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Congenital muscular dystrophy, caused by mutations in LAMA2 (the gene encoding laminin α2 chain), is a severe and incapacitating disease for which no therapy is yet available. We have recently demonstrated that proteasome activity is increased in laminin α2 chain-deficient muscle and that treatment with the nonpharmaceutical proteasome inhibitor MG-132 reduces muscle pathology in laminin α2 chain-
We show that the valence electrons of Ba3NaRu2O9, which has a quasimolecular structure, completely crystallize below 210 K. Using an extended Hubbard model, we show that the charge ordering instability results from long-range Coulomb interactions. However, orbital ordering, metal-metal bonding, and formation of a partial spin gap enforce the magnitude of the charge separation. The striped charge o
Application of polycrystalline cubic boron nitride (PCBN) tools as an alternative for ceramic and cemented carbide tools in machining superalloys has been frequently identified as a solution for enhancing process efficiency but only a limited number of studies has been done in this area. The current study explores the effect of the cutting speed, which was varied in a wide range (2-14m/s), on mach
The aim of the experimental study presented herein is the assessment and quantification of the behavior of individual dowels in multi-dowel connections loaded by a bending moment. For this purpose, double-shear, steel-to-timber connections with nine steel dowels arranged in different patterns and with different dowel diameters were tested in four-point bending. In order to achieve a ductile behavi
Tubules formed by self-assembly of organic molecules have a vast potential in nanotechnology applications and the introduction of sensitivity to stimuli into self-assembly tubules represents a particularly attractive feature. Here we report on the preparation and characterization of a molecule obtained by chemical modification of a natural bile acid, a biological surfactant, that self-assembles in
Antibacterial peptides of the innate immune system combat pathogenic microbes, but often have additional roles in promoting inflammation and as growth factors during tissue repair. Midkine (MK) and pleiotrophin (PTN) are the only two members of a family of heparin-binding growth factors. They show restricted expression during embryogenesis and are upregulated in neoplasia. In addition, MK shows co
A study on (137)Cs, (40)K, (226)Ra, (228)Ra, and (238)U was carried out along the EEZ of Qatar. Results serve as the first ever baseline data. The level of (137)Cs (mean value 1.6±0.4Bqm(-3)) in water filters was found to be in the same order of magnitude as reported by others in worldwide marine radioactivity studies. Results are also in agreement with values reported from other Gulf regions. The
Children with early symptomatic psychiatric disorders such as Attention-Deficit/Hyperactivity Disorder (ADHD) and Autism Spectrum Disorder (ASD) have been found to have high rates of motor and/or perception difficulties. However, there have been few large-scale studies reporting on the association between Conduct Disorder (CD) and motor/perception functions. The aim of the present study was to inv
Objective: To quantify the independent associations between objectively measured physical activity (PA), cardiorespiratory fitness (CRF), and anthropometry in European men and women. Methods: 2,056 volunteers from 12 centers across Europe were fitted with a heart rate and movement sensor at 2 visits 4 months apart for a total of 8 days. CRF (ml/kg/min) was estimated from an 8 minute ramped step te
In this study, we investigate which factors affect the false positive fraction (FPF) for digital breast tomosynthesis (DBT) compared to digital mammography (DM) in a screening population by using classification and regression trees (C&RT) and binary marginal generalized linear models. The data was obtained from the Malmö Breast Tomosynthesis Screening Trial, which aimed to compare the performa
The growth and ordering of {5,10,15,20-tetrakis(4-bromophenyl)porphyrinato}nickel(II) (NiTBrPP) molecules on the Au(111) surface have been investigated using scanning tunnelling microscopy, X-ray absorption, core-level photoemission, and microbeam low-energy electron diffraction. When deposited onto the substrate at room temperature, the NiTBrPP forms a well-ordered close-packed molecular layer in
In this paper we evaluate the Swedish Programme for improving energy efficiency in energy-intensive industries (PFE). Since 2005, some 100 energy-intensive companies have entered this five year voluntary agreement (VA) and been exempted from the EU minimum tax on electricity. In return, each company is required to: conduct an energy audit and analysis; identify and invest in profitable electricity
C-reactive protein (CRP) concentration is a heritable systemic marker of inflammation that is associated with cardiovascular disease risk. Genome-wide association studies have identified CRP-associated common variants associated in ∼25 genes. Our aims were to apply exome sequencing to (1) assess whether the candidate loci contain rare coding variants associated with CRP levels and (2) perform an e
The prediction of the influent load is of great importance for the improvement of the control system to a large wastewater treatment plant. A systematic data analysis method is presented in this paper in order to estimate and predict the periodicity of the influent flow rate and ammonia (NH3) concentrations: 1) data filtering using wavelet decomposition and reconstruction; 2) typical cycle identif
Respiratory motion during dynamic radiotherapy may affect the absorbed dose distribution both by dose-reducing smoothing and by more complicated interplay effects. In this study we present a novel method to determine the relative importance of these two effects. For the two dynamic deliveries studied in this work, the expected target dose reduction due to the smoothing effect was estimated by meas
Intraventricular hemorrhage (IVH) with posthemorrhagic ventricular dilatation (PHVD) is a common cause of hydrocephalus in infants. Dysregulation of cerebrospinal fluid (CSF) production by the choroid plexus may contribute to the development of PHVD. The aquaporins (AQPs), transmural water transporting proteins, are believed to contribute to CSF production. The aim of the study was to characterize
