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Leigh syndrome (LS)/Leigh-like syndrome (LLS) is one of the most common mitochondrial disease subtypes, caused by mutations in either the nuclear or mitochondrial genomes. Here, we identified a novel intronic mutation (c.82-2 A > G) and a novel exonic insertion mutation (c.290dupT) in TMEM126Bfrom a Chinese patient with clinical manifestations of LLS. In silico predictions, minigene splicing assays and patients’ RNA analyses determined that the c.82-2 A > G mutation resulted in complete exon 2 skipping, and the c.290dupT mutation provoked partial and complete exon 3 skipping, leading to translational frameshifts and premature termination. Functional analysis revealed the impaired mitochondrial function in patient-derived lymphocytes due to severe complex I content and assembly defect. Altogether, this is the first report of LLS in a patient carrying mutations in TMEM126B. Our data uncovers the functional effect and the molecular mechanism of the pathogenic variants c.82-2 A > G and c.290dupT, which expands the gene mutation spectrum of LLS and clinical spectrum caused by TMEM126Bmutations, and thus help to clinical diagnosis of TMEM126Bmutation‐related mitochondrial diseases.

• High power factor of 64.4 µW m−1 K−2 is achieved in PEDOT:PSS films at 360 K. • 80 vol.% DMAC solution boosts the electrical conductivity from 5 to 964 S cm−1. • 0.5 mol L−1 LAA improves the Seebeck coefficient from 18.7 to 25 µV K−1. • Mott variable range hopping model explains the thermoelectric transport behavior. Developing high-performance poly(3,4-ethylenedioxythiophene):poly(styrene sulfonate) (PEDOT:PSS) significantly widens the practical applications of flexible organic thermoelectric devices, while the water-based co-solvent dopants and/or post-treatments are still rarely studied so far. Here, we develop a one-step post-treatment to improve the power factor of PEDOT:PSS films by using a water-based solution, which is composed of co-solvent (polar solvent dimethylacetamide (DMAC) and deionized water) and organic reducing agent L-ascorbic acid (LAA). The 80 vol.% DMAC solution significantly boosts the room-temperature electrical conductivity of the films from 5 to 964 S cm−1, while the Seebeck coefficient can be further enhanced from 18.7 to 25 µV K−1 by treating with 0.5 mol L−1 LAA, contributing to a significantly improved power factor of 55.3 µW m−1 K−2. The boosted electrical conductivity is ascribed to the separated PEDOT and PSS phases triggered by the high dielectric constant and polarity of DMAC; while the improved Seebeck coefficient is attributed to the reduced oxidation degree of PEDOT from the reducing agent LAA, both confirmed by the comprehensive structural and morphological characterizations. Furthermore, a maximum power factor of 64.4 µW m−1 K−2 can be achieved at 360 K and the observed temperature-dependent electrical transport behavior can be well explained by the Mott variable range hopping model. Besides, a flexible thermoelectric device, assembled by the as-fabricated PEDOT:PSS films, exhibits a maximum output power of ∼23 nW at a temperature difference of 25 K, indicating the potential for applying to low-grade wearable electronics. [Display omitted] [ABSTRACT FROM AUTHOR]

Nowadays, trace CH4emitted from vehicle exhausts severely threaten the balance of the ecology system of our earth. Thereby, the development of active and stable catalysts capable of methane conversion under mild conditions is critical. Here, we present a convenient method to redisperse catalytically inert PdO nanoparticles (NPs) (>10 nm) into reactive PdOxnanoclusters (∼2 nm) anchored on a Ce-doped LaFeO3parent. Isothermally activated in an N2flow, the redispersed catalyst achieved a CH4conversion of 90% at 400 °C, which is significantly higher than the fresh and H2- and O2-treated counterparts (625, 616, and 641 °C, respectively), indicating the importance of the gas atmosphere in the redispersion of PdO NPs. In addition, the comprehensive catalyst characterizations demonstrated that the isolated Ce ions in the perovskite lattice play an irreplaceable role in the redispersion of reactive sites and the reduction of the energy barrier for C–H scission. More importantly, the Ce additive helps to stabilize the PdOxspecies by reducing overoxidation, resulting in significant lifetime extension. Through a thorough understanding of structural manipulation, this study sheds light on the design of highly performing supported catalysts for methane oxidation.

[Display omitted] • An in-situ synthesis method for non-noble metal Bi decorated mulberry-like BiVO 4 ; • Bi/BiVO 4 exhibits full-spectrum (UV–vis-NIR) responsive photocatalytic activities; • Surface plasmon resonance effects of Bi and synergetic effects between Bi and BiVO 4. • Density functional theory (DFT) calculation verify the electron transfer mechanism. Due to its appropriate bandgap (∼2.4 eV) and efficient light absorption, bismuth vanadate (BiVO 4) shows promising photocatalysis activity. However, the charge carrier recombination and poor electron transmission often induce poor photocatalytic performance. Herein, we report a new method to in-situ synthesize non-noble metal Bi decorated mulberry-like BiVO 4 by a two-step calcination process. Comprehensive characterizations reveal that non-noble metal Bi nanoparticles grown in-situ on BiVO 4 result in the red-shift of the absorbance edge, greatly extending the light absorption from the ultraviolet into the near-infrared region. The surface plasmon resonance excitation of Bi nanoparticles and synergetic effects between Bi and BiVO 4 effectively improve the photocatalytic efficiency and promote the separation of photoinduced electron-hole pairs in mulberry-like BiVO 4. Density functional theory (DFT) calculation results further verify that the electrons are transferred from Bi to BiVO 4 and the formation of OH radical in Bi/BiVO 4 is attributed to the lower simulated free energy, which supports our experimental outcomes. This work provides a novel strategy to enhance light absorption and promote efficient solar utilization of photocatalysts for practical applications. [ABSTRACT FROM AUTHOR]

In this article, we are interested in the following Kirchhoff-type problem (0.1)−a+b∫RN∣∇u∣2dxΔu+V(∣x∣)u=∣u∣2uinRN,u∈H1(RN),\left\{\begin{array}{l}-\left(a+b\mathop{\displaystyle \int }\limits_{{{\mathbb{R}}}^{N}}| \nabla u\hspace{-0.25em}{| }^{2}{\rm{d}}x\right)\Delta u+V\left(| x| )u=| u\hspace{-0.25em}{| }^{2}u\hspace{1.0em}{\rm{in}}\hspace{0.33em}{{\mathbb{R}}}^{N},\\ u\in {H}^{1}\left({{\mathbb{R}}}^{N}),\end{array}\right.where a,b>0,N=2a,b\gt 0,N=2or 3, the potential function VVis radial and bounded from below by a positive number. Because the nonlocal b∣∇u∣L2(RN)2Δub| \nabla u\hspace{-0.25em}{| }_{{L}^{2}\left({{\mathbb{R}}}^{N})}^{2}\Delta uis 3-homogeneous which is in complicated competition with the nonlinear term ∣u∣2u| u\hspace{-0.25em}{| }^{2}u. This causes that not all function in H1(RN){H}^{1}\left({{\mathbb{R}}}^{N})can be projected on the Nehari manifold and thereby the classical Nehari manifold method does not work. By introducing the Gersgorin Disk theorem and the Miranda theorem, via a limit approach and subtle analysis, we prove that for each positive integer kk, equation (0.1) admits a radial nodal solution Uk,4b{U}_{k,4}^{b}having exactly kknodes. Moreover, we show that the energy of Uk,4b{U}_{k,4}^{b}is strictly increasing in kkand for any sequence {bn}\left\{{b}_{n}\right\}with bn→0+,{b}_{n}\to {0}_{+},up to a subsequence, Uk,4bn{U}_{k,4}^{{b}_{n}}converges to Uk,40{U}_{k,4}^{0}in H1(RN){H}^{1}\left({{\mathbb{R}}}^{N}), which is a radial nodal solution with exactly kknodes of the classical Schrödinger equation −aΔu+V(∣x∣)u=∣u∣2uinRN,u∈H1(RN).\left\{\begin{array}{l}-a\Delta u+V\left(| x| )u=| u\hspace{-0.25em}{| }^{2}u\hspace{1.0em}{\rm{in}}\hspace{0.33em}{{\mathbb{R}}}^{N},\\ u\in {H}^{1}\left({{\mathbb{R}}}^{N}).\end{array}\right.Our results extend the existence result from the super-cubic case to the cubic case.

High-energy sodium-ion batteries have a significant prospective application as a next-generation energy storage technology. However, this technology is severely hindered by the lack of large-scale production of battery materials. Herein, a self-standing film, assembled with SnS–Sn/multiwalled carbon nanotubes encapsulated in carbon fibers (SnS–Sn/MCNTs@CFs), is prepared using ball milling and electrospinning techniques and used as sodium-ion battery anodes. To compensate the poor internal conductivity of SnS–Sn nanoparticles, MCNTs are used to interweave SnS–Sn nanoparticles to improve the conductivity. Moreover, the designed three-dimensional carbon fiber conductive network can effectively shorten the diffusion path of electron/Na+, accelerate the reaction kinetics, and provide abundant active sites for sodium absorption. Benefiting from these unique features, the self-standing film offers a high reversible capacity of 568 mA h g–1at 0.1 A g–1and excellent cycling stability at 1 A g–1with a reversible capacity of 359.3 mA h g–1after 1000 cycles. In the sodium-ion full cell device, the capacity is stable at 283.7 mA h g–1after 100 cycles at a current of 100 mA g–1. This work provides a new strategy for electrode design and facilitates the large-scale application of the sodium-ion battery.

Materials with low density, exceptional thermal and corrosion resistance, and ultrahigh mechanical and electromagnetic interference (EMI) shielding performance are urgently demanded for aerospace and military industries. Efficient design of materials’ components and microstructures is crucial yet remains highly challenging for achieving the above requirements. Herein, a strengthened reduced graphene oxide (SrGO)-reinforced multi-interfacial carbon–silicon carbide (C-SiC)nmatrix (SrGO/(C-SiC)n) composite is reported, which is fabricated by depositing a carbon-strengthening layer into rGO foam followed by alternate filling of pyrocarbon (PyC) and silicon carbide (SiC) viaa precursor infiltration pyrolysis (PIP) method. By increasing the number of alternate PIP sequences (n= 1, 3 and 12), the mechanical, electrical, and EMI shielding properties of SrGO/(C-SiC)ncomposites are significantly increased. The optimal composite exhibits excellent conductivity of 8.52 S·cm–1and powerful average EMI shielding effectiveness (SE) of 70.2 dB over a broad bandwidth of 32 GHz, covering the entire X-, Ku-, K-, and Ka-bands. The excellent EMI SE benefits from the massive conduction loss in highly conductive SrGO skeletons and polarization relaxation of rich heterogeneous PyC/SiC interfaces. Our composite features low density down to 1.60 g·cm–3and displays robust compressive properties (up to 163.8 MPa in strength), owing to the uniformly distributed heterogeneous interfaces capable of consuming great fracture energy upon loadings. Moreover, ultrahigh thermostructural stability (up to 2100 °C in Ar) and super corrosion resistance (no strength degradation after long-term acid and alkali immersion) are also discovered. These excellent comprehensive properties, along with ease of low-cost and scalable production, could potentially promote the practical applications of the SrGO/(C-SiC)ncomposite in the near future.

Hypomyelinating leukodystrophies (HLDs) are a rare group of disorders characterized by myelin deficit of the brain-based on MRI. Here, we studied 20 patients with unexplained HLD to uncover their genetic etiology through whole-exome sequencing (WES). Trio-based WES was performed for 20 unresolved HLDs families after genetic tests for the PLP1 duplication and a panel of 115 known leukodystrophy-related genes. Variants in both known genes that related to HLDs and promising candidate genes were analyzed. Minigene splicing assay was conducted to confirm the effect of splice region variant. All 20 patients were diagnosed with HLDs clinically based on myelin deficit on MRI and impaired motor ability. Through WES, in 11 of 20 trios, 15 causative variants were detected in seven genes TUBB4A, POLR1C, POLR3A, SOX10, TMEM106B, DEGS1, and TMEM63A. The last three genes have just been discovered. Of 15 variants, six were novel. Using minigene splicing assay, splice variant POLR3Ac.1770 + 5 G > C was proved to disrupt the normal splicing of intron 13 and led to a premature stop codon at position 618 (p.(P591Vfs*28)). Our analysis determined the molecular diagnosis of 11 HLDs patients. It emphasizes the heterogenicity of HLDs, the diagnostic power of trio-based WES for HLDs. Comprehensive analysis including a focus on candidate genes helps to discover novel disease-causing genes, determine the diagnosis for the first time, and improve the yield of WES. Moreover, novel mutations identified in TUBB4A, POLR3A, and POLR1Cexpand the mutation spectrum of these genes.

Thermoelectric (TE) performance of Ca3Co4O9(CCO) has been investigated extensively via a doping strategy in the past decades. However, the doping sites of different sublayers in CCO and their contributions to the TE performance remain unrevealed because of its strong correlated electronic system. In this work, Sr and Ti are chosen to realize doping at the [Ca2CoO3] and [CoO2] sublayers in CCO. It was found that figure of merit (ZT) at 957 K of Ti-doped CCO was improved 30% than that of undoped CCO whereas 1 at% Sr doping brought about a 150% increase in ZTas compared to undoped CCO. The significant increase in electronic conductivity and the Seebeck coefficient are attributed to the enhanced carrier concentration and spin-entropy of Co4+originating from the Sr doping effects in [Ca2CoO3] sublayer, which are evidenced by the scanning electron microscope (SEM), Raman, Hall, and X-ray photoelectron spectroscopy (XPS) analysis. Furthermore, the reduced thermal conductivity is attributed to the improved phonon scattering from heavier Sr doped Ca site in [Ca2CoO3] sublayer. Our findings demonstrate that doping at Ca sites of [Ca2CoO3] layer is a feasible pathway to boost TE performance of CCO material through promoting the electronic conductivity and the Seebeck coefficient, and reducing the thermal conductivity simultaneously. This work provides a deep understanding of the current limited ZTenhancement on CCO material and provides an approach to enhance the TE performance of other layered structure materials.

Increasing attention has been focused on potassium ion batteries (KIBs) as promising energy-storage system (ESS) owing to the abundance and low-cost of potassium resources. Here, SnS 2 /SnO 2 heterostructures were successfully fixed onto stainless steel mesh (SnS 2 /SnO 2 /SSM) through a facile two-step hydrothermal method and used as anodes for KIBs. Due to the advantages of SnS 2 /SnO 2 heterostructures and good conductivity of SSM substrate, the SnS 2 /SnO 2 /SSM anodes display enhanced electrochemical performance. The SnS 2 /SnO 2 /SSM anodes deliver specific capacity of 394 mA h g−1 at 50 mA g−1 over 100 cycles, better than SnO 2 /SSM. Even at 500 mA g-1 after 250 cycles, high capacity of 155 mA h g−1 can still be obtained. [ABSTRACT FROM AUTHOR]

The development of efficient non-noble metal catalysts is of critical importance for conversion of lignin toward value-added chemicals. The trans-metal Co catalysts and the counterparts promoted with a small amount of Ru were found highly selective for hydrogenolysis of lignin-derived aromatic ethers in decalin at relatively mild conditions. The physical and chemical properties of the as-prepared catalysts were fully investigated by various characterization methods, and Co species was found well dispersed in the reduced catalysts. The experimental results showed that Co/CeO2could selectively cleave the C–O bonds of diphenyl ether (DPE) to produce benzene and phenol, which can be further converted to cyclohexane and cyclohexanol. Upon 5Co/CeO2, 97% selectivity to those C–O bond ruptured products was achieved, while the direct hydrogenation products including dicyclohexyl ether and cyclohexyl account for only 3%. Moreover, benzene, featured as a typical intermediate product, could reach a maximum yield of 27.4% at around 80% conversion. The addition of a small amount of Ru (0.1 wt % and below) could significantly accelerate the hydrogenolysis reactivity but also result in the declined selectivity of benzene during the reaction due to the stronger hydrogenation ability.

IMPORTANCE: The gastric cancer (GC)–associated long noncoding RNA1 (lncRNA-GC1) plays an important role in gastric carcinogenesis. However, exosomal lncRNA-GC1 and its potential role in GC are poorly understood. OBJECTIVE: To evaluate the diagnostic value of circulating exosomal lncRNA-GC1 for early detection and monitoring progression of GC. DESIGN, SETTING, AND PARTICIPANTS: We performed a multiphase investigation of circulating exosomal lncRNA-GC1 for early detection of GC involving consecutive patients with GC (n = 522), patients with gastric precancerous lesions (n = 85), and healthy donor individuals (HDs; n = 219) from December 2016 to February 2019 at Chinese People’s Liberation Army General Hospital, China. LncRNA-GC1 was measured by reverse transcription–polymerase chain reaction by independent researchers who had no access to patients’ information. Receiver operating characteristic curves were used to calculate diagnostic efficiency in comparison between lncRNA-GC1 and 3 traditional biomarkers (carcinoembryonic antigen [CEA], cancer antigen 72-4 [CA72-4], and CA19-9). MAIN OUTCOMES AND MEASURES: Assessment of diagnostic efficiency on the basis of area under curve (AUC), specificity, and sensitivity. RESULTS: Of the 826 patients included in the study, 508 were men (61.5%), and the median age of all patients was 60 years (range, 28-82 years). In the test phase, lncRNA-GC1 achieved better diagnostic performance than the standard biomarkers CEA, CA72-4, and CA19-9 (AUC = 0.9033) for distinguishing between the patients with GC and HDs. Additionally, exosomal lncRNA-GC1 levels were significantly higher in culture media from GC cells compared with those of normal gastric epithelial cells (t = 5.310; P = .002). In the verification phase, lncRNA-GC1 retained its diagnostic efficiency in discriminating patients with GC from those with gastric precancerous lesions as well from HDs. Moreover, lncRNA-GC1 exhibited a higher AUC compared with those of CEA, CA72-4, and CA19-9 for early detection of GC with sufficient specificity and sensitivity, especially for patients with GC with negative standard biomarkers. Moreover, the levels of circulating exosomal lncRNA-GC1 were significantly associated with GC from early to advanced stages (HD vs stage I, t = 20.98; P < .001; stage I vs stage II, t = 2.787; P = .006; stage II vs stage III, t = 4.471; P < .001; stage III vs stage IV, t = 1.023; P = .30), independent of pathological grading and Lauren classification (pathological grading: HD vs G1, t = 21.09; P < .001; G1 vs G2, t = 0.3718; P = .71; G2 vs G3, t = 0.3598; P = .72; Lauren classification: t = 24.81; P <.001). In the supplemental phase, the levels of circulating exosomal lncRNA-GC1 were consistent with those in GC tissues and cells and were higher compared with those in normal tissues and cells. Furthermore, the levels of circulating lncRNA-GC1 were unchanged after exosomes were treated with RNase and remained constant after prolonged exposure to room temperature or after repeated freezing and thawing (t = 1.443; P = .39). Total circulating lncRNA-GC1 was nearly all packaged within exosomes rather than a free form in plasma. CONCLUSIONS AND RELEVENCE: Circulating exosomal lncRNA-GC1 may serve as a noninvasive biomarker for detecting early-stage GC and for monitoring disease progression. Combining circulating exosomal lncRNA-GC1 detection with endoscopy could improve the early diagnostic rate of GC.

In this work, we prepared flexible carbon-fiber/semimetal Bi nanosheet arrays from solvothermal-synthesized carbon-fiber/Bi2O2CO3nanosheet arrays via a reductive calcination process. The flexible carbon-fiber/semimetal Bi nanosheet arrays can function as photocatalysts and photoelectrocatalysts for 2,4-dinitorphenol oxidation. Compared with carbon-fiber/Bi2O2CO3nanosheet arrays, the newly designed flexible carbon-fiber/semimetal Bi nanosheet arrays show enhanced ultraviolet–visible (UV–vis) light absorption efficiency and photocurrent, photocatalytic, and photoelectrocatalytic activities. Photocatalytic analyses indicate that the surface plasmon resonance (SPR) of semimetal Bi occurs under solar-simulated light irradiation during the photocatalytic process. The carbon-fiber traps the hot electrons exerted from the SPR of semimetal Bi and creates holes in the semimetal Bi nanosheets, which boosts the photocatalytic activity of the carbon fiber through plasmonic sensitization. Both photocatalytic experiments and density functional theory (DFT) calculations indicate that the electrons transferred to the carbon fiber and the holes created in semimetal Bi contribute to the formation of •O2–and •OH, respectively. The synergistic effect between electrocatalysis and photocatalysis under the solar-simulated light results in almost complete degradation of 2,4-dinitorphenol during the photoelectrocatalytic process. This work realizes a non-noble-metal plasmonic catalyst and provides a new avenue for the commercialization of photocatalysis and photoelectrocatalysis using the separable and recyclable carbon-fiber/semimetal Bi nanosheet arrays in the environment-related field.

Condensation freezing inhibition is of great practical importance for anti-icing applications; however, no coatings with this performance have been reported. Here, we report the inhibition of condensation freezing on patterned polyelectrolyte coatings, including polyelectrolyte brush (PB), polyelectrolyte multilayer (PEM), and polyelectrolyte hydrogel (PH) surfaces, benefiting from their feature in regulating ice nucleation and propagation viachanging counterions. On the reported surfaces, ice nucleation can be initiated exclusively at the domains with the polyelectrolytes; moreover, spontaneous ice propagation can be achieved atop the patterned polyelectrolyte surface. Consequently, condensed water surrounding the frozen drops on the patterned polyelectrolyte surface evaporates due to the instantaneously released latent heat in the course of ice propagation. Afterward, ice grows specifically on polyelectrolyte surfaces viadesublimation as the saturated vapor pressure of ice is smaller than that of condensed water drops. As such, an ice-free region up to 96% of the entire surface area can be accomplished. We demonstrate that various polyelectrolyte coatings can be easily introduced on almost all surfaces, revealing great promise for anti-icing applications.

TOM70 is a member of the TOM complex that transports cytosolic proteins into mitochondria. Here, we identified two compound heterozygous variants in TOMM70[c.794C>T (p.T265M) and c.1745C>T (p.A582V)] from a patient with severe anemia, lactic acidosis, and developmental delay. Patient-derived immortalized lymphocytes showed decreased TOM70 expression, oligomerized TOM70 complex, and TOM 20/22/40 complex compared with expression in control lymphocytes. Functional analysis revealed that patient-derived cells exhibited multi-oxidative phosphorylation system (OXPHOS) complex defects, with complex IV being primarily affected. As a result, patient-derived cells grew slower in galactose medium and generated less ATP and more extracellular lactic acid than did control cells. In vitro cell model compensatory experiments confirmed the pathogenicity of TOMM70variants since only wild-type TOM70, but not mutant TOM70, could restore the complex IV defect and TOM70 expression in TOM70 knockdown U2OS cells. Altogether, we report the first case of mitochondrial disease-causing mutations in TOMM70and demonstrate that TOM70 is essential for multi-OXPHOS assembly. Mutational screening of TOMM70should be employed to identify mitochondrial disease-causing gene mutations in the future.

To ensure the biosafety of the domestic hot water system (DHWS) in the case of residual chlorine exhaustion and biofilm exfoliation, the secondary disinfection effects of chlorine alone or in combination with ultraviolet (UV) on microorganisms and its impact on biofilm bacterial community were investigated. A biofilm annular reactor was used to simulate DHWS conditions on a lab-scale and reproducibly. The results showed that even in the case of biofilm exfoliated into domestic hot water seriously, the chlorine combined with UV (chlorine-UV) disinfection could achieve high-level inactivation of suspended bacteria in a short time. Chlorine-UV disinfection effectively reduced the diversity of the bacterial community and affected bacterial community structure. It decreased the relative abundance of pathogenic bacteria, including Legionella, Staphylococcus. Chlorine-UV disinfection is suitable for the secondary disinfection of DHWS, which can ensure biosafety and effectively reduce bacterial contamination.

Due to the growing demand in the energy market, sodium-ion batteries (SIBs) have garnered significant attention as potential energy storage devices for large-scale applications. However, the sluggish kinetics and significant volume expansion during cycling bring about the poor electrochemical behavior of SIBs. In this work, a dual carbon including internal hard carbon core and external N-doped carbon shell confined MoS2hierarchical microspheres (HC@MoS2@NC) nanocomposites are prepared as anode for SIBs with superior cycling stability and good rate capability. The dual carbon confinement can improve the structural stability of MoS2. The dual carbon with good electronic conductivity ensures well battery rate capability of MoS2anode. Meanwhile, the larger interlayer spacing of 10.09 Å caused by NC insertion make MoS2store more Na+. As a consequence, the HC@MoS2@NC nanocomposite delivers a high capacity of 321 mAh g−1at 0.1 A g−1after 100 cycles. At large current density of 2 A g−1, a high capacity of 180 mAh g−1can still be achieved after 1000 cycles. Even at larger current density of 5 A g−1, the capacity can still be kept at 162 mAh g−1after 100 cycles.

In this study, an experimental study aimed at optimizing the factors affecting the regeneration amount of 4-chlorophenol (4-CP) from spent powdered activated carbon (PAC) is presented. For this response surface methodology (RSM) with Box-Behnken design was applied, which identified four variables (acoustic density, NaOH concentration, spent PAC dosage and ethanol concentration) in batch experiments. The physicochemical characteristics of the regenerated PAC under the optimal desorption condition was evaluated using thermogravimetric analysis, Fourier transform infrared spectroscopy (FTIR), Brunauer-Emmett-Teller (BET) and scanning electron microscopy, as compared with the virgin and spent PACs. Among the four variables, acoustic density and ethanol concentration (v/v, %) had stronger effect on the 4-CP desorption (p < 0.001). The predicted optimal 4-CP desorbing amount was 97.43 mg g–1, and this matched well the observed performance of (96.94 ± 0.70) mg g–1, obtained by acoustic density of 0.36 W mL–1, NaOH concentration of 0.10 mol L–1, spent PAC dosage of 0.93 g L–1and ethanol concentration (v/v) of 24%. The result of thermogravimetric pyrolysis profiles of regenerated PAC confirmed that the adsorption behavior of 4-CP exhibited a chemisorption feature and desorption process was dominated by chemical reaction. Analysis of FTIR, pore structure and BET surface area demonstrated that ultrasound mainly acted on the surface functionalities, macro-pore and meso-pore structure of PAC. Additionally, the SEM images indicated that cavitation effect affected the surface roughness and surface cavities of PAC. The results here provided an insight into the application of ultrasound to regenerate saturated PAC with 4-CP.

The water quality of domestic hot water can be controlled by disinfection with silver ions (Ag+). In order to maximize the antibacterial effect of Ag+and at the same time ensure the water is safe for human use, the adsorption and desorption properties of Ag+onto 304 stainless steel (SS) was studied. The effects of the initial Ag+concentration, the contact time, and the water temperature on the adsorption process were examined. The antibacterial properties of SS adsorbed with Ag+(SS-Ag) were also investigated. The results showed that with an increase of the Ag+concentration, water temperature or adsorption time, the equilibrium adsorption capacity increased gradually, and the adsorption process reached equilibrium after 72 h. Adsorption kinetic studies showed that the adsorption of Ag+on SS was best described by the pseudo-second order model. The adsorption isotherm data fitted well in the Langmuir isotherm model at 45°C (R2= 0.9894), which indicated that the adsorbed silver forms a monolayer on the SS. The thermodynamic modelling indicated that adsorption occurred by an endothermic reaction that occurred spontaneously under high temperature conditions. Elution was impossible with ultra-pure water and tap water was inefficient to desorb the Ag+from SS. It can be inferred that the shear action of a water flow inside a water pipe would not remove large amounts of adsorbed Ag+, so that adsorbed Ag+can maintain its antibacterial properties for extended periods. Solutions of sodium thiosulfate or thiourea could effectively desorb Ag+from the SS surface, and near to complete desorption (98.35%) was obtained with a 2 mg/L sodium thiosulfate solution. Antibacterial experiments indicated that the SS-Ag could inhibit the biofilm formation and effectively reduce the risk of biofilm pollution. The results presented here provide a theoretical basis for the use of silver in hot water supplies using SS pipes for disinfection purposes.

A series of Bi–Ti composited photocatalysts with different molar ratio of Bi/Ti (0.01:1(1%), 0.02:1(2%), 0.04:1(4%), and 0.05:1(5%)) and calcination temperature (400°C, 500°C, and 600°C) were prepared by sol-hydrothermal method. The properties of as-prepared photocatalysts were characterized by X-ray powder diffraction, scanning electron microscopy, transmission electron microscopy, X-ray photo-electron spectroscopy, Brunauer–Emmett–Teller, and ultraviolet–visible diffuse reflection spectroscopy. The selected photocatalyst (Bi-Ti-4%-600°C) was prepared under Bi/Ti molar ratio of 4% and calcination temperature of 600°C. Experimental variables during photocatalytic degradation were optimized for catalyst dosage, solution pH, initial bisphenol A (BPA) concentration, coexisting anions, and light source wavelength. Under optimal conditions of 1.0 g L–1catalyst, pH 3, 30 mg L–1initial BPA concentration, the degradation rate of BPA could reach up to 100% at 150 min under visible light irradiation. Compared with self-made pure TiO2and commercially available P25, Bi-Ti-4%- 600°C displayed an excellent photocatalytic activity for BPA degradation. Bi-Ti photocatalyst had a smaller crystal size, larger specific surface area, stronger visible light absorption ability, and lower band gap energy than self-made pure TiO2. Four intermediates of BPA degradation were identified and a possible degradation pathway was proposed.

Cobalamin G (cblG) and cobalamin J (cblJ) defects are rare disorders of cbl metabolism caused by MTRand ABCD4mutations, respectively. Patients with atypical biochemical features can be missed by current newborn screening using tandem mass spectrometry (MS/MS), in which total homocysteine (tHCY) in dried blood spots (DBS) is not a primary biomarker. Two Chinese patients suspected of cbl defect but missed by newborn screening were studied. Using comprehensive metabolic analyses including MS/MS assay for tHCY in DBS, slightly low methionine in Patient 1, methymalonic aciduria in Patient 2, and homocysteinemia in both patients were detected, and DBS tHCY of two patients were obviously elevated (59.22 μmol/L, 17.75 μmol/L) compared to 140 healthy controls (2.5th–97.5th percentile, 1.05–8.22 μmol/L). Utilizing whole-exome sequencing, we found two novel MTRvariants c.871C>T (p.Pro291Ser) and c.1771C>T (p.Arg591*) in Patient 1, and a ABCD4homozygous variant c.423C>G (p.Asn141Lys) in Patient 2. Our study identified the first cblG patient and cblJ patient in mainland China, and highlighted comprehensive metabolic analyses and genetic tests in patients suspected of cbl defects. It also indicated that supplementary MS/MS assay for tHCY in DBS may be practical for early diagnosis of homocysteinemia, without repeated blood sampling.

ABSTRACTUnderstanding the growth mechanisms of nanomaterials usually leads to the successful preparation of targeted micro-structures with excellent properties. In this paper, flower-like and roughly spherical ZnO nanocrystals have been successfully synthesised through facile wet chemical methods, i.e. hydrothermal pathways and homogeneous precipitation method. The photocatalytic activities of the prepared photocatalysts were evaluated by degradation of rhodamine B (RhB) and phenol under ultraviolet (UV) irradiation. Remarkably, the ZnO powder via homogeneous precipitation (urea/Zn2+ = 2.0) exhibited better photocatalytic performance and photostability than those of flower-like ZnO substrate. The enhanced photocatalytic properties could be attributed to more active catalytic sites and effective separation of carriers, which were confirmed by low-temperature nitrogen adsorption, photocurrent responses, and photoluminescence spectral analysis. A possible mechanism for the excellent photocatalytic activity of ZnO substrates was proposed.

Understanding the growth mechanisms of nanomaterials usually leads to the successful preparation of targeted micro-structures with excellent properties. In this paper, flower-like and roughly spherical ZnO nanocrystals have been successfully synthesised through facile wet chemical methods, i.e. hydrothermal pathways and homogeneous precipitation method. The photocatalytic activities of the prepared photocatalysts were evaluated by degradation of rhodamine B (RhB) and phenol under ultraviolet (UV) irradiation. Remarkably, the ZnO powder via homogeneous precipitation (urea/Zn2+?=?2.0) exhibited better photocatalytic performance and photostability than those of flower-like ZnO substrate. The enhanced photocatalytic properties could be attributed to more active catalytic sites and effective separation of carriers, which were confirmed by low-temperature nitrogen adsorption, photocurrent responses, and photoluminescence spectral analysis. A possible mechanism for the excellent photocatalytic activity of ZnO substrates was proposed.

Leigh syndrome is one of the most common subtypes of mitochondrial disease. Mutations in encoding genes of oxidative phosphorylation complexes have been frequently reported, of which, MTATP6was one of the most frequently reported genes for Leigh syndrome. In this study, by using next-generation sequencing targeted to MitoExome in a patient with clinical manifestations of Leigh syndrome, two missense mutations of NDUFS3(c.418 C > T/p.R140W and c.595 C > T/p.R199W) were identified, of which c.418 C > T was novel. Functionally, the patient derived lymphoblastoid cells showed decreased amount of NDUFS3 and complex I assembly when compared with two control cells. Although NDUFS3mutations have been related to late onset Leigh syndrome, we found that the patient carrying these two mutations developed an early onset Leigh syndrome. To our knowledge, this is the second study on patient carrying NDUFS3mutations. In conclusion, we identified a novel Leigh syndrome causing NDUFS3mutation and expanded the clinical spectrum caused by NDUFS3mutations in this study.

Two dimensional (2D) materials are promising gas sensing materials, but the most of them need to be heated to show promising sensing performance. Sensing structures with high sensing performance at room-temperature are urgent. Here, another 2D material, violet phosphorus (VP) nanoflake is investigated as gas sensing material. The VP nanoflakes have been effectively ablated to have layers of 1–5 layers by laser ablation in glycol. The VP nanoflakes are combined with graphene to form VP/G heterostructures-based NO sensor. An ultra-high gauge factor of 3 × 107for ppb-level sensing and high resistance response of 59.21% with ultra-short recovery time of 6s for ppm-level sensing have been obtained. The sensing mechanism is also analysed by density functional theory (DFT) calculations. The adsorption energy of VP/G is calculated to be −0.788 eV, resulting in electrons migration from P to N to form a P−N bond in the gap between VP and graphene sheet. This work provides a facile approach to ablate VP for mass production. The as-produced structures have also provided potential gas sensors with ultrasensitive performance as ppb-level room-temperature sensors.

In this article, we study the following Kirchhoff equation: (0.1)−(a+b‖∇u‖L2(R3)2)Δu+V(∣x∣)u=f(u)inR3,-(a+b\Vert \nabla u{\Vert }_{{L}^{2}\left({{\mathbb{R}}}^{3})}^{2})\Delta u+V\left(| x| )u=f\left(u)\hspace{1.0em}\hspace{0.1em}\text{in}\hspace{0.1em}\hspace{0.33em}{{\mathbb{R}}}^{3},where a,b>0a,b\gt 0, VVis a positive radial potential function, and f(u)f\left(u)is an asymptotically cubic term. The nonlocal term b‖∇u‖L2(R3)2Δub\Vert \nabla u{\Vert }_{{L}^{2}\left({{\mathbb{R}}}^{3})}^{2}\Delta uis 3-homogeneous in the sense that b‖∇tu‖L2(R3)2Δ(tu)=t3b‖∇u‖L2(R3)2Δub\Vert \nabla tu{\Vert }_{{L}^{2}\left({{\mathbb{R}}}^{3})}^{2}\Delta \left(tu)={t}^{3}b\Vert \nabla u{\Vert }_{{L}^{2}\left({{\mathbb{R}}}^{3})}^{2}\Delta u, so it competes complicatedly with the asymptotically cubic term f(u)f\left(u), which is totally different from the super-cubic case. By using the Miranda theorem and classifying the domain partitions, via the gluing method and variational method, we prove that for each positive integer kk, equation (0.1) has a radial nodal solution Uk,4b{U}_{k,4}^{b}, which has exactly k+1k+1nodal domains. Moreover, we show that the energy of Uk,4b{U}_{k,4}^{b}is strictly increasing in kk, and for any sequence {bn}→0+,\left\{{b}_{n}\right\}\to {0}_{+},up to a subsequence, Uk,4bn{U}_{k,4}^{{b}_{n}}converges strongly to Uk,40{U}_{k,4}^{0}in H1(R3){H}^{1}\left({{\mathbb{R}}}^{3}), where Uk,40{U}_{k,4}^{0}also has k+1k+1nodal domains exactly and solves the classical Schrödinger equation: −aΔu+V(∣x∣)u=f(u)inR3.-a\Delta u+V\left(| x| )u=f\left(u)\hspace{1.0em}\hspace{0.1em}\text{in}\hspace{0.1em}\hspace{0.33em}{{\mathbb{R}}}^{3}.Our results extend the ones in Deng et al. from the super-cubic case to the asymptotically cubic case.

Over the past few years, significant efforts have been made to create new fluorescent probes operating at longer wavelengths, particularly in the second near-infrared (NIR-II) window from 1000 to 1700 nm, offering enhanced tissue penetration compared to light in the visible and first near-infrared window (700–900 nm). However, most of the reported NIR-II fluorophores meet such dilemmas; they are excreted slowly and largely retained within the reticuloendothelial system. Here, we report a rapidly excreted NIR-II lanthanide complex Nd-DOTA (over 50% excreted through the kidneys within 3 h postinjection) with a molecular mass only 0.54 kDa. The NIR-II imaging quality of Nd-DOTA was far superior to that of clinically approved ICG with good photostability and deep tissue penetration (7 mm). Superior tumor-to-normal tissue ratio was successfully achieved to facilitate the abdominal ovarian metastases surgical delineation. Metastases with ≤1 mm can be completely excised under NIR-II bioimaging guidance. Significantly, since the Nd-DOTA structure is same to the clinically approved magnetic resonance imaging (MRI) contrast Gd-DOTA, it will speed up the clinical translation for this novel kind of NIR-II probes in the future.

Appropriate mixing conditions are always favorable during enhanced coagulation for the desirable performance. However, up to now, there are little studies systematically investigated the effects of mixing duration and intensity on coagulation–flocculation efficiency based on a comprehensive consideration. Different mixing intensity and duration were applied in coagulation and flocculation, respectively, using aluminum sulfate as the coagulant at a predetermined dosage where significant hydroxide precipitate occurs. Flocs formation, breakage and regrowth were monitored using a ‘turbidity fluctuation’ technique, photometric dispersion analyzer (PDA) measurement. In addition, the flocs images were in situ captured using a charged-coupled device camera to further the knowledge of flocs characteristics, such as fractal dimension and settleability. The results indicated that a slightly prolonged rapid mixing time (120 s) improved floc density with better settleability when the mixing intensity in coagulation stage was maintained at a central level. In contrast, a little higher mixing intensity (40.2 s–1) in flocculation stage induced more compact flocs, however, with smaller homogeneous size. The compensatory interaction of mixing conditions between coagulation and flocculation stages was verified. Particularly, this work was expected to provide a useful suggestion on optimizing coagulation and flocculation separately, and thus the desirable performance can be obtained by the optimized operational design.

Considering the earth powered by intermittent renewable energy in the coming future, solid oxide electrolysis cell (SOEC) will play an indispensable role in efficient energy conversion and storage on demand. The thermolytic and kinetic merits grant SOEC a bright potential to be directly integrated with electrical grid and downstream chemical synthesis process. Meanwhile, the scientific community are still endeavoring to pursue the SOEC assembled with better materials and operated at a more energy-efficient way. In this review article, at cell level, we focus on the recent development of electrolyte, cathode, anode and buffer layer materials for both steam and CO2electrolysis. On the other hand, we also discuss the next generation SOEC operated with the assistant of other fuels to further reduce the energy consumption and enhance the productivity of the electrolyzer. And stack level, the sealant, interconnect and stack operation strategies are collectively covered. Finally, the challenges and future research direction in SOECs are included.

To provide an insight into aluminum (Al) control during the treatment of micro-polluted water, recycling ferric water treatment sludge (Fe-WTS) as an alternative approach was innovatively investigated. Particular attention was paid to the recycling ratio of Fe-WTS when coagulated at an optimized dosage of both Al2(SO4)3and polyaluminum chloride (OH/Al = 2.0, denoted as PAC20) respectively. Coagulation performance, i.e., turbidity, organics and Al speciation were also examined. A morphological analysis, characterized via floc size and fractal dimension, was further conducted to explore the fundamental floc operational parameters and to elucidate the mechanism of different Al species removal. The results indicate that an appropriate recycling ratio yields improvements in residual Al control. In comparison, Fe-WTS is more effective in dissolved Al (AlD) removal when coagulated by Al2(SO4)3, while is proper to the reduction of colloidal Al (AlCO) when coagulated by PAC20. Flocs properties are significantly correlated with Al speciation during the treatment. Specifically, floc size is proportional to AlCOremoval rate, however, the value of floc fractal dimension is inversely proportional to AlDremoval rate.

Biofilm in secondary water supply system (SWSS) may affect tap water biological stability. The growth of biofilm and the diversity of biofilm microbial community are partly influenced by organic matter content. In this paper, the impact of organic matter content on biofilm bacteria growth and biofilm density was studied. The diversity and variation process of biofilm microbial community were also analyzed by Metagenomic sequencing. Biofilm bacteria growth and compactness were positive correlate with organic matter content. Biofilm grew with tap water of low organic matter content (permanganate oxygen consumption, viz., CODMn<3 mg/L) was of higher diversity level than that in the condition of CODMn>3 mg/L. It may because of the proportion of Proteobacteria, Firmicutes and Bacteroidetes changed in low CODMnconcentration condition in comparison to that in high CODMnconcentration condition. Proteobacteria and Firmicutes were dominant at phylum level in biofilm in different organic matter content in this experiment. Pathogenic bacteria including Pseudomonas, Acinetobacter, Ledionella and Flavobacterium could grow with tap water that met the Chinese drinking water standards (CODMn<3 mg/L).

By using next-generation sequencing targeted to MitoExome including the entire mtDNA and exons of 1033 genes encoding the mitochondrial proteome, we described here a novel m.11240C>T mutation in the mitochondrial ND4 genefrom a patient with Leigh syndrome. High mutant loads of m.11240C>T were detected in blood, urinary epithelium, oral mucosal epithelium cells, and skin fibroblasts of the patient. Decreased mitochondrial complex I activity was found in transmitochondrial cybrids containing the m.11240C>T mutation with biochemical analysis. Furthermore, functional investigations confirmed that mitochondria with the m.11240C>T variant exhibited lower adenosine triphosphate-related mitochondrial respiration. However, complex I assembly in mutant cybrids was not affected. While this mutation was located in the fourth hydrophobic trans-membrane region of ND4 gene, we suggested that mutation of m.11240C>T might impair the proton pumping channel of complex I but had little effect on the complex I assembly. In conclusion, we identified m.11240C>T as a novel mitochondrial disease-related mtDNA mutation.

AbstractIn this study, the nitrification performance in terms of ammonium (-N), nitrite (-N), and nitrifiers activity aftershock oxidation of sodium hypochlorite (NaOCl), chlorine dioxide (ClO2), or the combination of both (NaOCl/ClO2) within a raw water distribution system (RWDS) was investigated. The biofilm structure was evaluated to explore its role in nitrification recovery. Our results indicate that nitrification could be recovered, although oxidation initially played an adverse role in nitrification. Compared to individual NaOCl or ClO2, the combination of NaOCl/ClO2had a less negative effect on -N and -N removal, as well as on the regrowth of nitrifiers. The improved biofilm regrowth and consequential faster nitrification recovery after combined oxidation was mainly ascribed to the faster reaction between NaOCl/ClO2and surface biofilm, resulting in decreased limitation of nutrient transfer. These results revealed the synergistic effect of NaOCl/ClO2on nitrification recovery, and that the biofilm structure plays a key role in nitrification recovery within RWDS.

The longevity and the safety of artificial joint implants are determined by wear and corrosion properties. For many clinical cases, the released metallic ions and wear debris can cause adverse tissue reactions. Eventually, failure of such medical devices can be induced. Therefore, it is of great interest to increase the wear-corrosion resistance of implant materials. The release of metallic ions is related to the damage on the passive film which can spontaneously form on the surface of the cobalt–chromium–molybdenum (CoCrMo) alloy. In order to prevent the tribocorrosion reaction (the interaction between tribology and corrosion), a titanium nitride (TiN) film was applied on the surface by magnetron sputtering technology. The tribocorrosion behaviours of the titanium nitride and cobalt–chromium–molybdenum combination were studied. It has been shown that titanium nitride can greatly increase tribocorrosion resistance. However, if the titanium nitride film is damaged by tribological contact or the impact of the contact, the corrosion rate would dramatically increase.

Mutations in the fukutin-related protein (FKRP) gene have been associated with dystroglycanopathies, which are common in Europe but rare in Asia. Our study aimed to retrospectively analyze and characterize the clinical, myopathological and genetic features of 12 Chinese patients with FKRPmutations. Three patients were diagnosed with congenital muscular dystrophy type 1C (MDC1C) and nine patients were diagnosed with limb girdle muscular dystrophy type 2I (LGMD2I). Three muscle biopsy specimens had dystrophic changes and reduced glycosylated a-dystroglycan staining, and two showed reduced expression of laminin a2. Two known and 13 novel mutations were identified in our single center cohort. Interestingly, the c.545A>G mutation was found in eight of the nine LGMD2I patients as a founder mutation and this founder mutation in Chinese patients differs from the one seen in European patients. Moreover, patients homozygous for the c.545A>G mutation were clinically asymptomatic, a less severe phenotype than in compound heterozygous patients with the c.545A>G mutation. The 13 novel mutations of FKRP significantly expanded the mutation spectrum of MDC1C and LGMD2I, and the different founder mutations indicate the ethnic difference in FKRPmutations.