Academic literature on the topic 'SGCIE'

Myoclonus-dystonia (DYT-SGCE, formerly DYT11) is characterized by alcohol-sensitive, myoclonic-like appearance of fast dystonic movements. It is caused by mutations in the SGCE gene encoding ε-sarcoglycan leading to a dysfunction of this transmembrane protein, alterations in the cerebello-thalamic pathway and impaired striatal plasticity. To elucidate underlying pathogenic mechanisms, we investigated induced pluripotent stem cell (iPSC)-derived striatal medium spiny neurons (MSNs) from two myoclonus-dystonia patients carrying a heterozygous mutation in the SGCE gene (c.298T>G and c.304C>T with protein changes W100G and R102X) in comparison to two matched healthy control lines. Calcium imaging showed significantly elevated basal intracellular Ca2+ content and lower frequency of spontaneous Ca2+ signals in SGCE MSNs. Blocking of voltage-gated Ca2+ channels by verapamil was less efficient in suppressing KCl-induced Ca2+ peaks of SGCE MSNs. Ca2+ amplitudes upon glycine and acetylcholine applications were increased in SGCE MSNs, but not after GABA or glutamate applications. Expression of voltage-gated Ca2+ channels and most ionotropic receptor subunits was not altered. SGCE MSNs showed significantly reduced GABAergic synaptic density. Whole-cell patch-clamp recordings displayed elevated amplitudes of miniature postsynaptic currents and action potentials in SGCE MSNs. Our data contribute to a better understanding of the pathophysiology and the development of novel therapeutic strategies for myoclonus-dystonia.

ZusammenfassungMyoklonus-Dystonie (M-D, DYT11) zählt zu den »Dystonie-Plus-Syndromen«. Patienten zeigen neben fokalen oder segmentalen Dystonien, die sich meist als Schreibkrampf oder zervikale Dystonie manifestieren, v. a. kurz andauernde, »blitzartige« Myoklonien mit Betonung an Hals und oberen Extremitäten. Alkohol bessert die Bewegungsstörung in sehr vielen Fällen drastisch. Heterozygote Mutationen im ɛ-Sarkoglykangen (SGCE) kosegregieren mit der Erkrankung in Familien mit Myoklonus- Dystonie. Dabei handelt es sich in der überwiegenden Mehrzahl um Nonsense- oder Spleißstellenmutationen, die höchstwahrscheinlich zu einem Funktionsverlust des kodierten Proteins führen. Der pathogenetische Mechanismus, durch den ein Fehlen von ɛ-Sarkoglykan im Gehirn M-D auslöst, wurde bisher nicht experimentell aufgeklärt. Für die SGCE-Genregion auf Chromosom 7q21 ist maternales genomisches Imprinting bekannt. Dieser Mechanismus epigenetischer Modifikation führt durch spezifische Methylierung des maternalen SGCE-Promotors zu einer fast ausschließlichen Transkription des paternalen Allels. Dies erklärt die transmissionsspezifische Unterdrückung des M-D-Phänotyps nach Übertragung der Mutation durch die Mutter. M-D ist eine genetisch heterogene Erkrankung. Nur in jeweils einer Familie konnten eine Missense-Mutation im Gen für den D2-Dopamin- Rezeptor bzw. eine Deletion im TorsinA-Gen zusätzlich zu den SGCE-Mutationen nachgewiesen werden. Auf Chromosom 18p wurde bei einer SGCE-negativen M-DFamilie mit typischem Phänotyp ein weiterer Genlocus kartiert (DYT15).

ABSTRACT Imprinted genes are expressed from one allele according to their parent of origin, and many are essential to mammalian embryogenesis. Here we show that the ɛ-sarcoglycan gene (Sgce) andZac1 (Lot1) are both paternally expressed imprinted genes. They were identified in a subtractive screen for imprinted genes using a cDNA library made from novel parthenogenetic and wild-type fibroblast lines. Sgce is a component of the dystrophin-sarcoglycan complex, Zac1 is a nuclear protein inducing growth arrest and/or apoptosis, and Zac1 is a potential tumor suppressor gene. Sgce and Zac1 are expressed predominantly from their paternal alleles in all adult mouse tissues, except that Zac1 is biallelic in the liver andSgce is weakly expressed from the maternal allele in the brain. Sgce and Zac1 are broadly expressed in embryos, with Zac1 being highly expressed in the liver primordium, the umbilical region, and the neural tube.Sgce, however, is strongly expressed in the allantoic region on day 9.5 but becomes more widely expressed throughout the embryo by day 11.5. Sgce is located at the proximal end of mouse chromosome 6 and is a candidate gene for embryonic lethality associated with uniparental maternal inheritance of this region.Zac1 maps to the proximal region of chromosome 10, identifying a new imprinted locus in the mouse, homologous with human chromosome 6q24-q25. In humans, unipaternal disomy for this region is associated with fetal growth retardation and transient neonatal diabetes mellitus. In addition, loss of expression of ZAChas been described for a number of breast and ovarian carcinomas, suggesting that ZAC is a potential tumor suppressor gene.

In mammals, imprinted genes often exist in the form of clusters in specific chromosome regions. However, in pigs, genomic imprinting of a relatively few genes and clusters has been identified, and genes within or adjacent to putative imprinted clusters need to be investigated including those at the SGCE/PEG10 locus. The objective of this study was to, using porcine parthenogenetic embryos, investigate imprinting status of genes within the genomic region spans between the COL1A2 and ASB4 genes in chromosome 9. Whole-genome bisulfite sequencing (WGBS) and RNA sequencing (RNA-seq) were conducted with normal and parthenogenetic embryos, and methylome and transcriptome were analyzed. As a result, differentially methylated regions (DMRs) between the embryos were identified, and parental allele-specific expressions of the SGCE and PEG10 genes were verified. The pig imprinted interval was limited between SGCE and PEG10, since both the COL1A2 and CASD1 genes at the centromere-proximal region and the genes between PPP1R9A and ASB4 toward the telomere were non-imprinted and biallelically expressed. Consequently, our combining analyses of methylome, transcriptome, and informative polymorphisms revealed the boundary of imprinting cluster at the SGCE/PEG10 locus in pig chromosome 9 and consolidated the landscape of genomic imprinting in pigs.

Hereditary myoclonus dystonia is a rare movement disorder characterized with combination of myoclonic jerks with mild to moderate dystonia. Mostly caused due to changes in SGCE gene. Author report case of a 3 years old girl with atypical features of lower limb onset, mild dystonia, upper limb and neck myoclonic jerks and younger onset. She was detected to have pathogenic variant of SGCE gene. A diagnosis of myoclonus dystonia should be considered at an early age also like in our case so that treatment is initiated early for better results and improved quality of life and development.

ABSTRACT Sam68 (Src-associated in mitosis, 68 kDa) is a KH domain RNA binding protein implicated in a variety of cellular processes, including alternative pre-mRNA splicing, but its functions are not well understood. Using RNA interference knockdown of Sam68 expression and splicing-sensitive microarrays, we identified a set of alternative exons whose splicing depends on Sam68. Detailed analysis of one newly identified target exon in epsilon sarcoglycan (Sgce) showed that both RNA elements distributed across the adjacent introns and the RNA binding activity of Sam68 are necessary to repress the Sgce exon. Sam68 protein is upregulated upon neuronal differentiation of P19 cells, and many Sam68 RNA targets change in expression and splicing during this process. When Sam68 is knocked down by short hairpin RNAs, many Sam68-dependent splicing changes do not occur and P19 cells fail to differentiate. We also found that the differentiation of primary neuronal progenitor cells from embryonic mouse neocortex is suppressed by Sam68 depletion and promoted by Sam68 overexpression. Thus, Sam68 controls neurogenesis through its effects on a specific set of RNA targets.

Many cellular processes are governed by stochastic reaction events. These events do not necessarily occur in single steps of individual molecules, and, conversely, each birth or death of a macromolecule (e.g., protein) could involve several small reaction steps, creating a memory between individual events and thus leading to nonmarkovian reaction kinetics. Characterizing this kinetics is challenging. Here, we develop a systematic approach for a general reaction network with arbitrary intrinsic waiting-time distributions, which includes the stationary generalized chemical-master equation (sgCME), the stationary generalized Fokker–Planck equation, and the generalized linear-noise approximation. The first formulation converts a nonmarkovian issue into a markovian one by introducing effective transition rates (that explicitly decode the effect of molecular memory) for the reactions in an equivalent reaction network with the same substrates but without molecular memory. Nonmarkovian features of the reaction kinetics can be revealed by solving the sgCME. The latter 2 formulations can be used in the fast evaluation of fluctuations. These formulations can have broad applications, and, in particular, they may help us discover new biological knowledge underlying memory effects. When they are applied to generalized stochastic models of gene-expression regulation, we find that molecular memory is in effect equivalent to a feedback and can induce bimodality, fine-tune the expression noise, and induce switch.

Myoclonus dystonia (MD) is a rare autosomal-dominant combined dystonia movement disorder characterised by quick, involuntary muscle jerks (myoclonus) paired with sustained muscular contraction (dystonia). Although known to be genetically heterogeneous, the most common genetic factor is mutations within SGCE, the gene encoding ε-sarcoglycan, accounting for approximately 45% of cases. Previous linkage analyses conducted on a family displaying inherited MD without SGCE mutations lead to the identification of another critical region, DYT15. Preliminary data suggested that mutations within the long non-coding RNA (lncRNA) CD108131, found within the DYT15 locus, resulted in decreased expression of both the SGCE transcript, as well as the SGCE protein. Validation of the remaining variants of interest yielded no new candidate genes. A low coverage area coinciding with the entire sequence of TMEM200C was discovered, however subsequent sequencing data revealed no potential disease-causing variants. Therefore, to further characterise the relationship between CD108131 and SGCE suggested by the preliminary data, a CRISPR-Cas9 knockout was developed in HEK293 cells using a double-cut strategy that allowed for complete excision of the CD108131 gene. Stable CD108131 knockout mutant cell lines were examined for differences in gene expression. QRT-PCR analysis was conducted and revealed a significant decrease in SGCE expression in the absence of CD108131. Additionally, expression also trended towards a decrease for ZBTB14, however ARHGAP28 and RPPH1 were not significantly altered. This data demonstrates that the lncRNA CD108131 is likely to have a regulatory effect on SGCE, and perhaps ZBTB14, transcription.

Dissertação (mestrado) - Universidade Federal de Santa Catarina, Centro Tecnologico
Made available in DSpace on 2016-01-08T16:38:50Z (GMT). No. of bitstreams: 1 81150.pdf: 4132154 bytes, checksum: bd35b6a84ef44ff60af76b4676729ce9 (MD5) Previous issue date: 1990
O presente trabalho apresenta uma proposta de informatização das atividades operacionais de um sistema de CIFs, postos informativos do mercado de fretes rodoviários. Esta proposta consiste num sistema computacional (software) baseado em técnicas de inteligência artificial (busca heurística e processamento de linguagem natural), chamado SGCIF, desenvolvido não somente com o propósito de gerênciar as atividades das CIFs, mas também de apresentar uma maneira de maximizar o lucro dos transportadores. O SGCIF possibilita o gerênciamento de um sistema de CIFs de maneira interligada, isto é, todas as CIFs compartilham a mesma bolsa de fretes. Com isso pode haver, em cada CIF o conhecimento de todos os fretes disponíveis em dado momento. Isto amplia os benefícios aos usuários, uma vez que possibilita a escolha da melhor rota entre dois pontos da malha rodoviária, permitindo com isso, uma maximização no lucro do transportador. A determinação desta rota emprega informações heurísticas e algoritmos que constituem idéias sólidas de inteligência artificial. Além de ressaltar a complexidade do problema, este trabalho mostra a modelagem efetuada e sua solução através do algoritmo A* adaptado a função de maximização do lucro.

Dissertação de mestrado integrado em Engenharia e Gestão Industrial
As empresas consumidoras intensivas de energia, encontraram nas potencialidades da Cogeração a complementaridade ideal para a sustentabilidade financeira, através da racionalização dos consumos energéticos e, da venda de energia elétrica para o exterior. O Decreto-Lei 71/2008 de 15 de abril que define o SGCIE não faz erradamente a descriminação das empresas que possuem cogerações não tituladas como autónomas. Como consequência existe uma clara desvirtuação dos indicadores de avaliação do SGCIE. Com o intuito de demonstrar esta situação, este estudo realiza a análise dos consumos energéticos em 2011 na empresa Moda 21 segundo duas Perspetivas: a Perspetiva Balanço (inclui a cogeração no interior do Balanço) e a Perspetiva Moda 21 (exclui a cogeração no interior do Balanço). A Perspetiva Balanço que é a oficial, permite a redução de cerca de 1.158 tep de consumo de energia e de cerca de 1.600 tCO2e de gases de efeito de estufa emitidos para a atmosfera. A existência da unidade de Cogeração permitiu à empresa em 2011 ter um benefício anual de 1.321.758 euros em termos de despesas energéticas. A faturação de 2.266.801 euros da venda de energia elétrica à rede, influência grandemente este resultado. Paralelamente ao objetivo principal deste estudo, pensou-se pertinente analisar a tarifa de remuneração das Cogerações aplicável à Moda 21, definida na Portaria nº 58/2002 de 15 de janeiro. Apesar de ter sido recentemente publicada a Portaria nº140/2012 de 14 de maio, já alterada pela Portaria n.º 325-A/2012 de 16 de outubro, que define um novo regime remuneratório para as Cogerações, o tarifário de remuneração aplicável à Moda 21 mantêm-se até 2020 definido pela mesma Portaria. Aliás, das 166 unidades de Cogeração existentes em Portugal, apenas 32 transitam em 2012 para o novo regime remuneratório.
Companies that are intensive consumers of energy have found the ideal complement for financial sutainability in the potential for Cogeneration through the rationalisation of energy consumption and the sale of electricity to third parties. The Decree Law 71/2008 of 15 April that defines SGCIE mistakenly does not include companies that have unlisted cogeneration units as autonomous. As a consequence, there is a clear distortion of the evaluation indicators of the SGCIE. In order to highlight this situation, this study analyses the consumption of energy in the company “Moda 21” in 2011 from two perspectives: the Balance Sheet perspective (including cogeneration in the balance sheet) and the Moda 21 perspective (excluding cogeneration from the balance sheet). The Balance Sheet perspective, which is the official one, enables a reduction of approximately 1,158 tep in energy consumption and of approximately 1,600 tCO2e of greenhouse gases emitted into the atmosphere. In 2011 the existence of a cogeneration unit enabled the company to benefit annually by 1,321,758 euros in terms of energy expenditure. The sale of 2,266,801 euros of electricity to the national grid greatly influenced this result. In addition to the main aim of this study, it was also considered relevant to analyse the remuneration rate for cogeneration which is applicable to Moda 21 through Ordinance number 58 of January 15, 2002. Despite the recently published Ordinance number 140 of 14 May 2012, which has already been replaced by ordinance number 325-A/16 October, 2012, and defines a new remuneration scheme for cogeneration, the remuneration rate which is applicable to Moda 21 will remain in place until 2020. In fact, of 166 cogeneration units in Portugal, only 32 will change over to the new remuneration scheme in 2012.

Tese de mestrado integrado em Engenharia da Energia e do Ambiente, apresentada à Universidade de Lisboa, através da Faculdade de Ciências, 2014
A eficiência energética é uma matéria que tem vindo a assumir maior relevância na sociedade, face aos compromissos internacionais no combate ao desperdício de energia e às alterações climáticas. Neste contexto, o Governo Português avançou com um regulamento de Eficiência Energética mandatório destinado ao sector Industrial, logo em 1982, denominado por Regulamento da Gestão do Consumo de Energia (RGCE), que tem vindo a ser acompanhado e actualizado até aos dias de hoje, sendo actualmente denominado por Sistema de Gestão dos Consumos Intensivos de Energia (SGCIE) com a sua última revisão e actualização sido feita em Janeiro de 2013. O SGCIE comporta uma diversidade de intervenientes, com formações, ambições, prioridades e pontos de vista distintos, o que se traduz numa divergência de opiniões quanto à real eficácia do regulamento. Esta dissertação tem como objectivo principal analisar a evolução e efeito do regulamento, através da análise de dois casos de estudo reais e do benchmarking de políticas equivalentes de outros países. Os resultados e conclusões dos casos de estudo, bem como as entrevistas realizadas a parte dos intervenientes de todo o processo, permitem revelar algumas lacunas e pontos-críticos do regulamento. É apresentada informação minimamente detalhada relativa às políticas de eficiência energética na indústria do Reino Unido e Holanda, e informação mais generalizada de políticas de outros países. Desta forma, foi possível fazer uma comparação do funcionamento do SGCIE e respectivos pontos-críticos com outras políticas internacionais análogas. Pretendendo-se com isso, transportar de forma adequada e adaptada para o sistema Português, as soluções que as diversas políticas internacionais apresentam para esses pontos-críticos. Os resultados obtidos neste benchmarking levam à conclusão de que alguns dos pontos-críticos apontados ao SGCIE são comuns às políticas internacionais análogas, no entanto, estas apresentam um funcionamento mais saudável como resultado da melhor relação entre os governos e indústria, devido à existência de entidades intermédias como associações industriais sectoriais. Conclui-se, desta forma, que o melhoramento do SGCIE poderá passar pela introdução destas terceiras entidades em substituição ou actualização de uma ADENE multissectorial e relativamente próxima da entidade fiscalizadora, a DGEG, bem como pela criação de metas colectivas, adaptadas e mesuráveis por sector à responsabilidade da respectiva associação, em alternativa às metas fixas por instalação. A criação de mecanismos que permitam a introdução do modelo ESCO e de princípios de M&V (Medição e Verificação), no esquema de auditoria e acompanhamento de resultados do SGCIE, será também uma mais-valia no sentido em que os resultados obtidos serão quantificados em custos e consumos evitados reais, bem como com um menor nível de incerteza. A competência dos técnicos auditores deverá também ser garantida através de qualificações adequadas no âmbito dos princípios de M&V.
Energy efficiency issue has assumed greater relevance in world society, considering the international commitments to combat energy waste and climate change. In this context, the Portuguese Government has put forward a Regulation of Energy Efficiency bound to the Industrial sector, as early as 1982 labeled by Regulations on the Management of Energy Consumption (RGCE in the Portuguese acronym), which has been monitored and updated to the present day, currently labeled by Energy Intensive Consumption Management System (SGCIE in the Portuguese acronym) which the last review and update was made in January of 2013. The SGCIE comprises a diversity of stakeholders with distinct backgrounds, ambitions, priorities and points of view, which results into different types of opinions when it concerns the real effectiveness of this regulation. This thesis aims to analyze not only the evolution, but also the purpose of the regulation, through the analysis of two real case studies and with a benchmarking of similar policies in other countries. The results and conclusions of the case studies as well as the interviews with the stakeholders throughout the process SGCIE, allowed to reveal some gaps and other critical issues of the regulation. These issues and gaps are shown with their respective workarounds where it is possible. It is also given an information with some level of detail of equivalent policies in countries like UK and Netherlands, and more general information of equivalent systems of other countries, in order to identify points that can be adequately adapted to the Portuguese system of promoting energy efficiency in the industrial sector. The results obtained in this benchmarking lead to the conclusion that some of the SGCIE’s critical points are common to the similar international policies. However, these ones present an healthier application, as a result of the better link between the governments and the industry, because of the existence of intermediate entities, such as sectorial industrial associations. In conclusion, improving the SGCIE may not only involve the introduction of these third entities to replace or assist the ADENE, which is multisectoral and relatively close to the supervisory DGEG, but also the creation of collective goals, adapted and measurable by sector on the respective association’s responsibility, as an alternative to the fixed targets for each individual enterprise. The creation of mechanisms capable of allowing the introduction of the ESCO model and the M&V (Measurement and Verification)’s principles, in the auditing and monitoring processes of SGCIE scheme, will also be an asset, in order to obtain quantified results, in actual costs and consumptions avoided with a lower level of uncertainty. The technical competence of the auditors should also be ensured through appropriate qualifications within the M&V’s principles.

O presente Relatório tem como principal finalidade a apresentação da caracterização detalhada dos circuitos das traçagens da Prio Biocombustíveis S.A. obtida durante o Estágio realizado nesta empresa. A caraterização dos circuitos das traçagens iniciou-se com o reconhecimento dos circuitos no quadro geral das traçagens, seguindo-se a identificação dos mesmos no processo, fazendo correspondência aos circuitos do quadro. Este procedimento permitiu a elaboração dos esquemas elétricos das traçagens, a atualização dos documentos referentes à localização da atuação das traçagens no processo, o registo das temperaturas reguladas e do fluído em que atuam, e a etiquetagem das caixas de alimentação e derivação, de forma a identificar facilmente cada circuito. Este reconhecimento permitiu identificar a utilização de traçagens em partes de processo que não estão em atividade. Depois do reconhecimento dos circuitos no quadro procedeu-se à medição individual e global dos circuitos do quadro geral das traçagens. Terminadas as medições procedeu-se ao tratamento dos dados recolhidos, o que permitiu identificar uma redução de 13% da potência total relativamente ao valor apresentado na Auditoria Energética, realizada na empresa em 2015. Contudo, quando se fez o somatório das potências individuais de cada circuito ao longo do período do Estágio, o valor total voltou a aproximar-se do valor obtido em 2015, o que evidencia a influência das condições climatéricas na ativação dos circuitos das traçagens. Com base nos dados obtidos, foram analisadas e propostas algumas medidas de racionalização energética, nomeadamente desligar circuitos não utilizados, reduzir as temperaturas reguladas e colocar termómetros nas linhas. Destas, foram implementadas as duas primeiras medidas. Implementadas as medidas, foi realizada uma nova medição que permitiu verificar uma redução imediata de cerca de 10,4 kW na potência geral do quadro. Esta redução pode ser considerada ao longo de todo o ano, uma vez que as temperaturas registadas permitiram concluir que estes circuitos estão ligados o ano inteiro. Foi realizado um caso de estudo, que permitiu concluir que a utilização das traçagens por vezes não será necessária durante a atividade normal da fábrica. Assim, as traçagens devem ser analisadas de forma a perceber a sua correta utilização e verdadeira utilidade.

Dissertação de mestrado integrado em Engenharia Mecânica
Nesta dissertação foi feita uma análise energética à área de distribuição e arrefecimento de águas, de uma fábrica de papel, com o objectivo de seguir as orientações propostas pelo SGCIE (Sistema de Gestão dos Consumos Intensivos de Energia). Este sistema de gestão é regulado segundo o decreto-lei 71/2008 de 15 de Abril e pressupõe um balanço energético da instalação, que neste caso trata-se de uma área sectorial que só representa cerca de 5 % do consumo total de energia da fábrica. Após o balanço é feita uma auditoria à instalação com o objectivo de propor alterações que aumentem a eficiência energética, reduzindo consumos e rácios de emissão de CO2 sem prejudicar o processo produtivo. Foram feitos estudos da rentabilidade da alteração de motores eléctricos por outros com maior rendimento, do aproveitamento térmico das águas de retorno da unidade fabril, das medidas para redução do consumo do ar comprimido e aumento da eficiência na sua produção e da aplicação prática de variadores de frequência em diversas bombas centrífugas para transporte de água. Em conclusão, o aproveitamento térmico das águas de retorno, mediante a aplicação de um permutador de calor e algumas alterações na distribuição da água, provou ser capaz de poupanças em energia térmica na ordem dos 14%, relativamente à área de distribuição e arrefecimento de águas. Concluiu-se também, que a aplicação de variadores de frequência em bombas centrífugas é igualmente bastante rentável, permitindo poupanças de energia eléctrica superiores a 7% da área total auditada.
The main objective of this thesis is the energy analysis of the distribution and water cooling system in a paper mill in order to comply with the guidelines proposed by SGCIE (Management System for Intensive Energy Consumers). This management system is regulated according to the Decree-Law 71/2008 of 15 April and requires an energy balance of the installation, which in this case, includes a sectorial area that only represents about 5% of the total energy consumption in all the plant. The energy balance was followed by a detailed audit of the installation with the aim to recommend changes that will increase energy efficiency, reducing fuel consumption and CO2 emissions, without negatively affecting the production process. Studies were made showing the relevance of changing electric motors by others with higher efficiency, of the thermal recovery of returned water, of the actions to reduce compressed air consumption and increase efficiency and the practical application of variable frequency motors to several centrifugal pumps. In conclusion, the thermal recovery of returned water, through the application of a heat exchanger and some modifications in the water distribution, proved to be able to reach savings of around 14% in thermal energy, relative to the area of the distribution and water cooling. It is also concluded, that the application of variable frequency drives on centrifugal pumps is also fairly cost effective, with electrical power savings of more than 7% of the analyzed installation.

O aumento da competitividade do mercado e a crescente preocupação com o consumo energético, relativamente aos seus custos e impactos ambientais, têm vindo a pressionar as indústrias a implementar medidas de melhoria de desempenho, de modo a reduzir os seus consumos. A Herdade do Esporão (HE), inserida no grupo Esporão S.A., é uma de duas empresas portuguesas produtoras vitivinícolas certificadas pela ISO 50001. O seu consumo anual de cerca de 750 tep, permite caracterizá-la como um Consumidor Intensivo de Energia (CIE) que procura a melhoria contínua do seu desempenho energético. Neste sentido, caracterizou-se o consumo de utilidades, foram revistos e atualizados o Sistema de Gestão de Energia (SGE) e os Indicadores de Desempenho Energético (IDE) implementados, estabeleceram-se objetivos com a finalidade de controlo dos consumos, e foram propostas medidas de melhoria de eficiência energética. Da caracterização do consumo de utilidades foi possível determinar que os CIE são a Rega da Barragem (16%), a Linha de Enchimento (12%), o Enoturismo (12%), o Sistema de Refrigeração (11%), a ETAR (10%), o Lagar (9%) e a Central de Ar Comprimido (CAC) (8%). Foi possível, também, aferir 26 pontos de medição em falta de modo a controlar o consumo de todas as utilidades consumidas. Foram propostas 5 medidas de melhoria de eficiência energética, que permitem a redução de 6,15% de energia elétrica (EE), 1,51% da água e 1,00% da biomassa consumidas. As 3 medidas implementadas foram a substituição de iluminação, correções à CAC, e a substituição do filtro da Estação de Tratamento de Água. Estas, permitiram uma redução de 1,57% e 2,21% da EE e 1,51% da água consumidas, respetivamente. As medidas a implementar futuramente envolvem a redução de fugas de ar comprimido e as alterações ao sistema de monitorização que reduzirão 2,32% e 0,66% da EE e 1,00% da biomassa consumida. Propôs-se, ainda, a implementação de uma nova Central Fotovoltaica de 550 kWp, reduzindo em 22,87% a energia comprada à rede. Elevando para 40% o autoconsumo na HE e evitando a emissão de 205 tonCO2e.

For the lengthy process of different reactor types operation, huge amounts of spent graphite were accumulated in several countries. Spent reactor-grade graphite constitutes the largest fraction of the solid radioactive wastes accumulated all over the world. So, search for ways of spent graphite utilization is an urgent problem of international character. The purpose of the present work is to evaluate radioactive contamination of spent graphite sleeves from decommissioned reactors I-1, EI-2, ADE-3 of the Siberian Group of Chemical Enterprises and to consider a possibility for spent graphite sleeves utilization by incineration. Planning of actions on spent graphite management requires to take into account the reactor type, properties of spent graphite, peculiarities of spent graphite usage and storing. Beginning from 1996, authors have conducted the detailed studies on radioactive contamination of spent graphite from the SGCE reactors. In these studies, contents of some radionuclides in spent reactor-grade graphite were measured, including content of isotopes generated by neutron-induced activation of impurities (60Co, 3H, 14C, etc.) and the isotopes generated in result of uranium penetration into the graphite reactor components (137Cs, 90Sr, 241,243Am, 244Cm, Pu isotopes, etc.). It was revealed the largest contribution to the graphite radioactivity was given by 14C. Using data on inhalation and ingestion rate for different age groups, the evaluations were carried out for 14C intake into human organism with air and food. Analysis of the results obtained in the performed investigation enabled us to make the following conclusions: 1. the most part of the SGCE storage vaults where spent graphite sleeves are disposed of do not meet contemporary requirements; 2. the radioactive contamination level of spent graphite sleeves from the SGCE reactors allows their utilization by incineration at the SGCE territory; 14C exhaust into the atmosphere will not cause any significant risk of negative consequences for human health of cities Seversk and Tomsk population; 3. spent sleeves incineration and subsequent storing of incineration products can not lead to appearance of nuclear dangerous situation; 4. there is an available technology and associated equipment for conduction of experiment on spent graphite incineration. Thus, it can be concluded that nowadays there are all the conditions for planning, preparation and conduction of fullscale experiment on incineration of some spent graphite sleeves taken from the SGCE storage vaults. The results obtained in the test graphite incineration will allow to evaluate and, if necessary, improve the utilization technology and the applied equipment.

Abstract The paper is devoted to investigations of spent reactor graphite contamination. The study was based on the sampling from the graphite stacks and sleeves of three shut down plutonium-production reactors in Siberian Group of Chemical Enterprises (SGCE), Seversk (Tomsk-7). Several hundreds graphite samples were analysed by means of α-, β-, γ- and X-ray spectrometry. Contents of 3H, 14C, fission products, actinides, products of impurities neutron activation were determined. The inventories of above mentioned radionuclides were estimated. Correlation between content of various groups of radionuclides was studied. Graphite sleeves contamination was studied depending on the period of their irradiation. Comparison of obtained experimental data for the three SGCE reactors with calculated predictions showed substantial difference. The conclusion was made on applicability of the data obtained for estimations of spent graphite contamination in other reactor types including RBMK reactors. The main part of the studies was performed in the frame of the ISTC Projects No. 561 and No. 1409 fulfilment.

This report maps the African landscape of Open Science – with a focus on Open Data as a sub-set of Open Science. Data to inform the landscape study were collected through a variety of methods, including surveys, desk research, engagement with a community of practice, networking with stakeholders, participation in conferences, case study presentations, and workshops hosted. Although the majority of African countries (35 of 54) demonstrates commitment to science through its investment in research and development (R&D), academies of science, ministries of science and technology, policies, recognition of research, and participation in the Science Granting Councils Initiative (SGCI), the following countries demonstrate the highest commitment and political willingness to invest in science: Botswana, Ethiopia, Kenya, Senegal, South Africa, Tanzania, and Uganda. In addition to existing policies in Science, Technology and Innovation (STI), the following countries have made progress towards Open Data policies: Botswana, Kenya, Madagascar, Mauritius, South Africa and Uganda. Only two African countries (Kenya and South Africa) at this stage contribute 0.8% of its GDP (Gross Domestic Product) to R&D (Research and Development), which is the closest to the AU’s (African Union’s) suggested 1%. Countries such as Lesotho and Madagascar ranked as 0%, while the R&D expenditure for 24 African countries is unknown. In addition to this, science globally has become fully dependent on stable ICT (Information and Communication Technologies) infrastructure, which includes connectivity/bandwidth, high performance computing facilities and data services. This is especially applicable since countries globally are finding themselves in the midst of the 4th Industrial Revolution (4IR), which is not only “about” data, but which “is” data. According to an article1 by Alan Marcus (2015) (Senior Director, Head of Information Technology and Telecommunications Industries, World Economic Forum), “At its core, data represents a post-industrial opportunity. Its uses have unprecedented complexity, velocity and global reach. As digital communications become ubiquitous, data will rule in a world where nearly everyone and everything is connected in real time. That will require a highly reliable, secure and available infrastructure at its core, and innovation at the edge.” Every industry is affected as part of this revolution – also science. An important component of the digital transformation is “trust” – people must be able to trust that governments and all other industries (including the science sector), adequately handle and protect their data. This requires accountability on a global level, and digital industries must embrace the change and go for a higher standard of protection. “This will reassure consumers and citizens, benefitting the whole digital economy”, says Marcus. A stable and secure information and communication technologies (ICT) infrastructure – currently provided by the National Research and Education Networks (NRENs) – is key to advance collaboration in science. The AfricaConnect2 project (AfricaConnect (2012–2014) and AfricaConnect2 (2016–2018)) through establishing connectivity between National Research and Education Networks (NRENs), is planning to roll out AfricaConnect3 by the end of 2019. The concern however is that selected African governments (with the exception of a few countries such as South Africa, Mozambique, Ethiopia and others) have low awareness of the impact the Internet has today on all societal levels, how much ICT (and the 4th Industrial Revolution) have affected research, and the added value an NREN can bring to higher education and research in addressing the respective needs, which is far more complex than simply providing connectivity. Apart from more commitment and investment in R&D, African governments – to become and remain part of the 4th Industrial Revolution – have no option other than to acknowledge and commit to the role NRENs play in advancing science towards addressing the SDG (Sustainable Development Goals). For successful collaboration and direction, it is fundamental that policies within one country are aligned with one another. Alignment on continental level is crucial for the future Pan-African African Open Science Platform to be successful. Both the HIPSSA ((Harmonization of ICT Policies in Sub-Saharan Africa)3 project and WATRA (the West Africa Telecommunications Regulators Assembly)4, have made progress towards the regulation of the telecom sector, and in particular of bottlenecks which curb the development of competition among ISPs. A study under HIPSSA identified potential bottlenecks in access at an affordable price to the international capacity of submarine cables and suggested means and tools used by regulators to remedy them. Work on the recommended measures and making them operational continues in collaboration with WATRA. In addition to sufficient bandwidth and connectivity, high-performance computing facilities and services in support of data sharing are also required. The South African National Integrated Cyberinfrastructure System5 (NICIS) has made great progress in planning and setting up a cyberinfrastructure ecosystem in support of collaborative science and data sharing. The regional Southern African Development Community6 (SADC) Cyber-infrastructure Framework provides a valuable roadmap towards high-speed Internet, developing human capacity and skills in ICT technologies, high- performance computing and more. The following countries have been identified as having high-performance computing facilities, some as a result of the Square Kilometre Array7 (SKA) partnership: Botswana, Ghana, Kenya, Madagascar, Mozambique, Mauritius, Namibia, South Africa, Tunisia, and Zambia. More and more NRENs – especially the Level 6 NRENs 8 (Algeria, Egypt, Kenya, South Africa, and recently Zambia) – are exploring offering additional services; also in support of data sharing and transfer. The following NRENs already allow for running data-intensive applications and sharing of high-end computing assets, bio-modelling and computation on high-performance/ supercomputers: KENET (Kenya), TENET (South Africa), RENU (Uganda), ZAMREN (Zambia), EUN (Egypt) and ARN (Algeria). Fifteen higher education training institutions from eight African countries (Botswana, Benin, Kenya, Nigeria, Rwanda, South Africa, Sudan, and Tanzania) have been identified as offering formal courses on data science. In addition to formal degrees, a number of international short courses have been developed and free international online courses are also available as an option to build capacity and integrate as part of curricula. The small number of higher education or research intensive institutions offering data science is however insufficient, and there is a desperate need for more training in data science. The CODATA-RDA Schools of Research Data Science aim at addressing the continental need for foundational data skills across all disciplines, along with training conducted by The Carpentries 9 programme (specifically Data Carpentry 10 ). Thus far, CODATA-RDA schools in collaboration with AOSP, integrating content from Data Carpentry, were presented in Rwanda (in 2018), and during17-29 June 2019, in Ethiopia. Awareness regarding Open Science (including Open Data) is evident through the 12 Open Science-related Open Access/Open Data/Open Science declarations and agreements endorsed or signed by African governments; 200 Open Access journals from Africa registered on the Directory of Open Access Journals (DOAJ); 174 Open Access institutional research repositories registered on openDOAR (Directory of Open Access Repositories); 33 Open Access/Open Science policies registered on ROARMAP (Registry of Open Access Repository Mandates and Policies); 24 data repositories registered with the Registry of Data Repositories (re3data.org) (although the pilot project identified 66 research data repositories); and one data repository assigned the CoreTrustSeal. Although this is a start, far more needs to be done to align African data curation and research practices with global standards. Funding to conduct research remains a challenge. African researchers mostly fund their own research, and there are little incentives for them to make their research and accompanying data sets openly accessible. Funding and peer recognition, along with an enabling research environment conducive for research, are regarded as major incentives. The landscape report concludes with a number of concerns towards sharing research data openly, as well as challenges in terms of Open Data policy, ICT infrastructure supportive of data sharing, capacity building, lack of skills, and the need for incentives. Although great progress has been made in terms of Open Science and Open Data practices, more awareness needs to be created and further advocacy efforts are required for buy-in from African governments. A federated African Open Science Platform (AOSP) will not only encourage more collaboration among researchers in addressing the SDGs, but it will also benefit the many stakeholders identified as part of the pilot phase. The time is now, for governments in Africa, to acknowledge the important role of science in general, but specifically Open Science and Open Data, through developing and aligning the relevant policies, investing in an ICT infrastructure conducive for data sharing through committing funding to making NRENs financially sustainable, incentivising open research practices by scientists, and creating opportunities for more scientists and stakeholders across all disciplines to be trained in data management.