Notes

MuSK not MNGIE: Atypical MuSK-antibody myasthenia presenting being a innate condition.
Self-sustaining smoldering combustion is a promising technology for treating sewage sludge from wastewater treatment plants because of its low energy inputs. Proper use or disposal of the resulting ashes requires thorough characterization of their properties, including the content of total organic carbon (TOC). Here, we develop a two-step method for quantifying TOC in the raw ashes from sewage sludge smoldering combustion. The first step is to thermally treat the raw ashes at 500 °C in N2 for 1 h, followed by ultimate analysis of both the raw ashes and the thermally treated ashes. This enables the quantification of carbon released during thermal treatment. The second step is nonisothermal thermogravimetric analysis (TGA) of the thermally treated ashes, in which the samples are heated to 900 °C at 10 °C/min in air. The weight losses at 310-500 °C are confirmed to be caused by organic carbon oxidation, allowing us to determine the contents of carbon retained in the thermally treated ashes. The sums of these two portions of carbon represent the contents of TOC in the raw ashes, which are ∼0.85-6.52 wt % (dry basis), depending on their locations and particle sizes. The two-step (thermal treatment-TGA) method is validated via a method that includes acid washing of the thermally treated ashes, ultimate analysis of the acid-washed ashes, and TOC analysis of the leachates. The two-step method is simple and accurate and, importantly, provides thorough information on the thermal behavior of the raw and thermally treated ashes.Nuciferine is an aporphine alkaloid monomer that is extracted from the leaves of the lotus species Nymphaea caerulea and Nelumbo nucifera Gaertn. Nuciferine was reported to treat cerebrovascular diseases. However, the potential mechanism of the neuroprotective effects of nuciferine at the metabolomics level is still not unclear. The present research used neurological score, infarct volume, cerebral water content, and ultraperformance liquid chromatography to quadrupole time-of-flight mass spectrometry (UPLC-Q-TOF/MS)-based serum metabolomics to elucidate the anti-ischemic stroke effect and mechanisms of nuciferine. The results showed that nuciferine significantly improved neurological deficit scores and ameliorated cerebral edema and infarction. Multivariate data analysis methods were used to examine the differences in serum endogenous metabolism between groups, and the biomarkers of nuciferine on ischemic stroke were identified. Approximately 19 metabolites and 7 metabolic pathways associated with nuciferine on treatment of stroke were found, which indicated that nuciferine exerted a positive therapeutic action on cerebral ischemic by regulating metabolism. These results provided some data support for the study of anti-stroke effect of nuciferine from the perspective of metabolomics.Background Human urate transporter 1 (hURAT1) is the most pivotal therapeutic target for treating hyperuricemia. However, the molecular interactions between uric acid and URAT1 are still unknown due to lack of structural details. Methods In the present study, several methods (homology modeling, sequence alignment, docking, and mutagenesis) were used to explain the atomistic mechanisms of uric acid transport of hURAT1. Results Residues W357-F365 in the TMD7 and P484-R487 in the TMD11 present in the hURAT1 have unique roles in both binding to the uric acid and causing subsequent structural changes. These residues, located in the transport tunnel, were found to be related to the structural changes, as demonstrated by the reduced Vmax values and an unaltered expression of protein level. In addition, W357, G361, T363, F365, and R487 residues may confer high affinity for binding to uric acid. An outward-open homology model of hURAT1 revealed a crucial role for these two domains in the conformational changes of hURAT1. F241 and H245 in TMD5, and R477 and R487 in TMD11 may confer high affinity for uric acid, and as the docking analysis suggests, they may also enhance the affinity for the inhibitors. R477 relation to the structural changes was demonstrated by the Vmax values of the mutants and the contribution of positive charge to the uric acid selectivity. Conclusions W357-F365 in TMD7, P484-R487 in TMD11, and residues F241, H245, and R477 were found to be critical for the translocation and recognition of uric acid.In this work, a three-stage and easily scalable synthesis of 2,6-dicyano-4-pyrone (overall yield of 45%) as a new convenient building block has been developed from diethyl acetonedioxalate. It was shown that the transformation with hydroxylamine and [3 + 2]-cycloaddition, in contrast to the reactions with hydrazines, selectively proceed through the attack at the cyano groups without the pyrone ring-opening to give symmetrical and unsymmetrical pyrone-bearing heterocyclic triads containing 1,2,4- and 1,3,4-oxadiazoles as well as tetrazole moieties. The reaction of 2,6-bis(hetaryl)-4-pyrones with ammonia afforded 2,6-bis(hetaryl)pyridines in 63-87% yields. The 4-pyridone/4-pyridinol tautomerism of 2,6-bis(hetaryl)pyridinols and the influence of the nature of adjacent azolyl moieties on this equilibrium have been discussed.The reservoir heterogeneity is the major cause of poor volumetric sweep efficiency in sandstone and carbonate reservoirs. Displacing fluids (water, chemical solution, gas, and supercritical CO2 (sc-CO2)) flow toward the high permeable zone. A significant fraction of oil remains in the low permeable zone due to the permeability contrast. STA-9090 purchase This study used in situ sc-CO2 emulsion as a conformance control agent to plug the high permeable zone and improve the low permeable zone's volumetric sweep efficiency in carbonate formation. We investigated the effect of two types of conformance control patterns and the size of sc-CO2 emulsion on tertiary oil recovery performance by sc-CO2 miscible injection for carbonate reservoirs at reservoir conditions. The conformance control patterns are achieved using two different approaches. In the first approach, the low permeable zone was isolated, and the diverting gel system, a 0.4 pore volume slug, was injected into a high permeable zone. In the second approach, the simultaneous injection of the diverting gel system, a 0.
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