3,543 research outputs found

    Minimal ureagenesis is necessary for survival in the murine model of hyperargininemia treated by AAV-based gene therapy.

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    Hyperammonemia is less severe in arginase 1 deficiency compared with other urea cycle defects. Affected patients manifest hyperargininemia and infrequent episodes of hyperammonemia. Patients typically suffer from neurological impairment with cortical and pyramidal tract deterioration, spasticity, loss of ambulation, seizures and intellectual disability; death is less common than with other urea cycle disorders. In a mouse model of arginase I deficiency, the onset of symptoms begins with weight loss and gait instability, which progresses toward development of tail tremor with seizure-like activity; death typically occurs at about 2 weeks of life. Adeno-associated viral vector gene replacement strategies result in long-term survival of mice with this disorder. With neonatal administration of vector, the viral copy number in the liver greatly declines with hepatocyte proliferation in the first 5 weeks of life. Although the animals do survive, it is not known from a functional standpoint how well the urea cycle is functioning in the adult animals that receive adeno-associated virus. In these studies, we administered [1-13C] acetate to both littermate controls and adeno-associated virus-treated arginase 1 knockout animals and examined flux through the urea cycle. Circulating ammonia levels were mildly elevated in treated animals. Arginine and glutamine also had perturbations. Assessment 30 min after acetate administration demonstrated that ureagenesis was present in the treated knockout liver at levels as low at 3.3% of control animals. These studies demonstrate that only minimal levels of hepatic arginase activity are necessary for survival and ureagenesis in arginase-deficient mice and that this level of activity results in control of circulating ammonia. These results may have implications for potential therapy in humans with arginase deficiency

    En torno a la reforma del derecho civil matrimonial (Entrevista)

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    Crop diversification and digestate application effect on the productivity and efficiency of irrigated winter crop systems

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    Irrigated winter crops can reduce input demands when compared with irrigated summer cropping systems in the Mediterranean area. The sustainability of these systems can be further improved resorting to diverse rotations, but also to fertilisation with digestate, a by-product from anaerobic digestion of organic waste. Post-treatments such as drying and acidifying can improve the fertiliser value of this product. In this study, we compare wheat performance in a three-year full cereal rotation or in a diverse rotation, with pea and canola. Besides, untreated and dried acidified digestates are tested as fertilisers for all crops, comparing to mineral fertilisation at a rate of 140 kg N ha−1 and a control with no fertilisation. To assess productivity and efficiency of the different systems, grain yield and N concentration, N uptake efficiency (NUpE) and water use efficiency (WUE) were determined, along with soil nitrate dynamics and total N at the end of the experiment. Results showed an average wheat yield increase of 1.79 t ha−1 by the last year of the diverse crop rotation rather than the cereal rotation (p < 0.001). Although there was no yield increase in the previous year, wheat after pea showed higher grain N concentration (p < 0.001). However, the NUpE of wheat remained steady due to a higher soil N availability after pea, which suggests that fertilisation can be adjusted. Although wheat WUE increased due to canola and pea precedents (p < 0.001), the cereal rotations should be more adapted to systems with low water availability. Nonetheless, both rotations required about 30% of irrigated water than the typical irrigated summer crops of the Ebro valley region. Fertiliser effect on yields was variable according to the tested crop mainly due to differences in N demand along each growing cycle. Overall, dried acidified digestate application resulted in similar soil nitrate levels than the mineral fertiliser. The diverse rotation raised soil nitrates content compared to the cereal rotation at sowing and harvesting times (p = 0.002 and p < 0.001, respectively). Higher soil nitrate levels were found when associating these two practices, while nitrate levels after mineral fertilisation showed to be less dependent on the implemented rotation. The diverse rotation also raised soil total N at the end of the experiment (p = 0.023). Similarly, the dried acidified digestate application resulted in higher soil total N than mineral fertilisation (p = 0.011). These findings show how these management practices should allow for a chemical fertilisation reduction in irrigated winter crop systems.This work was supported by the European Union’s Horizon 2020 Research and Innovation programme [Grant Agreement No. 773649] and by the State Agency of Research (Spain) [Projects AGL 2017/84529-C3–3R and PID 2021–126343OB-C33]. G. Nascimento is recipient of an UdL-IRTA PhD fellowship. The IRTA authors acknowledge the CERCA programme (Generalitat de Catalunya) contribution.info:eu-repo/semantics/publishedVersio

    Recurrence for discrete time unitary evolutions

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    We consider quantum dynamical systems specified by a unitary operator U and an initial state vector \phi. In each step the unitary is followed by a projective measurement checking whether the system has returned to the initial state. We call the system recurrent if this eventually happens with probability one. We show that recurrence is equivalent to the absence of an absolutely continuous part from the spectral measure of U with respect to \phi. We also show that in the recurrent case the expected first return time is an integer or infinite, for which we give a topological interpretation. A key role in our theory is played by the first arrival amplitudes, which turn out to be the (complex conjugated) Taylor coefficients of the Schur function of the spectral measure. On the one hand, this provides a direct dynamical interpretation of these coefficients; on the other hand it links our definition of first return times to a large body of mathematical literature.Comment: 27 pages, 5 figures, typos correcte

    Characterization of quaternary ammonium compounds in Flourensia xerophytic communities and response to UV-B radiation

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    As part of ongoing studies aimed at characterizing molecular components involved in the ecophysiological adaptations of native xerophytic plants from central Argentina, we demonstrated the presence of compatible solutes in Flourensia campestris (FC) and Flourensia oolepis (FO), specifically glycine betaine (GB) through TLC, LC, 1H NMR and 13C-NMR. GB content (leaves: 38±7μmolg-1 DW; adult plants>seedlings), and distribution (capitula>vegetative leaves>reproductive leaves>shoots>roots) were similar to other quaternary ammonium compound (QAC) accumulators. Flourensia seedlings from both species protected from UV-B exposure - a major abiotic stress in these natural environments - showed a significant increase of GB in the leaves (p<0.01) and a significant decrease in the roots (p<0.05). In FC and FO xerophytic shrub-dominated communities QACs were detected for the first time in 41% of co-occurring species (N=39), 14 of 28 natives (50%) and 2 of 11 exotics (18%), being GB in natives only (57% of QAC accumulators). GB may be considered as a chemotaxonomical character for the genus Flourensia, since it was also detected in Flourensia hirta, Flourensia niederleinii, Flourensia riparia, Flourensia fiebrigii, Flourensia macroligulata and Flourensia heterolepis. Our controlled UV-B experiments, set up in the same natural environment where these species grow, clearly show that solar UV-B - and therefore oxidative stress - is involved in regulating GB contents and within-plant distribution in FC and FO seedlings. The findings in Flourensia co-occurring native species suggest that QACs accumulation may be considered as a community-specific ecophysiological trait in these xerophytic environments. .Fil: Piazza, Leonardo A.. Universidad de Buenos Aires. Facultad de Agronomia. Sede Polo Universitario Punilla Centro; ArgentinaFil: López, D.. Universidad de Buenos Aires. Facultad de Agronomia. Sede Polo Universitario Punilla Centro; ArgentinaFil: Silva, Mariana Paola. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Universidad de Buenos Aires. Facultad de Agronomia. Sede Polo Universitario Punilla Centro; ArgentinaFil: Lopez Rivilli, Marisa Juana. Universidad de Buenos Aires. Facultad de Agronomia. Sede Polo Universitario Punilla Centro; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; ArgentinaFil: Cantero, Juan Jose. Universidad Nacional de Río Cuarto. Facultad de Agronomía y Veterinaria; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto Multidisciplinario de Biología Vegetal. Universidad Nacional de Córdoba. Facultad de Ciencias Exactas Físicas y Naturales. Instituto Multidisciplinario de Biología Vegetal; ArgentinaFil: Tourn, G. M.. Universidad de Buenos Aires. Facultad de Agronomia. Sede Polo Universitario Punilla Centro; ArgentinaFil: Scopel, Ana Leonor. Consejo Nacional de Investigaciones Científicas y Técnicas. Oficina de Coordinación Administrativa Parque Centenario. Instituto de Investigaciones en Biociencias Agrícolas y Ambientales. Universidad de Buenos Aires. Facultad de Agronomía. Instituto de Investigaciones en Biociencias Agrícolas y Ambientales; Argentin

    Fractional Moment Estimates for Random Unitary Operators

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    We consider unitary analogs of dd-dimensional Anderson models on l2(Zd)l^2(\Z^d) defined by the product Uω=DωSU_\omega=D_\omega S where SS is a deterministic unitary and DωD_\omega is a diagonal matrix of i.i.d. random phases. The operator SS is an absolutely continuous band matrix which depends on parameters controlling the size of its off-diagonal elements. We adapt the method of Aizenman-Molchanov to get exponential estimates on fractional moments of the matrix elements of Uω(Uωz)1U_\omega(U_\omega -z)^{-1}, provided the distribution of phases is absolutely continuous and the parameters correspond to small off-diagonal elements of SS. Such estimates imply almost sure localization for UωU_\omega

    Mª Elena SÁNCHEZ JORDÁN: El régimen de segunda oportunidad del consumidor concursado

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    La autora, Catedrática de Derecho Civil de la Universidad de La Laguna, especialista reconocida en materia hipotecaria ofrece una monografía de máxima actualidad, social y jurídica. El prologuista, el Prof. Miquel, no ahorra calificativos de dura crítica al describir el marco legal y judicial del problema abordado. Escribe: «La grave crisis económica que ha tenido lugar en los últimos años ha puesto a prueba nuestro sistema jurídico y han quedado al descubierto algunas de sus debilidades». Así enumera algunas a continuación: Que el procedimiento de ejecución hipotecaria ha resultado excesivamente duro para los deudores de préstamos hipotecarios destinados a la adquisición de la vivienda; que muchas familias han perdido su vivienda quedando con el peso insoportable de una deuda enorme, inicuo resultado que ha dado lugar a movimientos sociales que justa y legítimamente han reclamado una política protectora del derecho a la vivienda tal como proclama la Constitución..

    A white dwarf catalogue from Gaia-DR2 and the Virtual Observatory

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    We present a catalogue of 73¿221 white dwarf candidates extracted from the astrometric and photometric data of the recently published Gaia-DR2 catalogue. White dwarfs were selected from the Gaia Hertzsprung–Russell diagram with the aid of the most updated population synthesis simulator. Our analysis shows that Gaia has virtually identified all white dwarfs within 100¿pc from the Sun. Hence, our sub-population of 8555 white dwarfs within this distance limit and the colour range considered, -0.52<(GBP-GRP)<0.80¿, is the largest and most complete volume-limited sample of such objects to date. From this sub-sample, we identified 8343 CO-core and 212 ONe-core white dwarf candidates and derived a white dwarf space density of 4.9±0.4×10-3pc-3¿. A bifurcation in the Hertzsprung–Russell diagram for these sources, which our models do not predict, is clearly visible. We used the Virtual Observatory SED Analyzer tool to derive effective temperatures and luminosities for our sources by fitting their spectral energy distributions, that we built from the ultraviolet to the near-infrared using publicly available photometry through the Virtual Observatory. From these parameters, we derived the white dwarf radii. Interpolating the radii and effective temperatures in hydrogen-rich white dwarf cooling sequences, we derived the surface gravities and masses. The Gaia 100¿pc white dwarf population is clearly dominated by cool (~8000¿K) objects and reveals a significant population of massive (¿M~0.8M¿¿) white dwarfs, of which no more than ~30--40 per cent can be attributed to hydrogen-deficient atmospheres, and whose origin remains uncertain.Peer ReviewedPreprin
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