Molecular effect of an OPTN common variant associated to Paget's disease of bone

Paget's disease of bone (PDB) is a chronic bone disorder and although genetic factors appear to play an important role in its pathogenesis, to date PDB causing mutations were identified only in the Sequestosome 1 (SQSTM1) gene at the PDB3 locus. PDB6 locus, also previously linked to PDB, contains several candidate genes for metabolic bone diseases. We focused our analysis in the most significantly associated variant with PDB, within the Optineurin (OPTN) gene, i.e. the common variant rs1561570. Although it was previously shown to be strongly associated with PDB in several populations, its contribution to PDB pathogenesis remains unclear. In this study we have shown that rs1561570 may contribute to PDB since its Tallele results in the loss of a methylation site in patients' DNA, leading to higher levels of OPTN gene expression and a corresponding increase in protein levels in patients' osteoclasts. This increase in OPTN expression leads to higher levels of NF-KB translocation into the nucleus and increasing expression of its target genes, which may contribute to the overactivity of osteoclasts observed in PDB. We also reported a tendency for a more severe clinical phenotype in the presence of a haplotype containing the rs1561570 T allele, which appear to be re-enforced with the presence of the SQSTM1/P392L mutation. In conclusion, our work provides novel insight towards understanding the functional effects of this variant, located in OPTN intron 7, and its implication in the contribution to PDB pathogenesis.national funds from Foundation for Science and Technology (FCT) [UID/Multi/04326/2013]; Canadian Institutes for Health Research, Canada [MOP130457]; CHU de Quebec Foundation; Canadian Foundation for Innovation; Fonds de recherche du Quebec-sante; Laval University; CHU de Quebec-Universite Laval Research Centre; FCT [SFRH/BD/77227/2011, SFRH/BPD/111898/2015]; Fonds de recherche Quebec-Sante (FRQ-S), Quebec, Canad

Information Flow Impediments in Disaster Relief Supply Chains

Supply Chain Management (SCM) is seldom more difficult than during disaster relief efforts. As supply chains quickly form in response to a disaster, a slow information flow presents a major hindrance to coordinating the allocation of resources necessary for disaster relief efforts. This paper identifies impediments to the flow of information through supply chains following large scale and catastrophic disasters. Given the scarce body of literature on this subject, a grounded theory case study was conducted to examine an extreme case. The study concentrates on the efforts of multiple organizations and individuals that provided relief in the aftermath of Hurricane Katrina, which battered the Gulf Coast of the southeastern United States in late 2005. Data was gathered from diverse sources, including government agencies, profit and non-profit organizations, and individuals, during and after the disaster. Based on our data analysis, we not only identify information flow impediments (i.e., inaccessibility, inconsistent data and information formats, inadequate stream of information, low information priority, source identification difficulty, storage media misalignment, unreliability, and unwillingness), but also identify likely sources of these impediments, and examine their consequences to organizations’ disaster recovery efforts. Our findings suggest some potential design principles for devising solutions capable of reducing or alleviating the impact of information flow impediments in future disasters

Housing in portugal (1992-2008)A multidimensional perspective on the behaviour of economic agents / Moradia em portugal (1992-2008)Uma perspectiva multidimensional do comportamento dos agentes econômicos

 Since the nation joined the European Union, the process that regulates housing policy in Portugal has been subject to specificities that are proper of the Portuguese situation. In a State which has a considerable housing deficit, it was the quantifier elements that judged the various governments regarding this matter. Almost all of the housing production depended on mortgage loans in which soft loans played an important part, together with tax benefits.  The negative effects produced by lease blocking were increasingly felt throughout the analyzed period of time. The changes in context due to the decrease of tax rates and the legislative alterations on soft loans, conditioned the agents behavior and, necessarily, the rhythm of Portugal’s housing market. Using the STATIS (Structuration of Tableaux A Trois Indices de la Statistique) methodology and through the analysis of the housing policy instruments, it was possible to identify the trajectories and changes in performance of the State, Owners and Families during the period between 1992 and 2008, as well as the most significant variables for understanding the problematic of housing in Portugal

Inventarios y liquidez en las empresas ferreteras, Callao 2020

La presente investigación planteó como objetivo, Determinar la relación entre el control de inventarios y la liquidez en las empresas Ferreteras, Callao 2020. El estudio fue de tipo básica, enfoque cuantitativo, diseño no experimental, nivel descriptivo correlacional, la población como objeto de estudio estuvo conformada por 6 empresas ferreteras del Callao y se tomó como unidad de análisis a 21 trabajadores. Para la recolección de datos se utilizó el cuestionario el mismo que fue validado mediante el juicio de 4 Expertos conocedores del tema de la universidad Cesar Vallejo, para el procesamiento de datos se utilizó el SPSS Versión 24 y se obtuvo como resultado un Alfa de Cronbach de 0,967 y para determinar la correlación entre ambas variables se realizó la prueba de hipótesis con Rho de Spearman cuyo resultado fue de 0,604, ya que algunas veces se planifican las compras en función a la liquidez, la necesidad de la empresa, y las ventas estimadas, La gerencia eventualmente evalúa la determinación de precios de los productos, e informa a ventas, no se registra los movimientos de mercadería en el kardex de manera clara y precisa, falta de elaboración y aplicación de los ratios para conocer la disponibilidad de liquidez

Planificación financiera y la toma de decisiones de las empresas ferreteras AA.HH. Bocanegra, Callao 2023

La presente investigación tuvo como objetivo general determinar la relación que existe entre la planificación financiera y la toma de decisiones en las empresas Ferreteras AA.HH. Bocanegra, Callao 2023, se enmarca dentro del ODS 12 Producción y consumo responsable. La metodología utilizada fue tipo básica, enfoque cuantitativo, diseño no experimental de corte transversal, nivel correlacional, método deductivo, la técnica utilizada para la recopilación de información fue la encuesta y como instrumento el cuestionario, la investigación fue censal. Se obtuvo como resultado que el 42.9% de encuestados consideran que una planificación financiera será mala cuando se toman malas decisiones. Se concluyó que existe una correlación alta positiva de 0.869 entre la planificación financiera y la toma de decisiones, esto permite afirmar que la planificación financiera se relaciona significativamente con la toma de decisiones, es decir la implementación de la planificación financiera facilitará la toma de decisiones. Se recomienda implementar procedimientos para mejorar: La gestión del plan financiero que aborde el control financiero, herramientas financieras y la identificación de los riesgos financieros; además realizar en análisis FODA, evaluar alternativas de financiamiento, comparar las tasas de interés, finalmente deben tomar en cuenta las habilidades técnicas y el liderazgo en la toma de decisiones

Análise da elaboração de instrumentos de ação pública na Agência Nacional de Aviação Civil (ANAC) : revisão da resolução n° 279

Trabalho de Conclusão de Curso (graduação)—Universidade de Brasília, Faculdade de Economia, Administração, Contabilidade e Gestão de Políticas Públicas, Departamento de Gestão de Políticas Públicas, 2019.Este trabalho tem a finalidade de investigar o processo de alteração de um instrumento de ação pública na Superintendência de Infraestrutura Aeroportuária (SIA) da Agência Nacional de Aviação Civil (ANAC). A finalidade da pesquisa é compreender como os atores se articularam para a produção de um instrumento de ação pública na ANAC, no caso, o primeiro módulo de revisão da resolução n° 279, que estabelece critérios r egulatórios quanto à implantação, operação e manutenção do Serviço de Prevenção, Salvamento e Combate a Incêndio em Aeródromos Civis (SESCINC), identificando as estratégias que utilizaram para a revisão de uma resolução, demonstrando a multiatorialidade envolvida com o processo e como diálogos informais auxiliaram na construção da norma. Para isso foi feito um estudo de caso, com pesquisa documental, em atas e relatórios, entrevistas com reguladores e re gulados e para a análise de dados, a análise de discurso foi utilizada. Como resultado, as estratégias que utilizaram para o primeiro módulo de revisão da resolução n° 279 foram encontradas, identificou se o workshop, a Análise de Impacto Regulatório (AIR) AIR), como etapas/estratégias na fase de estudos e na fase de elaboração da proposta de revisão do normativo, as fases da audiência pública e deliberação final, para além desses ritos processuais, identificou se os diálogos informais, que junto com os outros é componente de um repertório, repertório do módulo I de revisão da resolução n° 279, esses diálogos representam um repertório diferente de elaboração de ato normativo. Além disso, os atores que participaram do processo também foram identificados, os regu lados, operadores de aeródromo e os reguladores, Especialistas em Regulação de Aviação Civil, em especial da Gerência Técnica de Normas (GTNO) e da Gerência Técnica de Resposta à Emergência Aeroportuária (GTRE), lotadas na Superintendência de Infraestrutur a Aeroportuária (SIA) e Diretoria. É importante considerar como resultado, o protagonismo da Agência no processo, ainda reconhecendo que houveram dinâmicas de governança, como aponta Lascoumes e Le Galès (2012), práticas que envolvem múltiplos atores e mul tiformes. Todos os aspectos discutidos foram importantes para descobrir como os atores se articularam para a revisão da resolução n° 279, resultando na resolução n° 455, que altera critérios dispostos na n° 279

Avaliação do acesso aos serviços de saúde por imigrantes brasileiros em Portugal

A comunidade brasileira constitui-se na maior população estrangeira residente em terras lusitanas. Esta pesquisa objetivou conhecer as representações sociais de 70 imigrantes brasileiros residentes em Portugal há pelo menos um ano, que já tinham utilizado serviços públicos de saúde. O cenário da Pesquisa foi a “Casa do Brasil” em Lisboa, instituição de apoio sócio-jurídico aos imigrantes. Os instrumentos de coleta de dados foram um Teste de associação livre de palavras (TALP), com os estímulos: saúde, doença, acesso à serviços de saúde, além de entrevista semi-estruturada. A preocupação com adoecimento, dificuldades, adversidades e insegurança estão presentes na realidade desse grupo. Conclui-se ser necessário o delineamento de políticas e estratégias de intervenção, especialmente preventivas, mais resolutivas, abrangentes e eficazes para os imigrantes. É importante que eles sejam incluídos nas políticas sociais e de saúde, considerando que têm direitos e deveres como qualquer cidadão e como força de trabalho, ajudam o crescimento do país.The Brazilian community constitutes the largest foreign population resident in Portugal. The aim was to understand the social representations of 70 Brazilian immigrants living in Portugal for at least one year who had used public health services. The scenario of the search was the "House of Brazil" in Lisbon, the institution of socio-legal support to immigrants. The data collection instruments were a Test Free Word Association (TALP), with stimuli: health, disease, access to health services, as well as semi-structured interview. The preoccupation with illness, difficulty, adversity, insecurity is present in reality this group. It is concluded that the design of appropriate policies and intervention strategies, especially preventive, resolving most comprehensive and effective for immigrants. It is important they be included in social policy and health, considering they have any rights and duties as a citizen and as a workforce, help the country's growth

Removal of Hydrophobic Organic Pollutants and Copper by Alginate-Based and Polycaprolactone Materials

Funding Information: This research was funded by the FUNDAÇÃO PARA A CIÊNCIA e TECNOLOGIA (FCT—Portugal), grant number PTDC/BIA-MIB/31864/2017 and by LA/P/0045/2020 (ALiCE), UIDB/50020/2020 and UIDP/50020/2020 (LSRE-LCM), funded by national funds through FCT/MCTES (PIDDAC) and by FEDER funding CENTRO-01-0246-FEDER-000044. Publisher Copyright: © 2022 by the authors.Organic pollutants (OPs) and heavy metals are environmental toxicants associated with great concerns. Decontamination processes are urgent for both, and the possibility to achieve their simultaneous removal from polluted waters is highly interesting. Additionally, in many cases, the effect of organic matter in the removal process is overlooked and must be considered. This work aimed to study the potential of alginate-based and polycaprolactone (PCL) materials to remove OPs and copper ions in the absence and presence of organic matter. The OPs investigated were the polycyclic aromatic hydrocarbons anthracene and benzo[a]pyrene, and the pesticide chlorpyrifos, both hydrophobic compounds. Copper (II) ions were used as a model of heavy metals. Alginate-based spheres were prepared by gelation, and PCL microparticles were obtained by oil/water emulsion solvent evaporation. The materials with the highest efficiencies for OP removal from aqueous solutions were those with activated carbon and PCL. Furthermore, the spheres with activated carbon could remove anthracene and copper simultaneously, even in the presence of humic acid. This work points to activated carbon–alginate spheres as a multifunctional adsorbent able to remove different pollutants and to PCL for potential applications in OP decontamination processes.publishersversionpublishe