[B_Erandio_Martiartu] Documentación del conjunto formado por la Torre de Martiartu y la ermita de San Antonio (Erandio, Bizkaia)

[ES] El conjunto está formado por la torre de Martiartu (de unas 16 x 16 metros de planta), la pequeña ermita de la San Antonio y el área circundante.

Molecular diagnosis of distal renal tubular acidosis in Tunisian patients: proposed algorithm for Northern Africa populations for the ATP6V1B1, ATP6V0A4 and SCL4A1 genes

Background: Primary distal renal tubular acidosis (dRTA) caused by mutations in the genes that codify for the H+ -ATPase pump subunits is a heterogeneous disease with a poor phenotype-genotype correlation. Up to now, large cohorts of dRTA Tunisian patients have not been analyzed, and molecular defects may differ from those described in other ethnicities. We aim to identify molecular defects present in the ATP6V1B1, ATP6V0A4 and SLC4A1 genes in a Tunisian cohort, according to the following algorithm: first, ATP6V1B1 gene analysis in dRTA patients with sensorineural hearing loss (SNHL) or unknown hearing status. Afterwards, ATP6V0A4 gene study in dRTA patients with normal hearing, and in those without any structural mutation in the ATP6V1B1 gene despite presenting SNHL. Finally, analysis of the SLC4A1 gene in those patients with a negative result for the previous studies. Methods: 25 children (19 boys) with dRTA from 20 families of Tunisian origin were studied. DNAs were extracted by the standard phenol/chloroform method. Molecular analysis was performed by PCR amplification and direct sequencing. Results: In the index cases, ATP6V1B1 gene screening resulted in a mutation detection rate of 81.25%, which increased up to 95% after ATP6V0A4 gene analysis. Three ATP6V1B1 mutations were observed: one frameshift mutation (c.1155dupC; p.Ile386fs), in exon 12; a G to C single nucleotide substitution, on the acceptor splicing site (c.175-1G > C; p.?) in intron 2, and one novel missense mutation (c. 1102G > A; p. Glu368Lys), in exon 11. We also report four mutations in the ATP6V0A4 gene: one single nucleotide deletion in exon 13 (c.1221delG; p. Met408Cysfs* 10); the nonsense c.16C > T; p.Arg6*, in exon 3; and the missense changes c.1739 T > C; p.Met580Thr, in exon 17 and c.2035G > T; p.Asp679Tyr, in exon 19. Conclusion: Molecular diagnosis of ATP6V1B1 and ATP6V0A4 genes was performed in a large Tunisian cohort with dRTA. We identified three different ATP6V1B1 and four different ATP6V0A4 mutations in 25 Tunisian children. One of them, c.1102G > A; p.Glu368Lys in the ATP6V1B1 gene, had not previously been described. Among deaf since childhood patients, 75% had the ATP6V1B1 gene c. 1155dupC mutation in homozygosis. Based on the results, we propose a new diagnostic strategy to facilitate the genetic testing in North Africans with dRTA and SNHL.

Diseño y cálculo de la torre y la cimentación de un aerogenerador

El objeto del estudio consiste en el diseño y el cálculo de la torre y de la cimentación de un aerogenerador. El aerogenerador formará parte de un parque eólico que constará de 9 aerogeneradores ubicado en la zona de pico Bedures y Pico El peñón, en el municipio de Vegadeo, perteneciente a la comunidad autónoma de Asturias.

Análise da resposta dinâmica experimental de uma passarela tubular mista, aço-concreto, submetida ao caminhar humano.

Este trabalho de pesquisa apresenta como objetivo principal o desenvolvimento de investigação experimental dinâmica sobre estrutura real de uma passarela tubular mista aço-concreto. O sistema estrutural objeto deste trabalho corresponde a uma passarela composta por três vãos (32,5m, 17,5m e 20,0m, respectivamente) e dois balanços (7,50m e 5,0m, respectivamente), com comprimento total de 82,5m. A passarela com estrutura contínua de aço com as ligações soldadas se apoia em quatro pórticos também de aço. Estruturalmente está constituída por duas treliças planas que se interligam através de contraventamentos horizontais fixados na corda superior e inferior da treliça e lajes de concreto, formando um sistema misto com interação completa. A estrutura está submetida correntemente à travessia de pedestres e ciclistas. Testes experimentais foram realizados sobre o sistema estrutural e confrontados com resultados numéricos. Para a modelagem numérica do sistema são empregadas técnicas usuais de discretização, via método dos elementos finitos (MEF), por meio do programa ANSYS. Os resultados experimentais são analisados de acordo com a metodologia desenvolvida, sendo realizada análise modal experimental para a determinação das propriedades dinâmicas: freqüências, modos e taxa de amortecimento, enquanto que os resultados da estrutura, em termos de aceleração de pico, são comparados com os valores limites propostos por diversos autores, normas e recomendações de projeto, para uma avaliação do desempenho da estrutura em relação a vibração quando solicitada pelo caminhar dos pedestres no que diz respeito a critério para conforto humano.

Caracterização de juntas soldadas por Arame Tubular com proteção gasosa do aço ferrítico ASTM A335 P91, antes e após situação de duplo reparo.

Pouco se sabe a respeito das consequências da operação de reparo nas propriedades mecânicas e metalúrgicas das juntas do aço ASTM A335 grau P91 soldadas por Arame Tubular. Neste trabalho foi realizado um estudo para analisar e caracterizar juntas de um tubo do aço P91 soldadas pelo processo Arame Tubular com proteção gasosa, antes e após situação de duplo reparo. Foram utilizadas técnicas de microscopia eletrônica de varredura (MEV), análise química semiquantitativa por EDS (Energy Dispersive Spectroscopy), microscopia óptica (MO), além da obtenção de medidas de microdureza Vickers e realização de ensaios de impacto e tração à temperatura ambiente. A macrografia revelou a existência de uma nova zona termicamente afetada formada no centro da junta reparada e a análise micrográfica mostrou que esta zona apresentou uma pequena tendência a formação de grãos menores do que na zona de fusão que sofreu reparo, além disso, a região tinha grãos colunares que não são característicos de uma zona termicamente afetada e quantidade de carbonetos menor comparativamente. Não houve variações significativas nos valores de tração e microdureza. Os ensaios de impacto revelaram baixos valores de energia absorvida na zona de fusão do 2 reparo, no entanto, todas as outras zonas avaliadas das juntas com e sem reparo apresentaram valores dentro das especificações.

Tubular graphite cones with single-crystal nanotips and their antioxygenic properties.

Tubular graphite cones (TGCs) with a single-crystal nanotip have been achieved by means of microwave plasma-assisted chemical vapor deposition using in-situ-evaporated Fe catalysts. The absence of the disorder-induced D band in Raman spectra revealed the single-crystalline feature of the nanotip. TGCs were found to stem from Fe catalytic carbon spherules on the order of 100 mum diameter, whose critical role in promoting both nucleation and plasma annealing in the formation of highly crystalline TGCs is discussed. The crystalline quality of such TGCs can be further verified by the investigation of their oxidative stability in air. All TGCs can survive up to 600 degrees C without any structural variations, and a few TGCs still survive with an anisotropic etched and stepped nanotip at temperatures up to 800 degrees C, much better than CNTs. Thus, TGCs with single crystalline nanotips are potential candidates for scanning probes in high-temperature oxygen-containing environments.

Force ripple compensation in a tubular linear generator for marine renewable generation

Tubular permanent magnet linear generators are a promising generator technology for use in marine renewables. One aspect of their design relates to the conditions necessary for achieving a smooth thrust response from the generator, free from cogging and periodic variations due to spatial harmonics of the flux cutting the generator coils. This paper presents an experimental and finite element study of the sources of thrust ripple in a prototype linear generator for marine generation. A simple self-commutated control scheme is shown, which uses linear Hall-effect sensors and look-up-table based feed-forward compensation to derive the excitation currents required to drive the machine with constant force. Details of the controller's FPGA based implementation are given, including its strategy for detecting sensor failure. © 2011 IEEE.

Characterisation of friction and lubrication regimes in premium tubular connections

A friction test rig has been developed to carry out repeated sliding friction tests for premium tubular connections. The test rig enables accurate measurement of friction in various contact regimes which are relevant to the threaded connections between tubular components. Higher load tests can simulate the contact in metal-to-metal seals under very high contact pressures by using perpendicular pin-on-pin tests. The contact in the thread loading flank under intermediate pressures can be simulated by using larger radius coupon-on-coupon tests. The measured coefficient of friction is well correlated with a lubrication parameter combining lubricant film thickness and initial surface roughness. © 2012 Elsevier Ltd. All rights reserved.

Thermal modelling of a tubular linear machine for marine renewable generation

A lumped parameter thermal model has been constructed for a tubular linear machine that has been designed for use in a marine environment. It shows good correlation to both steady state and transient experimental tests on the machine. The model has been developed for a stationary machine in a laboratory environment - the modelling techniques used and enhancements to enable the application of the model directly to marine scenarios are discussed.

Detailed thermal modelling of a tubular linear machine for marine renewable generation

A detailed lumped-parameter thermal model is presented for a tubular linear machine that has been designed for use in a marine environment. The model has been developed for a static machine, the worst-case thermal scenario, and is used to establish a rating for the machine. The model has been validated against a large range of experimental tests and shows good correlation to both steady-state and transient experimental results. The model was constructed from a mostly theoretical basis with very little calibration, suggesting that the techniques used are applicable in a more general sense. © 2013 IEEE.

Raman scattering from an individual tubular graphite cone