Filetype
Abstract in UndeterminedLimb-girdle muscular dystrophy type 2A is an autosomal recessive disorder generated by inactivating mutations in the gene coding for the muscle specific protease calpain-3. It is mainly expressed in skeletal muscle as a monomeric multidomain protein characterized by three unique insertion sequences (NS, IS1, IS2). It is unstable, and undergoes very rapid autolysis in soluti
Since about 25 years ago work has been dedicated to the development of hydrodynamical model atmospheres for cool stars (of A to T spectral type). Despite their obviously sounder physical foundation in comparison with standard hydrostatic models, their general application has been rather limited. In order to understand why this is, and how to progress, we review the present status of hydrodynamical
Five protected analogues of beta-D-galctosyl-(5R)-5-hydroxy-L-lysine were prepared, in which the galactosyl moiety was modified by monodeoxygenation or inversion of stereochemistry at C-4. The building blocks were used in the solid-phase synthesis of a set of glycopeptides related to the peptide fragment CII256-273 from type II collagen. Evaluation of the glycopeptides revealed that T-cell hybrido
Intramyocellular triacylglycerol (TG) is an important energy store, and the energy content of this depot is higher than the energy content of the muscle glycogen depot. It has recently been shown that the mobilization of fatty acids from this TG pool may be regulated by the neutral lipase hormone-sensitive lipase (HSL). This enzyme is known to be rate limiting for intracellular TG hydrolysis in ad
Extracorporeal photochemotherapy (ECP) has demonstrated immunological effects. The proposed cytotoxic lymphocyte antigen 4 (CTLA-4) involvement, together with forkhead box P3 (FoxP3) and transforming growth factor (TGF)-beta are associated with regulatory T cell activity. The aim of the study was to evaluate the regulatory T cell-associated effect of ECP in recent onset type 1 diabetic (T1D) child
The folding kinetics of bovine acyl-CoA binding protein was studied by N-15 relaxation dispersion measurements under equilibrium conditions. Relaxation dispersion profiles were measured at several concentrations of guanidine hydrochloride (GuHCl). The unfolding rate constant (k(u)) was determined under conditions favoring folding, for which the folding rate constant (k(f)) dominates the relaxation
To assess the manifestation and location of airway inflammation in smokers with chronic bronchitis (CB) or chronic obstructive pulmonary disease (COPD), we lavaged the airways of 12 smokers with CB and 11 smokers with COPD and coexisting CB (OCB). For comparison, the airways of 5 asymptomatic smokers (AS) and 10 healthy nonsmokers (HNS) were lavaged. In all cases, the first lavage aliquot, labeled
Moraxella catarrhalis ubiquitous surface proteins A1 and A2 (UspA1/A2) interfere with the classical pathway of the complement system by binding C4b-binding protein. In this study we demonstrate that M. catarrhalis UspA1 and A2 noncovalently and in a dose-dependent manner bind both the third component of complement (C3) from EDTA-treated serum and methylamine-treated C3. In contrast, related Moraxe
Type 1 diabetes mellitus (T1DM) is an autoimmune disease in which the 0 cells in the pancreas are destroyed by the body's own immune system. IL12 plays a role in pathological situations, such as septic shock, tissue damage during inflammation, and organ-specific autoimmune diseases. In NOD mice, administration of IL12 induces T1DM and administration of IL12 antagonists prevents T1DM. Linkage and a
Seventeen globular clusters in the Large and Small Magellanic Clouds were observed in the mid-infrared wavelength region with the ISOCAM instrument on board the Infrared Space Observatory (ISO). Observations were made using the broadband filters LW1, LW2, and LW10, corresponding to the effective wavelengths of 4.5, 6.7, and 12 mum, respectively. We present the photometry of point sources in each c
Reduced capacity to produce ROS increases the severity of T cell-dependent arthritis in both mice and rats with polymorphisms in neutrophil cytosolic factor 1 (Ncf1) (p47phox). Since T cells cannot exert oxidative burst, we hypothesized that T cell responsiveness is downregulated by ROS produced by APCs. Macrophages have the highest burst capacity among APCs, so to study the effect of macrophage R
Very heavy elements (Pt, Au, Hg, Tl, and Bi) are found to be enhanced inthe atmospheres of the chemically peculiar stars of the upper mainsequence by up to a million times the solar system levels. Suchenhancements are believed to result from atmospheric dynamics (i.e.,diffusion) rather than scenarios that dredge up nuclear-processedmaterial to the surface or transfer processed material between bin
Using optimal control as a spectroscopic tool we decipher the details of the molecular dynamics of the essential multidimensional excited-state photoisomerization - a fundamental chemical reaction of key importance in biology. Two distinct nuclear motions are identified in addition to the overall bond-twisting motion: Initially, the reaction is dominated by motion perpendicular to the torsion coor
The present work studies the separated flow in a square channel with ribs on one wall by using the PIV method. In this study, the spacing between ribs is kept constant and the heights of rib are varied in order to investigate the influence of the pitch-to-height ratio, P/e on the flow structures. Depending on the various pitch ratios, the enclosure between ribs can be regarded as closed or open ca
