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41.
Jun Ogata Kentaro Hirao Kenya Nishioka Arisa Hayashida Yuanzhe Li Hiroyo Yoshino Soichiro Shimizu Nobutaka Hattori Yuzuru Imai 《International journal of molecular sciences》2021,22(7)
Leucine-rich repeat kinase 2 (LRRK2) is a major causative gene of late-onset familial Parkinson’s disease (PD). The suppression of kinase activity is believed to confer neuroprotection, as most pathogenic variants of LRRK2 associated with PD exhibit increased kinase activity. We herein report a novel LRRK2 variant—p.G2294R—located in the WD40 domain, detected through targeted gene-panel screening in a patient with familial PD. The proband showed late-onset Parkinsonism with dysautonomia and a good response to levodopa, without cognitive decline or psychosis. Cultured cell experiments revealed that p.G2294R is highly destabilized at the protein level. The LRRK2 p.G2294R protein expression was upregulated in the patient’s peripheral blood lymphocytes. However, macrophages differentiated from the same peripheral blood showed decreased LRRK2 protein levels. Moreover, our experiment indicated reduced phagocytic activity in the pathogenic yeasts and α-synuclein fibrils. This PD case presents an example wherein the decrease in LRRK2 activity did not act in a neuroprotective manner. Further investigations are needed in order to elucidate the relationship between LRRK2 expression in the central nervous system and the pathogenesis caused by altered LRRK2 activity. 相似文献
42.
Liangtao Yang Liang-Yin Kuo Juan Miguel López del Amo Prasant Kumar Nayak Katherine A. Mazzio Sebastian Maletti Daria Mikhailova Lars Giebeler Payam Kaghazchi Teófilo Rojo Philipp Adelhelm 《Advanced functional materials》2021,31(38):2102939
A known strategy for improving the properties of layered oxide electrodes in sodium-ion batteries is the partial substitution of transition metals by Li. Herein, the role of Li as a defect and its impact on sodium storage in P2-Na0.67Mn0.6Ni0.2Li0.2O2 is discussed. In tandem with electrochemical studies, the electronic and atomic structure are studied using solid-state NMR, operando XRD, and density functional theory (DFT). For the as-synthesized material, Li is located in comparable amounts within the sodium and the transition metal oxide (TMO) layers. Desodiation leads to a redistribution of Li ions within the crystal lattice. During charging, Li ions from the Na layer first migrate to the TMO layer before reversing their course at low Na contents. There is little change in the lattice parameters during charging/discharging, indicating stabilization of the P2 structure. This leads to a solid-solution type storage mechanism (sloping voltage profile) and hence excellent cycle life with a capacity of 110 mAh g-1 after 100 cycles. In contrast, the Li-free compositions Na0.67Mn0.6Ni0.4O2 and Na0.67Mn0.8Ni0.2O2 show phase transitions and a stair-case voltage profile. The capacity is found to originate from mainly Ni3+/Ni4+ and O2-/O2-δ redox processes by DFT, although a small contribution from Mn4+/Mn5+ to the capacity cannot be excluded. 相似文献
43.
《International Journal of Hydrogen Energy》2022,47(43):18663-18674
Developing non-precious metal-based catalysts as the substitution of precious catalysts (Pt/C) in oxygen reduction reaction (ORR) is crucial for energy devices. Herein, a template and organic solvent-free method was adopted to synthesize Fe, B, and N doped nanoflake-like carbon materials (Fe/B/N–C) by pyrolysis of monoclinic ZIF-8 coated with iron precursors and boric acid. Benefiting from introducing B into Fe–N–C, the regulated electron cloud density of Fe-Nx sites enhance the charge transfer and promotes the ORR process. The as-synthesized Fe/B/N–C electrocatalyst shows excellent ORR activity of a half-wave potential (0.90 V vs 0.87 V of Pt/C), together with superior long-term stability (95.5% current density retention after 27 h) in alkaline media and is even comparable to the commercial Pt/C catalyst (with a half-wave potential of 0.74 V vs 0.82 V of Pt/C) in an acidic electrolyte. A Zn-air battery assembled with Fe/B/N–C as ORR catalyst delivers a higher open-circuit potential (1.47 V), specific capacity (759.9 mA h g?1Zn at 10 mA cm?2), peak power density (62 mW cm?2), as well as excellent durability (5 mA cm?2 for more than 160 h) compared to those with commercial Pt/C. This work provides an effective strategy to construct B doped Fe–N–C materials as nonprecious ORR catalyst. Theoretical calculations indicate that introduction of B could induce Fe-Nx species electronic configuration and is favorable for activation of OH1 intermediates to promote ORR process. 相似文献
44.
《International Journal of Hydrogen Energy》2022,47(51):21613-21633
When planning large-scale 100% renewable energy systems (RES) for the year 2050, the system capacity is usually oversized for better supply-demand matching of electrical energy since solar and wind resources are highly intermittent. This causes excessive excess energy that is typically dissipated, curtailed, or sold directly. The public literature shows a lack of studies on the feasibility of using this excess for country-scale co-generation. This study presents the first investigation of utilizing this excess to generate green hydrogen gas. The concept is demonstrated for Jordan using three solar photovoltaic (PV), wind, and hybrid PV-wind RESs, all equipped with Lithium-Ion battery energy storage systems (ESSs), for hydrogen production using a polymer electrolyte membrane (PEM) system. The results show that the PV-based system has the highest demand-supply fraction (>99%). However, the wind-based system is more favorable economically, with installed RES, ESS, and PEM capacities of only 23.88 GW, 2542 GWh, and 20.66 GW. It also shows the highest hydrogen annual production rate (172.1 × 103 tons) and the lowest hydrogen cost (1.082 USD/kg). The three systems were a better option than selling excess energy directly, where they ensure annual incomes up to 2.68 billion USD while having payback periods of as low as 1.78 years. Furthermore, the hydrogen cost does not exceed 2.03 USD/kg, which is significantly lower than the expected cost of hydrogen (3 USD/kg) produced using energy from fossil fuel-based systems in 2050. 相似文献
45.
46.
Xiao-Lei Qiu Zi-Rui Fan Yang-Yang Liu Ding-Fu Wang Shi-Xin Wang Chun-Xia Li 《International journal of molecular sciences》2021,22(8)
A self-nanoemulsifying drug delivery system (SNEDDS) was developed to enhance the absorption of heparin after oral administration, in which heparin was compounded with phospholipids to achieve better fat solubility in the form of heparin-phospholipid (HEP-Pc) complex. HEP-Pc complex was prepared using the solvent evaporation method, which increased the solubility of heparin in n-octanol. The successful preparation of HEP-Pc complex was confirmed by differential scanning calorimetry (DSC), Fourier-transform infrared (FT-IR) spectroscopy, NMR, and SEM. A heparin lipid microemulsion (HEP-LM) was prepared by high-pressure homogenization and characterized. HEP-LM can enhance the absorption of heparin after oral administration, significantly prolong activated partial thromboplastin time (APTT) and thrombin time (TT) in mice, and reduce fibrinogen (FIB) content. All these outcomes indicate that HEP-LM has great potential as an oral heparin formulation. 相似文献
47.
《International Journal of Hydrogen Energy》2022,47(36):16165-16178
This study deals with the manufacturing of catalyst-coated membranes (CCMs) for newcomers in the field of coating. Although there are many studies on electrode ink composition for improving the performance of proton-exchange membrane fuel cells (PEMFCs), there are few papers dealing with electrode coating itself. Usually, it is a know-how that often remains secret and constitutes the added value of scientific teams or the business of industrialists. In this paper, we identify and clarify the role of key parameters to improve coating quality and also to correlate coating quality with fuel cell performance via polarization curves and electrochemical active surface area measurements. We found that the coating configurations can affect the performance of lab-made CCMs in PEMFCs. After the repeatability of the performance obtained by our coating method has been proved, we show that: (i) edge effects, due to mask shadowing - cannot be neglected when the active surface area is low, (ii) a heterogeneous thickness electrode produces performance lower than a homogeneous thickness electrode, and (iii) the origin and storage of platinum on carbon powders are a very important source of variability in the obtained results. 相似文献
48.
Young Been Kim Sung Hyeon Jung Dong Su Kim Nishad G. Deshpande Hee Won Suh Hak Hyeon Lee Ji Hoon Choi Ho Seong Lee Hyung Koun Cho 《Advanced functional materials》2021,31(38):2102439
Antimony triselenide (Sb2Se3) nanoflake-based nitrogen dioxide (NO2) sensors exhibit a progressive bifunctional gas-sensing performance, with a rapid alarm for hazardous highly concentrated gases, and an advanced memory-type function for low-concentration (<1 ppm) monitoring repeated under potentially fatal exposure. Rectangular and cuboid shaped Sb2Se3 nanoflakes, comprising van der Waals planes with large surface areas and covalent bond planes with small areas, can rapidly detect a wide range of NO2 gas concentrations from 0.1 to 100 ppm. These Sb2Se3 nanoflakes are found to be suitable for physisorption-based gas sensing owing to their anisotropic quasi-2D crystal structure with extremely enlarged van der Waals planes, where they are humidity-insensitive and consequently exhibit an extremely stable baseline current. The Sb2Se3 nanoflake sensor exhibits a room-temperature/low-voltage operation, which is noticeable owing to its low energy consumption and rapid response even under a NO2 gas flow of only 1 ppm. As a result, the Sb2Se3 nanoflake sensor is suitable for the development of a rapid alarm system. Furthermore, the persistent gas-sensing conductivity of the sensor with a slow decaying current can enable the development of a progressive memory-type sensor that retains the previous signal under irregular gas injection at low concentrations. 相似文献
49.
50.
The slight-alkalization of generator internal cooling water (GICW) is widely used to inhibit the corrosion of hollow copper conductor and thereby ensure the safe operation of the generator. CO2 inleakage is increasingly identified as a potential security risk for GICW system. In this paper, the influence of CO2 inleakage on the slight-alkalization of GICW was theoretically discussed. Based on the equilibriums of the CO2-NaOH-H2O system, CO2 inleakage saturation was derived to quantify the amount of the dissolved CO2 in GICW. This parameter can be directly calculated with the measured conductivity and the [Na+] of GICW. The influence of CO2 inleakage on the slight-alkalization conditioning of GICW and the measurement of its water quality parameters were then analyzed. The more severe the inleakage, the narrower the water quality operation ranges of GICW, resulting in the more difficult the slight-alkalization conditioning of GICW. The temperature calibrations of the conductivity and the pH value of GICW show non-linear correlations with the amount of CO2 inleakage and the NaOH dosage. This study provides insights into the influence of CO2 inleakage on the slight-alkalization of GICW, which can serve as the theoretical basis for the actual slight-alkalization when CO2 inleakage occurs. 相似文献