The ristocetin cofactor activity assay (VWF:RCo) is the reference method for assessing von Willebrand factor (VWF) activity but remains difficult to perform, and the coefficient of variation of the method is high (about 20-30%). This study evaluated and compared the performance for measuring the VWF activity of two newly commercialised assays [VWF:Ac Innovance (VWF:Ac) and VWF:RCo Acustar (VWF:RCo Acu)] with the reference VWF:RCo aggregation in 123 pathological plasma samples. The correlation and concordance between both new tests (VWF:RCo-Acu and VWF:Ac) and the reference VWF:RCo were good. The results of the VWF activity to VWF antigen ratio were also comparable whatever the method for the classification of VWF deficiency in all patients. Our results showed that both new tests could replace the "gold standard" VWF:RCo in aggregometry with several benefits: they are fully automated, easier and faster to perform, better adapted to emergency situations if necessary.

The infrared absorption spectrum of amorphous Mg(B 3 H 8) 2 (THF) 2 was measured in the mid-infrared region between 600 and 3300 cm-1. In the frequency range corresponding to B-H stretching vibrations, the observation of absorptions due to the movements of Mg-H-B bridging hydrides provides evidence of direct bond between octahydrotriborate ions and magnesium. Starting from this configuration, DFT and ab-initio calculations at the MP2, B3LYP and B97D level were utilized in order to find the lowest energy geometry of the Mg(B 3 H 8) 2 (THF) 2 complex. The results of all the computational studies indicate that the interaction of Mg with the [B 3 H 8 ]ions entails two B-Mg and two B-H-Mg bonds. The calculated infrared absorptions closely reproduce the experimental spectrum. Moreover, calculations provide evidence of the stabilization of the THF solvate of Mg(B 3 H 8) 2 and indicate that the THF complex has a greater stabilization energy than dialkyl ethers complexes by ~25 kJ/mol.

A series of density functional theory calculations were performed to understand the bonding and interaction of hydrogen adsorption on two-dimensional silicon carbide obtained from molecular dynamics simulation. The converged energy results pointed out that the H atom can sufficiently bond to 2D SiC at the top sites (atop Si and C), of which the most stable adsorption site is TSi. The vibrational properties along with the zero-point energy were incorporated into the energy calculations to further understand the phonon effect of the adsorbed H. Most of the 2D SiC structure deformations caused by the H atoms were found at the adsorbent atom along the vertical axis. For the first time, five SiC defect formations, including the quadrilateral-octagon linear defect (8-4), the silicon interstitial defect, the divacancy (4-10-4) defect, the divacancy (8-4-4-8) defect, and the divacancy (4-8-8-4) defect, were investigated and compared with previous 2D defect studies. The linear defect (8-4) has the lowest formation energy and is most likely to be formed for SiC materials. Furthermore, hydrogen atoms adsorb more readily on the defect surface than on the pristine SiC surface.

Background Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive paralysis due to motor neuron degeneration. It has been proposed that epigenetic modification and transcriptional dysregulation may contribute to motor neuron death. In this study, we investigate the basis for therapeutic approaches to target lysine-specific histone demethylase 1 (LSD1) and elucidate the mechanistic role of LSD1-histone H3K4 signaling pathway in ALS pathogenesis. Methods In order to examine the role of spermidine (SD), we administered SD to an animal model of ALS (G93A) and performed neuropathological analysis, body weight, and survival evaluation. Results Herein, we found that LSD1 activity is increased while levels of H3K4me2, a substrate of LSD1, is decreased in cellular and animal models of ALS. SD administration modulated the LSD1 activity and restored H3K4me2 levels in ChAT-positive motor neurons in the lumbar spinal cord of ALS mice. SD prevented cellular damage by improving the number and size of motor neurons in ALS mice. SD administration also reduced GFAP-positive astrogliogenesis in the white and gray matter of the lumbar spinal cord, improving the neuropathology of ALS mice. Moreover, SD administration improved the rotarod performance and gait analysis of ALS mice. Finally, SD administration delayed disease onset and prolonged the lifespan of ALS (G93A) transgenic mice. Conclusion Together, modulating epigenetic targets such as LSD1 by small compounds may be a useful therapeutic strategy for treating ALS.