GRACILE syndrome belongs to the Finnish disease heritage, and is caused by a point mutation in the BCS1L-gene encoding a mitochondrial protein. This leads to dysfunction of the complex III in the respiratory chain. Significant fetal growth disturbance is the primary manifestation. Within the first day the newborn infant develops severe lactic acidosis. Hypoglycemia, elevated serum ferritin and con

A search for supersymmetric partners of top quarks decaying as t˜1→cχ˜10 and supersymmetric partners of charm quarks decaying as c˜1→cχ˜10, where χ˜10 is the lightest neutralino, is presented. The search uses 36.1 fb−1pp collision data at a centre-of-mass energy of 13 TeV collected by the ATLAS experiment at the Large Hadron Collider and is performed in final states with jets identified as contain

This paper introduces the notion of surprising institutions. We often carry incorrect beliefs about the world surrounding us and we are often mistaken about the nature of the institutional structure. The case story in this paper shows that an institution may come as a surprise, even though its origins lies some 500 years back, and that the information we receive as feedback on our actions does not

In this study, the mechanical improvement of the platinum coated nanoporous gold is investigated by performing molecular dynamics simulations. The tensile properties of the structure is enhanced by a stress relief annealing which prevents the high stress concentration observed between the coating and the core materials. The annealing process is performed at 500K for 100ps with a heating and coolinIn this study, the mechanical improvement of the platinum coated nanoporous gold is investigated by performing molecular dynamics simulations. The tensile properties of the structure is enhanced by a stress relief annealing which prevents the high stress concentration observed between the coating and the core materials. The annealing process is performed at 500K for 100ps with a heating and coolin

The effects of mechanical load on optical properties of Y2SiO5 / Eu:Y2SiO5 crystals are investigated through application of first principles calculations. The crystals are first relaxed to reach equilibrium ground state using plane wave density functional theory (DFT). Once application of load on desired direction is performed the optical absorption profile of aforementioned crystals are plotted wThe effects of mechanical load on optical properties of Y2SiO5 / Eu:Y2SiO5 crystals are investigated through application of first principles calculations. The crystals are first relaxed to reach equilibrium ground state using plane wave density functional theory (DFT). Once application of load on desired direction is performed the optical absorption profile of aforementioned crystals are plotted w

Background: Even before the onset of age-related diseases, obesity might be a contributing factor to the cumulative burden of oxidative stress and chronic inflammation throughout the life course. Obesity may therefore contribute to accelerated shortening of telomeres. Consequently, obese persons are more likely to have shorter telomeres, but the association between body mass index (BMI) and leukoc

The elliptic (v2), triangular (v3), and quadrangular (v4) flow coefficients of π±, K±, p+p¯,Λ+Λ¯,KS0, and the ϕ-meson are measured in Pb-Pb collisions at sNN=5.02 TeV. Results obtained with the scalar product method are reported for the rapidity range |y| < 0.5 as a function of transverse momentum, pT, at different collision centrality intervals between 0–70%, including ultra-central (0–1%) collis

The first measurement of e+e− pair production at mid-rapidity (|ηe| < 0.8) in pp collisions at s=7 TeV with ALICE at the LHC is presented. The dielectron production is studied as a function of the invariant mass (mee < 3.3 GeV/c2), the pair transverse momentum (pT,ee < 8 GeV/c), and the pair transverse impact parameter (DCAee), i.e., the average distance of closest approach of the reconstructed el

Despite extensive studies, defining culture conditions in which hematopoietic stem cells can be expanded ex vivo has been challenging. Here we show that chemical inhibition of the NF- κB signaling pathway leads to a significant improvement of hematopoietic stem cell function from ex vivo cultured human umbilical cord blood derived CD34+ cells. We found a distinct peak of activation of the NF-κB pa

This paper addresses coherence at the levels of policy objectives and instruments. The existing literature usually links policy coherence to strong hierarchical coordination mechanisms. This paper builds on two cases of, respectively, hierarchical imposition and deliberation within the European Commission (the step‐up to a 30% greenhouse gas reduction target on the one hand, and the energy efficie

Intestinal degradation of dietary fibre in blanched and microwaved green beans was studied by using a rat experimental model. Content and composition of dietary fibre as well as molecular weight distribution of water-soluble polysaccharides (WSP) were analysed. There was a solubilization and a shift towards lower Mw of mainly uronic acid-containing polysaccharides with repeated microwave treatment

The effects of vegetables in mixed meals on satiety were evaluated by varying the dose (portions 150 and 250 g containing 4.3 and 7.2 g of dietary fibre, respectively) and structure (cut and minced) of microwaved spinach. A control meal without spinach and the test meals with spinach were balanced regarding energy (2000 kJ) and digestible carbohydrates (59 g, 51 E%) and with protein and fat as far

The influence of processing and cooking on the metabolic response to carrots in mixed meals was explored in two consecutive harvest years. The contribution of dietary fibre (4.4 g 1989 and 6.6 g 1990) from carrots was chosen to be different in order to compare effects with varying doses. The meals, composed of carrots, creamed potatoes, meat balls, lingonberry jam, white bread and light beer, were

Different vegetables--carrots, peas, Brussels sprouts and spinach--were added to a typical Swedish lunch test meal in portions of 96-164 g. The test and the control meals without vegetables were balanced with respect to digestible carbohydrates (59.7 g) and energy (2000 kJ, 475 kcal), and as far as possible also concerning protein and fat. The added vegetables contained 4.4 g dietary fibre. The me

The fermentative breakdown and fecal bulking capacity of dietary fiber from various vegetables (carrots, rutabagas, peas, green beans and Brussels sprouts) were evaluated in balance experiments in rats. The excretion of bile acids, protein and fat was also studied. The addition of blanched vegetables providing 10 g of fiber/100 g of diet caused a two-(rutabagas) to threefold (carrots) increase in