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The history of von Willebrand's disease (VWD) is fascinating because it demonstrates how good clinical observations, genetic studies and biochemical skills can improve basic understanding of a disease and its management. The continuous efforts of scientists and clinicians during the last 80 years have significantly improved the knowledge of von Willebrand factor (VWF) structure and function and th
In this paper, we present the expression and characterization of two novel enzymes from the a-amylase family exhibiting cyclomaltodextrinase specificity. The nucleotide sequences encoding the enzymes were isolated from the genomic DNA of two thermophilic bacterial strains originating from Icelandic hot springs and belonging to the genera Anoxybacillus (AfCda13) and Laceyella (LsCda13). The genes w
The formation of amylose-lipid complexes of form I (amorphous structure) and form II (crystalline structure) during heating was studied by differential scanning calorimetry (DSC) for a range of monoglycerides and for monoglyceride mixtures. The temperature treatment applied to amylose-monoglyceride-mixtures were either the first scan in DSC (10 °C/min, 15-144 °C) or a prolonged heat treatment wher
The aim of this study was to answer the question whether or not, after an operation for duodenal atresia, a transanastomotic feeding tube reduces the time to full preanastomotic feeding. The method used was a retrospective study and a prospective observation. 18 consecutive newborns with duodenal atresia, nine from each of two different centres of paediatric surgery, were studied retrospectively.
In the microelectronics industry, gold electrodes on silicon dioxide are produced using an adhesive layer of chromium or titanium. This method is applicable whenever a dry environment is present. However, when such an electrode is exposed to an electrolyte a galvanic cell is formed and the less noble metal will dissolve and eventually destroy the electrode. We describe here how a monolayer of merc
We reexamine spin-dependent transport properties of two-dimensional electrons modulated by the stray field of a ferromagnetic metal (FM) stripe on top. When the magnetization is along the transport direction of the electrons, the FM stripe generates not only a magnetic barrier but also an in-plane magnetic field component. Previous studies have omitted the in-plane magnetic field component and sho
Background., Hypertrophic cardiomyopathy is a common cause of sudden death in children. In this study, we aimed to identify clinical measures for stratification of this risk in childhood. Patients and methods: By means of a retrospective cohort study from six regional centres of paediatric cardiology, we identified 128 patients with hypertrophic cardiomyopathy presenting below 19 years of age, wit
Different lifetime broadenings in molecular-field-split 2p core levels in H2S are predicted theoretically and are identified in an experimental investigation of the S 2p Auger electron spectrum. The measurements were performed for the transition to the vibrationally resolved X(1)A(1) ground state of H2S2+. The lifetimes of the 3e(1/2) and 5e(1/2) levels of the 2p ionized molecule are found to be 6
The binding of Helicobacter pylori to glycosphingolipids was examined by binding of 35S-labeled bacteria to glycosphingolipids on thin-layer chromatograms. In addition to previously reported binding specificities, a selective binding to a non-acid tetraglycosylceramide of human meconium was found. This H. pylori binding glycosphingolipid was isolated and, on the basis of mass spectrometry, proton
Objective: The collagen VI muscular dystrophies, Bethlem myopathy and Ullrich congenital muscular dystrophy, form a continuum of clinical phenotypes. Glycine mutations in the triple helix have been identified in both Bethlem and Ullrich congenital muscular dystrophy, but it is not known why they cause these different phenotypes. Methods: We studied eight new patients who presented with a spectrum
Background: The frequencies with which physicians make different medical end-of-life decisions (ELDs) may differ between countries, but comparison between countries has been difficult owing to the use of dissimilar research methods. Methods: A written questionnaire was sent to a random sample of physicians from 9 specialties in 6 European countries and Australia to investigate possible differences
Molecularly imprinted microspheres were prepared as antibody binding mimics and used in scintillation proximity assay of a beta-adrenergic antagonist, (S)-propranolol. By using small polymer beads, we were able to place an organic scintillator and an "antenna" component in close proximity to the imprinted binding sites. When the radioactive template bound to the polymer, radiation energy was effec
Astrocytes are, as normal constituents of the brain, promising vehicles for ex vivo gene delivery to the central nervous system. In the present study, we have used a lentiviral vector encoding glial cell line-derived neurotrophic factor (GDNF) to transduce rat-derived primary astrocytes, in order to evaluate their potential for long-term transgene expression in vivo and neuroprotection in a rat mo
Culture of intact arteries is a frequently employed experimental model for investigating the mechanisms governing the regulation of vascular endothelin receptors. Endothelin type A (ETA) and type B (ETB) receptors on vascular smooth muscle cells are up-regulated in organ culture and the enhanced vasoconstriction mimics the changes that occur in cardiovascular disease. The effect of organ culture o
Regional levels of brain-derived neurotrophic factor protein were measured in the rat brain using enzyme immunoassay following seizures evoked by hippocampal kindling stimulations. One stimulation, which induced a brief, single episode of epileptiform activity in hippocampus and piriform cortex but not in parietal cortex or striatum, gave rise to a transient increase of brain-derived neurotrophic
The aim of the study was to quantify the workload on the upper extremity for fundamental work tasks during machine milking. Eleven milkers working in a loose-housing system with a milking parlour participated in the study. Muscle activity for the biceps and the forearm flexors, as well as positions and movements of the wrists were simultaneously measured by electromyography and electrogoniometry w
The macroscopic appearance and microstructure in the dilute corner of the ternary monoolein (MO)-sodium oleate (NaO)-water ((H2O)-H-2) system have been investigated by visual inspection and by using direct structural imaging with light microscopy and cryogenic transmission electron microscopy. The microstructural transformations that take place between the micellar phase (binary NaO-(H2O)-H-2 axis
