ATAXIA DE FRIEDREICH: RELATO DE UM CASO COM MANIFESTAÇÃO TARDIA

A Ataxia de Friedreich uma doena neurodegenerativa progressiva, de herana autossmica recessiva, que foi descrita pela primeira vez por Nicholaus Friedreich, em 1863. A mutao responsvel por essa doena se encontra no cromossomo nove, onde ocorre uma expanso de trinucleotdios GAA. O gene afetado tem a funo de codificar a protena mitocondrial frataxina, que est envolvida no metabolismo do ferro. O principal sintoma a ataxia (coordenao prejudicada), que, no incio, mais evidente nos membros inferiores e, posteriormente, atinge os membros superiores. Este estudo teve por objetivo relatar um caso de Ataxia de Friedreich, uma doena gentica rara de carter degenerativo que, ao contrrio do prognstico esperado, se manifestou mais tardiamente no indivduo afetado. Este trabalho tambm descreve a evoluo clnica, enfocando os sintomas e deficincias que o paciente apresentou antes e aps o diagnstico, bem como, discute as bases moleculares que podem ter contribudo para a manifestao tardia da doena, alm dos tratamentos emergentes

Determinação de Ácidos Graxos Livres em Óleos Vegetais por FT-NIR e Calibração Multivariada

Métodos instrumentais têm sido reportados como alternativas aos métodos convencionais comumente aplicados em análise químicas, dentre os quais a espectrometria no infravermelho próximo com transformada de Fourier (FT-NIR) tem superado a técnica convencional fornecendo procedimentos analíticos rápidos, diretos, precisos e não-invasivos. Este trabalho tem como objetivo a aplicação da espectroscopia FT-NIR acoplada à calibração multivariada na determinação do percentual de ácidos graxos livres (AGL) em óleos vegetais. O método analítico desenvolvido utilizou como matriz de calibração misturas de óleo de soja e ácido oléico em proporções de 0,3 a 38,0% (em massa) de ácido oléico. O método de mínimos quadrados parciais (PLS) com espectro derivado (1ª derivada) foi utilizado com sete fatores primários, fornecendo valores de erro médio quadrático de 0,2841 e 0,4613 para as curvas de calibração e predição respectivamente. Dentre as principais aplicações da metodologia desenvolvida está a avaliação do teor de AGL em óleos vegetais com potencialidade na produção de biodiesel

AVALIAÇÃO HIGIÊNICO-SANITÁRIA DO LEITE PRODUZIDO EM UMUARAMA (PARANÁ)

Este trabalho foi desenvolvido com o objetivo de avaliar a qualidade higiênico-sanitária do leite cru produzido em Umuarama (Paraná), mediante quantificação de Pseudomonas spp., de coliformes totais e termotolerantes em amostras de leite cru provenientes da Associação de Produtores. Propriedades produtoras de leite da região também foram avaliadas quanto aos procedimentos higiênicos adotados em visitas "in loco", mediante aplicação de questionário tipo "check-list". Para verificar a influência do armazenamento sob refrigeração inadequada na concentração de Pseudomonas spp. no leite cru realizou-se a enumeração desses micro-organismos logo após a ordenha (0h) e depois de 48 horas de armazenamento a 7°C. Observou-se diferença significativa ao nível de 5% nas concentrações de Pseudomonas spp. entre os tempos avaliados, comprovando que a refrigeração inadequada e prolongada contribui para o aumento da concentração desses micro-organismos. Verificaram-se contagens elevadas de coliformes totais e coliformes termotolerantes, indicando inadequação higiênico-sanitária das amostras de leite avaliadas. Também foram verificadas várias inadequações nas propriedades quando da aplicação do "check-list", indicando deficiência da higienização na obtenção do leite. Tal resultado reforça a importância de se investir em programas de qualidade, daquela conscientização e treinamento para os produtores da região

The inhibitory action of purple tea on in vivo starch digestion compared to other Camellia sinensis teas

In order to contribute to improve knowledge about the actions of Camellia sinensis extracts on starch digestion, several varieties were compared. The latter were green, oolong, white, black, and purple teas. The results are hoped to contribute to our understanding of the mode of action and potency of the various tea preparations as possible adjuvants in the control of post-prandial glycemia. The extracts were prepared in way similar to their form of consumption. All extracts decreased starch digestion, but the purple tea extract was the strongest inhibitor, their inhibitory tendency started at the dose of 50 mg/kg and was already maximal with 250 mg/kg. Maltose tolerance was not significantly affected by the extracts. Glucose tolerance was not affected by purple tea, but black tea clearly diminished it; green tea presented the same tendency. Purple tea was also the strongest inhibitor of pancreatic α-amylase, followed by black tea. The green tea, oolong tea, and white tea extracts tended to stimulate the pancreatic α-amylase at low concentrations, a phenomenon that could be counterbalancing its inhibitory effect on starch digestion. Based on chemical analyses and molecular docking simulations it was concluded that for both purple and black tea extracts the most abundant active component, epigallocatechin gallate, seems also to be the main responsible for the inhibition of the pancreatic α-amylase and starch digestion. In the case of purple tea, the inhibitory activity is likely to be complemented by its content in deoxyhexosidehexoside- containing polyphenolics, especially the kaempferol and myricetin derivatives. Polysaccharides are also contributing to some extent. Cyanidins, the compounds giving to purple tea its characteristic color, seem not to be the main responsible for its effects on starch digestion. It can be concluded that in terms of postprandial anti-hyperglycemic action purple tea presents the best perspectives among all the tea varieties tested in the present study.This work was financially supported by grants from the Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq-304090/ 2016-6), Coordenação do Aperfeiçoamento de Pessoal do Ensino Superior (CAPES) and Fundação Araucária (53/2019). The authors are grateful to the Foundation for Science and Technology (FCT, Portugal) for financial support through national funds FCT/MCTES to CIMO (UIDB/00690/2020) and through the institutional scientific employment program-contract for L. Barros and M.I. Dias contracts. This work was also funded by the European Regional Development Fund (ERDF) through the Regional Operational Program North 2020, within the scope of Project Mobilizador ValorNatural®: Norte-01-0247-FEDER-024479 and GreenHealth: Norte-01-0145-FEDER-000042.info:eu-repo/semantics/publishedVersio

Light conditions affect the growth, chemical composition, antioxidant and antimicrobial activities of the white-rot fungus Lentinus crinitus mycelial biomass

The mycelial biomass of basidiomycetes is a promising source of compounds and represents an alternative for industrial and biotechnological applications. Fungi use light as information and hold photoresponse mechanisms, in which sensors respond to light wavelengths and regulate various biological processes. Therefore, this study aimed to investigate the effects of blue, green, and red lights on the growth, chemical composition, and antioxidant and antimicrobial activity of Lentinus crinitus mycelial biomass. The chemical composition of the mycelial biomass was determined by chromatographic methods, antioxidant activity was analyzed by in vitro assays, and antimicrobial activity was investigated by the microdilution assay. The highest mycelial biomass yield was observed under blue-light cultivation. Many primordia arose under blue or green light, whereas the stroma was formed under red light. The presence of light altered the primary fungal metabolism, increasing the carbohydrate, tocopherol, fatty acid, and soluble sugar contents, mostly mannitol, and reducing the protein and organic acid concentrations. Cultivation under red light increased the phenol concentration. In contrast, cultivation under blue and green lights decreased phenol concentration. Benzoic and gallic acids were the main phenolic acids in the hydroalcoholic extracts, and the latter acids increased in all cultures under light, especially red light. Mycelial biomass cultivated under red light showed the highest antioxidant activity in the 2,2-diphenyl-1-picrylhydrazyl (DPPH) assay. The ferric reducing antioxidant power (FRAP) method showed that all light wavelengths increased the antioxidant activity of mycelial biomass, with the highest value under red light. Moreover, the β-carotene/linoleic acid co-oxidation (BCLA) assay demonstrated that the antioxidant activity was affected by light cultivation. Mycelial biomass grown under all conditions exhibited antibacterial and antifungal activities. Thus, mycelial biomass cultivation of L. crinitus under light conditions may be a promising strategy for controlling the mycelial chemical composition and biomass yield.info:eu-repo/semantics/publishedVersio

Characterisation of microbial attack on archaeological bone

As part of an EU funded project to investigate the factors influencing bone preservation in the archaeological record, more than 250 bones from 41 archaeological sites in five countries spanning four climatic regions were studied for diagenetic alteration. Sites were selected to cover a range of environmental conditions and archaeological contexts. Microscopic and physical (mercury intrusion porosimetry) analyses of these bones revealed that the majority (68%) had suffered microbial attack. Furthermore, significant differences were found between animal and human bone in both the state of preservation and the type of microbial attack present. These differences in preservation might result from differences in early taphonomy of the bones. © 2003 Elsevier Science Ltd. All rights reserved