58 research outputs found

    Countercyclical energy and climate policy for the US

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    Continuation of the U.S.'s historical pattern addressing energy problems only in times of crisis is unlikely to catalyze a transition to an energy system with fewer adverse social impacts. Instead, the U.S. needs to bolster support for energy innovation when the perceived urgency of energy-related problems appears to be receding. Because of the lags involved in both the energy system and the climate system, decarbonizing the economy will require extraordinary persistence over decades. This need for sustained commitment is in contrast to the last several decades, which have been marked by volatility and cycles of boom and bust. In contrast to the often-repeated phrase that one should 'never let a good crisis go to waste,' the U.S. needs to most actively foster energy innovation when aspects of energy and climate problems appear to be improving. We describe the rationale for a 'countercyclical' approach to energy and climate policy, which involves precommitment to a set of policies that go into effect once a set of trigger conditions are met

    Severe neurocognitive and growth disorders due to variation in THOC2, an essential component of nuclear mRNA export machinery

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    Highly conserved TREX-mediated mRNA export is emerging as a key pathway in neuronal development and differentiation. TREX subunit variants cause neurodevelopmental disorders (NDDs) by interfering with mRNA export from the cell nucleus to the cytoplasm. Previously we implicated four missense variants in the X-linked THOC2 gene in intellectual disability (ID). We now report an additional six affected individuals from five unrelated families with two de novo and threematernally inherited pathogenic or likely pathogenic variants in THOC2 extending the genotypic and phenotypic spectrum. These comprise three rare missense THOC2 variants that affect evolutionarily conserved amino acid residues and reduce protein stability and two with canonical splice-site THOC2 variants that result in C-terminally truncated THOC2 proteins.We present detailed clinical assessment and functional studies on a de novo variant in a female with an epileptic encephalopathy and discuss an additional four families with rare variants in THOC2 with supportive evidence for pathogenicity. Severe neurocognitive features, including movement and seizure disorders, were observed in this cohort. Taken together our data show that even subtle alterations to the canonical molecular pathways such asmRNAexport, otherwise essential for cellular life, can be compatible with life, but lead to NDDs in human

    Mastitis diagnostics and performance monitoring: a practical approach

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    In this paper a review is given of frequently used mastitis diagnostic methods in modern dairy practice. Methods used at the quarter, cow, herd and regional or national level are discussed, including their usability for performance monitoring in udder health. Future developments, such as systems in which milk-derived parameters are combined with modern analytical techniques, are discussed. It is concluded that, although much knowledge is available and science is still developing and much knowledge is available, it is not always fully exploited in practice

    Socio-economic variability of safespot cooperative safety systems

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    This paper describes the results of a socio-economic assessment of two cooperative safety systems. One of the systems is based on V2V communication, the other one is based on V2I communication. Both systems are composed of a bundle of applications which have been designed to provide road safety information to the driver. An integrated assessment approach was applied in this study using cost-benefit analysis and stakeholder analysis. The results of the impact assessment show a considerable increase of road safety for both systems. Benefit-cost ratios calculated for the V2V based system are acceptable from a society point of view, whereas the efficiency of the V2I system could not be proved in case of a large-scale equipment of infrastructure. The findings lead to the conclusion that added value can be achieved, if a combined solution (V2V plus V2I) is implemented and low-scale equipment of infrastructure concentrates on accident black spots

    μ‑Oxo Dimerization Effects on Ground- and Excited-State Properties of a Water-Soluble Iron Porphyrin CO<sub>2</sub> Reduction Catalyst

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    Iron 5,10,15,20-tetra(para-N,N,N-trimethylanilinium)porphyrin (Fe-p-TMA) is a water-soluble catalyst capable of electrochemical and photochemical CO2 reduction. Although its catalytic ability has been thoroughly investigated, the mechanism and associated intermediates are largely unknown. Previous studies proposed that Fe-p-TMA enters catalytic cycles as a monomeric species. However, we demonstrate herein that, in aqueous solutions, Fe-p-TMA undergoes formation of a μ-oxo porphyrin dimer that exists in equilibrium with its monomeric form. The propensity for μ-oxo formation is highly dependent on the solution pH and ionic strength. Indeed, the μ-oxo form is stabilized in the presence of electrolytes that are key components of catalytically relevant conditions. By leveraging the ability to chemically control and spectrally address both species, we characterize their ground-state electronic structures and excited-state photodynamics. Global fitting of ultrafast transient absorption data reveals two distinct excited-state relaxation pathways: a three-component sequential model consistent with monomeric relaxation and a two-component sequential model for the μ-oxo species. Relaxation of the monomeric species is best described as a ligand-to-metal charge transfer (τ1 = ∼500 fs), an ionic strength-dependent metal-to-ligand charge transfer (τ2 = 2–4 ps), and finally relaxation of a ligand field excited state to the ground state (τ3 = 5 ps). Conversely, excited-state relaxation of the μ-oxo species proceeds via cleavage of an FeIII–O bond to generate transient FeIVO and FeII porphyrin species (τ1 = 2 ps) that recombine to the ground-state μ-oxo species (τ2 = ∼1 ns). This latter lifetime extends to timescales relevant for chemical reactivity. It is therefore emphasized that further consideration of catalyst speciation and chemical microenvironments is necessary for elucidating the mechanisms of catalytic CO2 reduction reactions

    Large offset monochromators at PETRA III

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    For the beamlines of the new synchrotron radiation source PETRA III, fixed-exit double crystal monochromators with specific features were developed. To achieve a compact arrangement of the canted undulator beamlines at Sectors 2, 4 and 6, it is necessary to shift one of the two beamlines in vertical direction. This is done by so-called large offset monochromators. Two of these monochromators (LOM500 and Mono-P06) accept the white beam and are therefore cooled with liquid nitrogen. The third one accepts a monochromatic beam and has no cooling system. The challenge with this device (LOM1250) is the large offset of the beam by 1.25 m. The energy range in combination with the large vertical beam offset demands for a relative crystal movement of roughly 3 m along the beam direction. This is implemented by translating each crystal by up to 1.5 m. It is equipped with a visible laser-based stabilization which allows compensating thermal drift of the mechanical components involved in the positioning of the crystals. With this novel approach the X-ray beam is not attenuated by beam position monitors.</jats:p
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