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Diagnosis of immunodeficiency caused by a purine nucleoside phosphorylase defect by using tandem mass spectrometry on dried blood spots.

Purine nucleoside phosphorylase (PNP) deficiency is a rare form of autosomal recessive combined primary immunodeficiency caused by a enzyme defect leading to the accumulation of inosine, 2'-deoxy-inosine (dIno), guanosine, and 2'-deoxy-guanosine (dGuo) in all cells, especially lymphocytes. Treatments are available and curative for PNP deficiency, but their efficacy depends on the early approach. P

Synergism between vascular endothelial growth factor and placental growth factor contributes to angiogenesis and plasma extravasation in pathological conditions

Vascular endothelial growth factor (VEGF) stimulates angiogenesis by activating VEGF receptor-2 (VEGFR-2). The role of its homolog, placental growth factor (PlGF), remains unknown. Both VEGF and PlGF bind to VEGF receptor-1 (VEGFR-1), but it is unknown whether VEGFR-1, which exists as a soluble or a membrane-bound type, is an inert decoy or a signaling receptor for PlGF during angiogenesis. Here,

Artefactual Intelligence: The Development and Use of Cognitively Congenial Artefacts

How can tools help structure tasks to make them cognitively easier to perform? How do artefacts, and our strategies for using them, develop over time in cognitively beneficial ways? These are two of the main questions that are explored in the five papers collected in this thesis. The first paper details an ethnographic study conducted on people cooking in their homes. The study is a first pass ov

The production, allocation and cycling of carbon in a forest on fertile terra preta soil in eastern Amazonia compared with a forest on adjacent infertile soil

Background: Terra preta do indio or 'dark earth' soils formed as a result of a long-term addition of organic matter by indigenous peoples in Amazonia. Aims: Here we report on the first study of productivity, allocation and carbon cycling from a terra preta plot in eastern Amazonia (Caxiuana, Para, Brazil), and contrast its dynamics with a nearby plot on infertile soil (ferralsols). Methods: We det

Ecosystem services and biodiversity conservation: concepts and a glossary

The RUBICODE project draws on expertise from a range of disciplines to develop and integrate frameworks for assessing the impacts of environmental change on ecosystem service provision, and for rationalising biodiversity conservation in that light. With such diverse expertise and concepts involved, interested parties will not be familiar with all the key terminology. This paper defines the terms a

Using models to guide field experiments : a priori predictions for the CO2 response of a nutrient- and water-limited native Eucalypt woodland

The response of terrestrial ecosystems to rising atmospheric CO2 concentration (Ca), particularly under nutrient-limited conditions, is a major uncertainty in Earth System models. The Eucalyptus Free-Air CO2 Enrichment (EucFACE) experiment, recently established in a nutrient- and water-limited woodland presents a unique opportunity to address this uncertainty, but can best do so if key model uncer

Functional traits as indicators of biodiversity response to land use changes across ecosystems and organisms

Rigorous and widely applicable indicators of biodiversity are needed to monitor the responses of ecosystems to global change and design effective conservation schemes. Among the potential indicators of biodiversity, those based on the functional traits of species and communities are interesting because they can be generalized to similar habitats and can be assessed by relatively rapid field assess

Towards an integrated global framework to assess the impacts of land use and management change on soil carbon : Current capability and future vision

Intergovernmental Panel on Climate Change (IPCC) Tier 1 methodologies commonly underpin project-scale carbon accounting for changes in land use and management and are used in frameworks for Life Cycle Assessment and carbon footprinting of food and energy crops. These methodologies were intended for use at large spatial scales. This can introduce error in predictions at finer spatial scales. There

Impacts of experimentally imposed drought on leaf respiration and morphology in an Amazon rain forest

P>1. The Amazon region may experience increasing moisture limitation over this century. Leaf dark respiration (R) is a key component of the Amazon rain forest carbon (C) cycle, but relatively little is known about its sensitivity to drought. 2. Here, we present measurements of R standardized to 25 degrees C and leaf morphology from different canopy heights over 5 years at a rain forest subject to

Implementation of Industrial Robot Force Control Case Study: High Power Stub Grinding and Deburring

In this paper, the results from a joint industry-academia project in industrial robotic force control are presented. The extension and implementation of an external sensor system for an industrial robot system, which can be used for high-bandwidth force control, are described. Results from two industrial applications using the system are presented, a stub grinding application using a new compliant

Characterization and application of forced cooling channels for traction motors in HEVs

This paper presents the characterization of forced cooling channels with three different shapes which are applied in traction motors mounted in Hybrid Vehicles (HEVs) or Zero Emission Vehicles (ZEVs). Generally, there are two different positions of forced cooling channels in electric motors. One way is by drilling holes or cutting cooling channels inside the housing, which is called indirect cooli

H-2(e,e ' p)n reaction at high recoil momenta

The 2H(e,e(')p)n cross section was measured in Hall A of the Thomas Jefferson National Accelerator Facility near the top of the quasielastic peak (x(Bj)=0.964) at a four-momentum transfer squared, Q(2)=0.665 (GeV/c) (2) (omega=0.368 GeV, W=2.057 GeV), and for recoil momenta up to 550 MeV/c. The measured cross section deviates by 1-2sigma from a state-of-the-art calculation at low recoil momenta. A

Utilisation of antihyperglycaemic drugs in ten European countries: different developments and different levels.

Aims/hypothesis The aim of this study was to compare developments in the utilisation of antihyperglycaemic drugs (AHGDs) in ten European countries. Subhect and methods Data on the yearly utilisation of insulin and oral AHGDs were collected from public registers in Denmark, Finland, Norway, Sweden, Belgium, England, Germany, Italy, Portugal and Spain, and were expressed as defined daily doses per 1

The nutritional quality of an infant food from quinoa and its effect on the plasma level of insulin-like growth factor-1 (IGF-1) in undernourished children.

An infant food product was manufactured by drum drying a pre-cooked slurry of quinoa (Chenopodium quinoa, Willd) flour. The chemical composition shows that the product is a potential source of valuable nutrients, like protein (16%), vitamin E (19 mg/kg), thiamine (0.7 mg/100 g), iron (70 mg/kg), zinc (48 mg/kg) and magnesium (1.8 g/kg), all the values expressed on dry basis, to pre-school children

Early diagnosis of primary Sjögren's syndrome: EULAR-SS task force clinical recommendations.

Sjögren's syndrome (SjS) is a systemic autoimmune disease that mainly affects the exocrine glands, leading to generalized mucosal dryness. However, primary SjS may initially present with non-sicca (systemic) manifestations. When these features appear before the onset of an overt sicca syndrome, we may talk of an underlying 'occult' SjS. The European League Against Rheumatism (EULAR) has promoted a

GLP-1 inhibits and adrenaline stimulates glucagon release by differential modulation of N- and L-type Ca2+ channel-dependent exocytosis.

Glucagon secretion is inhibited by glucagon-like peptide-1 (GLP-1) and stimulated by adrenaline. These opposing effects on glucagon secretion are mimicked by low (1-10 nM) and high (10 muM) concentrations of forskolin, respectively. The expression of GLP-1 receptors in alpha cells is <0.2% of that in beta cells. The GLP-1-induced suppression of glucagon secretion is PKA dependent, is glucose indep

Stratospheric ozone depletion: High arctic tundra plant growth on Svalbard is not affected by enhanced UV-B after 7 years of UV-B supplementation in the field

The response of tundra plants to enhanced UV-B radiation simulating 15 and 30% ozone depletion was studied at two high arctic sites (Isdammen and Adventdalen, 78 degrees N, Svalbard).The set-up of the UV-B supplementation systems is described, consisting of large and small UV lamp arrays, installed in 1996 and 2002. After 7 years of exposure to enhanced UV-B radiation, plant cover, density, morpho