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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
Optically active C-2-symmetric (1S,2S,4S,5S)-bicyclo[2.2.2]octane-2,5-diol ((+)-12; 98% ee) and several selectivity protected optically active intermediates useful for synthetic transformations were synthesized via a 1,2-carbonyl transposition route starting from the easily available optically active (1R,4S,6S)-6-hydroxybicyclo[2.2.2]octan-2-one ((-)-2). The synthetic route also allowed the prepar
Background: Free and total human kallikrein 2 (hK2) might improve the discrimination between prostate cancer and benign prostatic hyperplasia. Concentrations of hK2 are 100-fold lower than concentrations of prostate-specific antigen (PSA); therefore, an hK2 assay must have a low detection limit and good specificity. Methods: PSA- and hK2-specific monoclonal antibodies were used in solid-phase, two
Decision-making in the European Union is subject to the risk of negotiation failure, because of governments' incentives to conceal their true preferences. This article argues that the EU Presidency possesses a set of informational and procedural resources that can help unlock incompatible negotiating positions and secure efficient agreements, while simultaneously allowing the government in office
We analyze the crystallization and collapse transition of a simple model for flexible polymer chains on simple-cubic and face-centered-cubic lattices by means of sophisticated chain-growth methods. In contrast to the bond-fluctuation polymer model in certain parameter ranges, where these two conformational transitions were found to merge in the thermodynamic limit, we conclude from our results tha
Previous studies indicate that 80-95% of grafted dopamine neurons die following implantation of embryonic ventral mesencephalic tissue into the striatum. It is believed that the majority die within the first 1-3 weeks after surgery. The aim of this study was to study when and where the implanted neurons die, using the novel fluorescent stain Fluoro-Jade. Fluoro-Jade has recently been shown to stai
Absorption spectra of a series of cyclic conjugated ketones and thioketones have been computed at the multiconfigurational second-order multistate perturbation level of theory, the CASSCF/MS-CASPT2 method. Excitation energies, transition dipole moments, oscillator strengths, and static dipole moments are reported and discussed for excited states with energies lower than approximate to7-8 eV. The m
This study investigated the effect of self-regulated learning, as indicated by academic self-concept, motivation and learning strategies, reading attitude and family based prerequisites on reading ability. Students (n=4018) in the eighth grade answered the IEA reading literacy test, the self-regulated learning questionnaire and a student questionnaire about their background. The exploratory factor
A condensed shale from the middle Silurian of Gotland (Sweden) is herein described with regard to its biotic and environmental significance. The fauna is characterised by an overall low diversity and anomalous abundance patterns, notably a mass-occurrence of scolecodonts. Other elements include abundant chitinozoans, dendroid and graptoloid graptolites, conodonts, eurypterids, and the brachiopod L
High-resolution (K3p sigma and pi)->sigma absorption spectrum of KF in the photon energy range of 18.2-19.4 eV was measured at the new FINEST beamline branch on the I3 beamline on MAX III. The experimental spectrum is presented and interpreted using nonrelativistic ab initio molecular calculations corrected with perturbation theory treatment to account for spin-orbit interaction. Resonant Auger de
Recombinant human cystatin C (cC), a cysteine protease inhibitor, contained methionine sulfoxide [Met(O)] residues when expressed in Escherichia coli under aerobic conditions or upon allowing osmotic shock solutions from anaerobically grown cultures to warm to room temperature. Oxidation occurred in the periplasmic space or intracellularly during aerobic expression. Both Met14 and Met41 were subje
Two conformationally different [1,2,7,8]tetrathiacyclododecino[4,3-b:5,6-b':10,9-b":11,12-b''']tetraind oles 9a and 9b have been isolated in good yields, and the existence of a third conformer 9c in solution was demonstrated by mass spectrometry and H-1 NMR spectroscopy. The interconversions of the tetraindoles 9a-c have also been studied. The conformation of 9b was confirmed by X-ray crystallogra
In a naturalistic newspaper reading study, two pairs of information graphics have been designed to study the effects of a) the spatial contiguity principle and b) the dual scripting principle by means of eye tracking measurements. Our data clearly show that different spatial layouts have a significant effect on readers’ eye movement behaviour. An integrated format with spatial contiguity between t
The hypothalamus is the main appetite-regulating center in the brain receiving peripheral signals regarding the metabolic status of the body. Pancreatic procolipase has recently been identified in rat hypothalamus. Procolipase is known mainly for its actions in the intestine where it is cleaved to colipase, an enzyme required for the maintenance of pancreatic lipase activity, and enterostatin, a p
Complex circuits involving both local intrinsic neurones (i.e. enteric nervous system; ENS) and extrinsic neurones achieve nervous control of digestive functions. The ENS is comprised of many functionally different types of neurons: sensory neurons, interneurons and secreto-motor neurons. Each neuronal population is required to manifest local reflex behavior and is central to the regulation of bot
We assess relative time courses of motor development in ferrets and rats and evaluate ferrets as experimental animals for studies of motor development. Motor behaviour of ferret pups was characterized in daily sessions from postnatal day (P) 2 to P63. Observations concerning rotational locomotion ('pivoting'), crawling, walking, upright standing ('rearing') and walking on a narrow path; righting o
