Induced pluripotent stem cell line (INSAi001-A) from a Gaucher disease type 3 patient compound heterozygote for mutations in the GBA1 gene

Gaucher Disease (GD) type 3 is a neurological form of a multisystemic autosomal recessive disorder belonging to the group of lysosomal storage diseases. Causal mutations in the glucocerebrosidase 1 (GBA1) commonly lead to abnormal protein and GD, heterozygosity is a genetic risk factor for Parkinson's disease. This work describes the use of a non-integrative approach using Sendai Virus delivery to establish induced Pluripotent Stem Cells (iPSCs) from fibroblasts from a GD type 3 patient. Differentiation of iPSCs can be employed to generate a variety of complex cell types with a high degree of genetic complexity that would otherwise be unattainable.Funding Agency Portuguese Foundation for Science and Technology PTDC/BIM-MEC/4762/2014info:eu-repo/semantics/publishedVersio

Analysis of a vegetable oil performance in a milling process by MQL Lubrication

In this work, we carried out a comparison between the dry machining of an aluminum block with conventional cutting oil and a block with vegetable oil. The two oils had different flow rates. Using the Taguchi method, it was possible to determine the matrices for optimizing the best parameters for each group of tests. Then, we studied the utility of using vegetable oil as a cutting lubricant. We found that the vegetable oil studied in this work had good properties in terms of reducing cutting temperatures but was less effective than conventional cutting oil in reducing the surface roughness of the machined part. Tribological tests were carried out to understand the influence of the selected lubricants in reducing friction and wear. After the sliding experiments, which were performed without lubrication in the presence of the same lubricants that were used in the machining tests and in the presence of distilled water, we concluded that vegetable oil has satisfactory lubricating properties that are similar to those of the conventional cutting fluid, indicating a potential for consideration as an effective alternative to the conventional cutting fluid, with economic, environmental, and health advantages.Financial support was provided by Portugal’s national funding FCT/MCTES (PIDDAC) to Centro de Investigação de Montanha (CIMO) (UIDB/00690/2020 and UIDP/00690/2020) and SusTEC (LA/P/0007/2020). Additionally, this work was partially supported by Portuguese FCT, under the reference projects UIDB/04077/2020, UIDB/00532/2020 and UIDB/04436/2020. This research was also partially funded by EXPL2021CIMO_01. Inês Afonso acknowledges the financial support of CIMO through EXPL2021CIMO_01

Analysis of a vegetable oil performance in a milling process by MQL lubrication

In this work, we carried out a comparison between the dry machining of an aluminum block with conventional cutting oil and a block with vegetable oil. The two oils had different flow rates. Using the Taguchi method, it was possible to determine the matrices for optimizing the best parameters for each group of tests. Then, we studied the utility of using vegetable oil as a cutting lubricant. We found that the vegetable oil studied in this work had good properties in terms of reducing cutting temperatures but was less effective than conventional cutting oil in reducing the surface roughness of the machined part. Tribological tests were carried out to understand the influence of the selected lubricants in reducing friction and wear. After the sliding experiments, which were performed without lubrication in the presence of the same lubricants that were used in the machining tests and in the presence of distilled water, we concluded that vegetable oil has satisfactory lubricating properties that are similar to those of the conventional cutting fluid, indicating a potential for consideration as an effective alternative to the conventional cutting fluid, with economic, environmental, and health advantages.Financial support was provided by Portugal’s national funding FCT/MCTES (PIDDAC) to Centro de Investigação de Montanha (CIMO) (UIDB/00690/2020 and UIDP/00690/2020) and SusTEC (LA/P/0007/2020). Additionally, this work was partially supported by Portuguese FCT, under the reference projects UIDB/04077/2020, UIDB/00532/2020 and UIDB/04436/2020. This research was also partially funded by EXPL2021CIMO_01. Inês Afonso acknowledges the financial support of CIMO through EXPL2021CIMO_01.info:eu-repo/semantics/publishedVersio

NOVO MARCO LEGAL À GERAÇÃO DISTRIBUÍDA DE ENERGIA ELÉTRICA NO BRASIL: UMA ABORDAGEM DAS PRINCIPAIS MUDANÇAS / New legal framework for the distributed generation of electric energy in Brazil: an approach to the main changes

This article presents the main changes in the distributed generation of electricity in Brazil, contemplating the approval of the new legal framework (Law 14.300/2022) for the area, which took its effect in January 2022.To answer the research in the field of energy generation, the research relies on bibliographic, documentary, exploratory-descriptive and qualitative studies. It starts from the understanding of the new legislation, which in a way provides greater legal certainty to the area, and ensures economic sustainability to the different agents of the Brazilian electricity sector. This way, despite the change in legislation being quite recent, with ongoing developments, and having impacts to be even better measured, it was concluded that the new legal framework balances the incentive for distributed generation and gives technical-economic feasibility in the medium and long term over Brazilian electric system, especially to electric energy distributors.El presente estudio presenta los principales cambios en la generación distribuida de energía eléctrica en Brasil, contemplando la aprobación del nuevo marco legal (Ley 14.300/2022) para el sector, que entró en vigor en enero de 2022. Para dar respuestas a la investigación acerca de generación de energía, esta pesquisa cuenta con estudios bibliográficos documentales y descriptivos, siendo en su mayoría un estudio exploratorio y cualitativo.  El punto inicial es la comprensión de la nueva legislación, que de alguna manera otorga mayor seguridad jurídica al sector y asegura sostenibilidad económica a los diferentes agentes del sector eléctrico brasileño. Así, a pesar de que el cambio de legislación es bastante reciente, con desarrollos continuos, con impactos aún mejor medidos, se concluyó que el nuevo marco legal equilibra el incentivo a la generación distribuida y la viabilidad técnico-económica en el mediano y largo plazo al sistema eléctrico brasileño, de manera especial a las distribuidoras de energía eléctrica.O presente artigo apresenta as principais mudanças na geração distribuída de energia elétrica no Brasil, contemplando a aprovação do novo marco legal (Lei 14.300/2022) do setor, que entrou em vigor em janeiro de 2022. Para responder à investigação no campo da geração de energia, a pesquisa conta com estudos bibliográficos, documentais, exploratório-descritiva e de natureza qualitativa. Parte-se da compreensão da nova legislação, que de certa forma apresenta maior segurança jurídica ao setor, assegura sustentabilidade econômica aos diferentes agentes do setor elétrico brasileiro. Desta forma, apesar de a mudança na legislação ser bastante recente, com desdobramentos em curso, com impactos a serem ainda melhor mensurados, concluiu-se que o novo marco legal equilibra o incentivo à geração distribuída e viabilidade técnico-econômica no médio e longo prazo ao sistema elétrico brasileiro, em especial às distribuidoras de energia elétrica. Abstract: This article presents the main changes in the distributed generation of electricity in Brazil, contemplating the approval of the new legal framework (Law 14.300/2022) for the area, which took its effect in January 2022.To answer the research in the field of energy generation, the research relies on bibliographic, documentary, exploratory-descriptive and qualitative studies. It starts from the understanding of the new legislation, which in a way provides greater legal certainty to the area, and ensures economic sustainability to the different agents of the Brazilian electricity sector. This way, despite the change in legislation being quite recent, with ongoing developments, and having impacts to be even better measured, it was concluded that the new legal framework balances the incentive for distributed generation and gives technical-economic feasibility in the medium and long term over Brazilian electric system, especially to electric energy distributors

NOVO MARCO LEGAL À GERAÇÃO DISTRIBUÍDA DE ENERGIA ELÉTRICA NO BRASIL: UMA ABORDAGEM DAS PRINCIPAIS MUDANÇAS / New legal framework for the distributed generation of electric energy in Brazil: an approach to the main changes

O presente artigo apresenta as principais mudanças na geração distribuída de energia elétrica no Brasil, contemplando a aprovação do novo marco legal (Lei 14.300/2022) do setor, que entrou em vigor em janeiro de 2022. Para responder à investigação no campo da geração de energia, a pesquisa conta com estudos bibliográficos, documentais, exploratório-descritiva e de natureza qualitativa. Parte-se da compreensão da nova legislação, que de certa forma apresenta maior segurança jurídica ao setor, assegura sustentabilidade econômica aos diferentes agentes do setor elétrico brasileiro. Desta forma, apesar de a mudança na legislação ser bastante recente, com desdobramentos em curso, com impactos a serem ainda melhor mensurados, concluiu-se que o novo marco legal equilibra o incentivo à geração distribuída e viabilidade técnico-econômica no médio e longo prazo ao sistema elétrico brasileiro, em especial às distribuidoras de energia elétrica. Abstract: This article presents the main changes in the distributed generation of electricity in Brazil, contemplating the approval of the new legal framework (Law 14.300/2022) for the area, which took its effect in January 2022.To answer the research in the field of energy generation, the research relies on bibliographic, documentary, exploratory-descriptive and qualitative studies. It starts from the understanding of the new legislation, which in a way provides greater legal certainty to the area, and ensures economic sustainability to the different agents of the Brazilian electricity sector. This way, despite the change in legislation being quite recent, with ongoing developments, and having impacts to be even better measured, it was concluded that the new legal framework balances the incentive for distributed generation and gives technical-economic feasibility in the medium and long term over Brazilian electric system, especially to electric energy distributors

Induced pluripotent stem cell line (INSAi002-A) from a Fabry Disease patient hemizygote for the rare p.W287X mutation

Fabry Disease (FD) is a multisystemic X-linked disorder that belongs to the group of lysosomal storage disorders (LSDs). Causal mutations on alpha-galactosidase A (α-Gal A) commonly lead to abnormal protein and consequently to FD. Since it is an X-linked disease, males are primarily affected. This work describes the generation of induced Pluripotent Stem Cells (iPSCs) from skin fibroblasts from a FD patient, using non-integrative episomal vectors. Differentiation of iPSCs can be applied to generate a variety of cell types with high degree of genetic complexity that would otherwise be difficult to obtain.FCT: PTDC/BIM-MEC/4762/2014 (PI-O.A.)/ UIDB/00211/2020/ PTDC/BIM-MEC/4762/2014.info:eu-repo/semantics/publishedVersio

Emergencia das zonas do perfil conceitual de calor em uma sequência didática

A noção de perfil conceitual foi proposta por Mortimer (1996), para abarcar a ideia de um conceito único apresentar vários modos de falar e de pensar, que representam pensamentos filosóficos e ontológicos distintos, usados em contextos diferentes. Neste trabalho procuramos analisar o discursode estudantes, em busca da emergência das zonas do perfil de calor (Amaral e Mortimer, 2001), bem como traços de tomada de consciência, utilizando uma sequência didática. Após a análise dos dados, registrados em vídeo e transcritos, percebemos uma construção de conhecimento relativo ao conceito de calor no discurso e, mesmo apresentando algumas irregularidades, percebemos que noções científicas foram construídas e confrontadas com as demais visões. Além disso, percebemos indícios de uma possível tomada de consciência da multiplicidade das zonas por parte dos estudantes