Search

Background: The pathogenic variation of CASK gene can cause CASK related mental disorders. The main clinical manifestations are microcephaly with pontine and cerebellar hypoplasia, X-linked mental disorders with or without nystagmus and FG syndrome. The main pathogenic mechanism is the loss of function of related protein caused by variant. We reported a Chinese male newborn with a de novo variant in CASK gene. Case presentation: We present an 18-day-old baby with growth retardation and brain hypoplasia. Whole-exome sequencing was performed, which detected a hemizygous missense variant c.764G > A of CASK gene. The variant changed the 255th amino acid from Arg to His. Software based bioinformatics analyses were conducted to infer its functional effect. Conclusions: In this paper, a de novo variant of CASK gene was reported. Moreover, a detailed description of all the cases described in the literature is reported. CASK variants cause a variety of clinical phenotypes. Its diagnosis is difficult due to the lack of typical clinical symptoms. Genetic testing should be performed as early as possible if this disease is suspected. This case provides an important reference for the diagnosis and treatment of future cases. [ABSTRACT FROM AUTHOR]

• A novel mathematical model of the transmission path effect is proposed. • Path effect depends only on the geometric parameters and material properties. • A phenomenological vibration model is used to simulate all vibration sources. • Vibration characteristics are analyzed and validated through experiments. Model-based vibration signal analysis to interpret and understand vibration characteristics can provide prior guidance for the condition monitoring and fault diagnosis of planetary gearboxes (PGs). Transmission path effects due to the rotational motion of planet gears with respect to the accelerometer mounted on the PG housing make understanding its real dynamic behavior challenging. To overcome this challenge, a novel mathematical model of the transmission path effect is proposed to support the construction of more realistic dynamic response of PGs. This model is derived only from the geometric parameters and material properties, and it has higher applicability than traditional models. Subsequently, a phenomenological vibration model is used to simulate all vibration sources, including sun–planet and ring–planet gear meshes. On these bases, a comprehensive vibration signal model is proposed by considering all vibration sources and the corresponding transmission path effects. Moreover, the theoretical spectral structure is deduced via Fourier series analysis. Finally, the vibration characteristics of the vibration signals obtained using the proposed mathematical model are analyzed and validated through simulations and experiments in the time and frequency domains. [ABSTRACT FROM AUTHOR]

• An improved phenomenological model of a gear pair with local fault is proposed. • Relative phases of planetary gearboxes are calculated by a modified phase model. • The local fault characteristic frequencies of gears are deduced based on models. • The conclusions are validated based on five experiments conducted on a test rig. Planetary gearboxes (PGs) are prone to gear local faults, such as cracking, pitting, and spalling, because of their difficult working environment. These faults may lead to significant capital losses and catastrophic events. Numerous phenomenological modulation models of vibration signals of PGs have been proposed for diagnosis purposes. However, local fault diagnosis remains extremely challenging due to two aspects: (1) Most vibration modulation models cannot reflect the local fault-induced pulse modulation characteristic. (2) Studies on local fault characteristic frequencies (LFCFs) of gears lack a unified mechanism explanation. To solve these problems, an improved phenomenological vibration model of a gear pair with local fault is first proposed. This model can reflect the pulse modulation characteristic. Then, relative phases between multiple gear pairs in the PG are calculated based on a modified phase model. Based on these, phenomenological vibration models of PGs with/without local faults are established, spectral structures are derived using Fourier series analysis, and LFCFs are deduced for local fault diagnosis. Simulation and experimental studies demonstrate the effectiveness of the proposed models, which exhibit higher descriptive capability than traditional models. [ABSTRACT FROM AUTHOR]

• A mathematical vibration model of multistage wind turbine gearboxes is proposed. • Modulation sidebands in the vibration spectrum are explained based on the model. • The equivalent transmission path functions in the multistage gearbox are proposed. • The conclusions are validated based on data from 2.0MW wind turbine gearboxes. The sensor-perceived vibration signals of multistage wind turbine gearboxes have more complex frequency features than that of single-stage gearboxes since there are more meshing vibration sources and more complicated transmission paths inside them, which lead to difficulties in condition monitoring and fault diagnosis. Aiming to understand the vibration frequency features of multistage wind turbine gearboxes, a mathematical vibration model of healthy wind turbine gearboxes is developed and the spectral structure of the model is deduced by adopting Fourier series analysis. In the vibration model, the time-varying transmission paths from all the meshing vibration sources to the fixed sensor are considered, and the equivalent transmission path functions of the time-varying transmission paths are proposed for the first time. The theoretical derivations are validated by both simulations and engineering tests using two cases of 2.0MW industrial wind turbine gearboxes. The findings in this work are not only applicable to the gearboxes studied in this paper, but also to other similar multistage gearboxes, which can provide a priori spectral structure for the multistage gearbox to assist its condition monitoring and fault diagnosis. [ABSTRACT FROM AUTHOR]

Si-based anodes are promising to be used in next-generation lithium ion batteries (LIBs), yet limited by low electrical conductivity and pulverization during cycling. The challenge is the simultaneous improvement of electrical conductivity and mechanical stability of the anodes under practical significance. Here, we constructed a ternary conductive 3D network based on hydroxyl-functionalized whisker-like carbon nanofibers (BH). We demonstrate that the hydrogen bond formed at the interactions between SiO x and the ternary conductive network can not only enhance electrode conductivity for the improved capacities and rate performance, but also increase interfacial adhesion strength for good structural stability. The ternary conductive 3D network constructed by BH, Super P, and SWCNTs at the optimal mass ratio of 2.95:7:0.05 enables the SiO x @C/graphite anode (10:90) a high initial discharge specific capacity (565 mAh g−1 at 0.1 C), high rate performance (320.1 mAh g−1 at 0.1 C), and outstanding cycling performance (381.9 mAh g−1 after 100 cycles at 0.2 C). Even the neat SiO x @C electrode with the optimized ternary conductive network delivers stable capacities of 1753.5 mAh g−1 at 0.5 C and 622.5 mAh g−1 at 10 C. The delicate design of 3D conductive networks in this work demonstrates great potential for the application of Si-based anodes in commercial LIBs with high energy densities. • WCNFs were collaboratively prepared through CVD and graphitization treatment. • Enhanced capacities and superior rate performance were attained through the integration of BH, Super P, and SWCNTs. • Ternary conductive agent can increase interfacial adhesion strength for good structural stability. • Suitable framework can buffer the volume expansion of SiO x and inhibit the pulverization during cycling process. [ABSTRACT FROM AUTHOR]

Epilepsy, a prevalent neurological disorder, necessitates precise and reliable electrophysiological recording for accurate study and diagnosis. Although traditional stereo‐electroencephalography and micro‐wire probes are widely used in epilepsy research, they are limited by recording site numbers and mechanical properties. This study explores the use of high‐density, ultra‐flexible neural probes in epilepsy monitoring, highlighting their advantages in terms of recording performance, scalability, and tissue compatibility. This work validates the effectiveness of the probes in detecting characteristic epileptic signals across various stages, including resting, preictal, and ictal stages, in two different mouse models of epilepsy. Additionally, the high spatial resolution of the probes allows to capture fine spatial propagation of epilepsy‐associated high‐frequency oscillations. Furthermore, the flexible probes exhibit superior biocompatibility, reducing inflammation and neuronal apoptosis compared to rigid electrodes. The results underscore the promising potential of ultra‐flexible neural probes for advancing epilepsy research, providing a powerful technological platform for future studies in the field. [ABSTRACT FROM AUTHOR]

Pre-lithiated multiwalled carbon nanotube anode was prepared by internal short circuit approach(ISC) for 5min, 30min, 60min and 120min respectively. Lithium ion capacitors (LICs) were assembled by using pre-lithiated multiwalled carbon nanotubes as anodes and activated carbon (AC) as cathodes. The structure of multiwalled carbon nanotubes and electrodes were investigated by scanning electron microscopy (SEM) and transmission electron microscopy (TEM). The electrochemical performance of pre-lithiated multiwalled carbon nanotube electrodes and pristine carbon nanotube electrodes were tested by galvanostatic charge/discharge and electrochemical impedance. The results indicated that pre-lithiation carbon nanotubes greatly improved the charge/discharge performance of LICs. The energy density was four times than conventional electric double-layer capacitors (EDLCs) at the current density of 100mA/g. The LICs achieved a specific capacitance of 59.3F/g at the current density of 100mA/g with 60min pre-lithiatiation process. The maximum energy density and power density was 96Wh/kg and 4035W/kg, respectively. The energy density still remained about 89.0% after 1000 cycles. The LIC showed excellent supercapacitor performance. Pre-lithiated multiwalled carbon nanotubes and activated carbon (AC) materials were used as anode and cathode respectively for lithium-ion capacitors (LICs). The pre-lithiatiation was performed using internal short circuit approach (ISC). The LIC showed excellent supercapacitor performance. The pre-lithiated MWCNTs have a potential application as anode for high performance lithium-ion capacitors. [ABSTRACT FROM AUTHOR]

Unlike thermoplastic petroleum-based materials, starch-based materials rely on aqueous systems but are incapable of hot melting, resulting in low processing efficiency and limited large-scale industrial applications. In this study, the combination of α-amylase liquefaction and urea plasticization was used for the first time to obtain enzymatic thermoplastic starch (ETPS) for hot melting by changing the molecular chain of starch. ETPS showed an apparent hot melting phenomenon when heated below 200 °C. Differential scanning calorimetry revealed that heat absorption peaks were obviously reduced, and the hot melting phenomenon occurred easily depending on the combination of enzymatic hydrolysis and plasticization. Dynamic mechanical analysis indicated that the combined modification effectively increased the number of freely movable chains. The red shift of -OH stretching vibration peaks indicated the formation of strengthened hydrogen bonds in ETPS. X-ray diffraction showed that the crystallinity of ETPS was reduced to 5.68 %, effectively reducing the regenerative phenomenon. Gel permeation chromatography revealed that the molecular weight of ETPS decreased, and the entanglements between molecular chains were reduced. A tensile test showed that the elongation at break of ETPS was as high as 235.29 %, which was much higher than those of enzymatic hydrolysis starch and thermoplastic starch. [Display omitted] [ABSTRACT FROM AUTHOR]

This article, published in Advanced Materials Technologies, discusses the use of flexible neural probes for high-resolution recording of neural activity in epilepsy. The cover image showcases a flexible electrode positioned within the brain, surrounded by neurons. The electrodes are designed to capture the signals transmitted by neurons during epileptic seizures, providing valuable insights into the intricate processes involved. The article, authored by Qiangqiang Liu, Tiger H. Tao, Liuyang Sun, and their colleagues, provides more detailed information on this topic. [Extracted from the article]

Multiwalled carbon nanotubes have been synthesized by floating reactant method in a vertical tubular reactor. An internal short approach process was developed to achieve the pre-lithiated multiwalled carbon nanotube MWCNTs/graphite composite anodes. Lithium-ion capacitors (LICs) were composed of a pre-lithiated multiwalled carbon nanotubes/graphite composite anode and an activated carbon (AC) cathode. The electrochemical performance of LICs with different MWCNTs content was investigated through galvanostatic charge/discharge and electrochemical impedance. The results show that the charge-discharge performance of LICs was greatly improved with the addition of MWCNTs in anodes. LIC25 (25 wt% MWCNTs) was found to achieve the optimal electrochemical performance. The LIC25 hold a specific capacitance of 58.2 F/g at the current density of 100 mA/g. The maximum energy density and power density were up to 96 Wh/kg and 10.1 kW/kg, respectively, in the current range of 100~8000 mA/g. The continuous galvanostatic charge-discharge cycling tests revealed that the LIC25 maintained excellent capacity retention of 86% after 3000 cycles. [ABSTRACT FROM AUTHOR]

In this investigation, multiwalled carbon nanotube (MWCNT) paper consists of MWCNTs and cellulose was fabricated by traditional paper-making method. It was applied directly as negative electrode in flexible lithium ion battery to replace ordinary electrode which is combined with anode material and current collector. The electrochemical performances of the as-produced MWCNT paper (AMP) and carbonized MWCNT paper (CMP) were evaluated in this study. The morphology and structure of the MWCNT papers were observed by scanning electron microscopy (SEM). The electrochemical performance of the battery was operated by cell test and electrochemical impedance spectroscopy (EIS) measurement. The charging and discharging results indicated that the CMP behaves with higher capacity than AMP. And the EIS analysis showed that a lower charge transfer resistance can be obtained in the CMP. The excellent electrochemical performance verifies the feasibility of MWCNT papers as a promising candidate for the anode in flexible lithium ion battery. Multiwalled carbon nanotube (MWCNT) paper consists of MWCNTs and cellulose was fabricated by traditional paper-making method. MWCNT paper can be directly used as electrode, and the current collector in LIB. The carbonized MWCNT paper, which turned the cellulose to amorphous carbon, has even better performance when applied as electrode. [ABSTRACT FROM AUTHOR]

A kind of flexible carbon nanotubes (CNTs) paper was investigated. Cellulose fibers were used as matrix and CNTs were used as conductive additive. The CNT papers were made by suction filtration method. The size and conductivity of the CNT paper can be arbitrarily tailored. The CNT papers show good electrical conductivity, electrochemical and electromagnetic interference (EMI) shielding properties. The electrical conductivity and (EMI) shielding effectiveness (SE) of CNT papers were enhanced with an addition of CNTs in the paper. The electrical conductivity increased from 9.92 S m −1 to 216.3 S m −1 and SE improved from 15 dB to 45 dB with a loading of CNTs from 10 wt% to 71 wt%. The CNT papers also show excellent applications for lithium ion battery, supercapacitors and zinc-manganese batteries. The testing results show that the capacity of supercapacitor reached 46 F/g at a scan rate of 5 mV/s. The reversible discharge capacity reached 474.0 mAh/g with the paper as anode current collector of lithium ion battery. The discharge capacity of Zn–Mn battery increased 100% when CNT paper replaced the graphite collector as current collector. [ABSTRACT FROM AUTHOR]

This study attempted to investigate the effect of coating porosity on the ability to protect the substrate from corrosion and the combined action mechanism of corrosion and erosion wear. In this study, five coatings with different porosity characteristics were deposited on the substrate. The microstructures and phase composition of coatings were characterized. Furthermore, corrosion and erosion wear behavior of coatings were analyzed. The erosion wear patterns of uncorroded and corroded coatings were revealed. The mechanism that erosion wear was influenced through corrosion was determined. Results indicated that corrosion and erosion wear resistance were remarkably affected by porosity. The unsealed cermet failed to protect the substrate and aggravated the corrosion of the substrate through galvanic corrosion. Porosity affected the microcutting performance by influencing hardness, whereas corrosion affected the microcutting and deformation wear performance by acting on hardness, resulting in the loosened structure. The main erosion mechanisms of uncorroded and corroded coatings were microcutting and deformation wear, respectively. Deformation wear was induced by corrosion, and microcutting. The qualitative analysis of the combined action of corrosion and erosion can provide guidance in coating design. • Unsealed coating promoted substrate galvanic corrosion, not surface corrosion. • Revealed negative effects of porosity on erosion and corrosion resistance • Revealed erosion wear patterns of corroded and uncorroded coatings • Revealed the mechanism that erosion wear affected by corrosion [ABSTRACT FROM AUTHOR]