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The congenital disorders of glycosylation: a multifaceted group of syndromes.

The congenital disorders of glycosylation (CDG) are a rapidly expanding group of metabolic syndromes with a wide symptomatology and severity. They all stem from deficient N-glycosylation of proteins. To date the group contains 18 different subtypes: 12 of Type I (disrupted synthesis of the lipid-linked oligosaccharide precursor) and 6 of Type II (malfunctioning trimming/processing of the protein-b

Combustion products generated by hetero-organic fuels on four different fire test scales

The scaling of results from combustion experiments with four organic substances containing heteroatoms, carried out on four different scales, is discussed. The scales ranged from the micro-scale, using about 2 x 10(-3) kg of the substances, to large scale using about 50 kg of the substances. The content of O-2, CO, CO2, NOx, HCl, HCN and SO2 in the combustion gases were determined with special att

Accurate dye tracer concentration estimations using image analysis

In this paper, the accuracy of dye tracer concentration estimations using image analysis is examined. The variability before and after application of different image correction methods was investigated in three experiments using a digital camera. In each of these experiments, one correction was applied and the remaining variability after correction was calculated as the SD of uniformly colored pat

Polybrominated diphenyl ethers (PBDES) at a solid waste incineration plant I: Atmospheric concentrations

In the first of two papers, the atmospheric poly brominated diphenyl ether (PBDE) concentrations at a municipal solid waste incineration (MSW) plant with electronic recycling is compared with that at an industrial urban reference site producing asphalt and concrete. In the second paper, atmospheric deposition and washout behaviour is presented (Atmos. Environ. (2004a)). PBDEs (BDE28, -47, -66, -10

Interaction of metals and thiols in cell damage and glutathione distribution: potentiation of mercury toxicity by dithiothreitol

In the present study, we have investigated the effects of extracellular redox status and metal/thiol interactions on glutathione distribution in HeLa cell cultures. No effects were seen on glutathione distribution after the addition of different thiols, whereas the pro-oxidant copper ions affected glutathione distribution in several ways. The addition of dithiothreitol (DTT) but not the other thio

In vitro molecular evolution of antibody genes mimicking receptor revision

Antibody evolution in vivo proceeds mainly by stepwise improvements, accomplished by single base pair substitutions. Lately, receptor revision, i.e. exchange of large parts of the V gene for another sequence, has been suggested to provide a complementary route for affinity maturation. By employing a receptor revision like evolution process in vitro using combinatorial libraries and phage display s

Phenotypic modulation of cultured bladder smooth muscle cells and the expression of inducible nitric oxide synthase.

Phenotypic modulation of smooth muscle is associated with various pathological conditions, including bladder dysfunction. Cytoskeletal dynamics modulate the cell phenotype and were recently shown to be involved in regulation of inducible nitric oxide synthase ( iNOS). We tested the hypothesis that the cell differentiation status affects iNOS expression, and that iNOS is preferentially expressed in

Visibility of current and shot noise in electrical Mach-Zehnder and Hanbury Brown Twiss interferometers

We investigate the visibility of the current and shot-noise correlations of electrical analogs of the optical Mach-Zehnder interferometer and the Hanbury Brown Twiss interferometer. The electrical analogs are discussed in conductors subject to high magnetic fields where electron motion is along edge states. The transport quantities are modulated with the help of an Aharonov-Bohm flux. We discuss t

Luminescence control in the marine bacterium Vibrio fischeri: An analysis of the dynamics of lux regulation

A mathematical model has been developed based on the fundamental properties of the control system formed by the lux genes and their products in Vibrio fischeri. The model Clearly demonstrates how the components of this system work together to create two, stable metabolic states corresponding to the expression of the luminescent and non-luminescent phenotypes. It is demonstrated how the cell can "s

HYDRO - A PROGRAM FOR PROTEIN HYDROPATHY PREDICTIONS

VAX/VMS programs for predicting protein hydropathy from amino acid sequence with several parameters are described. The hydropathy propensities of amino acids were normalised to make the different scales and predictions comparable. Another program package can be used to study similarity on the secondary structural level by comparing, for example, hydropathy predictions. These methods can provide va

Monte Carlo simulation of defect-free cross-linked polyelectrolyte gels

Model systems of cross-linked polyelectrolyte gels were investigated by means of Monte Carlo simulations. The model contained a charged defect-free th ee-dimensional network of a diamond-like topology and explicit counterions. Pressure vs volume relations and chain extensions were determined and compared to those of the corresponding polyelectrolyte solution. The structure of the gel was character

Binding of SDS to Ethyl(hydroxyethyl)cellulose. Effect of Hydrophobic Modification of the Polymer

The binding of SDS to cellulose polymers in the semidilute concentration regime has been studied by means of NMR, ion-selective electrode, and a time-resolved fluorescence technique. Two polymers have been used, differing only in a low degree of hydrophobic modification of one of them. NMR self-diffusion and activity measurements show that the binding of SDS to the nonmodified polymer has a fairly