the light source, generating reflexes and modulations. The result is a continuous variation of perspective visions. The works generate, at a first approach, an emotion of mystery and incomprehension, only a deeper contemplation lets one discover entangling and mutative details, evidencing the elegance of the lines and letting the meaning emerge. For more information : staff.association@cern.ch | Tél: 022 766 37 38

Novel histone deacetylase inhibitor AR-42 exhibits antitumor activity in pancreatic cancer cells by affecting multiple biochemical pathways.

Directory of Open Access Journals (Sweden)

Yi-Jin Chen

Full Text Available Pancreatic cancer is one of the most lethal types of cancer with a 5-year survival rate of ~5%. Histone deacetylases (HDACs participate in many cellular processes, including carcinogenesis, and pharmacological inhibition of HDACs has emerged as a potential therapeutic strategy. In this study, we explored antitumor activity of the novel HDAC inhibitor AR-42 in pancreatic cancer.Human pancreatic cancer cell lines BxPC-3 and PANC-1 were used in this study. Real-time PCR, RT-PCR, and western blotting were employed to investigate expression of specific genes and proteins, respectively. Translocation of apoptosis-inducing factor was investigated by immunofluorescence and subcellular fractionation. The number of apoptotic cells, cell cycle stages, and reactive oxygen species (ROS generation levels were determined by flow cytometry. Cell invasiveness was examined by the Matrigel invasion assay. Efficacy of AR-42 in vivo was evaluated by utilizing BxPC-3 xenograft mouse model.AR-42 inhibited pancreatic cancer cell proliferation by causing G2/M cell cycle arrest via regulating expression levels of genes and proteins involved in cell cycle. AR-42 also induced ROS generation and DNA damage, triggering apoptosis of pancreatic cancer cells via both caspase-3-dependent and caspase-3-independent pathways. In addition, AR-42 increased expression levels of negative regulators of p53 (miR-125b, miR-30d, and miR33, which could contribute to lower expression level of mutant p53 in pancreatic cancer cells. Cell invasion assay showed that AR-42 reduced cancer cell aggressiveness and significantly diminished BxPC-3 xenograft tumor growth in vivo.AR-42, a novel HDAC inhibitor, inhibited pancreatic cancer cells by regulating p53 expression, inducing cell cycle arrest, particularly at the G2/M stage, and activating multiple apoptosis pathways. Additionally, AR-42 inhibited cell invasiveness and potently suppressed pancreatic cancer tumors in vivo. We conclude that by

The Culture of Exhibitions and Conservation

Directory of Open Access Journals (Sweden)

Dimitrios Doumas

2013-11-01

Full Text Available The article reflects on temporary exhibitions from a theoretical as well as practical perspective. Regarded as a particularly effective mass-communication medium, exhibitions have a dual nature: they are scholarly undertakings, bringing off a curator’s vision and, simultaneously, they are projects with economic implications that need to be well managed and administered. The role of conservation in the making of temporary exhibitions, either in-house or touring, is here discussed in relation to how work is planned and prioritized as well as how time is managed and staff is allocated. Reference to weaknesses that lessen the crucial input of conservation in the decision-making process is also made. Much of the debate, which focuses on art exhibitions, concerns practicalities encountered in a private museum that extend from the very early stages of selecting objects for display to the mounting of an exhibition.

Enzymatic approaches to rare sugar production.

Science.gov (United States)

Zhang, Wenli; Zhang, Tao; Jiang, Bo; Mu, Wanmeng

Rare sugars have recently attracted much attention because of their potential applications in the food, nutraceutical, and pharmaceutical industries. A systematic strategy for enzymatic production of rare sugars, named Izumoring, was developed >10years ago. The strategy consists of aldose-ketose isomerization, ketose C-3 epimerization, and monosaccharide oxidation-reduction. Recent development of the Izumoring strategy is reviewed herein, especially the genetic approaches to the improvement of rare sugar-producing enzymes and the applications of target-oriented bioconversion. In addition, novel non-Izumoring enzymatic approaches are also summarized, including enzymatic condensation, phosphorylation-dephosphorylation cascade reaction, aldose epimerization, ulosonic acid decarboxylation, and biosynthesis of rare disaccharides. Copyright © 2017 Elsevier Inc. All rights reserved.

Rare Earth Polyoxometalates.

Science.gov (United States)

Boskovic, Colette

2017-09-19

Longstanding and important applications make use of the chemical and physical properties of both rare earth metals and polyoxometalates of early transition metals. The catalytic, optical, and magnetic features of rare earth metal ions are well-known, as are the reversible multielectron redox and photoredox capabilities of polyoxomolybdates and polyoxotungstates. The combination of rare earth ions and polyoxometalates in discrete molecules and coordination polymers is of interest for the unique combination of chemical and physical properties that can arise. This Account surveys our efforts to synthesize and investigate compounds with rare earth ions and polyoxometalates (RE-POMs), sometimes with carboxylate-based organic coligands. Our general synthetic approach is "bottom-up", which affords well-defined nanoscale molecules, typically in crystalline form and amenable to single-crystal X-ray diffraction for structure determination. Our particular focus is on elucidation of the physical properties conferred by the different structural components with a view to ultimately being able to tune these properties chemically. For this purpose, we employ a variety of spectroscopic, magnetochemical, electrochemical, and scattering techniques in concert with theoretical modeling and computation. Studies of RE-POM single-molecule magnets (SMMs) have utilized magnetic susceptibility, inelastic neutron scattering, and ab initio calculations. These investigations have allowed characterization of the crystal field splitting of the rare earth(III) ions that is responsible for the SMM properties of slow magnetic relaxation and magnetization quantum tunneling. Such SMMs are promising for applications in quantum computing and molecular spintronics. Photophysical measurements of a family of hybrid RE-POMs with organic ligands have afforded insights into sensitization of Tb(III) and Eu(III) emission through both organic and polyoxometalate chromophores in the same molecule. Detailed

Giant virus Megavirus chilensis encodes the biosynthetic pathway for uncommon acetamido sugars.

Science.gov (United States)

Piacente, Francesco; De Castro, Cristina; Jeudy, Sandra; Molinaro, Antonio; Salis, Annalisa; Damonte, Gianluca; Bernardi, Cinzia; Abergel, Chantal; Tonetti, Michela G

2014-08-29

Giant viruses mimicking microbes, by the sizes of their particles and the heavily glycosylated fibrils surrounding their capsids, infect Acanthamoeba sp., which are ubiquitous unicellular eukaryotes. The glycans on fibrils are produced by virally encoded enzymes, organized in gene clusters. Like Mimivirus, Megavirus glycans are mainly composed of virally synthesized N-acetylglucosamine (GlcNAc). They also contain N-acetylrhamnosamine (RhaNAc), a rare sugar; the enzymes involved in its synthesis are encoded by a gene cluster specific to Megavirus close relatives. We combined activity assays on two enzymes of the pathway with mass spectrometry and NMR studies to characterize their specificities. Mg534 is a 4,6-dehydratase 5-epimerase; its three-dimensional structure suggests that it belongs to a third subfamily of inverting dehydratases. Mg535, next in the pathway, is a bifunctional 3-epimerase 4-reductase. The sequential activity of the two enzymes leads to the formation of UDP-l-RhaNAc. This study is another example of giant viruses performing their glycan synthesis using enzymes different from their cellular counterparts, raising again the question of the origin of these pathways. © 2014 by The American Society for Biochemistry and Molecular Biology, Inc.

Construction of a controllable β-carotene biosynthetic pathway by decentralized assembly strategy in Saccharomyces cerevisiae.

Science.gov (United States)

Xie, Wenping; Liu, Min; Lv, Xiaomei; Lu, Wenqiang; Gu, Jiali; Yu, Hongwei

2014-01-01

Saccharomyces cerevisiae is an important platform organism for the synthesis of a great number of natural products. However, the assembly of controllable and genetically stable heterogeneous biosynthetic pathways in S. cerevisiae still remains a significant challenge. Here, we present a strategy for reconstructing controllable multi-gene pathways by employing the GAL regulatory system. A set of marker recyclable integrative plasmids (pMRI) was designed for decentralized assembly of pathways. As proof-of-principle, a controllable β-carotene biosynthesis pathway (∼16 kb) was reconstructed and optimized by repeatedly using GAL10-GAL1 bidirectional promoters with high efficiency (80-100%). By controling the switch time of the pathway, production of 11 mg/g DCW of total carotenoids (72.57 mg/L) and 7.41 mg/g DCW of β-carotene was achieved in shake-flask culture. In addition, the engineered yeast strain exhibited high genetic stability after 20 generations of subculture. The results demonstrated a controllable and genetically stable biosynthetic pathway capable of increasing the yield of target products. Furthermore, the strategy presented in this study could be extended to construct other pathways in S. cerevisisae. © 2013 Wiley Periodicals, Inc.

Thermochemistry of rare-earth trifluorides

International Nuclear Information System (INIS)

Kim, K.Y.; Johnson, C.E.

1981-01-01

Using the most recent crystallographic data, the Born-Lande equation was employed to calculate lattice energies of the rare-earth trifluorides. The excellent agreement ( 0 sub(f)(MX 3 ,c,298.15K) can be estimated. The magnitude of the monotonic change of ΔH 0 sub(f)(MX 3 ) for the rare-earth trihalides series (14 4f electrons) is comparable to the energy change between Sc and Ti in which only one 3d electron is added. This energy change is consistent with the chemical evidence that the electrons in the f-orbitals of rare earths contribute negligibly to the bonding. (author)

Expert services for rare anaemias across Europe

Directory of Open Access Journals (Sweden)

Beatrice Gulbis

2013-03-01

Full Text Available New challenges and priorities are given in the EU Health programme 2007-2013. The objectives of the programme are to improve citizens’ health security, to promote health to improve prosperity and solidarity, and to generate and disseminate health knowledge. If challenges and priorities have been defined globally for rare diseases by the European Commission, persons involved in rare anaemias have taken the opportunity to contribute to the empowerment of patients with rare anaemias. One of the ENERCA partners objectives was the mapping of existing centres that take care of patients with rare anaemias in Europe. Another goal was to obtain a directory of facilities available per centre for patients with rare anaemias. We thought that with those results it could realistically help to define a consensus regarding the criteria to be recognised as a centre of expertise for haemoglobinopathies and very rare anaemias.

Waveband specific transcriptional control of select genetic pathways in vertebrate skin (Xiphophorus maculatus).

Science.gov (United States)

Walter, Ronald B; Boswell, Mikki; Chang, Jordan; Boswell, William T; Lu, Yuan; Navarro, Kaela; Walter, Sean M; Walter, Dylan J; Salinas, Raquel; Savage, Markita

2018-05-10

Evolution occurred exclusively under the full spectrum of sunlight. Conscription of narrow regions of the solar spectrum by specific photoreceptors suggests a common strategy for regulation of genetic pathways. Fluorescent light (FL) does not possess the complexity of the solar spectrum and has only been in service for about 60 years. If vertebrates evolved specific genetic responses regulated by light wavelengths representing the entire solar spectrum, there may be genetic consequences to reducing the spectral complexity of light. We utilized RNA-Seq to assess changes in the transcriptional profiles of Xiphophorus maculatus skin after exposure to FL ("cool white"), or narrow wavelength regions of light between 350 and 600 nm (i.e., 50 nm or 10 nm regions, herein termed "wavebands"). Exposure to each 50 nm waveband identified sets of genes representing discrete pathways that showed waveband specific transcriptional modulation. For example, 350-400 or 450-500 nm waveband exposures resulted in opposite regulation of gene sets marking necrosis and apoptosis (i.e., 350-400 nm; necrosis suppression, apoptosis activation, while 450-500 nm; apoptosis suppression, necrosis activation). Further investigation of specific transcriptional modulation employing successive 10 nm waveband exposures between 500 and 550 nm showed; (a) greater numbers of genes may be transcriptionally modulated after 10 nm exposures, than observed for 50 nm or FL exposures, (b) the 10 nm wavebands induced gene sets showing greater functional specificity than 50 nm or FL exposures, and (c) the genetic effects of FL are primarily due to 30 nm between 500 and 530 nm. Interestingly, many genetic pathways exhibited completely opposite transcriptional effects after different waveband exposures. For example, the epidermal growth factor (EGF) pathway exhibits transcriptional suppression after FL exposure, becomes highly active after 450-500 nm waveband exposure, and again, exhibits strong

Zebra: searching for rare diseases

DEFF Research Database (Denmark)

Dragusin, Radu; Petcu, Paula; Lioma, Christina

2012-01-01

disease diagnostic hypotheses in the domain of medical IR. In this work, we build upon an existing vertical medical search engine, Zebra, that is focused on rare disease diagnosis. In previous work, Zebra has been evaluated using real-life medical cases of rare and difficult diseases, and has been found...

Pathway cross-talk network analysis identifies critical pathways in neonatal sepsis.

Science.gov (United States)

Meng, Yu-Xiu; Liu, Quan-Hong; Chen, Deng-Hong; Meng, Ying

2017-06-01

Despite advances in neonatal care, sepsis remains a major cause of morbidity and mortality in neonates worldwide. Pathway cross-talk analysis might contribute to the inference of the driving forces in bacterial sepsis and facilitate a better understanding of underlying pathogenesis of neonatal sepsis. This study aimed to explore the critical pathways associated with the progression of neonatal sepsis by the pathway cross-talk analysis. By integrating neonatal transcriptome data with known pathway data and protein-protein interaction data, we systematically uncovered the disease pathway cross-talks and constructed a disease pathway cross-talk network for neonatal sepsis. Then, attract method was employed to explore the dysregulated pathways associated with neonatal sepsis. To determine the critical pathways in neonatal sepsis, rank product (RP) algorithm, centrality analysis and impact factor (IF) were introduced sequentially, which synthetically considered the differential expression of genes and pathways, pathways cross-talks and pathway parameters in the network. The dysregulated pathways with the highest IF values as well as RPpathways in neonatal sepsis. By integrating three kinds of data, only 6919 common genes were included to perform the pathway cross-talk analysis. By statistic analysis, a total of 1249 significant pathway cross-talks were selected to construct the pathway cross-talk network. Moreover, 47 dys-regulated pathways were identified via attract method, 20 pathways were identified under RPpathways with the highest IF were also screened from the pathway cross-talk network. Among them, we selected 8 common pathways, i.e. critical pathways. In this study, we systematically tracked 8 critical pathways involved in neonatal sepsis by integrating attract method and pathway cross-talk network. These pathways might be responsible for the host response in infection, and of great value for advancing diagnosis and therapy of neonatal sepsis. Copyright © 2017

Online Exhibits & Concept Maps

Science.gov (United States)

Douma, M.

2009-12-01

Presenting the complexity of geosciences to the public via the Internet poses a number of challenges. For example, utilizing various - and sometimes redundant - Web 2.0 tools can quickly devour limited time. Do you tweet? Do you write press releases? Do you create an exhibit or concept map? The presentation will provide participants with a context for utilizing Web 2.0 tools by briefly highlighting methods of online scientific communication across several dimensions. It will address issues of: * breadth and depth (e.g. from narrow topics to well-rounded views), * presentation methods (e.g. from text to multimedia, from momentary to enduring), * sources and audiences (e.g. for experts or for the public, content developed by producers to that developed by users), * content display (e.g. from linear to non-linear, from instructive to entertaining), * barriers to entry (e.g. from an incumbent advantage to neophyte accessible, from amateur to professional), * cost and reach (e.g. from cheap to expensive), and * impact (e.g. the amount learned, from anonymity to brand awareness). Against this backdrop, the presentation will provide an overview of two methods of online information dissemination, exhibits and concept maps, using the WebExhibits online museum (www.webexhibits.org) and SpicyNodes information visualization tool (www.spicynodes.org) as examples, with tips on how geoscientists can use either to communicate their science. Richly interactive online exhibits can serve to engage a large audience, appeal to visitors with multiple learning styles, prompt exploration and discovery, and present a topic’s breadth and depth. WebExhibits, which was among the first online museums, delivers interactive information, virtual experiments, and hands-on activities to the public. While large, multidisciplinary exhibits on topics like “Color Vision and Art” or “Calendars Through the Ages” require teams of scholars, user interface experts, professional writers and editors

The formation of crystals in glasses containing rare earth oxides

Energy Technology Data Exchange (ETDEWEB)

Fadzil, Syazwani Mohd [Pohang University of Science and Technology (POSTECH), Pohang (Korea, Republic of); Hrma, Pavel [Pohang University of Science and Technology (POSTECH), Pohang, South Korea and Pacific Northwest National Laboratory, Richland, Washington (United States); Crum, Jarrod [Pacific Northwest National Laboratory, Richland, Washington (United States); Siong, Khoo Kok; Ngatiman, Mohammad Fadzlee; Said, Riduan Mt [National University of Malaysia, Bandar Baru Bangi, Selangor (Malaysia)

2014-02-12

Korean spent nuclear fuel will reach the capacity of the available temporary storage by 2016. Pyroprocessing and direct disposal seems to be an alternative way to manage and reuse spent nuclear fuel while avoiding the wet reprocessing technology. Pyroprocessing produces several wastes streams, including metals, salts, and rare earths, which must be converted into stabilized form. A suitable form for rare earth immobilization is borosilicate glass. The borosilicate glass form exhibits excellent durability, allows a high waste loading, and is easy to process. In this work, we combined the rare earths waste of composition (in wt%) 39.2Nd{sub 2}O{sub 3}–22.7CeO{sub 2}–11.7La{sub 2}O{sub 3}–10.9PrO{sub 2}–1.3Eu{sub 2}O{sub 3}–1.3Gd{sub 2}O{sub 3}–8.1Sm{sub 2}O{sub 3}–4.8Y{sub 2}O{sub 3} with a baseline glass of composition 60.2SiO{sub 2}–16.0B{sub 2}O{sub 3}–12.6Na{sub 2}O–3.8Al{sub 2}O{sub 3}–5.7CaO–1.7ZrO{sub 2}. Crystallization in waste glasses occurs as the waste loading increases. It may produce complicate glass processing and affect the product quality. To study crystal formation, we initially made glasses containing 5%, 10% and 15% of La{sub 2}O{sub 3} and then glasses with 5%, 10% and 15% of the complete rare earth mix. Samples were heat-treated for 24 hours at temperatures 800°C to 1150°C in 50°C increments. Quenched samples were analyzed using an optical microscope, scanning electron microscope with energy dispersive spectroscopy, and x-ray diffraction. Stillwellite (LaBSiO{sub 5}) and oxyapatite (Ca{sub 2}La{sub 8}Si{sub 6}O{sub 26}) were found in glasses containing La{sub 2}O{sub 3}, while oxyapatite (Ca{sub 2}La{sub 8}Si{sub 6}O{sub 26} and NaNd{sub 9}Si{sub 6}O{sub 26}) precipitated in glasses with additions of mixed rare earths. The liquidus temperature (T{sub L}) of the glasses containing 5%, 10% and 15% La{sub 2}O{sub 3} were 800°C, 959°C and 986°C, respectively; while T{sub L} was 825°C, 1059°C and 1267°C for glasses

A compounded rare-earth iron garnet single crystal exhibiting stable Faraday rotation against wavelength and temperature variation in the 1.55 μm band

International Nuclear Information System (INIS)

Xu, Z.C.; Huang, M.; Li Miao

2006-01-01

The Bi, Tb and Yb partially substituted iron garnet bulk single crystals of Tb 3- x - y Yb y Bi x Fe 5 O 12 were grown by using Bi 2 O 3 /B 2 O 3 as flux and accelerated crucible rotation technique for single-crystal growth. Faraday rotation (FR) spectra showed that the specific FR of the (Tb 0.91 Yb 1.38 Bi 0.71 )Fe 5 O 12 crystal under magnetic field at saturation was measured to be about -1617 o /cm at λ=1.55 μm, Faraday rotation wavelength coefficient (FWC, 0.009%/nm) in the wavelength range of 1.50-1.62 μm and Faraday rotation temperature coefficient (FTC, 3.92x10 -5 /K) at λ=1.55 μm were even smaller than that of YIG. It is proven that through combining two types of Bi-substituted rare-earth iron garnets with opposite FWC and FTC signs, the compound rare-earth iron garnets with low FWC and FTC may be obtained due to the compensation effect. The saturation magnetization of (Tb 0.91 Yb 1.38 Bi 0.71 ) Fe 5 O 12 crystal is 0.48x10 6 A/M and is also much smaller than that of YIG. We have found empirically that there is a simple relationship between the FR θ f (x) and Bi content x for Tb 3- x - y Yb y Bi x Fe 5 O 12 , which is given by θ f (x)=(-2759x+400) o /cm

Affordances and distributed cognition in museum exhibitions

DEFF Research Database (Denmark)

Achiam, Marianne; May, Michael; Marandino, Martha

2014-01-01

consistent framework. Here, we invoke the notions of affordance and distributed cognition to explain in a coherent way how visitors interact with exhibits and exhibit spaces and make meaning from those interactions, and we exemplify our points using observations of twelve visitors to exhibits at a natural...... history museum. We show how differences in exhibit characteristics give rise to differences in the interpretive strategies used by visitors in their meaning-making process, and conclude by discussing how the notions of affordance and distributed cognition can be used in an exhibit design perspective....

Preparation of rare earth fluorides from apatite concentrate

International Nuclear Information System (INIS)

Mulyarchuk, I.F.; Voloshchenko, M.V.; Zen'kovich, E.G.; Sumenkova, V.V.; AN Ukrainskoj SSR, Kiev. Inst. Problem Lit'ya)

1980-01-01

The processes of preparation of the rare earths element sum from apatite concentrate of the Khibins, connected with preliminary extraction of rare earth phosphates from nitric acid extract using solvent extraction or direct precipitation from the extract by solution of potassium and ammonium fluorides. The sequence of the processes of the first variant is the following: solvent extraction of rare earths by tributylphosphate from clarified nitric acid extract of apatite with subsequent reextraction of rare earths with water and precipitation of rare earth phosphates from aqueous solution during neutralization by ammonia. In case of fluoride preparation from rare earth phosphate the main attention is paid to precipitation and filtration of fluorides. Technological scheme and cost price of industry for the production of 1800 t of rare earth trifluorides a year are calculated. When taking account of TBP losses according to its solubility the industry cost price is 1O times lower the modern cost of rare earth fluorides

Holland at CERN – Industrial exhibition

CERN Multimedia

GS Department

2010-01-01

Sponsored by EVD, an agency of the Dutch Ministry of the Economy From 8 to 11 November 2010 Industrial Exhibition Administration Building Bldg. 61 9-00 - 17-30 Twenty seven companies will present their latest technology at the industrial exhibition "Holland at CERN". Dutch industry will exhibit products and technologies which are related to the field of particle physics. Individual interviews will take place directly at the stands in the Main Building. The firms will contact relevant users/technicians but any user wishing to make contact with a particular firm is welcome to use the contact details which are available from each departmental secretariat or at the following URL: http://gs-dep.web.cern.ch/gs-dep/groups/sem/ls/Industrial_Exhibitions.htm#Industrial_exhibitions You will find the list of exhibitors below. LIST OF EXHIBITORS: Schelde Exotech Vernooy BV Triumph Group INCAA Computers DeMaCo Holland bv TNO Science & Industry Janssen Precision Engi...

Comprehensive genotyping in dyslipidemia: mendelian dyslipidemias caused by rare variants and Mendelian randomization studies using common variants.

Science.gov (United States)

Tada, Hayato; Kawashiri, Masa-Aki; Yamagishi, Masakazu

2017-04-01

Dyslipidemias, especially hyper-low-density lipoprotein cholesterolemia and hypertriglyceridemia, are important causal risk factors for coronary artery disease. Comprehensive genotyping using the 'next-generation sequencing' technique has facilitated the investigation of Mendelian dyslipidemias, in addition to Mendelian randomization studies using common genetic variants associated with plasma lipids and coronary artery disease. The beneficial effects of low-density lipoprotein cholesterol-lowering therapies on coronary artery disease have been verified by many randomized controlled trials over the years, and subsequent genetic studies have supported these findings. More recently, Mendelian randomization studies have preceded randomized controlled trials. When the on-target/off-target effects of rare variants and common variants exhibit the same direction, novel drugs targeting molecules identified by investigations of rare Mendelian lipid disorders could be promising. Such a strategy could aid in the search for drug discovery seeds other than those for dyslipidemias.

Superconductive microstrip exhibiting negative differential resistivity

International Nuclear Information System (INIS)

Huebener, R.P.; Gallus, D.E.

1975-01-01

A device capable of exhibiting negative differential electrical resistivity over a range of values of current and voltage is formed by vapor-depositing a thin layer of a material capable of exhibiting superconductivity on an insulating substrate, establishing electrical connections at opposite ends of the deposited strip, and cooling the alloy into its superconducting range. The device will exhibit negative differential resistivity when biased in the current-induced resistive state

Sc, Y, La-Lu - Rare Earth Elements

International Nuclear Information System (INIS)

Anon.

1987-01-01

At present extensive efforts are being made in completing work on system number Rare Earth Elements. Part A is devoted to the occurrence of these elements on the earth and in the universe. Part B deals with the pure metals; the 7 volumes published cover the description of the separation from the raw materials, the preparation of pure metals,their uses and toxicology, the physical properties of nuclei, atoms, molecules, and isotopes; in addition the behavior of ions in solution and the electrochemical behavior of rare earth elements are described. The compounds are described in Part C. Part D with 6 volumes has been devoted to the description of coordination compounds and is completed. The volume ''Rare Earth Elements C 10'' deals with the rare earth tellurides, oxide tellurides, tellurates, telluride halides, tellurate halides, sulfide tellurides, selenide tellurides, and alkali rare earth tellurates. Another topic of this volume are the compounds of the rare earth elements with polonium. So far as meaningful and in accordance with all earlier volumes of ''Rare Earth Elements'' Series C, comparative data are presented in sections preceding treatment of the individual compounds and systems

Theory of Rare-Earth Alloys

DEFF Research Database (Denmark)

Lindgård, Per-Anker

1977-01-01

A mean-field random alloy theory combined with a simple calculation of the exchange interaction J(c,Q) is shown to quantitatively account for the phase diagrams for alloys of rare-earth metals with Y, Lu, Sc, and other rare-earth metals. A concentration-dependent J(c,Q) explains the empirical 2...

Rare-earth hafnium oxide materials for magnetohydrodynamic (MHD) generator application

Energy Technology Data Exchange (ETDEWEB)

Marchant, D. D; Bates, J. L.

1979-01-01

Several ceramic materials based on rare-earth hafnium oxides have been identified as potential high-temperature electrodes and low-temperature current leadouts for open cycle coal-fired MHD generator channels. The electrode-current leadouts combination must operate at temperatures between 400 and 2000K with an electrical conductivity greater than 10/sup -2/ ohm/sup -1/ cm/sup -1/. The electrodes will be exposed to flowing (linear flow rates up to 100 m/s) potassium seeded coal combustion gases (plasma core temperatures between 2400 to 3200/sup 0/K) and coal slag. During operation the electrodes must conduct direct electric current at densities near 1.5 amp/cm/sup 2/. Consequently, the electrodes must be resistant to electrochemical decompositions and interactions with both the coal slag and potassium salts (e.g., K/sub 2/SO/sub 4/, K/sub 2/CO/sub 3/). The current leadout materials are placed between the hot electrodes and the water-cooled copper structural members and must have electrical conductivities greater than 10/sup -2/ ohm/sup -1/ cm/sup -1/ between 1400 and 400/sup 0/K. The current leadouts must be thermally and electrochemically compatible with the electrode, copper, and potassium salts. Ideally, the electrodes and current leadouts should exhibit minimal ionic conductivity. The fabrication, electrical conductivity, and electrochemical corrosion of rare-earth hafnium oxide materials are discussed. (WHK)

Review of Signaling Pathways Governing MSC Osteogenic and Adipogenic Differentiation

Directory of Open Access Journals (Sweden)

Aaron W. James

2013-01-01

Full Text Available Mesenchymal stem cells (MSC are multipotent cells, functioning as precursors to a variety of cell types including adipocytes, osteoblasts, and chondrocytes. Between osteogenic and adipogenic lineage commitment and differentiation, a theoretical inverse relationship exists, such that differentiation towards an osteoblast phenotype occurs at the expense of an adipocytic phenotype. This balance is regulated by numerous, intersecting signaling pathways that converge on the regulation of two main transcription factors: peroxisome proliferator-activated receptor-γ (PPARγ and Runt-related transcription factor 2 (Runx2. These two transcription factors, PPARγ and Runx2, are generally regarded as the master regulators of adipogenesis and osteogenesis. This review will summarize signaling pathways that govern MSC fate towards osteogenic or adipocytic differentiation. A number of signaling pathways follow the inverse balance between osteogenic and adipogenic differentiation and are generally proosteogenic/antiadipogenic stimuli. These include β-catenin dependent Wnt signaling, Hedgehog signaling, and NELL-1 signaling. However, other signaling pathways exhibit more context-dependent effects on adipogenic and osteogenic differentiation. These include bone morphogenic protein (BMP signaling and insulin growth factor (IGF signaling, which display both proosteogenic and proadipogenic effects. In summary, understanding those factors that govern osteogenic versus adipogenic MSC differentiation has significant implications in diverse areas of human health, from obesity to osteoporosis to regenerative medicine.

Impact of care pathways for in-hospital management of COPD exacerbation: a systematic review.

Science.gov (United States)

Lodewijckx, C; Sermeus, W; Panella, M; Deneckere, S; Leigheb, F; Decramer, M; Vanhaecht, K

2011-11-01

In-hospital management of COPD exacerbation is suboptimal, and outcomes are poor. Care pathways are a possible strategy for optimizing care processes and outcomes. The aim of the literature review was to explore characteristics of existing care pathways for in-hospital management of COPD exacerbations and to address their impact on performance of care processes, clinical outcomes, and team functioning. A literature search was conducted for articles published between 1990 and 2010 in the electronic databases of Medline, CINAHL, EMBASE, and Cochrane Library. Main inclusion criteria were (I) patients hospitalized for a COPD exacerbation; (II) implementation and evaluation of a care pathway; (III) report of original research, including experimental and quasi experimental designs, variance analysis, and interviews of professionals and patients about their perception on pathway effectiveness. Four studies with a quasi experimental design were included. Three studies used a pre-post test design; the fourth study was a non randomized controlled trial comparing an experimental group where patients were treated according to a care pathway with a control group where usual care was provided. The four studied care pathways were multidisciplinary structured care plans, outlining time-specific clinical interventions and responsibilities by discipline. Statistic analyses were rarely performed, and the trials used very divergent indicators to evaluate the impact of the care pathways. The studies described positive effects on blood sampling, daily weight measurement, arterial blood gas measurement, referral to rehabilitation, feelings of anxiety, length of stay, readmission, and in-hospital mortality. Research on COPD care pathways is very limited. The studies described few positive effects of the care pathways on diagnostic processes and on clinical outcomes. Though due to limited statistical analysis and weak design of the studies, the internal validity of results is limited

Thermodynamics of rare earths in steelmaking

International Nuclear Information System (INIS)

Vahed, A.; Kay, D.A.R.

1976-01-01

The standard free energies of formation of the oxides, sulfides and oxysulfides of cerium and lanthanum under steelmaking conditions have been calculated and used to predict the behavior of rare earths in steelmaking. Deoxidation and desulfurization constants, expressed in terms of Henrian activities, have been used to construct a precipitation diagram which indicates the sequence of rare earth inclusion formation. An enrichment of lanthanum in (RE)-oxysulfide and cerium in (RE)-sulfide is predicted. It is also predicted that rare earths should be able to reduce the soluble oxygen and sulfur contents of liquid steel well below the contents presently found in most industrial and laboratory practices. A simple method of calculating steelmaking additions for complete rare earth control of inclusion composition is presented

Skeletal muscle wasting with disuse atrophy is multi-dimensional: the response and interaction of myonuclei, satellite cells and signaling pathways

Directory of Open Access Journals (Sweden)

Naomi Elisabeth Brooks

2014-03-01

Full Text Available Maintenance of skeletal muscle is essential for health and survival. There are marked losses of skeletal muscle mass as well as strength and physiological function under conditions of low mechanical load, such as space flight, as well as ground based models such as bed rest, immobilisation, disuse and various animal models. Disuse atrophy is caused by mechanical unloading of muscle and this leads to reduced muscle mass without fibre attrition. Skeletal muscle stem cells (satellite cells and myonuclei are integrally involved in skeletal muscle responses to environmental changes that induce atrophy. Myonuclear domain size is influenced differently in fast and slow twitch muscle, but also by different models of muscle wasting, a factor that is not yet understood. Although the myonuclear domain is 3-dimensional this is rarely considered. Apoptosis as a mechanism for myonuclear loss with atrophy is controversial, whereas cell death of satellite cells has not been considered. Molecular signals such as myostatin/SMAD pathway, MAFbx and MuRF1 E3 ligases of the ubiquitin proteasome pathway and IGF1-AKT-mTOR pathway are 3 distinctly different contributors to skeletal muscle protein adaptation to disuse. Molecular signalling pathways activated in muscle fibres by disuse are rarely considered within satellite cells themselves despite similar exposure to unloading or low mechanical load. These molecular pathways interact with each other during atrophy and also when various interventions are applied that could alleviate atrophy. Re-applying mechanical load is an obvious method to restore muscle mass, however how nutrient supplementation (e.g. amino acids may further enhance recovery (or reduce atrophy despite unloading or ageing is currently of great interest. Satellite cells are particularly responsive to myostatin and to growth factors. Recently, the hibernating squirrel has been identified as an innovative model to study resistance to atrophy.

Regulation of mat responses by a differentiation MAPK pathway in Saccharomyces cerevisiae.

Directory of Open Access Journals (Sweden)

Sheelarani Karunanithi

Full Text Available Fungal species exhibit diverse behaviors when presented with extracellular challenges. Pathogenic fungi can undergo cell differentiation and biofilm formation in response to fluctuating nutrient levels, and these responses are required for virulence. In the model fungal eukaryote Saccharomyces cerevisiae, nutrient limitation induces filamentous growth and biofilm/mat formation. Both responses require the same signal transduction (MAPK pathway and the same cell adhesion molecule (Flo11 but have been studied under different conditions. We found that filamentous growth and mat formation are aspects of a related response that is regulated by the MAPK pathway. Cells in yeast-form mats differentiated into pseudohyphae in response to nutrient limitation. The MAPK pathway regulated mat expansion (in the plane of the XY-axis and substrate invasion (downward in the plane of the Z-axis, which optimized the mat's response to extracellular nutrient levels. The MAPK pathway also regulated an upward growth pattern (in the plane of the Z-axis in response to nutrient limitation and changes in surface rigidity. Upward growth allowed for another level of mat responsiveness and resembled a type of colonial chemorepulsion. Together our results show that signaling pathways play critical roles in regulating social behaviors in which fungal cells participate. Signaling pathways may regulate similar processes in pathogens, whose highly nuanced responses are required for virulence.

Interaction of TGFβ and BMP signaling pathways during chondrogenesis.

Directory of Open Access Journals (Sweden)

Bettina Keller

2011-01-01

Full Text Available TGFβ and BMP signaling pathways exhibit antagonistic activities during the development of many tissues. Although the crosstalk between BMP and TGFβ signaling pathways is well established in bone development, the relationship between these two pathways is less well defined during cartilage development and postnatal homeostasis. We generated hypomorphic mouse models of cartilage-specific loss of BMP and TGFβ signaling to assess the interaction of these pathways in postnatal growth plate homeostasis. We further used the chondrogenic ATDC5 cell line to test effects of BMP and TGFβ signaling on each other's downstream targets. We found that conditional deletion of Smad1 in chondrocytes resulted in a shortening of the growth plate. The addition of Smad5 haploinsufficiency led to a more severe phenotype with shorter prehypertrophic and hypertrophic zones and decreased chondrocyte proliferation. The opposite growth plate phenotype was observed in a transgenic mouse model of decreased chondrocytic TGFβ signaling that was generated by expressing a dominant negative form of the TGFβ receptor I (ΔTβRI in cartilage. Histological analysis demonstrated elongated growth plates with enhanced Ihh expression, as well as an increased proliferation rate with altered production of extracellular matrix components. In contrast, in chondrogenic ATDC5 cells, TGFβ was able to enhance BMP signaling, while BMP2 significantly reduces levels of TGF signaling. In summary, our data demonstrate that during endochondral ossification, BMP and TGFβ signaling can have antagonistic effects on chondrocyte proliferation and differentiation in vivo. We also found evidence of direct interaction between the two signaling pathways in a cell model of chondrogenesis in vitro.

Biosynthetic routes of hydroxylated carotenoids (xanthophylls) in Marchantia polymorpha, and production of novel and rare xanthophylls through pathway engineering in Escherichia coli.

Science.gov (United States)

Takemura, Miho; Maoka, Takashi; Misawa, Norihiko

2015-03-01

MpBHY codes for a carotene β-ring 3(,3')-hydroxylase responsible for both zeaxanthin and lutein biosynthesis in liverwort. MpCYP97C functions as an ε-ring hydroxylase (zeinoxanthin 3'-hydroxylase) to produce lutein in liverwort. Xanthophylls are oxygenated or hydroxylated carotenes that are most abundant in the light-harvesting complexes of plants. The plant-type xanthophylls consist of α-xanthophyll (lutein) and β-xanthophylls (zeaxanthin, antheraxanthin, violaxanthin and neoxanthin). The α-xanthophyll and β-xanthophylls are derived from α-carotene and β-carotene by carotene hydroxylase activities, respectively. β-Ring 3,3'-hydroxylase that mediates the route of zeaxanthin from β-carotene via β-cryptoxanthin is present in higher plants and is encoded by the BHY (BCH) gene. On the other hand, CYP97A (or BHY) and CYP97C genes are responsible for β-ring 3-hydroxylation and ε-ring 3'-hydroxylation, respectively, in routes from α-carotene to lutein. To elucidate the evolution of the biosynthetic routes of such hydroxylated carotenoids from carotenes in land plants, we identified and functionally analyzed carotenoid hydroxylase genes of liverwort Marchantia polymorpha L. Three genes homologous to higher plants, BHY, CYP97A, and CYP97C, were isolated and named MpBHY, MpCYP97A, and MpCYP97C, respectively. MpBHY was found to code for β-ring hydroxylase, which is responsible for both routes starting from β-carotene and α-carotene. MpCYP97C functioned as an ε-ring hydroxylase not for α-carotene but for zeinoxanthin, while MpCYP97A showed no hydroxylation activity for β-carotene or α-carotene. These findings suggest the original functions of the hydroxylation enzymes of carotenes in land plants, which are thought to diversify in higher plants. In addition, we generated recombinant Escherichia coli cells, which produced rare and novel carotenoids such as α-echinenone and 4-ketozeinoxanthin, through pathway engineering using bacterial carotenogenic genes

Ethical and social aspects on rare diseases

Directory of Open Access Journals (Sweden)

Krajnović Dušanka

2012-01-01

Full Text Available Rare diseases are a heterogenic group of disorders with a little in common except of their rarity affecting by less than 5 : 10.000 people. In the world is registered about 6000-8000 rare diseases with 6-8% suffering population only in the European Union. In spite of rarity, they represent an important medical and social problem due to their incidence. For many rare diseases have no treatment, but if it exists and if started on time as being available to patients, there is a good prognosis for them to be able for normal life. The problems of patients affected by rare diseases are related to the lack of diagnosis and timely undergoing as well as their treatment or prevention. Orphan drugs are products intended for treatment, diagnosis or prevention of rare diseases, but for their development and marketing the industry has not been interested in yet because of their marketing reasons. Patients suffering from a rare disease although belonging to the vulnerable group for their specific health needs, is becoming invisible in the health care system due to their additional needs un properly recognized. Ethical problems faced by patients, but also health care professionals are related to the allocation of medical diagnostics, unequal approach to health care, inappropriately specialized social services as well as therapy and rare orphan drugs unavailability. Ethical questions related to clinical trails on orphan drugs, population screening and epidemiology testing on rare diseases will also be discussed in this paper. [Projekat Ministarstva nauke Republike Srbije, br. 41004: Rare diseases: Molecular pathophysiology, the diagnostic and therapeutical modalities, social, ethical and legal aspects

Rare KL decays at Fermilab

International Nuclear Information System (INIS)

Schnetzer, St.

1997-01-01

Recent results and the future prospects for rare K L decay at Fermilab are described. A summary of all rare decay results from E799 Phase I (the 1991 run) are presented. Three new results: K L → e + e - μ + μ - , K L → π 0 μe, and π 0 → e + e - e + e - are discussed in detail. Improvements for KTeV (the 1996-1997 run) are discussed and the expected sensitivities listed. Finally, the KAMI program for rare decays with the Main Injector (2000 and beyond) is presented with emphasis on a search for the decay K L → π 0 νν-bar at O(10 -12 ) single-event-sensitivity. (author)

Rare species are valued big time.

Directory of Open Access Journals (Sweden)

Elena Angulo

Full Text Available BACKGROUND: It has recently been postulated that the value humans place on rarity could cause the extinction of rare species. This is because people are willing to pay the high costs of exploiting the last individuals. Many hobbies, such as ecotourism or the keeping of exotic pets may cause this effect--known as the anthropogenic Allee effect. However, the entire theory relies on the insofar undemonstrated assumption that people do value rarity. METHODOLOGY/PRINCIPAL FINDINGS: In order to quantify how much people valued rare species relative to common ones, we created online slideshows of photographs of either rare or common species on an Internet web site. The slideshow with photographs of rare species attracted more visitors, and visitors spent, in general, more time waiting to view it. CONCLUSIONS/SIGNIFICANCE: We provide evidence that people value rare more than common species. As we did not target consumers of a specific market, this finding suggests that the anthropogenic Allee effect is likely be driven by a large part of the population. Given the substantial participation in our online experiment, we highlight the potential of the world wide web resource as a tool for conservation action. However, the evidence presented here that the general public value rare species, combined with the assumption that anthropogenic Allee effect is operating, implies that conservationists should be prudent when using rarity to promote conservation.

Protein-altering variants associated with body mass index implicate pathways that control energy intake and expenditure in obesity

DEFF Research Database (Denmark)

Turcot, Valérie; Lu, Yingchang; Highland, Heather M

2018-01-01

,734 individuals to discover rare and low-frequency (minor allele frequency (MAF) obesity, 2 variants...... were in genes (MC4R and KSR2) previously observed to be mutated in extreme obesity and 2 variants were in GIPR. The effect sizes of rare variants are ~10 times larger than those of common variants, with the largest effect observed in carriers of an MC4R mutation introducing a stop codon (p.Tyr35Ter......, MAF = 0.01%), who weighed ~7 kg more than non-carriers. Pathway analyses based on the variants associated with BMI confirm enrichment of neuronal genes and provide new evidence for adipocyte and energy expenditure biology, widening the potential of genetically supported therapeutic targets in obesity....

75 FR 47458 - TRICARE; Rare Diseases Definition

Science.gov (United States)

2010-08-06

... tribal governments, in aggregate or by the private sector, of $100 million or more in any one year... TRICARE; Rare Diseases Definition AGENCY: Office of the Secretary, DoD. ACTION: Final rule. SUMMARY: This final rule revises the definition of rare diseases to adopt the definition of a rare disease as...

Rare earths production and marketing opportunities

International Nuclear Information System (INIS)

Falconnet, P.G.

1988-01-01

The rare earths (RE) market is relatively small. The total production during 1968 was only 10000 tons (REO) which rose to 27000 tons (REO) during 1985. The three major areas of application, which are volume market for ceric rare earths are catalysts, glass ceramics and metallurgy. Among the other uses of rare earths, the permanent magnets, lamp phosphors and fine ceramics have registered significant growth in RE consumption. Monazite and bastnasite are the main natural source for rare earths and processing of these for one of the rare earths in high demand leads to over production of some others not in demand, thus creating a balance problem. The growth in RE market has always been influenced by the technology shifts and product substitution. For example, the RE consumption during 1974/76 for desulfurization of steel had substantially decreased due to the usage of calcium. Similarly, 1985 had witnessed a drastic cut in the use of REs in fluid cracking due to the introduction of stabilized zeolites which contain less REO. Thus, the overall compound growth rate of demand was only 3.9 % per year during the period 1970-1985. At present, 37 % of the rare earths production goes to the glass/ceramics industry, 33 % for catalyst and 25 % to metallurgy. The price of REs constantly shows a downward trend. This trend coupled with the rapid changes taking place in the various technological fields, demands greater flexibility and high marketing skills from the RE producers. The key factor for future expansion of RE market will be the development of 'high volume' application of ceric rare earths. (author) 2 figs., 8 tabs

Red Bell Pepper Chromoplasts Exhibit in Vitro Import Competency and Membrane Targeting of Passenger Proteins from the Thylakoidal Sec and ΔpH Pathways but Not the Chloroplast Signal Recognition Particle Pathway1

Science.gov (United States)

Summer, Elizabeth J.; Cline, Kenneth

1999-01-01

Chloroplast to chromoplast development involves new synthesis and plastid localization of nuclear-encoded proteins, as well as changes in the organization of internal plastid membrane compartments. We have demonstrated that isolated red bell pepper (Capsicum annuum) chromoplasts contain the 75-kD component of the chloroplast outer envelope translocon (Toc75) and are capable of importing chloroplast precursors in an ATP-dependent fashion, indicating a functional general import apparatus. The isolated chromoplasts were able to further localize the 33- and 17-kD subunits of the photosystem II O2-evolution complex (OE33 and OE17, respectively), lumen-targeted precursors that utilize the thylakoidal Sec and ΔpH pathways, respectively, to the lumen of an internal membrane compartment. Chromoplasts contained the thylakoid Sec component protein, cpSecA, at levels comparable to chloroplasts. Routing of OE17 to the lumen was abolished by ionophores, suggesting that routing is dependent on a transmembrane ΔpH. The chloroplast signal recognition particle pathway precursor major photosystem II light-harvesting chlorophyll a/b protein failed to associate with chromoplast membranes and instead accumulated in the stroma following import. The Pftf (plastid fusion/translocation factor), a chromoplast protein, integrated into the internal membranes of chromoplasts during in vitro assays, and immunoblot analysis indicated that endogenous plastid fusion/translocation factor was also an integral membrane protein of chromoplasts. These data demonstrate that the internal membranes of chromoplasts are functional with respect to protein translocation on the thylakoid Sec and ΔpH pathways. PMID:9952453

Chemosensitizing tumor cells by targeting the Fanconi anemia pathway with an adenovirus overexpressing dominant-negative FANCA.

Science.gov (United States)

Ferrer, Miriam; de Winter, Johan P; Mastenbroek, D C Jeroen; Curiel, David T; Gerritsen, Winald R; Giaccone, Giuseppe; Kruyt, Frank A E

2004-08-01

Fanconi anemia (FA) is a rare genetic disorder characterized by bone-marrow failure and cellular hypersensitivity to crosslinking agents, including cisplatin. Here, we studied the use of the FA pathway as a possible target for cancer gene therapy with the aim to sensitize tumor cells for cisplatin by interfering with the FA pathway. As proof-of-principle, FA and non-FA lymphoblast-derived tumors were grown subcutaneously in scid mice and treated with two different concentrations of cisplatin. As predicted, the antitumor response was considerably improved in FA tumors. An adenoviral vector encoding a dominant-negative form of FANCA, FANCA600DN, was generated that interfered with endogenous FANCA-FANCG interaction resulting in the disruption of the FA pathway as illustrated by disturbed FANCD2 monoubiquitination. A panel of cell lines, including non-small-cell lung cancer cells, could be sensitized approximately two- to three-fold for cisplatin after Ad.CMV.FANCA600DN infection that may increase upon enhanced infection efficiency. In conclusion, targeting the FA pathway may provide a novel strategy for the sensitization of solid tumors for cisplatin and, in addition, provides a tool for examining the role of the FA pathway in determining chemoresistance in different tumor types.

RNF4-mediated polyubiquitination regulates the Fanconi anemia/BRCA pathway.

Science.gov (United States)

Xie, Jenny; Kim, Hyungjin; Moreau, Lisa A; Puhalla, Shannon; Garber, Judy; Al Abo, Muthana; Takeda, Shunichi; D'Andrea, Alan D

2015-04-01

The Fanconi anemia/BRCA (FA/BRCA) pathway is a DNA repair pathway that is required for excision of DNA interstrand cross-links. The 17 known FA proteins, along with several FA-associated proteins (FAAPs), cooperate in this pathway to detect, unhook, and excise DNA cross-links and to subsequently repair the double-strand breaks generated in the process. In the current study, we identified a patient with FA with a point mutation in FANCA, which encodes a mutant FANCA protein (FANCAI939S). FANCAI939S failed to bind to the FAAP20 subunit of the FA core complex, leading to decreased stability. Loss of FAAP20 binding exposed a SUMOylation site on FANCA at amino acid residue K921, resulting in E2 SUMO-conjugating enzyme UBC9-mediated SUMOylation, RING finger protein 4-mediated (RNF4-mediated) polyubiquitination, and proteasome-mediated degradation of FANCA. Mutation of the SUMOylation site of FANCA rescued the expression of the mutant protein. Wild-type FANCA was also subject to SUMOylation, RNF4-mediated polyubiquitination, and degradation, suggesting that regulated release of FAAP20 from FANCA is a critical step in the normal FA pathway. Consistent with this model, cells lacking RNF4 exhibited interstrand cross-linker hypersensitivity, and the gene encoding RNF4 was epistatic with the other genes encoding members of the FA/BRCA pathway. Together, the results from our study underscore the importance of analyzing unique patient-derived mutations for dissecting complex DNA repair processes.

Enhanced pinning in mixed rare earth-123 films

Science.gov (United States)

Driscoll, Judith L [Los Alamos, NM; Foltyn, Stephen R [Los Alamos, NM

2009-06-16

An superconductive article and method of forming such an article is disclosed, the article including a substrate and a layer of a rare earth barium cuprate film upon the substrate, the rare earth barium cuprate film including two or more rare earth metals capable of yielding a superconductive composition where ion size variance between the two or more rare earth metals is characterized as greater than zero and less than about 10.times.10.sup.-4, and the rare earth barium cuprate film including two or more rare earth metals is further characterized as having an enhanced critical current density in comparison to a standard YBa.sub.2Cu.sub.3O.sub.y composition under identical testing conditions.

Silicon rich nitride ring resonators for rare - earth doped telecommunications-band amplifiers pumped at the O-band.

Science.gov (United States)

Xing, P; Chen, G F R; Zhao, X; Ng, D K T; Tan, M C; Tan, D T H

2017-08-22

Ring resonators on silicon rich nitride for potential use as rare-earth doped amplifiers pumped at 1310 nm with amplification at telecommunications-band are designed and characterized. The ring resonators are fabricated on 300 nm and 400 nm silicon rich nitride films and characterized at both 1310 nm and 1550 nm. We demonstrate ring resonators exhibiting similar quality factors exceeding 10,000 simultaneously at 1310 nm and 1550 nm. A Dysprosium-Erbium material system exhibiting photoluminescence at 1510 nm when pumped at 1310 nm is experimentally demonstrated. When used together with Dy-Er co-doped particles, these resonators with similar quality factors at 1310 nm and 1550 nm may be used for O-band pumped amplifiers for the telecommunications-band.

Possibility as monosaccharide laxative of rare sugar alcohols.

Science.gov (United States)

Oosaka, Kazumasa

2009-05-01

Allitol, D-talitol and L-iditol are sugar alcohols that are rare in nature. Due to their previous rarity, little is known about the laxative effects of these rare sugar alcohols. Therefore, reliable data on the laxative effect that these sugar alcohols cause in experimental animals could help to evaluate the effectiveness of new monosaccharide laxative drugs. To investigate the laxative effect of rare sugar alcohols, the study was designed to observe the diarrhea that occurred after oral administration of these sugar alcohols in mice. Moreover, to investigate the influence on intestinal function of rare sugar alcohols, the study was designed to examine small intestine transit and the luminal water content. Results indicated that rare sugar alcohols have a laxative effect in mice. Diarrhea started at a dose of 4.95 g/kg of rare sugar alcohols. There was a statistically significant laxative effect for D-talitol and L-iditol at a dose of 9.9 g/kg as compared to vehicle. Moreover, rare sugar alcohols significantly increased the small intestinal transit and the luminal water content of the small intestine and cecum in mice as compared to each vehicle. Overall, L-iditol greatly changes the function of intestine. In conclusion, rare sugar alcohols increase water content in small intestine and accelerate small intestine transit. These results support laxative effect of rare sugar alcohols. Therefore, rare sugar alcohols may be useful as monosaccharide laxatives and may be used to treat constipation.

A systematic random sampling scheme optimized to detect the proportion of rare synapses in the neuropil.

Science.gov (United States)

da Costa, Nuno Maçarico; Hepp, Klaus; Martin, Kevan A C

2009-05-30

Synapses can only be morphologically identified by electron microscopy and this is often a very labor-intensive and time-consuming task. When quantitative estimates are required for pathways that contribute a small proportion of synapses to the neuropil, the problems of accurate sampling are particularly severe and the total time required may become prohibitive. Here we present a sampling method devised to count the percentage of rarely occurring synapses in the neuropil using a large sample (approximately 1000 sampling sites), with the strong constraint of doing it in reasonable time. The strategy, which uses the unbiased physical disector technique, resembles that used in particle physics to detect rare events. We validated our method in the primary visual cortex of the cat, where we used biotinylated dextran amine to label thalamic afferents and measured the density of their synapses using the physical disector method. Our results show that we could obtain accurate counts of the labeled synapses, even when they represented only 0.2% of all the synapses in the neuropil.

Blocking the NOTCH pathway can inhibit the growth of CD133-positive A549 cells and sensitize to chemotherapy

Energy Technology Data Exchange (ETDEWEB)

Liu, Juntao; Mao, Zhangfan; Huang, Jie; Xie, Songping; Liu, Tianshu; Mao, Zhifu, E-mail: 48151660@qq.com

2014-02-21

Highlights: • Notch signaling pathway members are expressed lower levels in CD133+ cells. • CD133+ cells are not as sensitive as CD133− cells to chemotherapy. • GSI could inhibit the growth of both CD133+ and CD133− cells. • Blockade of Notch signaling pathway enhanced the effect of chemotherapy with CDDP. • DAPT/CDDP co-therapy caused G2/M arrest and elimination in CD133+ cells. - Abstract: Cancer stem cells (CSCs) are believed to play an important role in tumor growth and recurrence. These cells exhibit self-renewal and proliferation properties. CSCs also exhibit significant drug resistance compared with normal tumor cells. Finding new treatments that target CSCs could significantly enhance the effect of chemotherapy and improve patient survival. Notch signaling is known to regulate the development of the lungs by controlling the cell-fate determination of normal stem cells. In this study, we isolated CSCs from the human lung adenocarcinoma cell line A549. CD133 was used as a stem cell marker for fluorescence-activated cell sorting (FACS). We compared the expression of Notch signaling in both CD133+ and CD133− cells and blocked Notch signaling using the γ-secretase inhibitor DAPT (GSI-IX). The effect of combining GSI and cisplatin (CDDP) was also examined in these two types of cells. We observed that both CD133+ and CD133− cells proliferated at similar rates, but the cells exhibited distinctive differences in cell cycle progression. Few CD133+ cells were observed in the G{sub 2}/M phase, and there were half as many cells in S phase compared with the CD133− cells. Furthermore, CD133+ cells exhibited significant resistance to chemotherapy when treated with CDDP. The expression of Notch signaling pathway members, such as Notch1, Notch2 and Hes1, was lower in CD133+ cells. GSI slightly inhibited the proliferation of both cell types and exhibited little effect on the cell cycle. The inhibitory effects of DPP on these two types of cells were

Computer-assisted initial diagnosis of rare diseases

Directory of Open Access Journals (Sweden)

Rui Alves

2016-07-01

Full Text Available Introduction. Most documented rare diseases have genetic origin. Because of their low individual frequency, an initial diagnosis based on phenotypic symptoms is not always easy, as practitioners might never have been exposed to patients suffering from the relevant disease. It is thus important to develop tools that facilitate symptom-based initial diagnosis of rare diseases by clinicians. In this work we aimed at developing a computational approach to aid in that initial diagnosis. We also aimed at implementing this approach in a user friendly web prototype. We call this tool Rare Disease Discovery. Finally, we also aimed at testing the performance of the prototype. Methods. Rare Disease Discovery uses the publicly available ORPHANET data set of association between rare diseases and their symptoms to automatically predict the most likely rare diseases based on a patient’s symptoms. We apply the method to retrospectively diagnose a cohort of 187 rare disease patients with confirmed diagnosis. Subsequently we test the precision, sensitivity, and global performance of the system under different scenarios by running large scale Monte Carlo simulations. All settings account for situations where absent and/or unrelated symptoms are considered in the diagnosis. Results. We find that this expert system has high diagnostic precision (≥80% and sensitivity (≥99%, and is robust to both absent and unrelated symptoms. Discussion. The Rare Disease Discovery prediction engine appears to provide a fast and robust method for initial assisted differential diagnosis of rare diseases. We coupled this engine with a user-friendly web interface and it can be freely accessed at http://disease-discovery.udl.cat/. The code and most current database for the whole project can be downloaded from https://github.com/Wrrzag/DiseaseDiscovery/tree/no_classifiers.

Investigating multiple dysregulated pathways in rheumatoid arthritis based on pathway interaction network.

Science.gov (United States)

Song, Xian-Dong; Song, Xian-Xu; Liu, Gui-Bo; Ren, Chun-Hui; Sun, Yuan-Bo; Liu, Ke-Xin; Liu, Bo; Liang, Shuang; Zhu, Zhu

2018-03-01

The traditional methods of identifying biomarkers in rheumatoid arthritis (RA) have focussed on the differentially expressed pathways or individual pathways, which however, neglect the interactions between pathways. To better understand the pathogenesis of RA, we aimed to identify dysregulated pathway sets using a pathway interaction network (PIN), which considered interactions among pathways. Firstly, RA-related gene expression profile data, protein-protein interactions (PPI) data and pathway data were taken up from the corresponding databases. Secondly, principal component analysis method was used to calculate the pathway activity of each of the pathway, and then a seed pathway was identified using data gleaned from the pathway activity. A PIN was then constructed based on the gene expression profile, pathway data, and PPI information. Finally, the dysregulated pathways were extracted from the PIN based on the seed pathway using the method of support vector machines and an area under the curve (AUC) index. The PIN comprised of a total of 854 pathways and 1064 pathway interactions. The greatest change in the activity score between RA and control samples was observed in the pathway of epigenetic regulation of gene expression, which was extracted and regarded as the seed pathway. Starting with this seed pathway, one maximum pathway set containing 10 dysregulated pathways was extracted from the PIN, having an AUC of 0.8249, and the result indicated that this pathway set could distinguish RA from the controls. These 10 dysregulated pathways might be potential biomarkers for RA diagnosis and treatment in the future.

Preparing rare earth-silicon-iron-aluminum alloys

International Nuclear Information System (INIS)

Marchant, J.D.; Morrice, E.; Herve, B.P.; Wong, M.M.

1980-01-01

As part of its mission to assure the maximum recovery and use of the Nation's mineral resources, the Bureau of Mines, investigated an improved procedure for producing rare earth-silicon alloys. For example, a charge consisting of 681 grams of mixed rare-earth oxides, 309 grams of ferrosilicon (75 wt-pct Si), and 182 grams of aluminum metal along with a flux consisting of 681 grams of CaO and 45 grams of MgO was reacted at 1500 0 C in an induction furnace. Good slag-metal separation was achieved. The alloy product contained, in weight-percent, 53 RE, 28 Si, 11 Fe, and 4 Al with a rare earth recovery of 80 pct. In current industrial practice rare earth recoveries are usually about 60 pct in alloy products that contain approximately 30 wt-pct each of rare earths and silicon. Metallurgical evaluations showed the alloys prepared in this investigation to be as effective in controlling the detrimental effect of sulfur in steel and cast iron as the commercial rare earth-silicon-iron alloys presently used in the steel industry

Most of rare missense alleles in humans are deleterious:implications for evolution of complex disease and associationstudies

Energy Technology Data Exchange (ETDEWEB)

Kryukov, Gregory V.; Pennacchio, Len A.; Sunyaev, Shamil R.

2006-10-24

The accumulation of mildly deleterious missense mutations inindividual human genomes has been proposed to be a genetic basis forcomplex diseases. The plausibility of this hypothesis depends onquantitative estimates of the prevalence of mildly deleterious de novomutations and polymorphic variants in humans and on the intensity ofselective pressure against them. We combined analysis of mutationscausing human Mendelian diseases, human-chimpanzee divergence andsystematic data on human SNPs and found that about 20 percent of newmissense mutations in humans result in a loss of function, while about 27percent are effectively neutral. Thus, more than half of new missensemutations have mildly deleterious effects. These mutations give rise tomany low frequency deleterious allelic variants in the human populationas evident from a new dataset of 37 genes sequenced in over 1,500individual human chromosomes. Surprisingly, up to 70 percent of lowfrequency missense alleles are mildly deleterious and associated with aheterozygous fitness loss in the range 0.001-0.003. Thus, the low allelefrequency of an amino acid variant can by itself serve as a predictor ofits functional significance. Several recent studies have reported asignificant excess of rare missense variants in disease populationscompared to controls in candidate genes or pathways. These studies wouldbe unlikely to work if most rare variants were neutral or if rarevariants were not a significant contributor to the genetic component ofphenotypic inheritance. Our results provide a justification for thesetypes of candidate gene (pathway) association studies and imply thatmutation-selection balance may be a feasible mechanism for evolution ofsome common diseases.

Structural, electrical, magnetic and dielectric properties of rare-earth substituted cobalt ferrites nanoparticles synthesized by the co-precipitation method

Energy Technology Data Exchange (ETDEWEB)

Nikumbh, A.K., E-mail: aknik@chem.unipune.ac.in; Pawar, R.A.; Nighot, D.V.; Gugale, G.S.; Sangale, M.D.; Khanvilkar, M.B.; Nagawade, A.V.

2014-04-15

Pure nanoparticles of the rare-earth substituted cobalt ferrites CoRE{sub x}Fe{sub 2−x}O{sub 4} (where RE=Nd, Sm and Gd and x=0.1 and 0.2) were prepared by the chemical co-precipitation method. X-ray diffraction, Transmission electron microscopy (TEM), d.c. electrical conductivity, Magnetic hysteresis and Thermal analysis are utilized in order to study the effect of variation in the rare-earth substitution and its impact on particle size, magnetic properties like M{sub S}, H{sub C} and Curie temperature. The phase identification of the materials by X-ray diffraction reveals the single-phase nature of the materials. The lattice parameter increased with rare-earth content for x≤0.2. The Transmission electron micrographs of Nd-, Sm- and Gd-substituted CoFe{sub 2}O{sub 4} exhibit the particle size 36.1 to 67.8 nm ranges. The data of temperature variation of the direct current electrical conductivity showed definite breaks, which corresponds to ferrimagnetic to paramagnetic transitions. The thermoelectric power for all compound are positive over the whole range of temperature. The dielectric constant decreases with frequency and rare-earth content for the prepared samples. The magnetic properties of rare-earth substituted cobalt ferrites showed a definite hysteresis loop at room temperature. The reduction of coercive force, saturation magnetization, ratio M{sub R}/M{sub S} and magnetic moments may be due to dilution of the magnetic interaction.

Rare earth-ruthenium-magnesium intermetallics

Energy Technology Data Exchange (ETDEWEB)

Stein, Sebastian; Kersting, Marcel; Heletta, Lukas; Poettgen, Rainer [Muenster Univ. (Germany). Inst. fuer Anorganische und Analytische Chemie

2017-07-01

Eight new intermetallic rare earth-ruthenium-magnesium compounds have been synthesized from the elements in sealed niobium ampoules using different annealing sequences in muffle furnaces. The compounds have been characterized by powder and single crystal X-ray diffraction. Sm{sub 9.2}Ru{sub 6}Mg{sub 17.8} (a=939.6(2), c=1779(1) pm), Gd{sub 11}Ru{sub 6}Mg{sub 16} (a=951.9(2), c=1756.8(8) pm), and Tb{sub 10.5}Ru{sub 6}Mg{sub 16.5} (a=942.5(1), c=1758.3(4) pm) crystallize with the tetragonal Nd{sub 9.34}Ru{sub 6}Mg{sub 17.66} type structure, space group I4/mmm. This structure exhibits a complex condensation pattern of square-prisms and square-antiprisms around the magnesium and ruthenium atoms, respectively. Y{sub 2}RuMg{sub 2} (a=344.0(1), c=2019(1) pm) and Tb{sub 2}RuMg{sub 2} (a=341.43(6), c=2054.2(7) pm) adopt the Er{sub 2}RuMg{sub 2} structure and Tm{sub 3}Ru{sub 2}Mg (a=337.72(9), c=1129.8(4) pm) is isotypic with Sc{sub 3}Ru{sub 2}Mg. Tm{sub 3}Ru{sub 2}Mg{sub 2} (a=337.35(9), c=2671(1) pm) and Lu{sub 3}Ru{sub 2}Mg{sub 2} (a=335.83(5), c=2652.2(5) pm) are the first ternary ordered variants of the Ti{sub 3}Cu{sub 4} type, space group I4/mmm. These five compounds belong to a large family of intermetallics which are completely ordered superstructures of the bcc subcell. The group-subgroup scheme for Lu{sub 3}Ru{sub 2}Mg{sub 2} is presented. The common structural motif of all three structure types are ruthenium-centered rare earth cubes reminicent of the CsCl type. Magnetic susceptibility measurements of Y{sub 2}RuMg{sub 2} and Lu{sub 3}Ru{sub 2}Mg{sub 2} samples revealed Pauli paramagnetism of the conduction electrons.

Ainhum - A Rare Case Report.

Science.gov (United States)

Prabhu, Ravi; Kannan, Narayanasamy Subbaraju; Vinoth, Sundaresan; Praveen, Chinnappan Balasubramanian

2016-04-01

The term 'AINHUM' is derived from the African word meaning 'to saw or cut'. True ainhum otherwise called dactylolysis spontanea is a condition involving soft tissue or digits with constricting rings commonly presenting in fifth toes, usually bilateral. It is to be differentiated from Pseudo-ainhum that occurs secondary to some hereditary and nonhereditary diseases that lead to annular constriction of digits. We report a rare case of true ainhum involving the left fourth toe only. It is a very rare case and a very few were reported worldwide. The highest incidence of ainhum has been reported in South Africa and South America. It is rarely reported in India. Ainhum when diagnosed and treated in early stages can be prevented from progressing to mutilating deformities.

Students-exhibits interaction at a science center

Science.gov (United States)

Botelho, Agostinho; Morais, Ana M.

2006-12-01

In this study we investigate students' learning during their interaction with two exhibits at a science center. Specifically, we analyze both students' procedures when interacting with exhibits and their understanding of the scientific concepts presented therein. Bernstein's theory of pedagogic discourse (1990, 2000) provided the sociological foundation to assess the exhibit-student interaction and allowed analysis of the influence of the characteristics of students, exhibits, and interactions on students' learning. Eight students (ages 12ndash;13 years of age) with distinct sociological characteristics participated in the study. Several findings emerged from the results. First, the characteristics of the students, exhibits, and interactions appeared to influence student learning. Second, to most students, what they did interactively (procedures) seems not to have had any direct consequence on what they learned (concept understanding). Third, the data analysis suggest an important role for designers and teachers in overcoming the limitations of exhibit-student interaction.

Effect of deregulation of Sonic Hedgehog pathway on responses to DNA damage and cancer predisposition

International Nuclear Information System (INIS)

Charazac, Aurelie

2015-01-01

The Gorlin syndrome is a rare genetic disorder characterized by several developmental abnormalities. Due to mutations in PTCH1, a key player of the sonic hedgehog signaling pathway, clinical manifestations also includes hyper-radiosensitivity and an increased predisposition to the development of basal cell carcinomas. Given the implication of DNA repair system defects in hyper-radiosensitivity pathologies, we decided to study the effect of PTCH1 mutations on the DNA damage response in order to better understand the cellular and molecular mechanisms leading to Gorlin's phenotype.This study demonstrate a global failure of the DNA damage repair systems in Gorlin fibroblasts with respect to controls. It highlights in particular the collapse of the base excision repair pathway (BER) responsible for the repair of oxidative DNA damage. (author) [fr

A mouse model of the schizophrenia-associated 1q21.1 microdeletion syndrome exhibits altered mesolimbic dopamine transmission

DEFF Research Database (Denmark)

Nielsen, Jacob; Fejgin, Kim; Sotty, Florence

2017-01-01

on schizophrenia-related assays. Df(h1q21)/+ mice displayed increased hyperactivity in response to amphetamine challenge and increased sensitivity to the disruptive effects of amphetamine and phencyclidine hydrochloride (PCP) on prepulse inhibition. Probing of the direct dopamine (DA) pathway using the DA D1...... and basic functions such as reflexes, ASR, thermal pain sensitivity, and motor performance were unaltered. Similarly, anxiety related measures, baseline prepulse inhibition, and seizure threshold were unaltered. In addition to the central nervous system-related phenotypes, Df(h1q21)/+ mice exhibited reduced...

Processing of monazite at the rare earth division,Udyogamandal

International Nuclear Information System (INIS)

Narayanan, N.S.; Thulasidoss, S.; Ramachandran, T.V.; Swaminathan, T.V.; Prasad, K.R.

1988-01-01

The processing techniques adopted at the Rare Earth Division of the Indian Rare Earths Limited at Udyogamandal, for the production of rare earth compounds of various compositions and purity grades are reviewed. Over 100 different compounds are produced and marketed, and these include mixed rare earths chloride, crude thorium concentrate, cerium oxide, cerium hydrate, rare earths carbonate, didymium salts and individual rare earth oxides and salts. Also, the trisodium phosphate obtained as byproduct in the processing of monazite, is recovered and marketed. The process scheme for monazite essentially involves alkaline digestion of ground monazite, removal of the by-product trisodium phosphate, separation of thorium through preferential dissolution of rare earths hydroxide in hydrochloric acid under controlled pH and temperature conditions followed by purification, and evaporation of the chloride solution to yield pure rare earths chloride. Part of the chloride is utilised for the production of individual rare earth compounds after separation by solvent extraction and ion exchange processes. Individual rare earth compounds of 99.99 %+ purity are regularly produced to cater to the demand within the country. (author) 8 figs., 1 tab

Genetic profiling of a rare condition: co-occurrence of albinism and multiple primary melanoma in a Caucasian family.

Science.gov (United States)

De Summa, Simona; Guida, Michele; Tommasi, Stefania; Strippoli, Sabino; Pellegrini, Cristina; Fargnoli, Maria Concetta; Pilato, Brunella; Natalicchio, Iole; Guida, Gabriella; Pinto, Rosamaria

2017-05-02

Multiple primary melanoma (MPM) is a rare condition, whose genetic basis has not yet been clarified. Only 8-12% of MPM are due to germline mutations of CDKN2A. However, other genes (POT1, BRCA1/2, MC1R, MGMT) have been demonstrated to be involved in predisposition to this pathology.To our knowledge, this is the first family study based on two siblings with the rare coexistence of MPM and oculocutaneous albinism (OCA), an autosomal recessive disease characterized by the absence or decrease in pigmentation in the skin, hair, and eyes.In this study, we evaluated genes involved in melanoma predisposition (CDKN2A, CDK4, MC1R, MITF, POT1, RB1, MGMT, BRCA1, BRCA2), pathogenesis (BRAF, NRAS, PIK3CA, KIT, PTEN), skin/hair pigmentation (MC1R, MITF) and in immune pathways (CTLA4) to individuate alterations able to explain the rare onset of MPM and OCA in indexes and the transmission in their pedigree.From the analysis of the pedigree, we were able to identify a "protective" haplotype with respect to MPM, including MGMT p.I174V alteration. The second generation offspring is under strict follow up as some of them have a higher risk of developing MPM according to our model.

A high resolution cross section transmission electron microscopy study of epitaxial rare earth fluoride/GaAs(111) interfaces prepared by molecular beam epitaxy

International Nuclear Information System (INIS)

Chien, C.J.; Bravman, J.C.

1990-01-01

The authors report the HRXTEM study of epitaxial rare earth fluoride/GaAs(111) interfaces. Such interfaces are of interest because they are the starting point for growth of buried epitaxial rare earth/rare earth fluoride sandwich structures which exhibit interesting and non bulk-like magnetic properties. Also, the optical transitions in ultrathin epitaxial NdF 3 films may be influenced by strain and defects in the NdF 3 film and the nature of the interface to GaAs. The authors find that the rare earth fluoride/GaAs interfaces are semi-coherent but chemically abrupt with the transition taking place within 3 Angstrom. However, the interface is physically rough and multiple monolayer steps in the GaAs surface tend to tilt boundaries in the fluoride. The origin of these steps is believed to be thermal etching of the GaAs during the heat- cleaning stage prior to epitaxy. The surface of the fluoride film is much smoother than the initial GaAs surface indicating planarization during epitaxy

Putting a Face on Rare Diseases

Science.gov (United States)

... who have a rare and potentially dangerous disease, Ehlers-Danlos syndrome. Photo Courtesy of: Patricia Weltin That has been ... daughters with a rare and potentially dangerous disease, Ehlers-Danlos syndrome (EDS), a connective tissue disorder causing joint dislocations, ...

[SZCZECIN CITIZENS' KNOWLEDGE ABOUT RARE DISEASES].

Science.gov (United States)

Walat, Anna; Skoczylas, Michal Marian; Welnicka, Agnieszka; Kulig, Malgorzata; Rodak, Przemyslaw; Walczak, Zuzanna; Jablońska, Agata

2014-01-01

The aim of the study was to assess knowledge about rare diseases among citizens of Szczecin (Poland). The study was performed by questioning 242 adult customers of Turzyn Shopping Centre in Szczecin (149 females and 93 males). The survey was conducted in the shopping mall on 23 February 2013 (control group) and during the celebration of Rare Disease Day and the 12th Polish Nationwide Cystic Fibrosis Week ("Dolina Mukolinków") on 2 March 2013 (research group). The research tool was a questionnaire devised by the authors and filled out by the writing authors interviewer's answers. In the study group more people knew about the existence of Rare Disease Day than in the control group (86.02% vs 57.72%, chi-square test χ2 > χ2(1); 0.001, p χ2(1); 0.001, p < 0.001). The respondents from the research group knew more about Rare Disease Day and defined the idea of it as closed in a significantly higher degree than the control group. There was no significant difference in the detailed knowledge about rare diseases in either group. This might indicate the need to educate society and patients, along with their families.

Magnetic properties and structural transitions of fluorite-related rare earth osmates Ln3OsO7 (Ln=Pr, Tb)

International Nuclear Information System (INIS)

Hinatsu, Yukio; Doi, Yoshihiro

2013-01-01

Ternary rare-earth osmates Ln 3 OsO 7 (Ln=Pr, Tb) have been prepared. They crystallize in an ortho-rhombic superstructure of cubic fluorite with space group Cmcm. Both of these compounds undergo a structural phase transition at 130 K (Ln=Pr) and 580 K (Ln=Tb). These compounds show complex magnetic behavior at low temperatures. Pr 3 OsO 7 exhibits magnetic transitions at 8 and 73 K, and Tb 3 OsO 7 magnetically orders at 8 and 60 K. The Os moments become one-dimensionally ordered, and when the temperature is furthermore decreased, it provokes the ordering in the Ln 3+ sublattice that simultaneously becomes three-dimensionally ordered with the Os sublattice. - Graphical abstract: Ternary rare-earth osmates Ln 3 OsO 7 (Ln=Pr, Tb) have been prepared. They crystallize in an orthorhombic superstructure of cubic fluorite with space group Cmcm. Both of these compounds undergo a structural phase transition at 130 K (Ln=Pr) and 580 K (Ln=Tb). These compounds show complex magnetic behavior at low temperatures. Pr 3 OsO 7 exhibits magnetic transitions at 8 and 73 K, and Tb 3 OsO 7 magnetically orders at 8 and 60 K. Highlights: ► Ternary rare-earth osmates Ln 3 OsO 7 (Ln=Pr, Tb) with an ordered defect-fluorite structure have been prepared. ► Both of these compounds undergo a structural phase transition at 130 K (Ln=Pr) and 580 K (Ln=Tb). ► These compounds show complex magnetic behavior at low temperatures due to magnetic ordering of Ln and Os.

Low energy spin dynamics of rare-earth orthoferrites YFeO3 and LaFeO3

Science.gov (United States)

Park, Kisoo; Sim, Hasung; Leiner, Jonathan; Yoshida, Yoshiyuki; Eisaki, Hiroshi; Yano, Shinichiro; Gardner, Jason; Park, Je-Geun

YFeO3 and LaFeO3\\ are members of the rare-earth orthoferrites (RFeO3) family with Pbnm space group. With the strong superexchange interaction between Fe3 + ions, both compounds exhibit the room temperature antiferromagnetic order (TN >600 K) with a slight spin canting. Here we report low-energy magnetic excitation of YFeO3 and LaFeO3 using inelastic neutron scattering measurements, showing evidence of magnon mode splitting and a spin anisotropy gap at the zone center. Spin wave calculations with the spin Hamiltonian including both Dzyaloshinsky-Moriya interaction and single-ion anisotropy accounts for the observed features well. Our results offer insight into the underlying physics of other RFeO3\\ with magnetic rare-earth ions or related Fe3+-based multiferroic perovskites such as BiFeO3. The work at the IBS CCES (South Korea) was supported by the research program of the Institute for Basic Science (IBS-R009-G1).

Functional impact of global rare copy number variation in autism spectrum disorders.

Science.gov (United States)

Pinto, Dalila; Pagnamenta, Alistair T; Klei, Lambertus; Anney, Richard; Merico, Daniele; Regan, Regina; Conroy, Judith; Magalhaes, Tiago R; Correia, Catarina; Abrahams, Brett S; Almeida, Joana; Bacchelli, Elena; Bader, Gary D; Bailey, Anthony J; Baird, Gillian; Battaglia, Agatino; Berney, Tom; Bolshakova, Nadia; Bölte, Sven; Bolton, Patrick F; Bourgeron, Thomas; Brennan, Sean; Brian, Jessica; Bryson, Susan E; Carson, Andrew R; Casallo, Guillermo; Casey, Jillian; Chung, Brian H Y; Cochrane, Lynne; Corsello, Christina; Crawford, Emily L; Crossett, Andrew; Cytrynbaum, Cheryl; Dawson, Geraldine; de Jonge, Maretha; Delorme, Richard; Drmic, Irene; Duketis, Eftichia; Duque, Frederico; Estes, Annette; Farrar, Penny; Fernandez, Bridget A; Folstein, Susan E; Fombonne, Eric; Freitag, Christine M; Gilbert, John; Gillberg, Christopher; Glessner, Joseph T; Goldberg, Jeremy; Green, Andrew; Green, Jonathan; Guter, Stephen J; Hakonarson, Hakon; Heron, Elizabeth A; Hill, Matthew; Holt, Richard; Howe, Jennifer L; Hughes, Gillian; Hus, Vanessa; Igliozzi, Roberta; Kim, Cecilia; Klauck, Sabine M; Kolevzon, Alexander; Korvatska, Olena; Kustanovich, Vlad; Lajonchere, Clara M; Lamb, Janine A; Laskawiec, Magdalena; Leboyer, Marion; Le Couteur, Ann; Leventhal, Bennett L; Lionel, Anath C; Liu, Xiao-Qing; Lord, Catherine; Lotspeich, Linda; Lund, Sabata C; Maestrini, Elena; Mahoney, William; Mantoulan, Carine; Marshall, Christian R; McConachie, Helen; McDougle, Christopher J; McGrath, Jane; McMahon, William M; Merikangas, Alison; Migita, Ohsuke; Minshew, Nancy J; Mirza, Ghazala K; Munson, Jeff; Nelson, Stanley F; Noakes, Carolyn; Noor, Abdul; Nygren, Gudrun; Oliveira, Guiomar; Papanikolaou, Katerina; Parr, Jeremy R; Parrini, Barbara; Paton, Tara; Pickles, Andrew; Pilorge, Marion; Piven, Joseph; Ponting, Chris P; Posey, David J; Poustka, Annemarie; Poustka, Fritz; Prasad, Aparna; Ragoussis, Jiannis; Renshaw, Katy; Rickaby, Jessica; Roberts, Wendy; Roeder, Kathryn; Roge, Bernadette; Rutter, Michael L; Bierut, Laura J; Rice, John P; Salt, Jeff; Sansom, Katherine; Sato, Daisuke; Segurado, Ricardo; Sequeira, Ana F; Senman, Lili; Shah, Naisha; Sheffield, Val C; Soorya, Latha; Sousa, Inês; Stein, Olaf; Sykes, Nuala; Stoppioni, Vera; Strawbridge, Christina; Tancredi, Raffaella; Tansey, Katherine; Thiruvahindrapduram, Bhooma; Thompson, Ann P; Thomson, Susanne; Tryfon, Ana; Tsiantis, John; Van Engeland, Herman; Vincent, John B; Volkmar, Fred; Wallace, Simon; Wang, Kai; Wang, Zhouzhi; Wassink, Thomas H; Webber, Caleb; Weksberg, Rosanna; Wing, Kirsty; Wittemeyer, Kerstin; Wood, Shawn; Wu, Jing; Yaspan, Brian L; Zurawiecki, Danielle; Zwaigenbaum, Lonnie; Buxbaum, Joseph D; Cantor, Rita M; Cook, Edwin H; Coon, Hilary; Cuccaro, Michael L; Devlin, Bernie; Ennis, Sean; Gallagher, Louise; Geschwind, Daniel H; Gill, Michael; Haines, Jonathan L; Hallmayer, Joachim; Miller, Judith; Monaco, Anthony P; Nurnberger, John I; Paterson, Andrew D; Pericak-Vance, Margaret A; Schellenberg, Gerard D; Szatmari, Peter; Vicente, Astrid M; Vieland, Veronica J; Wijsman, Ellen M; Scherer, Stephen W; Sutcliffe, James S; Betancur, Catalina

2010-07-15

The autism spectrum disorders (ASDs) are a group of conditions characterized by impairments in reciprocal social interaction and communication, and the presence of restricted and repetitive behaviours. Individuals with an ASD vary greatly in cognitive development, which can range from above average to intellectual disability. Although ASDs are known to be highly heritable ( approximately 90%), the underlying genetic determinants are still largely unknown. Here we analysed the genome-wide characteristics of rare (variation in ASD using dense genotyping arrays. When comparing 996 ASD individuals of European ancestry to 1,287 matched controls, cases were found to carry a higher global burden of rare, genic copy number variants (CNVs) (1.19 fold, P = 0.012), especially so for loci previously implicated in either ASD and/or intellectual disability (1.69 fold, P = 3.4 x 10(-4)). Among the CNVs there were numerous de novo and inherited events, sometimes in combination in a given family, implicating many novel ASD genes such as SHANK2, SYNGAP1, DLGAP2 and the X-linked DDX53-PTCHD1 locus. We also discovered an enrichment of CNVs disrupting functional gene sets involved in cellular proliferation, projection and motility, and GTPase/Ras signalling. Our results reveal many new genetic and functional targets in ASD that may lead to final connected pathways.

Analyzing rare diseases terms in biomedical terminologies

Directory of Open Access Journals (Sweden)

Erika Pasceri

2012-03-01

Full Text Available Rare disease patients too often face common problems, including the lack of access to correct diagnosis, lack of quality information on the disease, lack of scientific knowledge of the disease, inequities and difficulties in access to treatment and care. These things could be changed by implementing a comprehensive approach to rare diseases, increasing international cooperation in scientific research, by gaining and sharing scientific knowledge about and by developing tools for extracting and sharing knowledge. A significant aspect to analyze is the organization of knowledge in the biomedical field for the proper management and recovery of health information. For these purposes, the sources needed have been acquired from the Office of Rare Diseases Research, the National Organization of Rare Disorders and Orphanet, organizations that provide information to patients and physicians and facilitate the exchange of information among different actors involved in this field. The present paper shows the representation of rare diseases terms in biomedical terminologies such as MeSH, ICD-10, SNOMED CT and OMIM, leveraging the fact that these terminologies are integrated in the UMLS. At the first level, it was analyzed the overlap among sources and at a second level, the presence of rare diseases terms in target sources included in UMLS, working at the term and concept level. We found that MeSH has the best representation of rare diseases terms.

A novel tridentate coordination mode for the carbonatonickel system exhibited in an unusual hexanuclear nickel(II) mu3-carbonato-bridged complex.

Science.gov (United States)

Anderson, James C; Blake, Alexander J; Moreno, Rafael Bou; Raynel, Guillaume; van Slageren, Joris

2009-11-14

The fixation of CO(2) at ambient temperature has been achieved by the reaction of Ni(cod)(2) and TMEDA in CO(2) saturated THF that yields a novel hexanuclear nickel(II) mu(3)-carbonato bridged complex [Ni(6)(mu(3)-CO(3))(4)(TMEDA)(6)(H(2)O)(12)](OH)(4) in 59% yield. The complex was characterised by MS analysis and the structure corroborated by single-crystal X-ray crystallography. The complex exhibits a rare carbonato binding mode for Ni(II) complexes and moderately strong antiferromagnetic interactions.

Spectroscopic and neutron detection properties of rare earth and titanium doped LiAlO 2 single crystals

Energy Technology Data Exchange (ETDEWEB)

Dickens, Peter T.; Marcial, José; McCloy, John; McDonald, Benjamin S.; Lynn, Kelvin G.

2017-10-01

In this study, LiAlO2 crystals doped with rare-earth elements and Ti were produced by the CZ method and spectroscopic and neutron detection properties were investigated. Photoluminescence revealed no clear luminescent activation of LiAlO2 by the rare-earth dopants though some interesting luminescence was observed from secondary phases within the crystal. Gamma-ray pulse height spectra collected using a 137Cs source exhibited only a Compton edge for the crystals. Neutron modeling using Monte Carlo N-Particle Transport Code revealed most neutrons used in the detection setup are thermalized, and while using natural lithium in the crystal growth, which contains 7.6 % 6Li, a 10 mm Ø by 10 mm sample of LiAlO2 has a 70.7 % intrinsic thermal neutron capture efficiency. Furthermore, the pulse height spectra collected using a 241Am-Be neutron source demonstrated a distinct neutron peak.

Exhibition - Mathematics, A Beautiful Elsewhere

CERN Multimedia

2011-01-01

From 21 October 2011 to 18 March 2012, the Fondation Cartier pour l’art contemporain will present the exhibition Mathematics: A Beautiful Elsewhere, an exhibition developed in association with the Institut des Hautes Études Scientifiques (IHÉS) and under the patronage of UNESCO. For this unprecedented event, the foundation invited mathematicians to work with artists with whom it has previously worked to create an exhibition that allows visitors to see, hear, do, interpret and think about mathematics. By bringing mathematics into its premises, the Fondation Cartier is itself undergoing the “sudden change of scenery” described by mathematician Alexandre Grothendieck. More information is available here. Fondation Cartier pour l’art contemporain 261, boulevard Raspail 75014 Paris http://fondation.cartier.com Private Visit For professors, researchers and all the staff of Mathematics departments...

Rare earth metal alloy magnets

International Nuclear Information System (INIS)

Harris, I.R.; Evans, J.M.; Nyholm, P.S.

1979-01-01

This invention relates to rare earth metal alloy magnets and to methods for their production. The technique is based on the fact that rare earth metal alloys (for e.g. cerium or yttrium) which have been crumbled to form a powder by hydride formation and decomposition can be used for the fabrication of magnets without the disadvantages inherent in alloy particle size reduction by mechanical milling. (UK)

A rare case of haboob in Tehran: Observational and numerical study

Science.gov (United States)

Karami, S.; Ranjbar, A.; Mohebalhojeh, A. R.; Moradi, M.

2017-03-01

A great dust storm occurred in Tehran on 2 June 2014 and caused severe damage to properties and involved loss of human life. From the visual evidence available, it can be regarded as a case of haboob. As a lower latitude phenomenon, its occurrence in Tehran was unprecedented in the last 50 years. This paper aims to present a detailed analysis of the weather conditions, the pathways by which dust particles were ingested by the haboob, as well as the impact of the urban boundary layer on the intensity and propagation of the dust storm. Using numerical simulation carried out by the WRF-Chem model and various observational techniques, the coupling of a low-level small-scale deformation field with a lower-tropospheric cold pool produced by precipitating mid-tropospheric clouds is identified as the main process involved in shaping this rare dust storm.

A novel dysregulated pathway-identification analysis based on global influence of within-pathway effects and crosstalk between pathways

Science.gov (United States)

Han, Junwei; Li, Chunquan; Yang, Haixiu; Xu, Yanjun; Zhang, Chunlong; Ma, Jiquan; Shi, Xinrui; Liu, Wei; Shang, Desi; Yao, Qianlan; Zhang, Yunpeng; Su, Fei; Feng, Li; Li, Xia

2015-01-01

Identifying dysregulated pathways from high-throughput experimental data in order to infer underlying biological insights is an important task. Current pathway-identification methods focus on single pathways in isolation; however, consideration of crosstalk between pathways could improve our understanding of alterations in biological states. We propose a novel method of pathway analysis based on global influence (PAGI) to identify dysregulated pathways, by considering both within-pathway effects and crosstalk between pathways. We constructed a global gene–gene network based on the relationships among genes extracted from a pathway database. We then evaluated the extent of differential expression for each gene, and mapped them to the global network. The random walk with restart algorithm was used to calculate the extent of genes affected by global influence. Finally, we used cumulative distribution functions to determine the significance values of the dysregulated pathways. We applied the PAGI method to five cancer microarray datasets, and compared our results with gene set enrichment analysis and five other methods. Based on these analyses, we demonstrated that PAGI can effectively identify dysregulated pathways associated with cancer, with strong reproducibility and robustness. We implemented PAGI using the freely available R-based and Web-based tools (http://bioinfo.hrbmu.edu.cn/PAGI). PMID:25551156

A Bacterial Pathogen Targets a Host Rab-Family GTPase Defense Pathway with a GAP.

Science.gov (United States)

Spanò, Stefania; Gao, Xiang; Hannemann, Sebastian; Lara-Tejero, María; Galán, Jorge E

2016-02-10

Cell-autonomous defense mechanisms are potent strategies that protect individual cells against intracellular pathogens. The Rab-family GTPase Rab32 was previously shown to restrict the intracellular human pathogen Salmonella Typhi, but its potential broader role in antimicrobial defense remains unknown. We show that Rab32 represents a general cell-autonomous, antimicrobial defense that is counteracted by two Salmonella effectors. Mice lacking Rab-32 or its nucleotide exchange factor BLOC-3 are permissive to S. Typhi infection and exhibit increased susceptibility to S. Typhimurium. S. Typhimurium counters this defense pathway by delivering two type III secretion effectors, SopD2, a Rab32 GAP, and GtgE, a specific Rab32 protease. An S. Typhimurium mutant strain lacking these two effectors exhibits markedly reduced virulence, which is fully restored in BLOC-3-deficient mice. These results demonstrate that a cell-autonomous, Rab32-dependent host defense pathway plays a central role in the defense against vacuolar pathogens and describe a mechanism evolved by a bacterial pathogen to counter it. Copyright © 2016 Elsevier Inc. All rights reserved.

Lectures on rare B decays

International Nuclear Information System (INIS)

Masiero, A.

1989-01-01

This paper provides a pedagogical presentation of the class of the rare charmless B decays with strange particles in the final state. These processes involve the flavour changing neutral current transition b → s and, thus, they are GIM suppresses. They constitute a specially promising ground to test the Standard Model and the possible presence of new physics beyond it. In particular I show that the radiative decays b → s + π and b → s + g are priviledged places to look for large supersymmetric enhancements. The author reviews both the present experimental and theoretical situations in this field and point out the challenges that we have to meet in the 90's to have rare B physics play a role comparable to that of rare K physics

Transgenic Citrus Expressing an Arabidopsis NPR1 Gene Exhibit Enhanced Resistance against Huanglongbing (HLB; Citrus Greening).

Science.gov (United States)

Dutt, Manjul; Barthe, Gary; Irey, Michael; Grosser, Jude

2015-01-01

Commercial sweet orange cultivars lack resistance to Huanglongbing (HLB), a serious phloem limited bacterial disease that is usually fatal. In order to develop sustained disease resistance to HLB, transgenic sweet orange cultivars 'Hamlin' and 'Valencia' expressing an Arabidopsis thaliana NPR1 gene under the control of a constitutive CaMV 35S promoter or a phloem specific Arabidopsis SUC2 (AtSUC2) promoter were produced. Overexpression of AtNPR1 resulted in trees with normal phenotypes that exhibited enhanced resistance to HLB. Phloem specific expression of NPR1 was equally effective for enhancing disease resistance. Transgenic trees exhibited reduced diseased severity and a few lines remained disease-free even after 36 months of planting in a high-disease pressure field site. Expression of the NPR1 gene induced expression of several native genes involved in the plant defense signaling pathways. The AtNPR1 gene being plant derived can serve as a component for the development of an all plant T-DNA derived consumer friendly GM tree.

Transgenic Citrus Expressing an Arabidopsis NPR1 Gene Exhibit Enhanced Resistance against Huanglongbing (HLB; Citrus Greening.

Directory of Open Access Journals (Sweden)

Manjul Dutt

Full Text Available Commercial sweet orange cultivars lack resistance to Huanglongbing (HLB, a serious phloem limited bacterial disease that is usually fatal. In order to develop sustained disease resistance to HLB, transgenic sweet orange cultivars 'Hamlin' and 'Valencia' expressing an Arabidopsis thaliana NPR1 gene under the control of a constitutive CaMV 35S promoter or a phloem specific Arabidopsis SUC2 (AtSUC2 promoter were produced. Overexpression of AtNPR1 resulted in trees with normal phenotypes that exhibited enhanced resistance to HLB. Phloem specific expression of NPR1 was equally effective for enhancing disease resistance. Transgenic trees exhibited reduced diseased severity and a few lines remained disease-free even after 36 months of planting in a high-disease pressure field site. Expression of the NPR1 gene induced expression of several native genes involved in the plant defense signaling pathways. The AtNPR1 gene being plant derived can serve as a component for the development of an all plant T-DNA derived consumer friendly GM tree.

Human Exhibitions

DEFF Research Database (Denmark)

Andreassen, Rikke

light on the staging of exhibitions, the daily life of the exhibitees, the wider connections between shows across Europe and the thinking of the time on matters of race, science, gender and sexuality. A window onto contemporary racial understandings, the book presents interviews with the descendants...... of displayed people, connecting the attitudes and science of the past with both our (continued) modern fascination with ‘the exotic’, and contemporary language and popular culture. As such, it will be of interest to scholars of sociology, anthropology and history working in the areas of gender and sexuality...

Structural elucidation and magnetic behavior evaluation of rare earth (La, Nd, Gd, Tb, Dy) doped BaCoNi-X hexagonal nano-sized ferrites

Energy Technology Data Exchange (ETDEWEB)

Majeed, Abdul, E-mail: abdulmajeed2276@gmail.com [Department of Physics, The Islamia University of Bahawalpur, Bahawalpur 63100 (Pakistan); Khan, Muhammad Azhar, E-mail: azhar.khan@iub.edu.pk [Department of Physics, The Islamia University of Bahawalpur, Bahawalpur 63100 (Pakistan); Raheem, Faseeh ur; Hussain, Altaf; Iqbal, F. [Department of Physics, The Islamia University of Bahawalpur, Bahawalpur 63100 (Pakistan); Murtaza, Ghulam [Centre for Advanced Studies in Physics, Government College University, Lahore 54000 (Pakistan); Akhtar, Majid Niaz [Department of Physics, COMSATS Institute of Information Technology, Lahore 54000 (Pakistan); Shakir, Imran [Deanship of Scientific Research, College of Engineering, King Saud University, PO Box 800, Riyadh 11421 (Saudi Arabia); Warsi, Muhammad Farooq [Department of Chemistry, The Islamia University of Bahawalpur, Bahawalpur 63100 (Pakistan)

2016-06-15

Rare-earth (RE=La{sup 3+}, Nd{sup 3+}, Gd{sup 3+}, Tb{sup 3+}, Dy{sup 3+}) doped Ba{sub 2}NiCoRE{sub x}Fe{sub 28−x}O{sub 46} (x=0.25) hexagonal ferrites were synthesized for the first time via micro-emulsion route, which is a fast chemistry route for obtaining nano-sized ferrite powders. These nanomaterials were investigated by X-ray diffraction (XRD), Fourier transform infrared spectroscopy (FTIR), as well as vibrating sample magnetometer (VSM). The XRD analysis exhibited that all the samples crystallized into single X-type hexagonal phase. The crystalline size calculated by Scherrer's formula was found in the range 7–19 nm. The variations in lattice parameters elucidated the incorporation of rare-earth cations in these nanomaterials. FTIR absorption spectra of these X-type ferrites were investigated in the wave number range 500–2400 cm{sup −1.} Each spectrum exhibited absorption bands in the low wave number range, thereby confirming the X-type hexagonal structure. The enhancement in the coercivity was observed with the doping of rare-earth cations. The saturation magnetization was lowered owing to the redistribution of rare-earth cations on the octahedral site (3b{sub VI}). The higher values of coercivity (664–926 Oe) of these nanomaterials suggest their use in longitudinal recording media. - Graphical abstract: Nano-sized rare-earth (RE=La{sup 3+}, Nd{sup 3+}, Gd{sup 3+}, Tb{sup 3+}, Dy{sup 3+}) doped Ba{sub 2}NiCoRE{sub x}Fe{sub 28−x}O{sub 46} (x=0.25) hexagonal ferrites were synthesized for the first time via micro-emulsion route and the crystallite size was found in the range 7–19 nm. The enhancement in the coercivity was observed with the doping of rare-earth cations. The higher values of coercivity (664–926 Oe) of these nanomaterials suggest their use in longitudinal recording media. - Highlights: • Micro-emulsion route was used to synthesize Ba{sub 2}NiCoRE{sub x}Fe{sub 28−x}O{sub 46} ferrites. • The crystallite size was found

Anniversary Exhibition. Nechvolodov.

Directory of Open Access Journals (Sweden)

- -

2006-03-01

Full Text Available On the 10th of August, 2005 in Tartu (the second biggest educational and cultural city in Estonia Stanislav Nechvolodov's exhibition was opened to show the 5-year cycle of his work, traditional for the author and his admirers. At the opening ceremony Nechvolodov said that the exhibition was the last one and appointed on his 70th anniversary.The architectural and building society in Irkutsk remembers Stanislav Nechvolodov as an architect working on dwelling and civil buildings in 1960-70s. Below are some extracts from the Estonian press.«Postimees» newspaper, December 1993. The interview «Expressionistic naturalist, conservative Nechvolodov» by journalist Eric Linnumyagi. He asks about all the details and describes the troubles experienced by Nechvolodov during the perestroika period in Estonia, for example: the Tartu University refused to install the sculpture of Socrat, the art school refused to engage him as an instructor, the sculpture of Socrat moved to Vrotzlav, Poland, and Nechvolodov moved to Poland to read lectures there.«Tartu» newspaper, November 2000. Mats Oun, artist, says in the article «Nechvolodov: a man of Renaissance»: «Nechvolodov works in Estonia, his works are placed in many local and foreign museums. Regardless some insignificant faults, he deserves a high estimation, and his manysided open exhibition can be an example for other artists. He is a man of Renaissance».

Annual research review: Rare genotypes and childhood psychopathology--uncovering diverse developmental mechanisms of ADHD risk.

Science.gov (United States)

Scerif, Gaia; Baker, Kate

2015-03-01

Through the increased availability and sophistication of genetic testing, it is now possible to identify causal diagnoses in a growing proportion of children with neurodevelopmental disorders. In addition to developmental delay and intellectual disability, many genetic disorders are associated with high risks of psychopathology, which curtail the wellbeing of affected individuals and their families. Beyond the identification of significant clinical needs, understanding the diverse pathways from rare genetic mutations to cognitive dysfunction and emotional-behavioural disturbance has theoretical and practical utility. We overview (based on a strategic search of the literature) the state-of-the-art on causal mechanisms leading to one of the most common childhood behavioural diagnoses - attention deficit hyperactivity disorder (ADHD) - in the context of specific genetic disorders. We focus on new insights emerging from the mapping of causal pathways from identified genetic differences to neuronal biology, brain abnormalities, cognitive processing differences and ultimately behavioural symptoms of ADHD. First, ADHD research in the context of rare genotypes highlights the complexity of multilevel mechanisms contributing to psychopathology risk. Second, comparisons between genetic disorders associated with similar psychopathology risks can elucidate convergent or distinct mechanisms at each level of analysis, which may inform therapeutic interventions and prognosis. Third, genetic disorders provide an unparalleled opportunity to observe dynamic developmental interactions between neurocognitive risk and behavioural symptoms. Fourth, variation in expression of psychopathology risk within each genetic disorder points to putative moderating and protective factors within the genome and the environment. A common imperative emerging within psychopathology research is the need to investigate mechanistically how developmental trajectories converge or diverge between and within

Alaska's rare earth deposits and resource potential

Science.gov (United States)

Barker, James C.; Van Gosen, Bradley S.

2012-01-01

Alaska’s known mineral endowment includes some of the largest and highest grade deposits of various metals, including gold, copper and zinc. Recently, Alaska has also been active in the worldwide search for sources of rare earth elements (REE) to replace exports now being limitedby China. Driven by limited supply of the rare earths, combined with their increasing use in new ‘green’ energy, lighting, transportation, and many other technological applications, the rare earth metals neodymium, europium and, in particular, the heavy rare earth elements terbium, dysprosium and yttrium are forecast to soon be in critical short supply (U.S. Department of Energy, 2010).

Creating Virtual Exhibitions for Educational and Cultural Development

Directory of Open Access Journals (Sweden)

Gabriela DUMITRESCU

2014-01-01

Full Text Available The paper presents different tools and mechanisms to implement a virtual exhibition in different cultural areas, such as museums and libraries. Quality characteristics of virtual exhibitions are identified and described. The possibility to create native mobile applications for virtual exhibitions presentation is analyzed. The functional flow of creating a virtual exhibition is presented and discussed. The Seals - History Treasure exhibition is presented and significant historical documents are revealed.

A Rare Form of Corneal Opacity Associated with Spondyloepiphyseal Dysplasia Congenita

Directory of Open Access Journals (Sweden)

Yuichiro Ishida

2018-02-01

Full Text Available A 13-year-old Japanese female diagnosed with spondyloepiphyseal dysplasia congenita (SEDC was referred for ophthalmologic evaluation. Examination with slit-lamp and optical coherence tomography revealed bilateral thin cornea with diffuse corneal opacity which was localised at the posterior stromal depth in the central cornea. Unlike the two previously reported cases of diffuse and nodular patterns of corneal opacity in SEDC, the current case exhibited a rare form of corneal opacity. SEDC is one of the type II collagenopathies, characterised by dwarfism because the mutations in COL2A1 prevent bone growth. Although the existence of type II collagen has not been reported in the human corneal stroma, the aetiology of the opacity in the corneal stroma in SEDC type II collagenopathy is of interest.

Chemiluminescent Diagnostics of Free-Radical Processes in an Abiotic System and in Liver Cells in the Presence of Nanoparticles Based on Rare-Earth Elements nReVO4:Eu3+ (Re = Gd, Y, La) and CeO2

Science.gov (United States)

Averchenko, E. A.; Kavok, N. S.; Klochkov, V. K.; Malyukin, Yu. V.

2014-11-01

We have used luminol-dependent chemiluminescence with Fenton's reagent to study the effect of nanoparticles based on rare-earth elements of different sizes and shapes on free-radical processes in abiotic and biotic cell-free systems, and also in isolated cells in vitro. We have estimated the effects of rare-earth orthovanadate nanoparticles of spherical (GdYVO4:Eu3+, 1-2 nm), spindle-shaped (GdVO4:Eu3+, 25 ×8 nm), and rod-shaped (LaVO4:Eu3+, 57 × (6-8) nm) nanoparticles and spherical CeO2 nanoparticles (sizes 1-2 nm and 8-10 nm). We have shown that in contrast to the abiotic system, in which all types of nanoparticles exhibit antiradical activity, in the presence of biological material, extra-small spherical (1-2 nm) nanoparticles of both types exhibit pro-oxidant activity, and also enhance pro-oxidant induced oxidative stress (for the pro-oxidants hydrogen peroxide and tert-butyl hydroperoxide). The effect of rare-earth orthovanadate spindle and rod shaped nanoparticles in this system was neutral; a moderate antioxidant effect was exhibited by 8-10 nm CeO2 nanoparticles.

Anatomy of hierarchy: Feedforward and feedback pathways in macaque visual cortex

Science.gov (United States)

Markov, Nikola T; Vezoli, Julien; Chameau, Pascal; Falchier, Arnaud; Quilodran, René; Huissoud, Cyril; Lamy, Camille; Misery, Pierre; Giroud, Pascale; Ullman, Shimon; Barone, Pascal; Dehay, Colette; Knoblauch, Kenneth; Kennedy, Henry

2013-01-01

The laminar location of the cell bodies and terminals of interareal connections determines the hierarchical structural organization of the cortex and has been intensively studied. However, we still have only a rudimentary understanding of the connectional principles of feedforward (FF) and feedback (FB) pathways. Quantitative analysis of retrograde tracers was used to extend the notion that the laminar distribution of neurons interconnecting visual areas provides an index of hierarchical distance (percentage of supragranular labeled neurons [SLN]). We show that: 1) SLN values constrain models of cortical hierarchy, revealing previously unsuspected areal relations; 2) SLN reflects the operation of a combinatorial distance rule acting differentially on sets of connections between areas; 3) Supragranular layers contain highly segregated bottom-up and top-down streams, both of which exhibit point-to-point connectivity. This contrasts with the infragranular layers, which contain diffuse bottom-up and top-down streams; 4) Cell filling of the parent neurons of FF and FB pathways provides further evidence of compartmentalization; 5) FF pathways have higher weights, cross fewer hierarchical levels, and are less numerous than FB pathways. Taken together, the present results suggest that cortical hierarchies are built from supra- and infragranular counterstreams. This compartmentalized dual counterstream organization allows point-to-point connectivity in both bottom-up and top-down directions. PMID:23983048

Rare muon processes: Experiment

International Nuclear Information System (INIS)

Walter, H.K.

1998-01-01

The decay properties of muons, especially their rare decays, can be used to study very accurately deviations from the Standard Model. Muons with extremely low energies and good spatial definition are preferred for the majority of such studies. With the upgrade of the 590-MeV ring accelerator, PSI possesses the most powerful cyclotron in the world. This makes it possible to operate high-intensity beams of secondary pions and muons. A short review on rare muon processes is presented, concerning μ-e conversion and muonium-antimuonium oscillations. A possible new search for μ→eγ is also mentioned

Rare thoracic cancers, including peritoneum mesothelioma

NARCIS (Netherlands)

Siesling, Sabine; van der Zwan, Jan Maarten; Izarzugaza, Isabel; Jaal, Jana; Treasure, Tom; Foschi, Roberto; Ricardi, Umberto; Groen, Harry; Tavilla, Andrea; Ardanaz, Eva

Rare thoracic cancers include those of the trachea, thymus and mesothelioma (including peritoneum mesothelioma). The aim of this study was to describe the incidence, prevalence and survival of rare thoracic tumours using a large database, which includes cancer patients diagnosed from 1978 to 2002,

Rare thoracic cancers, including peritoneum mesothelioma

NARCIS (Netherlands)

Siesling, Sabine; Zwan, J.M.V.D.; Izarzugaza, I.; Jaal, J.; Treasure, T.; Foschi, R.; Ricardi, U.; Groen, H.; Tavilla, A.; Ardanaz, E.

2012-01-01

Rare thoracic cancers include those of the trachea, thymus and mesothelioma (including peritoneum mesothelioma). The aim of this study was to describe the incidence, prevalence and survival of rare thoracic tumours using a large database, which includes cancer patients diagnosed from 1978 to 2002,

Sustainability evaluation of essential critical raw materials: cobalt, niobium, tungsten and rare earth elements

Science.gov (United States)

Tkaczyk, A. H.; Bartl, A.; Amato, A.; Lapkovskis, V.; Petranikova, M.

2018-05-01

The criticality of raw materials has become an important issue in recent years. As the supply of certain raw materials is essential for technologically-advanced economies, the European Commission and other international counterparts have started several initiatives to secure reliable and unhindered access to raw materials. Such efforts include the EU Raw Materials Initiative, European Innovation Partnership on Raw Materials, US Critical Materials Institute, and others. In this paper, the authors present a multi-faceted and multi-national review of the essentials for the critical raw materials (CRMs) Co, Nb, W, and rare earth elements (REEs). The selected CRMs are of specific interest as they are considered relevant for emerging technologies and will thus continue to be of increasing major economic importance. This paper presents a ‘sustainability evaluation’ for each element, including essential data about markets, applications and recycling, and possibilities for substitution have been summarized and analysed. All the presented elements are vital for the advanced materials and processes upon which modern societies rely. These elements exhibit superior importance in ‘green’ applications and products subject to severe conditions. The annual production quantities are quite low compared to common industrial metals. Of the considered CRMs, only Co and REE gross production exceed 100 000 t. At the same time, the prices are quite high, with W and Nb being in the range of 60 USD kg‑1 and some rare earth compounds costing almost 4000 USD kg‑1. Despite valiant effort, in practice some of the considered elements are de facto irreplaceable for many specialized applications, at today’s technological level. Often, substitution causes a significant loss of quality and performance. Furthermore, possible candidates for substitution may be critical themselves or available in considerably low quantities. It can be concluded that one preferred approach for the

The exploration of the exhibition informatization

Science.gov (United States)

Zhang, Jiankang

2017-06-01

The construction and management of exhibition informatization is the main task and choke point during the process of Chinese exhibition industry’s transformation and promotion. There are three key points expected to realize a breakthrough during the construction of Chinese exhibition informatization, and the three aspects respectively are adopting service outsourcing to construct and maintain the database, adopting advanced chest card technology to collect various kinds of information, developing statistics analysis to maintain good cutomer relations. The success of Chinese exhibition informatization mainly calls for mature suppliers who can provide construction and maintenance of database, the proven technology, a sense of data security, advanced chest card technology, the ability of data mining and analysis and the ability to improve the exhibition service basing on the commercial information got from the data analysis. Several data security measures are expected to apply during the process of system developing, including the measures of the terminal data security, the internet data security, the media data security, the storage data security and the application data security. The informatization of this process is based on the chest card designing. At present, there are several types of chest card technology: bar code chest card; two-dimension code card; magnetic stripe chest card; smart-chip chest card. The information got from the exhibition data will help the organizers to make relevant service strategies, quantify the accumulated indexes of the customers, and improve the level of the customer’s satisfaction and loyalty, what’s more, the information can also provide more additional services like the commercial trips, VIP ceremonial reception.

Rare beauty and charm decays

International Nuclear Information System (INIS)

Blake, T.

2016-01-01

Rare beauty and charm decays can provide powerful probes of physics beyond the Standard Model. These proceedings summarise the latest measurements of rare beauty and charm decays from the LHCb experiment at the end of Run 1 of the LHC. Whilst the majority of the measurements are consistent with SM predictions, small differences are seen in the rate and angular distribution of b → sℓ"+ℓ"− decay processes.

Rare kaon, muon, and pion decay

International Nuclear Information System (INIS)

Littenberg, L.

1998-01-01

The author discusses the status of and prospects for the study of rare decays of kaons, muons, and pions. Studies of rare kaon decays are entering an interesting new phase wherein they can deliver important short-distance information. It should be possible to construct an alternative unitarity triangle to that determined in the B sector, and thus perform a critical check of the Standard Model by comparing the two. Rare muon decays are beginning to constrain supersymmetric models in a significant way, and future experiments should reach sensitivities which this kind of model must show effects, or become far less appealing

Health activism and the logic of connective action. A case study of rare disease patient organisations.

Science.gov (United States)

Vicari, Stefania; Cappai, Franco

2016-11-01

This exploratory work investigates the role of digital media in expanding health discourse practices in a way to transform traditional structures of agency in public health. By focusing on a sample of rare disease patient organisations as representative of contemporary health activism, this study investigates the role of digital communication in the development of (1) bottom-up sharing and co-production of health knowledge, (2) health public engagement dynamics and (3) health information pathways. Findings show that digital media affordances for patient organisations go beyond the provision of social support for patient communities; they ease one-way, two-way and crowdsourced processes of health knowledge sharing, exchange and co-production, provide personalised routes to health public engagement and bolster the emergence of varied pathways to health information where experiential knowledge and medical authority are equally valued. These forms of organisationally enabled connective action can help the surfacing of personal narratives that strengthen patient communities, the bottom-up production of health knowledge relevant to a wider public and the development of an informational and eventually cultural context that eases patients' political action.

X-ray Absorption Spectroscopy of the Rare Earth orthophosphates

International Nuclear Information System (INIS)

Shuh, D.K.; Terminello, L.J.; Boatner, L.A.; Abraham, M.M.

1993-06-01

X-ray Absorption Spectroscopy (XAS) of the Rare Earth (RE) 3d levels yields sharp peaks near the edges as a result of strong, quasi-atomic 3d 10 4f n → 3d- 9 4f n+1 transitions and these transitions exhibit a wealth of spectroscopic features. The XAS measurements of single crystal REPO 4 (RE = La, Ce, Pr, Nd, Sm, Eu, Gd, Tb, Dy, Er) at the 3d edge were performed in the total yield mode at beam line 8-2 at the Stanford Synchrotron Radiation Laboratory (SSRL). The XAS spectra of the RE ions in the orthophosphate matrix generally resemble the XAS of the corresponding RE metal. This is not unexpected and emphasizes the major contribution of the trivalent state to the electronic transitions at the RE 3d edges. These spectra unequivocally identify the transitions originating from well-characterized RE cores and correlate well with previous theoretical investigations

Krukenberg tumor in a young woman: A rare presentation

Directory of Open Access Journals (Sweden)

Deepa Hatwal

2014-01-01

Full Text Available Krukenberg tumors mostly occur after 40 years. Metastatic ovarian tumors in young age are very rare and reported to be 2% of all the cases. Thirty percent of all ovarian neoplasms occurring during childhood and adolescence are malignant. A 25-year-old woman, parity- 2, presented with abdominal distension, pain in abdomen and amenorrhea. On examination, 18 weeks lump was palpable, firm to hard in consistency, non-tender and mobile. On ultrasonography bilateral ovarian tumors were reported, without any peritoneal free fluid. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Microscopic examination revealed signet ring cells with glandular differentiation, diffusely invading the ovarian parenchyma. Tumor cells exhibited strong, diffuse immunopositivity for CEA with focal strong immunopositivity for CK7 and CK20 and immunonegativity for SATB2. Diagnosis of Krukenberg tumor was made. Endoscopic biopsy confirmed the diagnosis of adenocarcinoma stomach. This case is reported because of its rarity in younger age group.

Digital Natives: Creating Emergent Exhibitions through Digital Technologies

DEFF Research Database (Denmark)

Smith, Rachel Charlotte; Iversen, Ole Sejer; Dindler, Christian

2011-01-01

. In this way, digital technology can contribute to the creation of emergent exhibitions in which the exhibition is created in dialogue between audiences and the museum. We present experiences from a current research project, the Digital Natives exhibition, in which digital technology was designed......Digital Technology can support the creation of dialogical spaces in the museum, both playful and reflective, that allow audiences to engage in the ongoing construction and reproduction of cultural heritage creating novel connections between self and others and between past, present and future...... as an integral part of the exhibition to encourage dialogue between audiences and the exhibition materials and thereby investigate how the exhibition emerge as a result of this dialogic co-construction inside the exhibition space. In short, the opportunities offered by digital technologies prompts us to consider...

Rare event simulation using Monte Carlo methods

CERN Document Server

Rubino, Gerardo

2009-01-01

In a probabilistic model, a rare event is an event with a very small probability of occurrence. The forecasting of rare events is a formidable task but is important in many areas. For instance a catastrophic failure in a transport system or in a nuclear power plant, the failure of an information processing system in a bank, or in the communication network of a group of banks, leading to financial losses. Being able to evaluate the probability of rare events is therefore a critical issue. Monte Carlo Methods, the simulation of corresponding models, are used to analyze rare events. This book sets out to present the mathematical tools available for the efficient simulation of rare events. Importance sampling and splitting are presented along with an exposition of how to apply these tools to a variety of fields ranging from performance and dependability evaluation of complex systems, typically in computer science or in telecommunications, to chemical reaction analysis in biology or particle transport in physics. ...

Artefacts and the performance of an exhibition

DEFF Research Database (Denmark)

Svabo, Connie

2008-01-01

The article explores the role of mediating artefacts in children's encounters with a museum of natural history. Using actor network theory it explores how a specific artefact shapes the way users relate to exhibited objects and how the artefact guides users' movements in the exhibition....... The mediated performance of an exhibition is explored through an empirical case....

Rare earth metals for automotive exhaust catalysts

International Nuclear Information System (INIS)

Shinjoh, Hirohumi

2006-01-01

The usage of rare earth metals for automotive exhaust catalysts is demonstrated in this paper. Rare earth metals have been widely used in automotive catalysts. In particular, three-way catalysts require the use of ceria compounds as oxygen storage materials, and lanthana as both a stabilizer of alumina and a promoter. The application for diesel catalysts is also illustrated. Effects of inclusion of rare earth metals in automotive catalysts are discussed

A content-oriented model for science exhibit engineering

DEFF Research Database (Denmark)

Achiam, Marianne

2013-01-01

Recently, science museums have begun to review their educational purposes and redesign their pedagogies. At the most basic level, this entails accounting for the performance of individual exhibits, and indeed, in some cases, research indicates shortcomings in exhibit design: While often successful......: as a means to operationalize the link between exhibit features and visitor activities; and as a template to transform scientists’ practices in the research context into visitors’ activities in the exhibit context. The resulting model of science exhibit engineering is presented and exemplified, and its...... implications for science exhibit design are discussed at three levels: the design product, the design process, and the design methodology....

Sintered cobalt-rare earth intermetallic product

International Nuclear Information System (INIS)

Benz, M.G.

1975-01-01

This patent describes a sintered product having substantially stable permanent magnet properties in air at room temperature. It comprises compacted particulate cobalt--rare earth alloy consisting essentially of a Co 5 R intermetallic phase and a CoR intermetallic phase which is richer in rare earth metal content than the Co 5 R phase, where R is a rare earth metal. The Co 5 R intermetallic phase is present in an amount of at least 65 percent by weight of the sintered product and the CoR intermetallic phase which is richer in rare earth metal content than the Co 5 R phase is present in a positive amount having a value ranging up to about 35 percent by weight of the product. The sintered product has a density of at least 87 percent and has pores which are substantially noninterconnecting and wherein the component grains have an average size less than 30 microns

France at CERN – Industrial exhibition

CERN Multimedia

FP Department

2012-01-01

Industrial Exhibition Administration Building Bldg 61 – 1st Floor Tuesday 27 March: 9 a.m. – 5.30 p.m. Wednesday 28 March: 9 a.m. – 2 p.m.   About thirty French companies are presenting their latest technological advances during the industrial exhibition "France at CERN", featuring products and technologies specifically related to CERN activities. Individual B2B meetings can be organized with the sales and technical representatives of participating firms and will take place at either the companies’ exhibition stands or in conference rooms in the Main Building. Individuals wishing to make contact with one or more companies must use the contact details available from each secretariat of department or by using this link. B2B meetings will be coordinated by UBIFRANCE. You will also find the list of exhibiting and participating companies online here. This event is sponsored by the French subsidiary of RS Components, the most important distri...

Study of the oxides nature effect of rare and rare earth elements on the aluminium-chromium catalyst properties

International Nuclear Information System (INIS)

Dadashev, B.A.; Abbasov, S.G.; Sarydzhanov, A.A.; AN Azerbajdzhanskoj SSR, Baku. Inst. Neftekhimicheskikh Protsessov)

1975-01-01

Adsorption studies have shown that oxides of rare and rare earth elements REE appreciably influence the structure of aluminium-chrome catalyst. Alkaline promotors, unlike REE, contribute to the formation of developed contact surface. Electrophysical investigations show that oxides of rare elements introduced into the catalyst increase its conductivity and activation energy. As for REE oxides, they decrease the conductivity and increase the activation energy. Catalysts with developed surface and high conductivity are also more active in the reaction of isopentane dehydration

Energy of solution of rare gases in metals; Energie de dissolution des gaz rares dans les metaux

Energy Technology Data Exchange (ETDEWEB)

Blin, J. [Commissariat a l' Energie Atomique, Saclay (France).Centre d' Etudes Nucleaires

1959-07-01

In order to calculate the energy of solution of rare gases in metals, a method which has given good results in the case of solid solutions in metals has been applied. Nevertheless, it was necessary for this, to know the compressibility of gases under conditions which are not feasible in a laboratory. H. Jensen has studied this compressibility in a precise way for the rare gases Ar, Kr, Xe. It has thus been possible to calculate the energy of solution of these gases in different metals. These calculations have been carried out most thoroughly for the case of uranium. (author) [French] Nous avons applique au calcul de l'energie de dissolution des gaz rares dans les metaux, une methode qui a donne de bons resultats dans le cas des solutions solides metalliques. Il fallait pour cela connaitre la compressibilite des gaz rares dans des conditions impossibles a realiser en laboratoire. Cette compressibilite a ete etudiee par H. Jensen de facon precise pour les gaz rares A, Kr, Xe. Nous avons pu, de ce fait, calculer les energies de dissolution de ces gaz dans les differents metaux. Les calculs ont ete faits plus completement dans le cas de l'uranium. (auteur)

Energy of solution of rare gases in metals; Energie de dissolution des gaz rares dans les metaux

Energy Technology Data Exchange (ETDEWEB)

Blin, J [Commissariat a l' Energie Atomique, Saclay (France).Centre d' Etudes Nucleaires

1959-07-01

In order to calculate the energy of solution of rare gases in metals, a method which has given good results in the case of solid solutions in metals has been applied. Nevertheless, it was necessary for this, to know the compressibility of gases under conditions which are not feasible in a laboratory. H. Jensen has studied this compressibility in a precise way for the rare gases Ar, Kr, Xe. It has thus been possible to calculate the energy of solution of these gases in different metals. These calculations have been carried out most thoroughly for the case of uranium. (author) [French] Nous avons applique au calcul de l'energie de dissolution des gaz rares dans les metaux, une methode qui a donne de bons resultats dans le cas des solutions solides metalliques. Il fallait pour cela connaitre la compressibilite des gaz rares dans des conditions impossibles a realiser en laboratoire. Cette compressibilite a ete etudiee par H. Jensen de facon precise pour les gaz rares A, Kr, Xe. Nous avons pu, de ce fait, calculer les energies de dissolution de ces gaz dans les differents metaux. Les calculs ont ete faits plus completement dans le cas de l'uranium. (auteur)

Temporary and Travelling Exhibitions. Museums and Monuments, X.

Science.gov (United States)

Daifuku, Hiroshi; And Others

The permanent exhibition, the most typical form of museum exhibition, has failed to attract repeated visitation, since visitors quickly become familiar with the objects shown. The temporary exhibition evolved as a result for the need of repeated visitation. The temporary exhibition, set up for a period of one to six months, introduces fresh…

Omphalocele with Dextrocardia - A Rare Association

OpenAIRE

Vikal Chandra Shakya; C S Agrawal; N R Shrestha; K Dhungel; S Adhikary

2009-01-01

Omphalocele is frequently associated with many other congenital malformations. In cardiac anomalies, association of omphalocele with dextrocardia has been rarely noticed before. We present here a child with dextrocardia and omphalocele alongwith a brief review of the literature on this rare association. Key Words: congenital malformations, dextrocardia, omphalocele

Rare earth impact on glass structure and alteration kinetics

International Nuclear Information System (INIS)

Molieres, E.

2012-01-01

This work is related to the question of the geological deep repository of high-level waste glass. These wastes include fission products and minor actinides, elements which can be simulated by rare earths. As new glass compositions could enable increased rare earth concentrations, it is crucial to know and understand rare earth impact on glass structure on the one hand, and on glass alteration kinetics or their incorporation into an altered layer. This work studied simplified borosilicate glasses in order to limit synergetic effects between rare earths and other elements. Various complementary techniques were used to characterize pristine and altered glasses (solid-high resolution NMR, Raman spectroscopy, fluorescence, SIMS, SAXS). Firstly, the structural role of a rare earth is discussed and is compared to a calcium cation. The local environment of rare earths is also probed. Secondly, rare earth (nature and concentration) impact on several alteration regimes was studied (initial rate, rate drop). Then, after alteration, rare earth elements being retained within the altered layer, the structural impact of rare earth elements (and their local environment) in this alteration layer was also investigated. (author) [fr

Periosteal chondrosarcoma: a histopathological and molecular analysis of a rare chondrosarcoma subtype.

Science.gov (United States)

Cleven, Arjen H G; Zwartkruis, Evita; Hogendoorn, Pancras C W; Kroon, Herman M; Briaire-de Bruijn, Inge; Bovée, Judith V M G

2015-10-01

Periosteal chondrosarcoma is a rare, malignant cartilage-forming neoplasm originating from the periosteal surface of bone. We collected 38 cases from the archives of the Netherlands Committee on Bone Tumours, with the aim of studying histological features and evaluating the involvement of isocitrate dehydrogenase 1 (IDH1), EXT, Wnt/β-catenin, the pRB pathway (CDK4 and p16), and the TP53 pathway (p53 and MDM2). Histology showed a moderately cellular matrix with mucoid-myxoid changes and, in 42% of cases, formation of a neocortex. Occasional intramedullary extension (26%) and subsequent host bone entrapment (40%) were seen. Histological grading revealed grade 1 (53%) and grade 2 (45%). The EXT1 protein was normally expressed, and mutations in IDH1 were observed in only 15% of cases. pRb signalling was deregulated by loss of p16 expression in 50% of cases, and Wnt signalling was lost in 89%. No alterations were found in CDK4, p53, or MDM2. We report the first large histological and molecular study on periosteal chondrosarcoma showing that histopathological examination and molecular aberrations do not predict prognosis. Although the mutation frequency of IDH1 was low, we confirm the supposed relationship with central chondrosarcoma. Moreover, we identify loss of canonical Wnt signalling and deregulation of pRb signalling as possible events contributing to its histogenesis. © 2015 John Wiley & Sons Ltd.

CERN Industrials Exhibitions - Over 30 Years of Tradition

CERN Multimedia

2001-01-01

Industrial exhibitions have been part of the CERN landscape for 33 years. At least once each year several companies from the same country come to CERN to present their products and services. Now, a new scheme of one-day visits is giving potential exhibitors at CERN a lighter option. The United Kingdom inaugurated the Industrial Exhibitions in 1968, and it wasn't till 1971 that other countries staged exhibitions at CERN. This photo was taken in 1969, at the second British exhibition, where 16 companies were present. Four years after joining CERN, Poland inaugurated its first exhibition at CERN in 1995 in the presence of the former Director-General Chris Llewellyn-Smith. Almost all the Member States have held industrial exhibitions at the Organization. May '68 wasn't only revolutionary in Paris. For the very first time, an industrial exhibition took place at CERN. Great Britain was first to come with eight companies and remains until this day the most devoted country to the programme with 17 exhibitions and ...

Distribution of rare earths in liver of mice administered with chloride compounds of 12 rare earths

International Nuclear Information System (INIS)

Shinohara, A.; Chiba, M.; Inaba, Y.

1998-01-01

Full text: Rare earths are used in high technology field, however, the information on their biological effects are not sufficient. The behaviour of rare earths in biology is of interest in connection with their toxicity. In the present study, the distribution of rare earths in liver of mice administered with these elements was investigated. The effects on Ca and other biological essential elements were also determined. Male mice (5 weeks old) were injected with one of 12 kinds of rare earths (chlorides of Y, La, Ce, Nd, Sm, Eu, Gd, Tb, Dy, Ho, Er and Yb) at the dose of 25 mg/KXg body weight. After 20 hours of administration, mice were sacrificed, then liver and other organs were taken out. Liver was homogenized and separated by centrifugation. The concentrations of rare earths administered were measured by microwave-induced plasma-mass spectrometry (MIP-MS) after acid digestion. The concentrations of administered elements in whole liver were about 100μg/g (wet weight), where the difference between elements was few. Distribution amounts of elements administered in four fractions were following order; 700μg precipitate > mitocondrial fraction > microsomal fraction > cytosol. The relative contents in these fractions, however, was different depending on the element administered. Calcium concentrations in liver of administered mice were higher than those of control mice. Increase of Ca concentrations were observed in all four fractions and the increase ratio was also dependent on the elements administered

Expert services for rare anaemias across Europe

OpenAIRE

Beatrice Gulbis

2013-01-01

New challenges and priorities are given in the EU Health programme 2007-2013. The objectives of the programme are to improve citizens’ health security, to promote health to improve prosperity and solidarity, and to generate and disseminate health knowledge. If challenges and priorities have been defined globally for rare diseases by the European Commission, persons involved in rare anaemias have taken the opportunity to contribute to the empowerment of patients with rare anaemias. One o...

Artemisinic acid exhibits antitumor activity in MCF-7 breast cancer cells through the inhibition of angiogenesis, VEGF, m-TOR and AKT signalling pathways

Directory of Open Access Journals (Sweden)

Yan Cui

2016-09-01

Full Text Available The aim of the present study was to evaluate the antitumor and anti-angiogenic effects of artemisinic acid in MCF-7 human breast cancer cells. Various cell signalling pathways (VEGF, m-TOR and AKT signalling pathways and MTT assay were used. The in vivo antitumor activity of artemisinic acid was evaluated by means of tumor xenograft mouse model. Transwell cell migration assay was used to examine the chemotactic motility of the human umbilical vascular endothelial cells (HUVECs, while as endothelial cell capillary-like tube formation assay was used to evaluate the effect of artemisinic acid on the tube formation in HUVECs. We found that artemisinic acid considerably reduced both the volume and weight of concrete tumors and reduced angiogenesis in a xenograft mouse tumor model in vivo. Further, artemisinic acid suppressed the VEGF-induced cell migration and capillary-like tube formation of HUVECs in a dose-dependent manner. Artemisinic acid was found to suppress the VEGF-induced phosphorylation of VEGFR2 and also the activity of AKT and m-TOR.

Drosophila mutants of the autism candidate gene neurobeachin (rugose) exhibit neuro-developmental disorders, aberrant synaptic properties, altered locomotion, and impaired adult social behavior and activity patterns.

Science.gov (United States)

Wise, Alexandria; Tenezaca, Luis; Fernandez, Robert W; Schatoff, Emma; Flores, Julian; Ueda, Atsushi; Zhong, Xiaotian; Wu, Chun-Fang; Simon, Anne F; Venkatesh, Tadmiri

2015-01-01

Autism spectrum disorder (ASD) is a neurodevelopmental disorder in humans characterized by complex behavioral deficits, including intellectual disability, impaired social interactions, and hyperactivity. ASD exhibits a strong genetic component with underlying multigene interactions. Candidate gene studies have shown that the neurobeachin (NBEA) gene is disrupted in human patients with idiopathic autism ( Castermans et al., 2003 ). The NBEA gene spans the common fragile site FRA 13A and encodes a signal scaffold protein ( Savelyeva et al., 2006 ). In mice, NBEA has been shown to be involved in the trafficking and function of a specific subset of synaptic vesicles. ( Medrihan et al., 2009 ; Savelyeva et al., 2006 ). Rugose (rg) is the Drosophila homolog of the mammalian and human NBEA. Our previous genetic and molecular analyses have shown that rg encodes an A kinase anchor protein (DAKAP 550), which interacts with components of the epidermal growth factor receptor or EGFR and Notch-mediated signaling pathways, facilitating cross talk between these and other pathways ( Shamloula et al., 2002 ). We now present functional data from studies on the larval neuromuscular junction that reveal abnormal synaptic architecture and physiology. In addition, adult rg loss-of-function mutants exhibit defective social interactions, impaired habituation, aberrant locomotion, and hyperactivity. These results demonstrate that Drosophila NBEA (rg) mutants exhibit phenotypic characteristics reminiscent of human ASD and thus could serve as a genetic model for studying ASDs.

Rare earth industries: Upstream business

International Nuclear Information System (INIS)

2011-01-01

Evidently, many factors contribute to the rush to invest in the unprecedented revival of rare earths. One major reason has to do with the rapidly growing world demand. The other reason relates to the attractive price of rare earths which is projected to stay strong in the coming years. This is because supply is predicted to have difficulty keeping pace with demand. Experts believe a major driver of global rare earths demand is the forecasted expansion in the green economy. Climate change is a major driver of the green economy. With climate change, there is concern that the uncontrolled emission of the greenhouse gases, especially carbon dioxide, can lead to catastrophic consequences for the world. This has been documented in countless studies and reports. Another important driver of the green economy is the growing shortfall in many resources. The world is now experiencing declines in key resources to meet a growing global demand. With more than 6 billion people now in the world and growing, the pressure exerted on global resources including energy, water and food is a major concern. Recent demand surge in China and India has dented the supply position of major world resources. The much quoted Stern Report from the UK has warned that, unless immediate steps are taken to reduce greenhouse gas emissions, it may be a costly exercise to undertake the corrections later. Since energy use, especially fossil fuels, is a major contributor to climate change, greener options are being sought. Add to that the fact that the fossil energy resources of the world are declining, the need to seek alternatives becomes even more urgent. One option is to change to renewable energy sources. These include such potentials as solar, wind and biomass. Rare earths have somehow become a critical feature of the technologies in such renewable. Another option is to improve the efficient use of energy in transport, buildings and all the other energy intensive industries. Again the technologies in

RARE BRANCHIAL ARCH ANOMALIES

Directory of Open Access Journals (Sweden)

Jayanta Kumar

2016-03-01

Full Text Available AIM Amongst the branchial arch anomalies third arch anomaly occurs rarely and more so the fourth arch anomalies. We present our experience with cases of rare branchial arch anomalies. PATIENTS AND METHODS From June 2006 to January 2016, cases having their external opening in the lower third of sternocleidomastoid muscle with the tract going through thyroid gland and directing to pyriform sinus (PFS or cysts with internal opening in the PFS were studied. RESULTS No fourth arch anomaly was encountered. One cyst with internal opening which later on formed a fistula, three fistulae from beginning and two sinuses were encountered. The main stay of diagnosis was the fistula in the PFS and the tract lying posterior to the internal carotid artery. Simple excision technique with a small incision around the external opening was done. There was no recurrence. CONCLUSION Third arch fistula is not very rare as it was thought. Internal fistula is found in most of the cases. Though radiological investigations are helpful, fistulae can be diagnosed clinically and during operation. Extensive operation of the neck, mediastinum and pharynx is not required.

Memory and Mourning: An Exhibit History

Science.gov (United States)

Eberle, Scott G.

2005-01-01

Mounted by the Strong Museum in Rochester, New York, in 1993, and traveling nationally thereafter, the exhibit Memory and Mourning provided historical and contemporary perspectives to help museum guests explore their own reactions to loss and grief. In the process the exhibit's development team encountered a range of philosophical, historical,…

Rare (Earth Elements [score

Directory of Open Access Journals (Sweden)

Camilo Méndez

2014-12-01

Full Text Available Rare (Earth Elements is a cycle of works for solo piano. The cycle was inspired by James Dillon’s Book of Elements (Vol. I-V. The complete cycle will consist of 14 pieces; one for each selected rare (earth element. The chosen elements are Neodymium, Erbium, Tellurium, Hafnium, Tantalum, Technetium, Indium, Dysprosium, Lanthanium, Cerium, Europium, Terbium, Yttrium and Darmstadtium. These elements were selected due to their special atomic properties that in many cases make them extremely valuable for the development of new technologies, and also because of their scarcity. To date, only 4 works have been completed Yttrium, Technetium, Indium and Tellurium.

Low-grade central osteosarcoma in proximal humerus: a rare entity

Directory of Open Access Journals (Sweden)

Tang F

2017-10-01

Full Text Available Fan Tang,1,2 Li Min,1,2 Yong Zhou,1 Yi Luo,1 Chongqi Tu1 1Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, People’s Republic of China; 2Sarcoma Biology Laboratory, Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital, Boston, MA, USA Abstract: Low-grade central osteosarcoma is a rare subtype of tumor with low-grade malignancy. Currently, wide resection with negative resection margin is the standard treatment for this disease. The role of neoadjuvant chemotherapy in low-grade central osteosarcoma was controversial and was mostly considered for tumors containing high-grade focal areas. Local tumor recurrences often exhibited a tumor with higher histologic grade or differentiation with the potential for metastases. In low-grade central osteosarcoma, timely wide resection after definite diagnosis can result in 5-year survival for almost 90%. However, the relatively nonspecific radiological and pathological findings make diagnosis very difficult. MDM2 and CDK4 are specific and provide sensitive markers for the diagnosis of low-grade central osteosarcoma, helping to differentiate low-grade central osteosarcoma from some benign lesions, including fibrous dysplasia, bone giant cell tumor, and chondrosarcoma. Here, we report the case of a 19-year-old woman with low-grade central osteosarcoma located at the proximal humerus. The affected site was rare, but the sensitive biomarkers CDK4 and MDM2 were positive. The patient recovered well after wide tumor resection following a proximal humerus endoprosthesis replacement. Our case highlighted the management strategies in low-grade central osteosarcoma. Being familiar with radiographic features, understanding the biological characteristics, and mastering diagnostic biomarkers can help oncologists avoid embarrassing situations in treatment when this rare tumor is highly suspected, even when located at an uncommon site. The discussion in this report

Precipitation of the rare earth double sodium and rare earths from the sulfuric liquor and the conversion into rare earth hydroxides through meta ethic reaction

International Nuclear Information System (INIS)

Abreu, Renata D.; Oliveira, Ester F.; Brito, Walter de; Morais, Carlos A.

2007-01-01

This work presents the purification study of the rare earths through precipitation of rare earth and sodium (Na TR (SO 4 ) 2 . x H 2 O)) double sulfate and his conversion to rare earths hydroxide TR(OH) 3 by meta ethic reaction through the addition of sodium hydroxide solution to the solid double sulfate. The study used the sulfuric liquor as rare earth sample, generated in the chemical processing of the monazite with sulfuric acid by the Industrias Nucleares do Brasil - INB, Brazil, after the thorium and uranium extraction. The work investigated the influence of the main variables involved in the precipitation of Na TR(SO 4 ) 2 .xH 2 O and in the conversion for the TR(OH) 3 , as follows: type and excess of the precipitation agent, temperature and time reaction. The obtained solid composites were characterized by X-ray diffraction, infrared and chemical analysis. The double sulfate diffractogram indicated the Na TR(SO 4 ) 2 mono-hydrated. The characterization of the metatese products has shown that, for obtaining the complete conversion of NaTR(SO 4 ) 2 .H 2 O into TR(OH) 3 , the reaction must be hot processed (∼70 deg C) and with small excess of Na OH (≤ 5 percent). (author)

DMPD: Regulatory pathways in inflammation. [Dynamic Macrophage Pathway CSML Database

Lifescience Database Archive (English)

Full Text Available 17967718 Regulatory pathways in inflammation. Mantovani A, Garlanda C, Locati M, Ro....html) (.csml) Show Regulatory pathways in inflammation. PubmedID 17967718 Title Regulatory pathways in infl

First-Year Engineering Students' Portrayal of Engineering in a Proposed Museum Exhibit for Middle School Students

Science.gov (United States)

Mena, Irene B.; Diefes-Dux, Heidi A.

2012-04-01

Students' perceptions of engineering have been documented through studies involving interviews, surveys, and word associations that take a direct approach to asking students about various aspects of their understanding of engineering. Research on perceptions of engineering rarely focuses on how students would portray engineering to others. First-year engineering student teams proposed a museum exhibit, targeted to middle school students, to explore the question "What is engineering?" The proposals took the form of a poster. The overarching research question focuses on how these students would portray engineering to middle school students as seen through their museum exhibit proposals. A preliminary analysis was done on 357 posters to determine the overall engineering themes for the proposed museum exhibits. Forty of these posters were selected and, using open coding, more thoroughly analyzed to learn what artifacts/objects, concepts, and skills student teams associate with engineering. These posters were also analyzed to determine if there were any differences by gender composition of the student teams. Building, designing, and teamwork are skills the first-year engineering students link to engineering. Regarding artifacts, students mentioned those related to transportation and structures most often. All-male teams were more likely to focus on the idea of space and to mention teamwork and designing as engineering skills; equal-gender teams were more likely to focus on the multidisciplinary aspect of engineering. This analysis of student teams' proposals provides baseline data, positioning instructors to develop and assess instructional interventions that stretch students' self-exploration of engineering.

Sotos syndrome is associated with deregulation of the MAPK/ERK-signaling pathway.

Directory of Open Access Journals (Sweden)

Remco Visser

Full Text Available Sotos syndrome (SoS is characterized by tall stature, characteristic craniofacial features and mental retardation. It is caused by haploinsufficiency of the NSD1 gene. In this study, our objective was to identify downstream effectors of NSD1 and to map these effectors in signaling pathways associated with growth. Genome-wide expression studies were performed on dermal fibroblasts from SoS patients with a confirmed NSD1 abnormality. To substantiate those results, phosphorylation, siRNA and transfection experiments were performed. A significant association was demonstrated with the Mitogen-Activated Protein Kinase (MAPK pathway. Members of the fibroblast growth factor family such as FGF4 and FGF13 contributed strongly to the differential expression in this pathway. In addition, a diminished activity state of the MAPK/ERK pathway was demonstrated in SoS. The Ras Interacting Protein 1 (RASIP1 was identified to exhibit upregulated expression in SoS. It was shown that RASIP1 dose-dependently potentiated bFGF induced expression of the MAPK responsive SBE reporter providing further support for a link between NSD1 and the MAPK/ERK signaling pathway. Additionally, we demonstrated NSD1 expression in the terminally differentiated hypertrophic chondrocytes of normal human epiphyseal growth plates. In short stature syndromes such as hypochondroplasia and Noonan syndrome, the activation level of the FGF-MAPK/ERK-pathway in epiphyseal growth plates is a determining factor for statural growth. In analogy, we propose that deregulation of the MAPK/ERK pathway in SoS results in altered hypertrophic differentiation of NSD1 expressing chondrocytes and may be a determining factor in statural overgrowth and accelerated skeletal maturation in SoS.

A rare cause of hemoptysis

Directory of Open Access Journals (Sweden)

Meghan Aversa

2014-01-01

Full Text Available Angiosarcomas are rare, malignant, endothelial-cell tumors of vascular origin that can arise at any body site. They frequently metastasize to the lung, heralded by dyspnea, hemoptysis, chest pain, pneumothoraces, and diffuse pulmonary hemorrhage. However, in most cases lung metastases are discovered after the diagnosis of a primary angiosarcoma has already been established. Very rarely will an undiagnosed metastatic angiosarcoma present as diffuse pulmonary hemorrhage. We describe the case of a 59-year-old male who presented to hospital with dyspnea and hemoptysis. CT chest revealed rapidly progressing nodular changes and broncho-alveolar lavage returns were progressively bloody. Open lung wedge biopsy ultimately revealed metastatic angiosarcoma and extensive pulmonary hemorrhage. Our case highlights the key clinical, radiological, and pathological features of this rare malignancy that frequently metastasizes to the lung and reminds clinicians to consider it as a cause of hemoptysis and pulmonary hemorrhage.

Teaching Biochemical Pathways Using Concept Maps

Directory of Open Access Journals (Sweden)

Simon Brown

2013-08-01

expected to help and, eventually, everyone will be the volunteer. Thereafter, I act as moderator, provide support to any nervous students and, very rarely, offer some clarification, correction or suggestion. The sessions are very lively and highly productive. We usually spend one week going over the details of the reactions in all the pathways we have considered, and in the next tutorial we generate a simpler outline of the reactions and superimpose the regulation of the pathways. In addition to the generation of a coherent overview of the pathways and processes, the advantages of this approach include instant feedback on the suggestions made by students (often provided by other students; real discussion of the biochemistry; identification of particular points of difficulty; the voluntary inclusion of almost every student, which gives me some indication of those who might be struggling; and laughter. The feedback is consistently positive and students actually ask when the sessions are going to happen as we approach the end of semester. A similar approach works just as well when summarizing a semester’s work on cell and molecular biology.

e-ENERCA: telemedicine platform for rare anaemias

OpenAIRE

Béatrice Gulbis; María del Mar Mañú Pereira; Emilio J. Armaza Armaza; Pilar Nicolás; for the ENERCA working group

2014-01-01

The creation of a telemedicine, tele-expertise platform opens a new challenge within the European Network for Rare and Congenital Anaemias (ENERCA; www.enerca.org). This is a cornerstone in the field of rare anaemias, in which national expertise is usually scarce and a significant number of patients remain undiagnosed. Experts in rare diseases are specially needed of shared knowledge platforms offering the possibility of a faster and more accurate diagnosis and the availability of a better pa...

Rare earth - no case for government intervention

OpenAIRE

Georg Zachmann

2010-01-01

China has officially restricted exports of rare earth for several years and announced this year it will further tighten exports. Rare earth is a group of 17 different metals, usually found clustered together. These metals have hundreds of different industry applications. For example, they are used in certain high capacity magnets, batteries and lasers. As the rare earth elements are used in sectors that are assumed to have an over-proportionate growth potential (eg. green-technology), policy ...

Protein-altering variants associated with body mass index implicate pathways that control energy intake and expenditure in obesity.

Science.gov (United States)

Turcot, Valérie; Lu, Yingchang; Highland, Heather M; Schurmann, Claudia; Justice, Anne E; Fine, Rebecca S; Bradfield, Jonathan P; Esko, Tõnu; Giri, Ayush; Graff, Mariaelisa; Guo, Xiuqing; Hendricks, Audrey E; Karaderi, Tugce; Lempradl, Adelheid; Locke, Adam E; Mahajan, Anubha; Marouli, Eirini; Sivapalaratnam, Suthesh; Young, Kristin L; Alfred, Tamuno; Feitosa, Mary F; Masca, Nicholas G D; Manning, Alisa K; Medina-Gomez, Carolina; Mudgal, Poorva; Ng, Maggie C Y; Reiner, Alex P; Vedantam, Sailaja; Willems, Sara M; Winkler, Thomas W; Abecasis, Gonçalo; Aben, Katja K; Alam, Dewan S; Alharthi, Sameer E; Allison, Matthew; Amouyel, Philippe; Asselbergs, Folkert W; Auer, Paul L; Balkau, Beverley; Bang, Lia E; Barroso, Inês; Bastarache, Lisa; Benn, Marianne; Bergmann, Sven; Bielak, Lawrence F; Blüher, Matthias; Boehnke, Michael; Boeing, Heiner; Boerwinkle, Eric; Böger, Carsten A; Bork-Jensen, Jette; Bots, Michiel L; Bottinger, Erwin P; Bowden, Donald W; Brandslund, Ivan; Breen, Gerome; Brilliant, Murray H; Broer, Linda; Brumat, Marco; Burt, Amber A; Butterworth, Adam S; Campbell, Peter T; Cappellani, Stefania; Carey, David J; Catamo, Eulalia; Caulfield, Mark J; Chambers, John C; Chasman, Daniel I; Chen, Yii-Der I; Chowdhury, Rajiv; Christensen, Cramer; Chu, Audrey Y; Cocca, Massimiliano; Collins, Francis S; Cook, James P; Corley, Janie; Corominas Galbany, Jordi; Cox, Amanda J; Crosslin, David S; Cuellar-Partida, Gabriel; D'Eustacchio, Angela; Danesh, John; Davies, Gail; Bakker, Paul I W; Groot, Mark C H; Mutsert, Renée; Deary, Ian J; Dedoussis, George; Demerath, Ellen W; Heijer, Martin; Hollander, Anneke I; Ruijter, Hester M; Dennis, Joe G; Denny, Josh C; Di Angelantonio, Emanuele; Drenos, Fotios; Du, Mengmeng; Dubé, Marie-Pierre; Dunning, Alison M; Easton, Douglas F; Edwards, Todd L; Ellinghaus, David; Ellinor, Patrick T; Elliott, Paul; Evangelou, Evangelos; Farmaki, Aliki-Eleni; Farooqi, I Sadaf; Faul, Jessica D; Fauser, Sascha; Feng, Shuang; Ferrannini, Ele; Ferrieres, Jean; Florez, Jose C; Ford, Ian; Fornage, Myriam; Franco, Oscar H; Franke, Andre; Franks, Paul W; Friedrich, Nele; Frikke-Schmidt, Ruth; Galesloot, Tessel E; Gan, Wei; Gandin, Ilaria; Gasparini, Paolo; Gibson, Jane; Giedraitis, Vilmantas; Gjesing, Anette P; Gordon-Larsen, Penny; Gorski, Mathias; Grabe, Hans-Jörgen; Grant, Struan F A; Grarup, Niels; Griffiths, Helen L; Grove, Megan L; Gudnason, Vilmundur; Gustafsson, Stefan; Haessler, Jeff; Hakonarson, Hakon; Hammerschlag, Anke R; Hansen, Torben; Harris, Kathleen Mullan; Harris, Tamara B; Hattersley, Andrew T; Have, Christian T; Hayward, Caroline; He, Liang; Heard-Costa, Nancy L; Heath, Andrew C; Heid, Iris M; Helgeland, Øyvind; Hernesniemi, Jussi; Hewitt, Alex W; Holmen, Oddgeir L; Hovingh, G Kees; Howson, Joanna M M; Hu, Yao; Huang, Paul L; Huffman, Jennifer E; Ikram, M Arfan; Ingelsson, Erik; Jackson, Anne U; Jansson, Jan-Håkan; Jarvik, Gail P; Jensen, Gorm B; Jia, Yucheng; Johansson, Stefan; Jørgensen, Marit E; Jørgensen, Torben; Jukema, J Wouter; Kahali, Bratati; Kahn, René S; Kähönen, Mika; Kamstrup, Pia R; Kanoni, Stavroula; Kaprio, Jaakko; Karaleftheri, Maria; Kardia, Sharon L R; Karpe, Fredrik; Kathiresan, Sekar; Kee, Frank; Kiemeney, Lambertus A; Kim, Eric; Kitajima, Hidetoshi; Komulainen, Pirjo; Kooner, Jaspal S; Kooperberg, Charles; Korhonen, Tellervo; Kovacs, Peter; Kuivaniemi, Helena; Kutalik, Zoltán; Kuulasmaa, Kari; Kuusisto, Johanna; Laakso, Markku; Lakka, Timo A; Lamparter, David; Lange, Ethan M; Lange, Leslie A; Langenberg, Claudia; Larson, Eric B; Lee, Nanette R; Lehtimäki, Terho; Lewis, Cora E; Li, Huaixing; Li, Jin; Li-Gao, Ruifang; Lin, Honghuang; Lin, Keng-Hung; Lin, Li-An; Lin, Xu; Lind, Lars; Lindström, Jaana; Linneberg, Allan; Liu, Ching-Ti; Liu, Dajiang J; Liu, Yongmei; Lo, Ken S; Lophatananon, Artitaya; Lotery, Andrew J; Loukola, Anu; Luan, Jian'an; Lubitz, Steven A; Lyytikäinen, Leo-Pekka; Männistö, Satu; Marenne, Gaëlle; Mazul, Angela L; McCarthy, Mark I; McKean-Cowdin, Roberta; Medland, Sarah E; Meidtner, Karina; Milani, Lili; Mistry, Vanisha; Mitchell, Paul; Mohlke, Karen L; Moilanen, Leena; Moitry, Marie; Montgomery, Grant W; Mook-Kanamori, Dennis O; Moore, Carmel; Mori, Trevor A; Morris, Andrew D; Morris, Andrew P; Müller-Nurasyid, Martina; Munroe, Patricia B; Nalls, Mike A; Narisu, Narisu; Nelson, Christopher P; Neville, Matt; Nielsen, Sune F; Nikus, Kjell; Njølstad, Pål R; Nordestgaard, Børge G; Nyholt, Dale R; O'Connel, Jeffrey R; O'Donoghue, Michelle L; Olde Loohuis, Loes M; Ophoff, Roel A; Owen, Katharine R; Packard, Chris J; Padmanabhan, Sandosh; Palmer, Colin N A; Palmer, Nicholette D; Pasterkamp, Gerard; Patel, Aniruddh P; Pattie, Alison; Pedersen, Oluf; Peissig, Peggy L; Peloso, Gina M; Pennell, Craig E; Perola, Markus; Perry, James A; Perry, John R B; Pers, Tune H; Person, Thomas N; Peters, Annette; Petersen, Eva R B; Peyser, Patricia A; Pirie, Ailith; Polasek, Ozren; Polderman, Tinca J; Puolijoki, Hannu; Raitakari, Olli T; Rasheed, Asif; Rauramaa, Rainer; Reilly, Dermot F; Renström, Frida; Rheinberger, Myriam; Ridker, Paul M; Rioux, John D; Rivas, Manuel A; Roberts, David J; Robertson, Neil R; Robino, Antonietta; Rolandsson, Olov; Rudan, Igor; Ruth, Katherine S; Saleheen, Danish; Salomaa, Veikko; Samani, Nilesh J; Sapkota, Yadav; Sattar, Naveed; Schoen, Robert E; Schreiner, Pamela J; Schulze, Matthias B; Scott, Robert A; Segura-Lepe, Marcelo P; Shah, Svati H; Sheu, Wayne H-H; Sim, Xueling; Slater, Andrew J; Small, Kerrin S; Smith, Albert V; Southam, Lorraine; Spector, Timothy D; Speliotes, Elizabeth K; Starr, John M; Stefansson, Kari; Steinthorsdottir, Valgerdur; Stirrups, Kathleen E; Strauch, Konstantin; Stringham, Heather M; Stumvoll, Michael; Sun, Liang; Surendran, Praveen; Swift, Amy J; Tada, Hayato; Tansey, Katherine E; Tardif, Jean-Claude; Taylor, Kent D; Teumer, Alexander; Thompson, Deborah J; Thorleifsson, Gudmar; Thorsteinsdottir, Unnur; Thuesen, Betina H; Tönjes, Anke; Tromp, Gerard; Trompet, Stella; Tsafantakis, Emmanouil; Tuomilehto, Jaakko; Tybjaerg-Hansen, Anne; Tyrer, Jonathan P; Uher, Rudolf; Uitterlinden, André G; Uusitupa, Matti; Laan, Sander W; Duijn, Cornelia M; Leeuwen, Nienke; van Setten, Jessica; Vanhala, Mauno; Varbo, Anette; Varga, Tibor V; Varma, Rohit; Velez Edwards, Digna R; Vermeulen, Sita H; Veronesi, Giovanni; Vestergaard, Henrik; Vitart, Veronique; Vogt, Thomas F; Völker, Uwe; Vuckovic, Dragana; Wagenknecht, Lynne E; Walker, Mark; Wallentin, Lars; Wang, Feijie; Wang, Carol A; Wang, Shuai; Wang, Yiqin; Ware, Erin B; Wareham, Nicholas J; Warren, Helen R; Waterworth, Dawn M; Wessel, Jennifer; White, Harvey D; Willer, Cristen J; Wilson, James G; Witte, Daniel R; Wood, Andrew R; Wu, Ying; Yaghootkar, Hanieh; Yao, Jie; Yao, Pang; Yerges-Armstrong, Laura M; Young, Robin; Zeggini, Eleftheria; Zhan, Xiaowei; Zhang, Weihua; Zhao, Jing Hua; Zhao, Wei; Zhao, Wei; Zhou, Wei; Zondervan, Krina T; Rotter, Jerome I; Pospisilik, John A; Rivadeneira, Fernando; Borecki, Ingrid B; Deloukas, Panos; Frayling, Timothy M; Lettre, Guillaume; North, Kari E; Lindgren, Cecilia M; Hirschhorn, Joel N; Loos, Ruth J F

2018-01-01

Genome-wide association studies (GWAS) have identified >250 loci for body mass index (BMI), implicating pathways related to neuronal biology. Most GWAS loci represent clusters of common, noncoding variants from which pinpointing causal genes remains challenging. Here we combined data from 718,734 individuals to discover rare and low-frequency (minor allele frequency (MAF) < 5%) coding variants associated with BMI. We identified 14 coding variants in 13 genes, of which 8 variants were in genes (ZBTB7B, ACHE, RAPGEF3, RAB21, ZFHX3, ENTPD6, ZFR2 and ZNF169) newly implicated in human obesity, 2 variants were in genes (MC4R and KSR2) previously observed to be mutated in extreme obesity and 2 variants were in GIPR. The effect sizes of rare variants are ~10 times larger than those of common variants, with the largest effect observed in carriers of an MC4R mutation introducing a stop codon (p.Tyr35Ter, MAF = 0.01%), who weighed ~7 kg more than non-carriers. Pathway analyses based on the variants associated with BMI confirm enrichment of neuronal genes and provide new evidence for adipocyte and energy expenditure biology, widening the potential of genetically supported therapeutic targets in obesity.

Leptoquarks meet ɛ '/ ɛ and rare Kaon processes

Science.gov (United States)

Bobeth, Christoph; Buras, Andrzej J.

2018-02-01

We analyse for the first time the CP violating ratio ɛ '/ ɛ in K → ππ decays in leptoquark (LQ) models. Assuming a mass gap to the electroweak (EW) scale, the main mechanism for LQs to contribute to ɛ ' /ɛ is EW gauge-mixing of semi-leptonic into non-leptonic operators, which we treat in the Standard Model effective theory (SMEFT). We perform also the one-loop decoupling for scalar LQs, finding that in all models with both left-handed and right-handed LQ couplings box-diagrams generate numerically strongly enhanced EW-penguin operators Q 8,8' already at the LQ scale. We then investigate correlations of ɛ ' /ɛ with rare Kaon processes ( {K}_L\\to {π}^0ν \\overline{ν} , {K}+\\to {π}+ν \\overline{ν} , {K}_L\\to {π}^0ℓ \\overline{ℓ} , {K}_S\\to μ \\overline{μ} , Δ M K and ɛ K ) and find that even imposing only a moderate enhancement of ( ɛ ' /ɛ)NP = 5 × 10-4 to explain the current anomaly hinted by the Dual QCD approach and RBC-UKQCD lattice QCD calculations leads to conflicts with experimental upper bounds on rare Kaon processes. They exclude all LQ models with only a single coupling as an explanation of the ɛ ' /ɛ anomaly and put strong-to-serious constraints on parameter spaces of the remaining models. Future results on {K}+\\to {π}+ν \\overline{ν} from the NA62 collaboration, {K}_L\\to {π}^0ν \\overline{ν} from the KOTO experiment and {K}_S\\to μ \\overline{μ} from LHCb will even stronger exhibit the difficulty of LQ models in explaining the measured ɛ ' /ɛ, in case the ɛ ' /ɛ anomaly will be confirmed by improved lattice QCD calculations. Hopefully also improved measurements of {K}_L\\to {π}^0ℓ \\overline{ℓ} decays will one day help in this context.

CERN exhibition a big hit in Bulgaria

CERN Multimedia

2005-01-01

The first CERN exhibition in Bulgaria attracted many visitors. In the first ever CERN exhibition to be held in Bulgaria, over 1,400 visitors, many of them students and young physicists, visited the 10-day event in Sofia. The CERN mini-exhibition took place at the National Earth and Mankind Museum between 8 and 17 November. Permanently staffed by young physicists from Sofia University, there were exhibits on display about research activities at CERN, as well as four additional posters describing Bulgaria's participation. The inauguration took place on the morning of 8 November in the presence of the Vice-Minister for Science and Education, Mrs. Vanya Dobreva, and some 200 guests. A series of short speeches were followed by a visit to the exhibition. CERN's representative at the event, Ray Lewis, was then asked by Professor Matey Mateev, President of the Union of Physicists in Bulgaria, to say a few words on behalf of the Organization. Numerous journalists were also present at the inauguration. A painting enti...

CERN’s travelling exhibition goes to Austria

CERN Multimedia

Mélissa Lanaro

2011-01-01

Since April 2009 CERN’s travelling exhibition has been touring through some of the Organization's Member States. After Italy and Denmark it has been on show since February at Austria’s Hartberg Ökopark, a very popular science museum situated some one hundred kilometres from Vienna. To coincide with the CERN exhibition, Austria’s scientific community has organised a series of events for the general public which have had marked success. The exhibition's next destination will be Portugal and preparations are already underway to ensure that it is another resounding success   The travelling exhibition was designed in collaboration with the University of Geneva, as part of the celebrations for its 450th anniversary, and has already notched up a good number of kilometres as it travels from country to country. “In 2010 the exhibition already had around 55,000 visitors,” explains Rolf Landua, who heads the Education Group. Since its inauguration ...

“Accelerating Science” exhibition zooms to Turkey

CERN Multimedia

Joannah Caborn Wengler

2012-01-01

'Accelerating Science', CERN’s travelling science outreach exhibition, has just arrived at the Middle East Technical University (METU) in Ankara, Turkey for a four-month stay there. This is the first time it has moved outside the circle of the Member States. The Turkish venue will inaugurate some new exhibits that have recently been developed by CERN’s software developers.   “It’s been a very busy day,” says Bilge Demirkoz, an associate professor of physics at METU and a member of AMS-02, who had been overseeing the unloading of the lorries when we spoke to her. “As the University doesn’t have a specific exhibition space, the CERN exhibits are going to be housed in the covered tennis courts just behind the cultural and congress centre. It’s a beautiful venue, and there are plenty of parking spaces.” The University has sent invitations to the exhibition to high schools and to about 100 ...

Magnetic properties of Kramers rare earth ions in aluminium and gallium garnets; Proprietes magnetiques des ions de kramers des terres rares dans les grenats de terres rares et d'aluminium et les grenats de terres rares et de gallium

Energy Technology Data Exchange (ETDEWEB)

Capel, H [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

1964-07-01

The magnetic properties of Kramers rare earth ions in aluminium and gallium garnets (MAlG and MGaG) are discussed by means of a molecular field treatment. The symmetry properties of the space group permit to establish a parametrization for the magnetic dipolar and exchange couplings. The magnetic properties of the system can be expressed in terms of these parameters and the g factors of the rare earth ions. We have calculated the transition temperatures, the sub-lattice magnetizations, the susceptibility in the paramagnetic region and the antiferromagnetic susceptibility for a special type of magnetic ordering. The influence of the excited Kramers doublets is described by means of a generalization of the usual g tensor. (authors) [French] Les proprietes magnetiques des ions de Kramers des terres rares dans les grenats de terre rare et d'aluminium et les grenats de terre rare et de gallium sont discutees a l'aide d'un traitement du champ moleculaire. Les proprietes de symmetrie du groupe d'espace permettent d'exprimer les couplages dipolaires et les interactions d'echange en fonction de quelques parametres. Les proprietes magnetiques peuvent etre exprimees en fonction de ces parametres et les facteurs g des ions de terre rare. Nous avons calcule les temperatures de transition, les aimantations des sous-reseaux pour 0

Characterization of a SAM-dependent fluorinase from a latent biosynthetic pathway for fluoroacetate and 4-fluorothreonine formation in Nocardia brasiliensis.

Science.gov (United States)

Wang, Yaya; Deng, Zixin; Qu, Xudong

2014-01-01

Fluorination has been widely used in chemical synthesis, but is rare in nature. The only known biological fluorination scope is represented by the fl pathway from Streptomyces cattleya that produces fluoroacetate (FAc) and 4-fluorothreonine (4-FT). Here we report the identification of a novel pathway for FAc and 4-FT biosynthesis from the actinomycetoma-causing pathogen Nocardia brasiliensis ATCC 700358. The new pathway shares overall conservation with the fl pathway in S. cattleya. Biochemical characterization of the conserved domains revealed a novel fluorinase NobA that can biosynthesize 5'-fluoro-5'-deoxyadenosine (5'-FDA) from inorganic fluoride and S-adenosyl-l-methionine (SAM). The NobA shows similar halide specificity and characteristics to the fluorination enzyme FlA of the fl pathway. Kinetic parameters for fluoride ( K m 4153 μM, k cat 0.073 min (-1)) and SAM ( K m 416 μM, k cat 0.139 min (-1)) have been determined, revealing that NobA is slightly (2.3 fold) slower than FlA. Upon sequence comparison, we finally identified a distinct loop region in the fluorinases that probably accounts for the disparity of fluorination activity.

Cake kidney: a rare anomaly of renal fusion

Directory of Open Access Journals (Sweden)

Guilherme Lippi Ciantelli

2012-06-01

Full Text Available ABSTRACT The cake kidney is a rare congenital anomaly of the urinogenital tract that can be diagnosed at any age. Few more than 20 cases have been described in the literature. The authors describe in this article another case of this rare malformation. Key-words: kidney, congenital abnormalities, rare diseases.

Plant for removing radioactive rare gases

International Nuclear Information System (INIS)

An, Buzai; Kanazawa, Toshio

1977-01-01

The outline of the pilot plant to remove and recover radioactive rare gases generated from nuclear power plants, reprocessing installations for nuclear fuel, nuclear research installations, etc. is described below. Among the studies of various processes such as liquefaction and distillation, absorption into solvents, active carbon adsorption, diaphragm method, etc., the liquefaction and distillation process by rectification at low temperature has been positively developed. It is in the stage of practical application for removing rare gases in waste gases from reprocessing and nuclear power plants. This is the process with high safety and excellent rare gas removing capability. Further research and development have been also made for selective adsorption and desorption method at low temperature which is very efficient as there is no release of long life nuclides such as Krypton-85. Rare gases recovered by the above mentioned removal systems must be stored safely for a long time as their half lives are long and specific radioactivities are high. The study has been made continuously on the storage methods including adsorption in cylinders and remotely automatically sealing storing system. (Kobatake, H.)

Method for storing radioactive rare gases

International Nuclear Information System (INIS)

Watabe, Atsushi; Nagao, Hiroyuki; Takiguchi, Yukio; Kanazawa, Toshio; Soya, Masataka.

1975-01-01

Object: To safely and securely store radioactive rare gases for a long period of time. Structure: The waste gases produced in nuclear power plant are cooled by a cooler and then introduced into a low temperature adsorbing device so that the gases are adsorbed by adsorbents, and then discharged into atmosphere through the purifying gas discharge line. When the radioactive rare gases reach a level of saturation in the amount of adsorption, they are heated and extracted by a suction pump and heated by a heater. The gases are then introduced into an oxygen-impurity removing device and the purified rare gases containing no oxygen and impurities are cooled by a cooler and fed into a gas holder. When the amount of radioactive rare gases stored within the gas holder reaches a given level, they are compressed and sealed by a compressure into a storing cylinder and residual gases in the piping are sucked and recovered into the gas holder, after which the cylinder is removed and stored in a fixed room. (Kamimura, M.)

Rare diseases in the media - Report April-June 2014 - Observatory for Rare Diseases FEDER (OBSER

Directory of Open Access Journals (Sweden)

Josep Solves Almela

2015-12-01

Full Text Available This report presents the analysis of how Spanish mass media dealt with the so-called rare deseases during the months of April, May and June of 2014. The report has the same general objective of the first one for the previous three months: understand how rare diseases are presented in the Spanish media and, correspondingly, how that media representation evolves. In this report, the data of the first trimester is compared to the second one.

Methods of assessing total doses integrated across pathways

International Nuclear Information System (INIS)

Grzechnik, M.; Camplin, W.; Clyne, F.; Allott, R.; Webbe-Wood, D.

2006-01-01

future years. C) Construct Individuals with high rates of consumption or occupancy across all pathways are used to derive rates for each pathway. These are applied in future years. D) Top-Two High and average consumption and occupancy rates for each pathway are derived. Doses can be calculated for all combinations where two pathways are considered at high rates and the remainder as average E) Profiling A profile is derived by calculating consumption and occupancy rates for each pathway for individuals who exhibit high rates for a single pathway. Other profiles may be built by repeating for other pathways. Total dose is the highest dose for any profile, and that profile becomes known as the critical group. Method A was used as a benchmark, with methods B -E compared according to the previously specified criteria. Overall the profiling method of total dose calculation was adopted due to its favourable overall comparison with the individual method and the homogeneity of the critical group selected. (authors)

Residual levels of rare earth elements in freshwater and marine fish and their health risk assessment from Shandong, China

International Nuclear Information System (INIS)

Yang, Luping; Wang, Xining; Nie, Hongqian; Shao, Lijun; Wang, Guoling; Liu, Yongjun

2016-01-01

The total concentrations of rare earth elements (ΣREE) were quantified in 251 samples from 10 common species of freshwater and marine fish in seventeen cities of Shandong, China. ΣREE obtained from the freshwater fish ranged from 34.0 to 37.9 ngg −1 (wet weight) and marine fish from 12.7 to 37.6 ngg −1 . The ratio of LREE to HREE was 13.7:1 and 10:1 for freshwater and marine fish, respectively. This suggests that freshwater fish exhibit greater REE concentrations than marine fish and the biological effects of LREE are higher than HREE. Results revealed a similar REE distribution pattern between those fish and coastal sediments, abiding the “abundance law”. The health risk assessment demonstrated the EDIs of REEs in fish were significantly lower than the ADI, indicating that the consumption of these fish presents little risk to human health. - Highlights: •Concentrations of rare earth elements in freshwater and marine fishes from the coastlines of Shandong, China are reported. •Statistical analysis and comparison indicates that freshwater fishes exhibit greater REE concentrations than marine fishes. •A similar REE distribution pattern was found between those of fish and coastal sediments, abiding the "abundance law". •Health risk assessment demonstrated that consuming fish is presently at little risk to human health.

p53 downregulates the Fanconi anaemia DNA repair pathway.

Science.gov (United States)

Jaber, Sara; Toufektchan, Eléonore; Lejour, Vincent; Bardot, Boris; Toledo, Franck

2016-04-01

Germline mutations affecting telomere maintenance or DNA repair may, respectively, cause dyskeratosis congenita or Fanconi anaemia, two clinically related bone marrow failure syndromes. Mice expressing p53(Δ31), a mutant p53 lacking the C terminus, model dyskeratosis congenita. Accordingly, the increased p53 activity in p53(Δ31/Δ31) fibroblasts correlated with a decreased expression of 4 genes implicated in telomere syndromes. Here we show that these cells exhibit decreased mRNA levels for additional genes contributing to telomere metabolism, but also, surprisingly, for 12 genes mutated in Fanconi anaemia. Furthermore, p53(Δ31/Δ31) fibroblasts exhibit a reduced capacity to repair DNA interstrand crosslinks, a typical feature of Fanconi anaemia cells. Importantly, the p53-dependent downregulation of Fanc genes is largely conserved in human cells. Defective DNA repair is known to activate p53, but our results indicate that, conversely, an increased p53 activity may attenuate the Fanconi anaemia DNA repair pathway, defining a positive regulatory feedback loop.

Drugs for rare disorders.

Science.gov (United States)

Cremers, Serge; Aronson, Jeffrey K

2017-08-01

Estimates of the frequencies of rare disorders vary from country to country; the global average defined prevalence is 40 per 100 000 (0.04%). Some occur in only one or a few patients. However, collectively rare disorders are fairly common, affecting 6-8% of the US population, or about 30 million people, and a similar number in the European Union. Most of them affect children and most are genetically determined. Diagnosis can be difficult, partly because of variable presentations and partly because few clinicians have experience of individual rare disorders, although they may be assisted by searching databases. Relatively few rare disorders have specific pharmacological treatments (so-called orphan drugs), partly because of difficulties in designing trials large enough to determine benefits and harms alike. Incentives have been introduced to encourage the development of orphan drugs, including tax credits and research aids, simplification of marketing authorization procedures and exemption from fees, and extended market exclusivity. Consequently, the number of applications for orphan drugs has grown, as have the costs of using them, so much so that treatments may not be cost-effective. It has therefore been suggested that not-for-profit organizations that are socially motivated to reduce those costs should be tasked with producing them. A growing role for patient organizations, improved clinical and translational infrastructures, and developments in genetics have also contributed to successful drug development. The translational discipline of clinical pharmacology is an essential component in drug development, including orphan drugs. Clinical pharmacologists, skilled in basic pharmacology and its links to clinical medicine, can be involved at all stages. They can contribute to the delineation of genetic factors that determine clinical outcomes of pharmacological interventions, develop biomarkers, design and perform clinical trials, assist regulatory decision

A study on dehydration of rare earth chloride hydrate

International Nuclear Information System (INIS)

Cho, Yong Zun; Eun, Hee Chul; Son, Sung Mo; Lee, Tae Kyo; Hwang, Taek Sung

2012-01-01

The dehydration schemes of rare earth (La, Ce, Nd, Pr, Sm. Eu, Gd, Y) chloride hydrates was investigated by using a dehydration apparatus. To prevent the formation of the rare earth oxychlorides, the operation temperature was changed step by step (80→150→230 degree C) based on the TGA (thermo-gravimetric analysis) results of the rare earth chloride hydrates. A vacuum pump and preheated Ar gas were used to effectively remove the evaporated moisture and maintain an inert condition in the dehydration apparatus. The dehydration temperature of the rare earth chloride hydrate was increased when the atomic number of the rare earth nuclide was increased. The content of the moisture in the rare earth chloride hydrate was decreased below 10% in the dehydration apparatus.

Clinical report: a rare co-occurrence of tuberous sclerosis complex and Rett syndrome in a girl with mental retardation, epilepsy and autism

OpenAIRE

Belousova, Elena; Sukhorukov, Vladimir; Dorofeeva, Marina; Shagam, Lev; Vlodavetz, Dmitrii V.

2017-01-01

Introduction. There are some genetic disorders with combination of mental retardation, epilepsy and autism in which the abnormal mammalian Target of Rapamycin (m-TOR) signaling is implicated. The most important of them is tuberous sclerosis complex (TSC), but the disturbances of the m-TOR pathway can also be detected in Rett syndrome (RS), Fragile X syndrome and Down syndrome. We describe the rare case of co-occurrence of TSC and RS. Case study. The female child was born at term by normal de...

"Big Science" exhibition at Balexert

CERN Multimedia

2008-01-01

CERN is going out to meet those members of the general public who were unable to attend the recent Open Day. The Laboratory will be taking its "Big Science" exhibition from the Globe of Science and Innovation to the Balexert shopping centre from 19 to 31 May 2008. The exhibition, which shows the LHC and its experiments through the eyes of a photographer, features around thirty spectacular photographs measuring 4.5 metres high and 2.5 metres wide. Welcomed and guided around the exhibition by CERN volunteers, shoppers at Balexert will also have the opportunity to discover LHC components on display and watch films. "Fun with Physics" workshops will be held at certain times of the day. Main hall of the Balexert shopping centre, ground floor, from 9.00 a.m. to 7.00 p.m. Monday to Friday and from 10 a.m. to 6 p.m. on the two Saturdays. Call for volunteers All members of the CERN personnel are invited to enrol as volunteers to help welcom...

Soya bean Gα proteins with distinct biochemical properties exhibit differential ability to complement Saccharomyces cerevisiae gpa1 mutant.

Science.gov (United States)

Roy Choudhury, Swarup; Wang, Yuqi; Pandey, Sona

2014-07-01

Signalling pathways mediated by heterotrimeric G-proteins are common to all eukaryotes. Plants have a limited number of each of the G-protein subunits, with the most elaborate G-protein network discovered so far in soya bean (Glycine max, also known as soybean) which has four Gα, four Gβ and ten Gγ proteins. Biochemical characterization of Gα proteins from plants suggests significant variation in their properties compared with the well-characterized non-plant proteins. Furthermore, the four soya bean Gα (GmGα) proteins exhibit distinct biochemical activities among themselves, but the extent to which such biochemical differences contribute to their in vivo function is also not known. We used the yeast gpa1 mutant which displays constitutive signalling and growth arrest in the pheromone-response pathway as an in vivo model to evaluate the effect of distinct biochemical activities of GmGα proteins. We showed that specific GmGα proteins can be activated during pheromone-dependent receptor-mediated signalling in yeast and they display different strengths towards complementation of yeast gpa1 phenotypes. We also identified amino acids that are responsible for differential complementation abilities of specific Gα proteins. These data establish that specific plant Gα proteins are functional in the receptor-mediated pheromone-response pathway in yeast and that the subtle biochemical differences in their activity are physiologically relevant.

An Overview of Rare Earth Science and Technology

Science.gov (United States)

Gschneidner, Karl, Jr.

2012-02-01

Currently rare earth science and technology is robust: this includes all the major branches of science -- biochemistry, chemistry, materials and physics. There are, however, currently some anomalies and distortions especially in the technology and applications sector of the rare earth field, which is caused by the dominance of China on the sales of rare earths and rare earth containing products. For the past 5 to 10 years ˜95% of rare earths utilized in commerce came from China. Although Chinese actions have lead to sudden and large price spikes and export embargoes, the rare earths are still available but at a higher cost. The start up of production in 2011 at mines in the USA and Australia will alleviate this situation in about two years. Basic and applied research on the condensed matter physics/materials science has hardly been impacted by these events, but new research opportunities are opening up especially with regard to the USA's military and energy security. Magnets seems to be the hottest topic, but research on battery materials, phosphors and catalysts are also (or should be) strongly considered.

Determination of rare earths in their extraction processing

International Nuclear Information System (INIS)

You Jiannan; Zhang Yuqin

1989-01-01

A method for determination of rare earths in ores, ion-exchange resins and solution samples has been developed. The ore is molten with sodium peroxide and the molten sample is leached with triethenol amine and sodium citrate. In weak acid medium, the rare earths can be extracted by PMBP-phenol solution, and stripped with formic acid. In the acetic acidsodium acetate buffer medium of pH3, the spectrophotometric determination of rare earths with arsenazo M has been made. The rare earths in ion-exchange resins can be directly determined by spectrophotometry after being leached with hydrochloric acid and at heated condition. The rare earths with arsenazo M or a red complex. The maximum absorption of the complex is at 640 nm, and the molar absorption is 8.0 x 10 4 L centre dot mol -1 centre dot cm -1 . While the range of determination is 0.005%-0.5% and 0.001-1.0 g/L, the relative standard deviation is less than 5%, and recovery of rare earths is 98.5-105%. The method is rather simple and rapid

[A rare form of granuloma annulare].

Science.gov (United States)

Bogdanowski, T; Wygledowska-Kania, M

1995-01-01

We present a four-year-old girl with a doubly rare form of granuloma annulare with non-typical localisation of superficial nodules on the palms and predisposition to ulceration which is very rare in this type of superficial nodules. The diagnosis was proved by histological examination. After the local cryotherapy (ethyl chloride) the lesions almost completely disappeared.

Rare earth oxyhydrides and preparation process

International Nuclear Information System (INIS)

Diaz, H.

1986-01-01

Rare earth oxyhydrides of formula RE 1-q Th q Ni 5-p M p O x H y are claimed. RE is a rare earth, Th can be replaced by Yt, M is Cu, Mn, Al, Fe, Cr or Co, o O C and the hydrides are oxidized. They are catalysts for various chemical reactions [fr

Rare Earth Elements Distribution in Beryl

International Nuclear Information System (INIS)

El Gawish, H.K.; Nada, N.; Ghaly, W.A.; Helal, A.I.

2012-01-01

Laser ablation method is applied to a double focusing inductively coupled plasma mass spectrometer to determine the rare earth element distribution in some selected beryl samples. White, green and blue beryl samples are selected from the Egyptian eastern desert. Distributions of chondrite- normalized plot for the rare earth element in the selected beryl samples are investigated

Rare Earth-Activated Silica-Based Nanocomposites

Directory of Open Access Journals (Sweden)

C. Armellini

2007-01-01

Full Text Available Two different kinds of rare earth-activated glass-based nanocomposite photonic materials, which allow to tailor the spectroscopic properties of rare-earth ions: (i Er3+-activated SiO2-HfO2 waveguide glass ceramic, and (ii core-shell-like structures of Er3+-activated silica spheres obtained by a seed growth method, are presented.

Protein-altering variants associated with body mass index implicate pathways that control energy intake and expenditure in obesity

OpenAIRE

Turcot, Valérie; Lu, Yingchang; Highland, Heather M; Schurmann, Claudia; Justice, Anne E; Fine, Rebecca S; Bradfield, Jonathan P; Esko, Tõnu; Giri, Ayush; Graff, Mariaelisa; Guo, Xiuqing; Hendricks, Audrey E; Karaderi, Tugce; Lempradl, Adelheid; Locke, Adam E

2018-01-01

Genome-wide association studies (GWAS) have identified >250 loci for body mass index (BMI), implicating pathways related to neuronal biology. Most GWAS loci represent clusters of common, noncoding variants from which pinpointing causal genes remains challenging. Here we combined data from 718,734 individuals to discover rare and low-frequency (minor allele frequency (MAF) < 5%) coding variants associated with BMI. We identified 14 coding variants in 13 genes, of which 8 variants were in...

Waterpipe industry products and marketing strategies: analysis of an industry trade exhibition.

Science.gov (United States)

Jawad, Mohammed; Nakkash, Rima T; Hawkins, Ben; Akl, Elie A

2015-12-01

Understanding product development and marketing strategies of transnational tobacco companies (TTCs) has been of vital importance in developing an effective tobacco control policy. However, comparatively little is known of the waterpipe tobacco industry, which TTCs have recently entered. This study aimed to gain an understanding of waterpipe tobacco products and marketing strategies by visiting a waterpipe trade exhibition. In April 2014, the first author attended an international waterpipe trade exhibition, recording descriptions of products and collecting all available marketing items. We described the purpose and function of all products, and performed a thematic analysis of messages in marketing material. We classified waterpipe products into four categories and noted product variation within categories. Electronic waterpipe products (which mimic electronic cigarettes) rarely appeared on waterpipe tobacco marketing material, but were displayed just as widely. Claims of reduced harm, safety and quality were paramount on marketing materials, regardless of whether they were promoting consumption products (tobacco, tobacco substitutes), electronic waterpipes or accessories. Waterpipe products are diverse in nature and are marketed as healthy and safe products. Furthermore, the development of electronic waterpipe products appears to be closely connected with the electronic cigarette industry, rather than the waterpipe tobacco manufacturers. Tobacco control policy must evolve to take account of the vast and expanding array of waterpipe products, and potentially also charcoal products developed for waterpipe smokers. We recommend that tobacco substitutes be classified as tobacco products. Continued surveillance of the waterpipe industry is warranted. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Sharing Rare Attitudes Attracts.

Science.gov (United States)

Alves, Hans

2018-04-01

People like others who share their attitudes. Online dating platforms as well as other social media platforms regularly rely on the social bonding power of their users' shared attitudes. However, little is known about moderating variables. In the present work, I argue that sharing rare compared with sharing common attitudes should evoke stronger interpersonal attraction among people. In five studies, I tested this prediction for the case of shared interests from different domains. I found converging evidence that people's rare compared with their common interests are especially potent to elicit interpersonal attraction. I discuss the current framework's theoretical implications for impression formation and impression management as well as its practical implications for improving online dating services.

Rare-earth nuclei: Radii, isotope-shifts and deformation properties in the relativistic mean-field theory

International Nuclear Information System (INIS)

Lalazissis, G.A.; Ring, P.

1996-01-01

A systematic study of the ground-state properties of even-even rare earth nuclei has been performed in the framework of the Relativistic Mean-Field (RMF) theory using the parameter set NL-SH. Nuclear radii, isotope shifts and deformation properties of the heavier rare-earth nuclei have been obtained, which encompass atomic numbers ranging from Z=60 to Z=70 and include a large range of isospin. It is shown that RMF theory is able to provide a good and comprehensive description of the empirical binding energies of the isotopic chains. At the same time the quadrupole deformations β 2 obtained in the RMF theory are found to be in good agreement with the available empirical values. The theory predicts a shape transition from prolate to oblate for nuclei at neutron number N=78 in all the chains. A further addition of neutrons up to the magic number 82 brings about the spherical shape. For nuclei above N=82, the RMF theory predicts the well-known onset of prolate deformation at about N=88, which saturates at about N=102. The deformation properties display an identical behaviour for all the nuclear chains. A good description of the above deformation transitions in the RMF theory in all the isotopic chains leads to a successful reproduction of the anomalous behaviour of the empirical isotopic shifts of the rare-earth nuclei. The RMF theory exhibits a remarkable success in providing a unified and microscopic description of various empirical data. (orig.)

Photosynthetic pathway types of evergreen rosette plants (Liliaceae) of the Chihuahuan desert.

Science.gov (United States)

Kemp, Paul R; Gardetto, Pietra E

1982-11-01

Diurnal patterns of CO 2 exchange and titratable acidity were monitored in six species of evergreen rosette plants growing in controlled environment chambers and under outdoor environmental conditions. These patterns indicated that two of the species, Yucca baccata and Y. torreyi, were constituitive CAM plants while the other species, Y. elata, Y. campestris, Nolina microcarpa and Dasylirion wheeleri, were C 3 plants. The C 3 species did not exhibit CAM when grown in any of several different temperature, photoperiod, and moisture regimes. Both photosynthetic pathway types appear adapted to desert environments and all species show environmentally induced changes in their photosynthetic responses consistent with desert adaptation. The results of this study do not indicate that changes in the photosynthetic pathway type are an adaptation in any of these species.

Characterisation of ethylene pathway components in non-climacteric capsicum.

Science.gov (United States)

Aizat, Wan M; Able, Jason A; Stangoulis, James C R; Able, Amanda J

2013-11-28

Climacteric fruit exhibit high ethylene and respiration levels during ripening but these levels are limited in non-climacteric fruit. Even though capsicum is in the same family as the well-characterised climacteric tomato (Solanaceae), it is non-climacteric and does not ripen normally in response to ethylene or if harvested when mature green. However, ripening progresses normally in capsicum fruit when they are harvested during or after what is called the 'Breaker stage'. Whether ethylene, and components of the ethylene pathway such as 1-aminocyclopropane 1-carboxylate (ACC) oxidase (ACO), ACC synthase (ACS) and the ethylene receptor (ETR), contribute to non-climacteric ripening in capsicum has not been studied in detail. To elucidate the behaviour of ethylene pathway components in capsicum during ripening, further analysis is therefore needed. The effects of ethylene or inhibitors of ethylene perception, such as 1-methylcyclopropene, on capsicum fruit ripening and the ethylene pathway components may also shed some light on the role of ethylene in non-climacteric ripening. The expression of several isoforms of ACO, ACS and ETR were limited during capsicum ripening except one ACO isoform (CaACO4). ACS activity and ACC content were also low in capsicum despite the increase in ACO activity during the onset of ripening. Ethylene did not stimulate capsicum ripening but 1-methylcyclopropene treatment delayed the ripening of Breaker-harvested fruit. Some of the ACO, ACS and ETR isoforms were also differentially expressed upon treatment with ethylene or 1-methylcyclopropene. ACS activity may be the rate limiting step in the ethylene pathway of capsicum which restricts ACC content. The differential expression of several ethylene pathway components during ripening and upon ethylene or 1-methylclopropene treatment suggests that the ethylene pathway may be regulated differently in non-climacteric capsicum compared to the climacteric tomato. Ethylene independent pathways may

Catalogue of Korean manuscripts and rare books

DEFF Research Database (Denmark)

Lerbæk Pedersen, Bent

2014-01-01

Catalogue of Korean manuscripts and rare books in The Royal Library, Copenhagen and the National Museum of Denmark......Catalogue of Korean manuscripts and rare books in The Royal Library, Copenhagen and the National Museum of Denmark...

Rare Events

Science.gov (United States)

2009-10-01

Limited Operational Exercise 1. 1A Limited Operational Exercise is a multiplayer experiment designed to exploit and study information sharing and...1.4 Summary of the Study The “rare event” of interest is an extreme, deliberate act of violence , destruction or socioeconomic disruption, such as an...connection with terrorism inves- tigations. The programs then use some combination of doctrinal revision and rewards to induce the people to abandon violence

Estrogen receptor β exhibited anti-tumor effects on osteosarcoma cells by regulating integrin, IAP, NF-kB/BCL-2 and PI3K/Akt signal pathway

Directory of Open Access Journals (Sweden)

Minfei Yang

2017-11-01

Full Text Available This study aimed to investigate the effects of Estrogen receptor β (ERβ on osteosarcoma cells, and explore the regulatory mechanisms involved in this process. Osteosarcoma U2-OS cells consisted four groups, and treated by E2, E2 + LY294002 (ERβ agonists, E2 + ERβ siRNA, E2 + ERβ siRNA + LY294002, respectively. Cell counting kit 8 (CCK-8 assay was performed to detect the cell viability of U2-OS cells in each group. The effects of ERβ on the migration and invasion ability of U2-OS cells were examined by wound healing assay and transwell cell culture chamber, respectively. The expression of Inhibitor of apoptosis protein (IAP and integrin α5 in U2-OS cells of each group was detected by quantitative RT-PCR, and the expression of phosphorylated p65 (p-p65, p-AKT and Bcl-2 was detected by western blotting. The cell viability, migration and invasion ability of U2-OS cells were significantly increased by ERβ siRNA, but inhibited by ERβ agonists LY294002 (p < 0.05. ERβ siRNA significantly downregulated Integrin α5 and unregulated IAP in U2-OS cells (p < 0.05. The expression of p-p65, p-AKT and Bcl-2 was significantly reduced by LY294002, but increased by ERβ siRNA (p < 0.05. In conclusion, ERβ exhibited obvious anti-tumor effects on osteosarcoma cells by regulating integrin, IAP, NF-kBBCL-2 and PI3K/Akt signal pathway. Keywords: Estrogen receptor β, Osteosarcoma, Anti-tumor, Regulatory mechanism

Rare earth activated yttrium aluminate phosphors with modulated luminescence.

Science.gov (United States)

Muresan, L E; Popovici, E J; Perhaita, I; Indrea, E; Oro, J; Casan Pastor, N

2016-06-01

Yttrium aluminate (Y3 A5 O12 ) was doped with different rare earth ions (i.e. Gd(3+) , Ce(3+) , Eu(3+) and/or Tb(3+) ) in order to obtain phosphors (YAG:RE) with general formula,Y3-x-a Gdx REa Al5 O12 (x = 0; 1.485; 2.97 and a = 0.03). The synthesis of the phosphor samples was done using the simultaneous addition of reagents technique. This study reveals new aspects regarding the influence of different activator ions on the morpho-structural and luminescent characteristics of garnet type phosphor. All YAG:RE phosphors are well crystallized powders containing a cubic-Y3 Al5 O12 phase as major component along with monoclinic-Y4 Al2 O9 and orthorhombic-YAlO3 phases as the impurity. The crystallites dimensions of YAG:RE phosphors vary between 38 nm and 88 nm, while the unit cell slowly increase as the ionic radius of the activator increases. Under UV excitation, YAG:Ce exhibits yellow emission due to electron transition in Ce(3+) from the 5d level to the ground state levels ((2) F5/2 , (2) F7/2 ). The emission intensity of Ce(3+) is enhanced in the presence of the Tb(3+) ions and is decreased in the presence of Eu(3+) ions due to some radiative or non-radiative processes that take place between activator ions. By varying the rare earth ions, the emission colour can be modulated from green to white and red. Copyright © 2015 John Wiley & Sons, Ltd. Copyright © 2015 John Wiley & Sons, Ltd.

Genetic analysis of pathway regulation for enhancing branched-chain amino acid biosynthesis in plants

KAUST Repository

Chen, Hao

2010-08-01

The branched-chain amino acids (BCAAs) valine, leucine and isoleucine are essential amino acids that play critical roles in animal growth and development. Animals cannot synthesize these amino acids and must obtain them from their diet. Plants are the ultimate source of these essential nutrients, and they synthesize BCAAs through a conserved pathway that is inhibited by its end products. This feedback inhibition has prevented scientists from engineering plants that accumulate high levels of BCAAs by simply over-expressing the respective biosynthetic genes. To identify components critical for this feedback regulation, we performed a genetic screen for Arabidopsis mutants that exhibit enhanced resistance to BCAAs. Multiple dominant allelic mutations in the VALINE-TOLERANT 1 (VAT1) gene were identified that conferred plant resistance to valine inhibition. Map-based cloning revealed that VAT1 encodes a regulatory subunit of acetohydroxy acid synthase (AHAS), the first committed enzyme in the BCAA biosynthesis pathway. The VAT1 gene is highly expressed in young, rapidly growing tissues. When reconstituted with the catalytic subunit in vitro, the vat1 mutant-containing AHAS holoenzyme exhibits increased resistance to valine. Importantly, transgenic plants expressing the mutated vat1 gene exhibit valine tolerance and accumulate higher levels of BCAAs. Our studies not only uncovered regulatory characteristics of plant AHAS, but also identified a method to enhance BCAA accumulation in crop plants that will significantly enhance the nutritional value of food and feed. © 2010 Blackwell Publishing Ltd.

State of rare earth impurities in gallium and indium antimonides

International Nuclear Information System (INIS)

Evgen'ev, S.B.; Kuz'micheva, G.M.

1990-01-01

State of rare earth impurities in indium and gallium antimonides was studied. Results of measuring density and lattice parameter of samples in GaSb-rare earth and InSb-rare earth systems are presented. It is shown that during rare earth dissolution in indium and gallium antimonides rare earth atoms occupy interstitial positions or, at least, are displaced from lattice points

An Automated Pipeline for Engineering Many-Enzyme Pathways: Computational Sequence Design, Pathway Expression-Flux Mapping, and Scalable Pathway Optimization.

Science.gov (United States)

Halper, Sean M; Cetnar, Daniel P; Salis, Howard M

2018-01-01

Engineering many-enzyme metabolic pathways suffers from the design curse of dimensionality. There are an astronomical number of synonymous DNA sequence choices, though relatively few will express an evolutionary robust, maximally productive pathway without metabolic bottlenecks. To solve this challenge, we have developed an integrated, automated computational-experimental pipeline that identifies a pathway's optimal DNA sequence without high-throughput screening or many cycles of design-build-test. The first step applies our Operon Calculator algorithm to design a host-specific evolutionary robust bacterial operon sequence with maximally tunable enzyme expression levels. The second step applies our RBS Library Calculator algorithm to systematically vary enzyme expression levels with the smallest-sized library. After characterizing a small number of constructed pathway variants, measurements are supplied to our Pathway Map Calculator algorithm, which then parameterizes a kinetic metabolic model that ultimately predicts the pathway's optimal enzyme expression levels and DNA sequences. Altogether, our algorithms provide the ability to efficiently map the pathway's sequence-expression-activity space and predict DNA sequences with desired metabolic fluxes. Here, we provide a step-by-step guide to applying the Pathway Optimization Pipeline on a desired multi-enzyme pathway in a bacterial host.

The Fanconi Anemia Pathway in Replication Stress and DNA Crosslink Repair