O atendimento educacional aos alunos com altas habilidades/superdotação na Rede Pública Estadual do Paraná: uma história recente

Metadados do Trabalho de Conclusão de Curso: O atendimento educacional aos alunos com altas habilidades/superdotação na Rede Pública Estadual do Paraná: uma história recente, pela discente: Ana Paula Leandro Gonçalves, sob Orientação de Catarina Costa Fernandes do curso de Especialização em Atendimento Educacional Especializado na perspectiva da Educação Inclusiva (2015-2016) da Universidade Federal da Integração Latino-Americana (UNILA), no Repositório Institucional da UNILA (RI-UNILA).O atendimento educacional aos alunos com altas habilidades/superdotação na Rede Pública Estadual do Paraná: uma história recent

PKU mutation p.G46S prevents the stereospecific binding of l-phenylalanine to the dimer of human phenylalanine hydroxylase regulatory domain

Mammalian phenylalanine hydroxylase (PAH) has a potential allosteric regulatory binding site for l‐phenylalanine (l‐Phe), in addition to its catalytic site. This arrangement is supported by a crystal structure of a homodimeric truncated form of the regulatory domain of human PAH (hPAH‐RD1–118/19–118) [Patel D et al. (2016) Sci Rep doi: 10.1038/srep23748]. In this study, a fusion protein of the domain (MBP‐(pepXa)‐hPAH‐RD1–120) was overexpressed and recovered in a metastable and soluble state, which allowed the isolation of a dimeric and a monomeric fusion protein. When cleaved from MBP, hPAH‐RD forms aggregates which are stereospecifically inhibited by l‐Phe (> 95%) at low physiological concentrations. Aggregation of the cleaved dimer of the mutant form hPAH‐G46S‐RD was not inhibited by l‐Phe, which is compatible with structurally/conformationally changed βαββαβ ACT domain folds in the mutant.publishedVersio

Phenylketonuria as a protein misfolding disease: The mutation pG46S in phenylalanine hydroxylase promotes self-association and fibril formation

AbstractThe missense mutation pG46S in the regulatory (R) domain of human phenylalanine hydroxylase (hPAH), associated with a severe form of phenylketonuria, generates a misfolded protein which is rapidly degraded on expression in HEK293 cells. When overexpressed as a MBP-G46S fusion protein, soluble and fully active tetrameric/dimeric forms are assembled and recovered in a metastable conformational state. When MBP is cleaved off, G46S undergoes a conformational change and self-associates with a lag phase and an autocatalytic growth phase (tetramers≫dimers), as determined by light scattering. The self-association is controlled by pH, ionic strength, temperature, protein concentration and the phosphorylation state of Ser16; the net charge of the protein being a main modulator of the process. A superstoichiometric amount of WT dimers revealed a 2-fold enhancement of the rate of G46S dimer self-association. Electron microscopy demonstrates the formation of higher-order oligomers and linear polymers of variable length, partly as a branching network, and partly as individual long and twisted fibrils (diameter ~145–300Å). The heat-shock proteins Hsp70/Hsp40, Hsp90 and a proposed pharmacological PAH chaperone (3-amino-2-benzyl-7-nitro-4-(2-quinolyl)-1,2-dihydroisoquinolin-1-one) partly inhibit the self-association process. Our data indicate that the G46S mutation results in a N-terminal extension of α-helix 1 which perturbs the wild-type α–β sandwich motif in the R-domain and promotes new intermolecular contacts, self-association and non-amyloid fibril formation. The metastable conformational state of G46S as a MBP fusion protein, and its self-association propensity when released from MBP, may represent a model system for the study of other hPAH missense mutations characterized by misfolded proteins

On the enumeration of finite L-algebras

We use Constraint Satisfaction Methods to construct and enumerate finite L-algebras up to isomorphism. These objects were recently introduced by Rump and appear in Garside theory, algebraic logic, and the study of the combinatorial Yang–Baxter equation. There are 377,322,225 isomorphism classes of L-algebras of size eight. The database constructed suggests the existence of bijections between certain classes of L-algebras and well-known combinatorial objects. We prove that Bell numbers enumerate isomorphism classes of finite linear L-algebras. We also prove that finite regular L-algebras are in bijective correspondence with infinite-dimensional Young diagrams

Portuguese and Brazilian family business: in between urgency and delay perceptions in the succession process

Family business has been the focus of several studies over the last two decades and its relevance has been supported by the interdisciplinary perspectives in the fields of management, entrepreneurship, economics, psychology, and sociology. Despite that, there is still insufficient knowledge about the key role of family influences in the business, namely the intergenerational management succession, its planning and effectiveness. According to a recent research focused on the entrepreneurial succession in Portugal (AEP, 2011), 50% of family businesses are not passed on to the second generation and only 20% reach the third generation. In fact, business succession planning has been identified as one of the most challenging steps in the life of the family firm, both in maintaining the competiveness of the business, and in overcoming intra/ inter family conflicts. Nonetheless, resistance to succession, relationship founder/ successor, planning of succession, and type of organisational culture, among others, explain how executive succession is one of the most important and hardest tasks in organisational life (Zahra, 2005). This paper will be supported mainly by qualitative data, taking into account the main results from the project “Roadmap for Portuguese Family Businesses” (NORTE2020/FEDER) developed in Portugal (Marques, 2018) and in Brazil (Silva, 2018), which analyses in-depth interviews conducted to Portuguese (N 23) and Brazilian (N 11) founders/managers/owners. In the present article we wish to discuss the main management challenges of a family business, particularly the importance of succession preparation and the role of the family in the socialisation of the second (third or subsequent) generation.NORTE-02-0853-FEDER-00001

Access to information, and concerns, myths and truths about food safety during the COVID-19 pandemic: an overview of the Portuguese population

The COVID-19 pandemic raised questions and concerns about the possibility of the virus being transmitted through food, as the virus was found in sewage, shrimps and packages of frozen food. During the first wave of COVID-19, concerns about the transmission of SARS-CoV-2 through food arose. As the number of cases began to increase rapidly, so did the availability of information regarding the virus and ways to prevent infection. A significant portion of this information was disseminated by the media and the general public. Identifying and understanding the main doubts and concerns about food hygiene and safety raised by the Portuguese population during the first wave of COVID-19 is important in order to understand whether these issues have influenced their practices and what lessons can be learnt for food safety and hygiene education. The aims of this work were (1) to understand the doubts and concerns of the Portuguese population regarding food safety and hygiene during the first wave of COVID-19, and how these issues were clarified, (2) to analyze the population’s opinion on food/hygiene myths and truths related to the transmission and prevention of the infection, and (3) to understand how the first wave of COVID-19 may have influenced the population’s practices linked to food handling and consumption. The main doubts of the respondents were related to food handling (41.6%) and the possibility of transmission of COVID-19 through food (17%). Television was the main source of information used to clarify these doubts (32.9%), followed by a guideline issued by the Directorate-General of Health (30.7%). However, most respondents (50.9%) said that they had only found answers to some of their questions. Most respondents reported washing and disinfecting hands before (85% and 63.4%, respectively) and after (73.8% and 57.3%, respectively) the handling and organization of food purchases. Most respondents did not believe the myths about COVID-19 and food safety, but this depended on their level of education. Some practices may have changed as a result of the pandemic, particularly with regard to washing and disinfecting hands and food, as well as kitchen hygiene.info:eu-repo/semantics/publishedVersio