Molecular basis of human mitochondrial very-long-chain acyl-CoA dehydrogenase deficiency causing cardiomyopathy and sudden death in childhood.

beta-Oxidation of long-chain fatty acids provides the major source of energy in the heart. Defects in enzymes of the beta-oxidation pathway cause sudden, unexplained death in childhood, acute hepatic encephalopathy or liver failure, skeletal myopathy, and cardiomyopathy. Very-long-chain acyl-CoA dehydrogenase [VLCAD; very-long-chain-acyl-CoA:(acceptor) 2,3-oxidoreductase, EC 1.3.99.13] catalyzes the first step in beta-oxidation. We have isolated the human VLCAD cDNA and gene and determined the complete nucleotide sequences. Polymerase chain reaction amplification of VLCAD mRNA and genomic exons defined the molecular defects in two patients with VLCAD deficiency who presented with unexplained cardiac arrest and cardiomyopathy. In one, a homozygous mutation in the consensus dinucleotide of the donor splice site (g+1-->a) was associated with universal skipping of the prior exon (exon 11). The second patient was a compound heterozygote, with a missense mutation, C1837-->T, changing the arginine at residue 613 to tryptophan on one allele and a single base deletion at the intron-exon 6 boundary as the second mutation. This initial delineation of human mutations in VLCAD suggests that VLCAD deficiency reduces myocardial fatty acid beta-oxidation and energy production and is associated with cardiomyopathy and sudden death in childhood.

Produção de energia por mini e micro hidrelétricas na rede de distribuição de água.

A geração de energia hidrelétrica enfrenta uma crescente restrição a sua expansão, diretamente relacionada a fatores ambientais e da limitação de terrenos com potencial economicamente aproveitável. A partir deste fato, é relacionada uma possível fonte de energia hidrelétrica, resultante do aproveitamento dos potenciais presentes na rede de distribuição de água das cidades, ainda sem nenhum aproveitamento. O desenvolvimento desta fonte de energia se dá com a instalação de mini e micro centrais hidrelétricas nos condutos da rede distribuidora de água. Este trabalho tem por objetivo avaliar o potencial de aproveitamento hidrelétrico por mini e micro hidrelétricas por meio de técnicas de modelagem e de otimização, para agilizar e facilitar o procedimento de identificação dos potenciais e a instalação na rede de abastecimento. O trabalho leva em conta as diversas peculiaridades das redes de distribuição de água e dos equipamentos eletro-hidráulicos, discorrendo sobre a possível complementariedade da geração de energia durante os picos de consumo. Discorre também sobre a contribuição para a rede de distribuição elétrica, na logística e nos custos de implantação além de discutir a tipologia das turbinas capazes de aproveitar o potencial energético. É avaliado, com o auxilio de modelos hidráulicos e de otimização, o posicionamento das centrais geradoras na rede e os possíveis benefícios, restrições e impedimentos ao seu uso, desenvolvendo uma metodologia para facilitar a tomada de decisão quanto ao aproveitamento para geração, ou não, em redes diversas. A construção deste procedimento e ferramenta são desenvolvidos a partir do estudo de caso do sistema distribuidor de água do Município de Piquete no estado de São Paulo, Brasil.

Estudo de catalisadores de Ni/ZnO promovidos com CeO2 aplicados em reações de reforma a vapor do etanol e acetona

A produção de energia é um dos grandes desafios deste século, principalmente com a necessidade no desenvolvimento de processos que utilizem preferencialmente fontes renováveis. Nesse contexto, é claro o interesse por pesquisas baseada no hidrogênio. Porém, os ganhos ambientais efetivos estão associados também à matéria-prima usada no processo de geração do hidrogênio, senso assim mais significativo quando do uso de fontes renováveis. No presente trabalho foi estudado o efeito da adição de diferentes teores de CeO2 em catalisadores de NiZnO, preparados pelo método da co-precipitação. Os catalisadores foram estudados frente à reação de reforma a vapor do etanol (RVE) para produção de H2, e no decorrer do trabalho foi pertinente incluir o estudo da reforma a vapor da acetona (RVA), como complemento dos estudos da RVE. Além disso, esta é uma molécula modelo para reforma a vapor do bio-óleo. Nos sistemas catalíticos sintetizados houve a formação da liga NiZn3, o que aparenta proporcionar um efeito sinérgico entre esses elementos. Observou-se um efeito altamente promotor do CeO2 com relação a diminuição do coque formado, devido a uma menor da formação da acetona, durante as etapas de reforma do etanol. O que consequentemente interferiu na produção de H2. O catalisador contendo 20% de CeO2 denominado NiZn20Ce apresentou um desempenho altamente promissor, pois de acordo com as análises de DRIFT, a presença do CeO2 levou a formação de espécies de formiato, o que consequentemente interferiu em uma menor formação de coque e maior produção de H2. Foi comprovado também que a natureza dos depósitos carbonáceos depende tanto do substrato utilizado quanto das etapas precursoras inicias que levarão a formação desse coque, sendo o ponto chave para um melhor desempenho do catalisador.

Risco de mercado na comercialização de energia elétrica: uma análise estruturada com foco no ambiente de contratação livre - ACL.

A motivação para o desenvolvimento desse trabalho surge em um momento em que se verifica uma participação cada vez mais significativa das fontes energéticas renováveis não convencionais no País. Não obstante, o cenário de evolução evidencia que o arcabouço regulatório e as regras de mercado não acompanharam as especificidades inerentes à exploração dessas fontes. Assim, para que se mantenha adequado ritmo de inserção na matriz energética, devem ser buscadas opções para que fontes alternativas sejam cada vez mais competitivas na atual configuração do mercado energético. A contribuição dessa pesquisa, portanto, centra-se na análise dos riscos de mercado incorridos por esses geradores de fontes intermitentes de energia ao comercializarem energia no ambiente de contratação livre. Nessa perspectiva, a Dissertação foi desenvolvida abordando tipos de geração de energia e suas características técnicas e econômicas, legislação do setor elétrico, regras de comercialização, balanço energético do sistema, formação de preços no mercado de curto prazo e precificação de contratos no ACL, diferença de preços entre submercados, requisitos de flexibilidade e sazonalidade nos contratos de venda a consumidores livres e seu impacto na precificação de contratos, identificação de comportamento energético complementar para mitigação de riscos de mercado entre fontes renováveis e rebatimento na formulação de mecanismo de hedge, análise de portfólio de contratos e estratégia ótima de contratação de energia para agentes geradores atuando no ACL. Como resposta ao desafio de equacionar o impasse surgido na comercialização de fontes de produção sazonal, propõe-se um modelo para definir estratégias de contratação para agentes geradores e comercializadores a partir da complementação energética entre diferentes tipos de fontes, de forma a maximizar os ganhos de comercialização para um risco estabelecido. Busca-se a composição ideal dessas fontes na carteira de um comercializador para minimizar o risco de exposição à volatilidade dos preços do mercado de curto prazo. Isso é possível em virtude das compensações energéticas feitas entre as diferentes fontes em um portfólio combinado, mitigando a receita em risco decorrente das variações que existem nos preços de curto prazo e na produção energética. De forma complementar, estruturou-se um modelo de negócio no qual uma empresa detentora de ativos de geração hidrelétrica compra os direitos de produção de uma eólica e/ou biomassa para incorporar ao seu portfólio e vender como contrato por quantidade. Determinou-se o volume de energia a ser comprado de cada fonte, o preço, a estratégia mais indicada de contratação e a mitigação de fatores de risco contemplados nos contratos de venda, buscando maximizar os ganhos de comercialização condicionada a critérios de risco pré-fixados.

The Effects of Oil and Gas Exploration on Greater Sage Grouse Populations

Energy production throughout the greater Rocky Mountain Region has increased dramatically in the past decade. Pristine habitat areas historically home to the greater sage grouse are being impaired by these energy development actions. Ongoing studies by state and federal biologists have shown a decrease in overall reproductive activity and a decrease in population on historical mating grounds. This project identifies actions to conserve and restore the habitat of sage grouse populations and reverse the decline of the species. Recommended measures presented in this project include using directional drilling of multiple wells from one single drill pad, creating a GIS mapping data base, funding additional scientific studies regarding impacts from energy development and improved cooperation between state, federal and private agencies.

Crystallographic orientation and electrode nature are key factors for electric current generation by Geobacter sulfurreducens

We have investigated the influence of electrode material and crystallographic structure on electron transfer and biofilm formation of Geobacter sulfurreducens. Single-crystal gold - Au(110), Au(111), Au(210) - and platinum - Pt(100), Pt(110), Pt(111), Pt(210) - electrodes were tested and compared to graphite rods. G. sulfurreducens electrochemically interacts with all these materials with different attachment kinetics and final current production, although redox species involved in the electron transfer to the anode are virtually the same in all cases. Initial bacterial colonization was fastest on graphite up to the monolayer level, whereas gold electrodes led to higher final current densities. Crystal geometry showed to have an important influence, with Au(210) sustaining a current density of up to 1442 (± 101) μA cm- 2 at the steady state, over Au(111) with 961 (± 94) μA cm- 2 and Au(110) with 944 (± 89) μA cm- 2. On the other hand, the platinum electrodes displayed the lowest performances, including Pt(210). Our results indicate that both crystal geometry and electrode material are key parameters for the efficient interaction of bacteria with the substrate and should be considered for the design of novel materials and microbial devices to optimize energy production.

Sub-Cellular Localization and Complex Formation by Aminoacyl-tRNA Synthetases in Cyanobacteria: Evidence for Interaction of Membrane-Anchored ValRS with ATP Synthase

tRNAs are charged with cognate amino acids by aminoacyl-tRNA synthetases (aaRSs) and subsequently delivered to the ribosome to be used as substrates for gene translation. Whether aminoacyl-tRNAs are channeled to the ribosome by transit within translational complexes that avoid their diffusion in the cytoplasm is a matter of intense investigation in organisms of the three domains of life. In the cyanobacterium Anabaena sp. PCC 7120, the valyl-tRNA synthetase (ValRS) is anchored to thylakoid membranes by means of the CAAD domain. We have investigated whether in this organism ValRS could act as a hub for the nucleation of a translational complex by attracting other aaRSs to the membranes. Out of the 20 aaRSs, only ValRS was found to localize in thylakoid membranes whereas the other enzymes occupied the soluble portion of the cytoplasm. To investigate the basis for this asymmetric distribution of aaRSs, a global search for proteins interacting with the 20 aaRSs was conducted. The interaction between ValRS and the FoF1 ATP synthase complex here reported is of utmost interest and suggests a functional link between elements of the gene translation and energy production machineries.

Thermal model for charring rate calculation in wooden cellular slabs under fire

Wood is a natural and traditional building material, as popular today as ever, and presents advantages. Physically, wood is strong and stiff, but compared with other materials like steel is light and flexible. Wood material can absorb sound very effectively and it is a relatively good heat insulator. But dry wood burns quite easily and produces a great deal of heat energy. The main disadvantage is the high level of combustion when exposed to fire, where the point at which it catches fire is from 200–400°C. After fire exposure, is need to determine if the charred wooden structures are safe for future use. Design methods require the use of computer modelling to predict the fire exposure and the capacity of structures to resist those action. Also, large or small scale experimental tests are necessary to calibrate and verify the numerical models. The thermal model is essential for wood structures exposed to fire, because predicts the charring rate as a function of fire exposure. The charring rate calculation of most structural wood elements allows simple calculations, but is more complicated for situations where the fire exposure is non-standard and in wood elements protected with other materials. In this work, the authors present different case studies using numerical models, that will help professionals analysing woods elements and the type of information needed to decide whether the charred structures are adequate or not to use. Different thermal models representing wooden cellular slabs, used in building construction for ceiling or flooring compartments, will be analysed and submitted to different fire scenarios (with the standard fire curve exposure). The same numerical models, considering insulation material inside the wooden cellular slabs, will be tested to compare and determine the fire time resistance and the charring rate calculation.

Isotopic power and thermionic conversion : a literature search /

Included are 88 references on thermionic conversion of heat energy and the use of radioisotopes as power sources. References on thermoelectric conversion are included if the primary energy source is a radioisotope.

An isotopic analysis of geothermal brine and calcite scaling from the Blue Mountain geothermal field, Winnemucca, Nevada

Understanding, and controlling, the conditions under which calcite precipitates within geothermal energy production systems is a key step in maintaining production efficiency. In this study, I apply methods of bulk and clumped isotope thermometry to an operating geothermal energy facility in northern Nevada to see how those methods can better inform the facility owner, AltaRock Energy, Inc., about the occurrence of calcite scale in their power plant. I have taken water samples from five production wells, the combined generator effluent, shallow cold-water wells, monitoring wells, and surface water. I also collected calcite scale samples from within the production system. Water samples were analyzed for stable oxygen isotope composition (d18O). Calcite samples were analyzed for stable oxygen and carbon (d13C) composition, and clumped isotope composition (D47). With two exceptions, the water compositions are very similar, likely indicating common origin and a well-mixed hydrothermal system. The calcite samples are likewise similar to one another. Apparent temperatures calculated from d18O values of water and calcite are lower than those recorded for the system. Apparent temperatures calculated from D47 are several degrees higher than the recorded well temperatures. The lower temperatures from the bulk isotope data are consistent with temperatures that could be expected during a de-pressurization of the production system, which would cause boiling in the pipes, a reduction in system temperature, and rapid precipitation of calcite scale. However, the high apparent temperature indicated by the D47 data suggests that the calcite is depleted in clumped isotopes given the known temperature of the system, which is inconsistent with this hypothesis. This depletion could instead result from disequilibrium isotopic fractionation during the aforementioned boil events, which would make both the apparent d18O-based and D47-based temperatures unrepresentative of the actual water temperature. This research can help improve our understanding of how isotopic analyses can better inform us about the movement of water through geothermal systems of the past and how it now moves through modern systems. Increased understanding of water movement in these systems could potentially allow for more efficient utilization of geothermal energy as a renewable resource.

Gene expression in profiling in individual cases reveals consistent transcriptional changes in alcoholic human brain

Chronic alcohol exposure induces lasting behavioral changes, tolerance, and dependence. This results, at least partially, from neural adaptations at a cellular level. Previous genome-wide gene expression studies using pooled human brain samples showed that alcohol abuse causes widespread changes in the pattern of gene expression in the frontal and motor cortices of human brain. Because these studies used pooled samples, they could not determine variability between different individuals. In the present study, we profiled gene expression levels of 14 postmortem human brains (seven controls and seven alcoholic cases) using cDNA microarrays (46 448 clones per array). Both frontal cortex and motor cortex brain regions were studied. The list of genes differentially expressed confirms and extends previous studies of alcohol responsive genes. Genes identified as differentially expressed in two brain regions fell generally into similar functional groups, including metabolism, immune response, cell survival, cell communication, signal transduction and energy production. Importantly, hierarchical clustering of differentially expressed genes accurately distinguished between control and alcoholic cases, particularly in the frontal cortex.

Alcohol-responsive genes in the frontal cortex and nucleus accumbens of human alcoholics

The molecular processes underlying alcohol dependence are not fully understood. Many characteristic behaviours result from neuroadaptations in the mesocorticolimbic system. In addition, alcoholism is associated with a distinct neuropathology. To elucidate the molecular basis of these features, we compared the RNA expression profile of the nucleus accumbens and prefrontal cortex of human brain from matched individual alcoholic and control cases using cDNA microarrays. Approximately 6% of genes with a marked alcohol response were common to the two brain regions. Alcohol-responsive genes were grouped into 11 functional categories. Predominant alcohol-responsive genes in the prefrontal cortex were those encoding DNA-binding proteins including transcription factors and repair proteins. There was also a down-regulation of genes encoding mitochondrial proteins, which could result in disrupted mitochondrial function and energy production leading to oxidative stress. Other alcohol-responsive genes in the prefrontal cortex were associated with neuroprotection/apoptosis. In contrast, in the nucleus accumbens, alcohol-responsive genes were associated with vesicle formation and regulation of cell architecture, which suggests a neuroadaptation to chronic alcohol exposure at the level of synaptic structure and function. Our data are in keeping with the previously reported alcoholism-related pathology characteristic of the prefrontal cortex, but suggest a persistent decrease in neurotransmission and changes in plasticity in the nucleus accumbens of the alcoholic.

Enzyme Electrochemistry - Biocatalysis on an electrode

Oxidoreductase enzymes catalyze single- or multi-electron reduction/oxidation reactions of small molecule inorganic or organic substrates, and they are integral to a wide variety of biological processes including respiration, energy production, biosynthesis, metabolism, and detoxification. All redox enzymes require a natural redox partner such as an electron-transfer protein ( e. g. cytochrome, ferredoxin, flavoprotein) or a small molecule cosubstrate ( e. g. NAD(P)H, dioxygen) to sustain catalysis, in effect to balance the substrate/product redox half-reaction. In principle, the natural electron-transfer partner may be replaced by an electrochemical working electrode. One of the great strengths of this approach is that the rate of catalysis ( equivalent to the observed electrochemical current) may be probed as a function of applied potential through linear sweep and cyclic voltammetry, and insight to the overall catalytic mechanism may be gained by a systematic electrochemical study coupled with theoretical analysis. In this review, the various approaches to enzyme electrochemistry will be discussed, including direct and indirect ( mediated) experiments, and a brief coverage of the theory relevant to these techniques will be presented. The importance of immobilizing enzymes on the electrode surface will be presented and the variety of ways that this may be done will be reviewed. The importance of chemical modification of the electrode surface in ensuring an environment conducive to a stable and active enzyme capable of functioning natively will be illustrated. Fundamental research into electrochemically driven enzyme catalysis has led to some remarkable practical applications. The glucose oxidase enzyme electrode is a spectacularly successful application of enzyme electrochemistry. Biosensors based on this technology are used worldwide by sufferers of diabetes to provide rapid and accurate analysis of blood glucose concentrations. Other applications of enzyme electrochemistry are in the sensing of macromolecular complexation events such as antigen - antibody binding and DNA hybridization. The review will include a selection of enzymes that have been successfully investigated by electrochemistry and, where appropriate, discuss their development towards practical biotechnological applications.

Comparative gene expression in brain regions of human alcoholics

The mesocorticolimbic system is the reward centre of the brain and the major target for drugs of abuse including alcohol. Neuroadaptive changes in this region are thought to underlie the process of tolerance and dependence. Recently, several research groups have searched for alcohol-responsive genes using high-throughput microarrays and well-characterized human post-mortem material. Comparison of data from these studies of cortical regions highlights the differences in experimental approach and selection of cases. However, alcohol-responsive gene sets associated with transcription, oxidative stress and energy production were common to these studies. In marked contrast, alcohol-responsive genes in the nucleus accumbens and the ventral tegmental area are primarily associated with changes in neurotransmission and signal transduction. These data support the concept that, within cortical regions, changes in gene expression are associated with alcoholism-related pathology. In the dopaminergic tract of the mesocorticolimbic system, alcohol-responsive gene sets suggest long-term neuroplastic changes in synaptic transmission.

Effect of free ammonia and free nitrous acid concentration on the anabolic and catabolic processes of an enriched Nitrosomonas culture

The effects of free ammonia (FA; NH3) and free nitrous acid (FNA; HNO2) concentrations on the metabolisms of an enriched ammonia oxidizing bacteria (AOB) culture were investigated using a method allowing the decoupling of growth and energy generation processes. A lab-scale sequencing batch reactor (SBR) was operated for the enrichment of an AOB culture. Fluorescent in-situ hybridization (FISH) analysis showed that 82% of the bacterial population in the SBR bound to the NEU probe specifically designed for Nitrosomonas europaea. Batch tests were carried out to measure the oxygen and ammonium consumption rates by the culture at various FA and FNA levels, in the presence or absence of inorganic carbon (CO2, HCO3, and CO32-). It was revealed that FA of up to 16.0 mgNH(3)-N (.) L-1, which was the highest concentration used in this study, did not have any inhibitory effect on either the catabolic or anabolic processes of the Nitrosomonas culture. In contrast, FNA inhibited both the growth and energy production capabilities of the Nitrosomonas culture. The inhibition on growth initiated at approximately 0.10 mgHNO(2)-(NL-1)-L-., and the data suggested that the biosynthesis was completely stopped at an FNA concentration of 0.40 mgHNO(2)-N (.) L-1. The inhibition on energy generation initiated at a slightly lower level but the Nitrosomonas culture was still oxidizing ammonia at half of the maximum rate at an FNA concentration of 0.50-0.63 mgHNO(2)-N (.) L-1. The affinity constant of the Nitrosomonas culture with respect to ammonia was determined to be 0.36 mgNH3-N (.) L-1, independent of the presence or absence of inorganic carbon. (c) 2006 Wiley Periodicals, Inc.