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  • 1
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    Secondary coenzyme Q10 deficiency and oxidative stress in cultured fibroblasts from patients with riboflavin responsive multiple Acyl-CoA dehydrogenation deficiency (2013)
    Oxford University Press
    Details
    Publication Date: 2013-09-08
    Description: Coenzyme Q10 (CoQ10) is essential for the energy production of the cells and as an electron transporter in the mitochondrial respiratory chain. CoQ10 links the mitochondrial fatty acid β-oxidation to the respiratory chain by accepting electrons from electron transfer flavoprotein-ubiquinone oxidoreductase (ETF-QO). Recently, it was shown that a group of patients with the riboflavin responsive form of multiple acyl-CoA dehydrogenation deficiency (RR-MADD) carrying inherited amino acid variations in ETF-QO also had secondary CoQ10 deficiency with beneficial effects of CoQ10 treatment, thus adding RR-MADD to an increasing number of diseases involving secondary CoQ10 deficiency. In this study, we show that moderately decreased CoQ10 levels in fibroblasts from six unrelated RR-MADD patients were associated with increased levels of mitochondrial reactive oxygen species (ROS). Treatment with CoQ10, but not with riboflavin, could normalize the CoQ10 level and decrease the level of ROS in the patient cells. Additionally, riboflavin-depleted control fibroblasts showed moderate CoQ10 deficiency, but not increased mitochondrial ROS, indicating that variant ETF-QO proteins and not CoQ10 deficiency are the causes of mitochondrial ROS production in the patient cells. Accordingly, the corresponding variant Rhodobacter sphaeroides ETF-QO proteins, when overexpressed in vitro , bind a CoQ10 pseudosubstrate, Q10Br, less tightly than the wild-type ETF-QO protein, suggesting that molecular oxygen can get access to the electrons in the misfolded ETF-QO protein, thereby generating superoxide and oxidative stress, which can be reversed by CoQ10 treatment.
    Print ISSN: 0964-6906
    Electronic ISSN: 1460-2083
    Topics: Biology , Medicine
    Published by Oxford University Press
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  • 2
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    Core surface magnetic field evolution 2000–2010 (2012)
    Oxford University Press
    Details
    Publication Date: 2012-03-16
    Description: SUMMARY We present new dedicated core surface field models spanning the decade from 2000.0 to 2010.0. These models, called gufm-sat , are based on CHAMP, Ørsted and SAC-C satellite observations along with annual differences of processed observatory monthly means. A spatial parametrization of spherical harmonics up to degree and order 24 and a temporal parametrization of sixth-order B-splines with 0.25 yr knot spacing is employed. Models were constructed by minimizing an absolute deviation measure of misfit along with measures of spatial and temporal complexity at the core surface. We investigate traditional quadratic or maximum entropy regularization in space, and second or third time derivative regularization in time. Entropy regularization allows the construction of models with approximately constant spectral slope at the core surface, avoiding both the divergence characteristic of the crustal field and the unrealistic rapid decay typical of quadratic regularization at degrees above 12. We describe in detail aspects of the models that are relevant to core dynamics. Secular variation and secular acceleration are found to be of lower amplitude under the Pacific hemisphere where the core field is weaker. Rapid field evolution is observed under the eastern Indian Ocean associated with the growth and drift of an intense low latitude flux patch. We also find that the present axial dipole decay arises from a combination of subtle changes in the southern hemisphere field morphology.
    Print ISSN: 0956-540X
    Electronic ISSN: 1365-246X
    Topics: Geosciences
    Published by Oxford University Press on behalf of The Deutsche Geophysikalische Gesellschaft (DGG) and the Royal Astronomical Society (RAS).
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  • 3
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    Review of thermal processing of biomass and waste in molten salts for production of renewable fuels and chemicals (2012)
    Oxford University Press
    Details
    Publication Date: 2012-11-09
    Description: Renewable energy has gained great attention and interest in recent years due to growing energy consumption and greater environmental concerns. Biomass is regarded as a promising candidate for replacing fossil-derived products, through either thermal, biological, or physical processes. This review focuses on thermal processing of biomass in molten salts for production of renewable fuels and chemicals, concepts based on dispersion of biomass or waste particles in a molten salt bath. Inorganic salts have very high heat capacities and good thermal stability at high temperatures. Some molten salts have catalytic properties, and in thermal processing of biomass, the product yields and compound compositions of products can be adjusted by varying compositions and amount of molten salts. In addition, molten salts will retain noxious contaminants, and it is thus possible to use difficult convertible- and/or contaminated biomass as feedstock.
    Print ISSN: 1748-1317
    Electronic ISSN: 1748-1325
    Topics: Energy, Environment Protection, Nuclear Power Engineering , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Published by Oxford University Press
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  • 4
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    Movements and site fidelity of harbour seals (Phoca vitulina) in Kattegat, Denmark, with implications for the epidemiology of the phocine distemper virus (2012)
    Oxford University Press
    Details
    Publication Date: 2012-12-12
    Description: Dietz, R., Teilmann, J., Andersen S. M. Rigét, F., and Olsen, M. T. 2013. Movements and site fidelity of harbour seals ( Phoca vitulina ) in Kattegat, Denmark, with implications for the epidemiology of the phocine distemper virus. – ICES Journal of Marine Science, 70:186–195. Twenty-seven harbour seals were caught and tagged at the island of Anholt in central Kattegat, Denmark, the epicentre of the phocine distemper virus (PDV) outbreaks in 1988 and 2002 that killed 50–60% of the populations. The satellite tagging shows that harbour seals from Anholt moved widely across Kattegat with a maximum distance of 249 km from the tagging haul-out site. Overall, females travelled over a wider area compared with males [90% kernel home range (KHR) females, 5189 km 2 ; males, 3293 km 2 ). KHR calculated for yearlings (6414 km 2 ) is larger than for subadults (2534 km 2 ), which again is larger than for adult seals (1713 km 2 ), showing a strong site fidelity, indicating limited gene flow between haul-out sites. Distances moved and home range sizes increased across autumn, peaked in February–March, and decreased through spring. During the breeding season in spring, all seals were very stationary around Anholt. The onset of the PDV epizootics in 1988 and 2002 took place when the Anholt harbour seals congregate on the Island during April. Anholt seal were also documented to have contact with infected seal locations at Hesselø, Læsø, and the Swedish west coast, although this contact takes place during winter prior to the documented summer outbreaks.
    Print ISSN: 1054-3139
    Electronic ISSN: 1095-9289
    Topics: Biology , Geosciences , Physics
    Published by Oxford University Press
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  • 5
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    Core surface magnetic field evolution 2000–2010 (2012)
    Oxford University Press
    Details
    Publication Date: 2012-04-15
    Description: SUMMARY We present new dedicated core surface field models spanning the decade from 2000.0 to 2010.0. These models, called gufm-sat , are based on CHAMP, Ørsted and SAC-C satellite observations along with annual differences of processed observatory monthly means. A spatial parametrization of spherical harmonics up to degree and order 24 and a temporal parametrization of sixth-order B-splines with 0.25 yr knot spacing is employed. Models were constructed by minimizing an absolute deviation measure of misfit along with measures of spatial and temporal complexity at the core surface. We investigate traditional quadratic or maximum entropy regularization in space, and second or third time derivative regularization in time. Entropy regularization allows the construction of models with approximately constant spectral slope at the core surface, avoiding both the divergence characteristic of the crustal field and the unrealistic rapid decay typical of quadratic regularization at degrees above 12. We describe in detail aspects of the models that are relevant to core dynamics. Secular variation and secular acceleration are found to be of lower amplitude under the Pacific hemisphere where the core field is weaker. Rapid field evolution is observed under the eastern Indian Ocean associated with the growth and drift of an intense low latitude flux patch. We also find that the present axial dipole decay arises from a combination of subtle changes in the southern hemisphere field morphology.
    Print ISSN: 0956-540X
    Electronic ISSN: 1365-246X
    Topics: Geosciences
    Published by Oxford University Press on behalf of The Deutsche Geophysikalische Gesellschaft (DGG) and the Royal Astronomical Society (RAS).
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  • 6
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    Adjoint analysis of the source and path sensitivities of basin-guided waves (2012)
    Oxford University Press
    Details
    Publication Date: 2012-04-15
    Description: SUMMARY Simulations of earthquake rupture on the southern San Andreas Fault (SAF) reveal large amplifications in the San Gabriel and Los Angeles Basins (SGB and LAB) apparently associated with long-range path effects. Geometrically similar excitation patterns can be recognized repeatedly in different SAF simulations (e.g. Love wave-like energy with predominant period around 4 s, channelled southwestwardly from the SGB into LAB), yet the amplitudes with which these distinctive wavefield patterns are excited change, depending upon source details (slip distribution, direction and velocity of rupture). We describe a method for rapid calculation of the sensitivity of such predicted wavefield features to perturbations of the source kinematics, using a time-reversed (adjoint) wavefield simulation. The calculations are analogous to those done in adjoint tomography, and the same time-reversed calculation also yields path-sensitivity kernels that give further insight into the excitation mechanism. For rupture on the southernmost 300 km of SAF, LAB excitation is greatest for slip concentrated between the northern Coachella Valley and the transverse ranges, propagating to the NE and with rupture velocities between 3250 and 3500 m s –1 along that fault segment; that is, within or slightly above the velocity range (between Rayleigh and S velocities) that is energetically precluded in the limit of a sharp rupture front, highlighting the potential value of imposing physical constraints (such as from spontaneous rupture models) on source parametrizations. LAB excitation is weak for rupture to the SW and for ruptures in either direction located north of the transverse transverse ranges, whereas Ventura Basin (VTB) is preferentially excited by NE ruptures situated north of the transverse ranges. Path kernels show that LAB excitation is mediated by surface waves deflected by the velocity contrast along the southern margin of the transverse ranges, having most of their energy in basement rock until they impinge on the eastern edge of SGB, through which they are then funnelled into LAB. VTB amplification is enhanced by a similar waveguide effect.
    Print ISSN: 0956-540X
    Electronic ISSN: 1365-246X
    Topics: Geosciences
    Published by Oxford University Press on behalf of The Deutsche Geophysikalische Gesellschaft (DGG) and the Royal Astronomical Society (RAS).
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  • 7
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    The response of herring to high crowding densities in purse-seines: survival and stress reaction (2012)
    Oxford University Press
    Details
    Publication Date: 2012-08-22
    Description: Tenningen, M., Vold, A., and Olsen, R. E. 2012. The response of herring to high crowding densities in purse-seines: survival and stress reaction. – ICES Journal of Marine Science, 69: . To study the effects of crowding in purse-seines on the survival and stress response of herring ( Clupea harengus ), large-scale field experiments were conducted in the North Sea during 2008 and 2009. The mortality was 28% at a crowding density of 221 kg m –3 and increased further with increasing density. Crowding densities 〈150 kg m –3 did not result in any additional mortality compared with the control group (0.9–2.0%). Smaller herring and herring with a lower condition factor were more vulnerable to the effects of crowding. Blood analyses showed a significant increase in cortisol, lactate, and blood ions in the crowded fish. Lactate returned to control levels 2 d post-stress, whereas cortisol and blood ion levels continued to increase during the 4- to 5-d monitoring period. Furthermore, plasma glucose appeared to be substantially reduced at the end of the trial, indicating that the herring were incapable of restoring homeostasis and were approaching energy exhaustion. These results provide important information about the crowding densities that can be tolerated in the purse-seine fisheries for herring and will benefit future development of guidelines for purse-seine slipping operations.
    Print ISSN: 1054-3139
    Electronic ISSN: 1095-9289
    Topics: Biology , Geosciences , Physics
    Published by Oxford University Press
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  • 8
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    Molecular mechanisms of riboflavin responsiveness in patients with ETF-QO variations and multiple acyl-CoA dehydrogenation deficiency (2012)
    Oxford University Press
    Details
    Publication Date: 2012-07-10
    Description: Riboflavin-responsive forms of multiple acyl-CoA dehydrogenation deficiency (RR-MADD) have been known for years, but with presumed defects in the formation of the flavin adenine dinucleotide (FAD) co-factor rather than genetic defects of electron transfer flavoprotein (ETF) or electron transfer flavoprotein-ubiquinone oxidoreductase (ETF-QO). It was only recently established that a number of RR-MADD patients carry genetic defects in ETF-QO and that the well-documented clinical efficacy of riboflavin treatment may be based on a chaperone effect that can compensate for inherited folding defects of ETF-QO. In the present study, we investigate the molecular mechanisms and the genotype–phenotype relationships for the riboflavin responsiveness in MADD, using a human HEK-293 cell expression system. We studied the influence of riboflavin and temperature on the steady-state level and the activity of variant ETF-QO proteins identified in patients with RR-MADD, or non- and partially responsive MADD. Our results showed that variant ETF-QO proteins associated with non- and partially responsive MADD caused severe misfolding of ETF-QO variant proteins when cultured in media with supplemented concentrations of riboflavin. In contrast, variant ETF-QO proteins associated with RR-MADD caused milder folding defects when cultured at the same conditions. Decreased thermal stability of the variants showed that FAD does not completely correct the structural defects induced by the variation. This may cause leakage of electrons and increased reactive oxygen species, as reflected by increased amounts of cellular peroxide production in HEK-293 cells expressing the variant ETF-QO proteins. Finally, we found indications of prolonged association of variant ETF-QO protein with the Hsp60 chaperonin in the mitochondrial matrix, supporting indications of folding defects in the variant ETF-QO proteins.
    Print ISSN: 0964-6906
    Electronic ISSN: 1460-2083
    Topics: Biology , Medicine
    Published by Oxford University Press
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  • 9
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    Identification of supershear transition mechanisms due to material contrast at bimaterial faults (2012)
    Oxford University Press
    Details
    Publication Date: 2012-06-01
    Description: SUMMARY Numerical modelling of dynamic rupture is conducted along faults separating similar and dissimilar materials. Supershear transition is enhanced in the direction of slip of the stiffer material (the negative direction) due to the bimaterial effect whereby a decrease in normal stress in front of the crack tip supports yielding ahead of the rupture. In the direction of slip of the more compliant material (the positive direction), an increase in normal stress ahead of the rupture tip delays or prevents the supershear transition, whereas the impact of the bimaterial effect on subshear ruptures is to promote rupture in the positive direction due to the tensile stress perturbation behind the rupture tip in this direction. We demonstrate that the material contrast and the parameter S control whether the transition from sub- to supershear velocity (supershear transition) is smooth or follows the Burridge–Andrews mechanism. Supershear transition along interfaces separating dissimilar materials is possible for higher values of the parameter S than supershear transition along material interfaces separating similar materials. The difference between pulse-like and crack-like rupture is small with regard to the supershear transition type.
    Print ISSN: 0956-540X
    Electronic ISSN: 1365-246X
    Topics: Geosciences
    Published by Oxford University Press on behalf of The Deutsche Geophysikalische Gesellschaft (DGG) and the Royal Astronomical Society (RAS).
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  • 10
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    Is embedding entailed in consumer valuation of food safety characteristics? (2011)
    Oxford University Press
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    Publication Date: 2011-11-24
    Description: Consumers' preferences for food safety characteristics are investigated with a particular focus on the existence of an embedding effect. Embedding exists if consumer valuation of food safety is insensitive to scope. We conduct between-attribute external tests for embedding in two choice experiments concerning the value of food safety attributes in minced pork and chicken breasts. We find no evidence of embedding neither when using food safety attributes that are not close substitutes and which exhibit both private and public good characteristics, nor when using food safety attributes that are closer substitutes and which have primarily private good characteristics.
    Keywords: Q10 - General, Q13 - Agricultural Markets and Marketing ; Cooperatives ; Agribusiness, Q18 - Agricultural Policy ; Food Policy
    Print ISSN: 0165-1587
    Electronic ISSN: 1464-3618
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition , Economics
    Published by Oxford University Press
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