Aberrant copper metabolism and hepatic inflammation cause neurological manifestations in a mouse model of Wilson's disease.

Pathogenic germline mutations in the P-type copper-transporting ATPase (ATP7B) gene cause Wilson's disease (WD), a hereditary disorder characterized by disrupted copper metabolism. The Arg778Leu (R778L) mutation in exon 8 is prevalent among individuals with WD in East Asia and is associated with more severe phenotypes. In this study, we generated a WD mouse model harboring R778L mutation (R778L mice) using CRISPR/Cas9.

Activated Co in Thiospinel Boosting LiCO Decomposition in Li-CO Batteries.

Catalytic reactions mainly depend on the adsorption properties of reactants on the catalyst, which provides a perspective for the design of reversible lithium-carbon dioxide (Li-CO) batteries including CO reduction (CORR) and CO evolution (COER) reactions. However, due to the complex reaction process, the relationship between the adsorption configuration and CORR/COER catalytic activity is still unclear in Li─CO batteries. Herein, taking CoS as a model system, nickel (Ni substitution in the tetrahedral site to activate cobalt (Co) atom for forming multiatom catalytic domains in NiCoS is utilized.

Steering the Orbital Hybridization to Boost the Redox Kinetics for Efficient Li-CO Batteries.

The sluggish CO reduction and evolution reaction kinetics are thorny problems for developing high-performance Li-CO batteries. For the complicated multiphase reactions and multielectron transfer processes in Li-CO batteries, exploring efficient cathode catalysts and understanding the interplay between structure and activity are crucial to couple with these pendent challenges. In this work, we applied the CoS as a model catalyst and adjusted its electronic structure by introducing sulfur vacancies to optimize the d-band and p-band centers, which steer the orbital hybridization and boost the redox kinetics between Li and CO, thus improving the discharge platform of Li-CO batteries and altering the deposition behavior of discharge products.

A locally solvent-tethered polymer electrolyte for long-life lithium metal batteries.

Solid polymer electrolytes exhibit enhanced Li conductivity when plasticized with highly dielectric solvents such as N,N-dimethylformamide (DMF). However, the application of DMF-containing electrolytes in solid-state batteries is hindered by poor cycle life caused by continuous DMF degradation at the anode surface and the resulting unstable solid-electrolyte interphase. Here we report a composite polymer electrolyte with a rationally designed Hofmann-DMF coordination complex to address this issue.

Recycled Tandem Catalysts Promising Ultralow Overpotential Li-CO Batteries.

Lithium-carbon dioxide (Li-CO ) batteries are regarded as a prospective technology to relieve the pressure of greenhouse emissions but are confronted with sluggish CO redox kinetics and low energy efficiency. Developing highly efficient and low-cost catalysts to boost bidirectional activities is craved but remains a huge challenge. Herein, derived from the spent lithium-ion batteries, a tandem catalyst is subtly synthesized and significantly accelerates the CO reduction and evolution reactions (CO RR and CO ER) kinetics with an in-built electric field (BEF).

Plasma Fluorinated Nano-SiO Enhances the Surface Insulation Performance of Glass Fiber Reinforced Polymer.

With the extensive application of glass fiber reinforced polymer (GFRP) in the field of high voltage insulation, its operating environment is becoming more and more complex, and the surface insulation failure has gradually become a pivotal problem affecting the safety of equipment. In this paper, nano-SiO was fluorinated by Dielectric barrier discharges (DBD) plasma and doped with GFRP to enhance the insulation performance. Through Fourier Transform Ioncyclotron Resonance (FTIR) and X-ray Photoelectron Spectroscopy (XPS) characterization of nano fillers before and after modification, it was found that plasma fluorination can graft a large number of fluorinated groups on the surface of SiO.

Loss of circSRY reduces γH2AX level in germ cells and impairs mouse spermatogenesis.

on the Y chromosome is the master switch of sex determination in mammals. It has been well established that encodes a transcription factor that is transiently expressed in somatic cells of the male gonad, leading to the formation of testes. In the testis of adult mice, is expressed as a circular RNA (circRNA) transcript.