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Deficiency of ornithine-δ-aminotransferase (OAT) in humans results in gyrate atrophy. Early diagnosis may allow initiation of treatment before irreversible damage has occurred. However, diagnosis is commonly delayed well into adulthood because of the nonspecific character of initial symptoms. Here, we report findings in a neonate who was evaluated because of a positive family history of OAT defici
A search for the electroweak production of charginos and sleptons decaying into final states with two electrons or muons is presented. The analysis is based on 139 fb- 1 of proton–proton collisions recorded by the ATLAS detector at the Large Hadron Collider at s=13 TeV. Three R-parity-conserving scenarios where the lightest neutralino is the lightest supersymmetric particle are considered: the pro
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The emission of floral volatiles requires coordinated expression of biosynthetic genes. In the regulatory network of the volatile benzenoid/phenylpropanoid pathway in Petunia hybrida two master regulators of the pathway have been identified. The R2R3-MYB transcription factor EMISSION OF BENZENOIDS II (EOBII) utilizes a specific MYB binding site to activate the expression of the R2R3-MYB ODORANT1 (
The health and productivity of global land resources are declining, while demand for those resources is increasing. The aim of land degradation neutrality (LDN) is to maintain or enhance land-based natural capital and its associated ecosystem services. The Scientific Conceptual Framework for Land Degradation Neutrality has been developed to provide a scientific approach to planning, implementing a
The Crafoord Prize in Polyarthritis ranks as one of the most prestigious prizes and can be awarded only if the Royal Swedish Academy of Sciences decides the likelihood of prize worthy progress in the field, and at most every 4th year. This has happened only four times since 1982. This year the 5th Laureates were Shimon Sakaguchi, Fred Ramsdell, and Alexander Rudensky with the motivation "for their
Objective: Adult-onset IgA vasculitis (Henoch-Schönlein) (IgAV) is a rare systemic vasculitis characterized by IgA1-dominant deposits. The treatment of adult-onset IgAV is controversial and is based on the combination of glucocorticoids and immunosuppressive agents, but many patients have refractory or relapsing disease despite treatment. Rituximab (RTX) is a B cell–depleting antibody of proven ef
Transition narratives are stories promoting particular pathways for development, promoting specific actions, strategies, and interventions to enable certain outcomes in socio-technical transitions. Narratives centered around biorefineries take a significant role in the growing bioeconomy discourse, yet they express remarkably different visions for the transition. The paper uses Q methodology to id
Sexual dimorphism in eye structure is attributed to sexual selection in animals that employ vision for locating mates. In many male insects, large eyes and eye regions of higher acuity are believed to facilitate the location of females. Here, we compare various features of male and female eyes in three sympatric carpenter bee species, which include two diurnal species (Xylocopa tenuiscapa and X. l
Describes a method to measure changes in the mid-latency auditory evoked potential (MLAEP) during anesthesia. It is claimed that the position of the Nb-trough of the MLAEP indicates the level of consciousness. The component shows graded changes corresponding to the dose of anesthetic and it exhibits stable reproducible properties between different subjects. The authors propose a system that reduce
Heart failure occurs in 2 - 3% of the adult population in the developed world. With decompensation of cardiac function, haemodynamic stability can be achieved by using intravenous vasodilators, diuretics and inotropes. Unlike traditional inotropes, Ca2+, sensitisers enhance cardiac function without significantly increasing cardiac oxygen consumption, promoting arrhythmia or impairing lusitropy. Th
A review and expansion of the fundamental processes of the vapor-liquid-solid (VLS) growth mechanism for nanowires is presented. Although the focus is on nanowires, most of the concepts may be applicable to whiskers, nanotubes, and other unidirectional growth. Important concepts in the VLS mechanism such as preferred deposition, supersaturation, and nucleation are examined. Nanowire growth is feas
Abstract. In this paper we examine subalgebras on two generators in the univariate polynomial ring. A set, S, of polynomials in a subalgebra of a polynomial ring is called a canonical basis (also referred to as SAGBI basis) for the subalgebra if all lead monomials in the subalgebra are products of lead monomials of polynomials in S. In this paper we prove that a pair of polynomials ff; gg is a can
Our purpose is to find factors that are important for expected returns and risk of Swedish industrial portfolios during 1980–1997. The tested factors are supposed to be essential for a small open economy. We take into account the small sample problem that surfaces in the form of firms dominating the value weighted test portfolios. An extreme bound analysis (EBA) investigates the robustness of the
A time-gated ballistic imaging instrument is used to obtain high-spatial-resolution, single-shot images of the liquid core in a water spray issuing into a gaseous crossflow. We describe further development of the diagnostic technique to improve spatial resolution and present images and statistics for various jets under crossflow experimental conditions (different Weber numbers). Series of these im
Here, a conceptual model is presented for the development of Phytophthora disease in pedunculate oak. The model is presented using the causal loop diagram tool and gives an overview of how various abiotic and biotic factors, such as soil moisture, nutrient availability and mycorrhizal colonization, may affect the reproduction and the infective capacity of soil-borne Phytophthora species, the susce
Rheological properties of isotropic solutions and gel structures of lysozyme-sodium dodecyl sulfate mixtures in water are investigated. Isotropic solutions behave as Newtonian fluids with very low viscosity values. For the lysozyme solutions the intrinsic viscosity and the Huggins coefficient were calculated on the basis of the Mooney equation. Above a certain yield stress value, the viscosity of
