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Abstract Background Fabry disease is an X-linked lysosomal storage disease caused by the impairment of α-galactosidase A. The complex intronic haplotype (CIH) variants, located in promoter and intronic regulatory lesions, has been found in patients with classical forms of Fabry disease. We present a case of Fabry disease in female monozygotic twins exhibiting the CIH mutation and classical manifestations. Case presentation A 61-year-old woman with a history of stroke, carotid artery occlusion, hypertrophic cardiomyopathy, and chronic kidney disease was referred to the nephrology clinic for management of her chronic kidney disease. Her monozygotic twin sister also presented with hypertrophic cardiomyopathy, atrial flutter, carotid stenosis, and proteinuria. Clinical symptoms and a comprehensive family history strongly suggested the presence of Fabry disease. Genetic analysis revealed the presence of 5 variants within a complex intronic haplotype (CIH): c.−10 C > T, c.369 + 990 C > A, c.370 − 81_370–77delCAGCC, c.640–16 A > G, and c.1000–22 C > T. We conducted a review of the patient’s previous kidney biopsy findings, which demonstrated the presence of lamellated inclusion bodies in electron microscopy. Remarkably, both the monozygotic twin sister and her son exhibited the same genetic mutation. Enzyme replacement therapy was initiated for the patient. Her kidney function decreased throughout a thorough 2-year follow-up period, while there was a slight decrease in the left ventricular mass index. Conclusions This is the first reported case of female monozygotic twins with the CIH variants representing cardiac, cerebrovascular, and renal manifestations suggestive of Fabry disease.

Serious climate changes and energy-related environmental problems are currently critical issues in the world. In order to reduce carbon emissions and save our environment, renewable energy harvesting technologies will serve as a key solution in the near future. Among them, triboelectric nanogenerators (TENGs), which is one of the most promising mechanical energy harvesters by means of contact electrification phenomenon, are explosively developing due to abundant wasting mechanical energy sources and a number of superior advantages in a wide availability and selection of materials, relatively simple device configurations, and low-cost processing. Significant experimental and theoretical efforts have been achieved toward understanding fundamental behaviors and a wide range of demonstrations since its report in 2012. As a result, considerable technological advancement has been exhibited and it advances the timeline of achievement in the proposed roadmap. Now, the technology has reached the stage of prototype development with verification of performance beyond the lab scale environment toward its commercialization. In this review, distinguished authors in the world worked together to summarize the state of the art in theory, materials, devices, systems, circuits, and applications in TENG fields. The great research achievements of researchers in this field around the world over the past decade are expected to play a major role in coming to fruition of unexpectedly accelerated technological advances over the next decade.

Alkoxide precursors have been highlighted for depositing carbon-free films, but their use in Atomic Layer Deposition (ALD) often exhibits a non-saturated growth. This indicates no self-limiting growth due to the chain reaction of hydrolysis or ligand decomposition caused by β-hydride elimination. In the previous study, we demonstrated that self-limiting growth of ALD can be achieved using our newly developed precursor, hafnium cyclopentadienyl tris(N-ethoxy-2,2-dimethyl propanamido) [HfCp(edpa)3]. To elucidate the growth mechanism and the role of cyclopentadienyl (Cp) ligand in a heteroleptic alkoxide precursor, herein, we compare homoleptic and heteroleptic Hf precursors consisting of N-ethoxy-2,2-dimethyl propanamido (edpa) ligands with and without cyclopentadienyl ligand—hafnium tetrakis(N-ethoxy-2,2-dimethyl propanamido) [Hf(edpa)4] and HfCp(edpa)3. We also investigate the role of a Cp ligand in growth characteristics. By substituting an alkoxide ligand with a Cp ligand, we could modify the surface reaction during ALD, preventing undesired reactions. The last remaining edpa after Hf(edpa)4 adsorption can undergo a hydride elimination reaction, resulting in surface O–H generation. In contrast, Cp remains after the HfCp(edpa)3 adsorption. Accordingly, we observe proper ALD growth with self-limiting properties. Thus, a comparative study of different ligands of the precursors can provide critical clues to the design of alkoxide precursors for obtaining typical ALD growth with a saturation behavior. [ABSTRACT FROM AUTHOR]

Electro‐ionic artificial muscles, consisting of a configuration where an electrolyte membrane is flanked by two active electrodes, have emerged as transformative components in the field of soft robotics. Despite this, the current actuation performance falls short for many practical applications, because most existing electrode materials exhibit limitations in terms of their properties. Here, a multifunctional active electrode material is reported for an electro‐ionic artificial muscle, employing a triazine framework that incorporates 6,6′‐Dicyano‐2,2′‐bipyridyl (DCB‐TF). The multifunctional DCB‐TF boasts fully conjugated bonds, facilitating fast electron transfer, high porosity for ion accommodation, a substantial surface area of 1800 m2 g−1 for charge storage, numerous active sites for ion interactions, and complete aromatic rings contributing to stability. The application of DCB‐TF to the active electrodes in the electro‐ionic artificial muscles yields remarkable actuation performance, including a substantial bending displacement of 24 mm at 0.5 V and 0.2 Hz, a rapid response time of 2 s, and outstanding durability sustained over 3.4 million continuous cycles. Consequently, these soft actuators have enabled a dragonfly demonstration as a form of kinetic art. [ABSTRACT FROM AUTHOR]

MXenes are promising passive components that enable lithium‐sulfur batteries (LSBs) by effectively trapping lithium polysulfides (LiPSs) and facilitating surface‐mediated redox reactions. Despite numerous studies highlighting the potential of MXenes in LSBs, there are no systematic studies of MXenes' composition influence on polysulfide adsorption, which is foundational to their applications in LSB. Here, a comprehensive investigation of LiPS adsorption on seven MXenes with varying chemistries (Ti2CTx, Ti3C2Tx, Ti3CNTx, Mo2TiC2Tx, V2CTx, Nb2CTx, and Nb4C3Tx), utilizing optical and analytical spectroscopic methods is performed. This work reports on the influence of polysulfide concentration, interaction time, and MXenes' chemistry (transition metal layer, carbide and carbonitride inner layer, surface terminations and structure) on the amount of adsorbed LiPSs and the adsorption mechanism. These findings reveal the formation of insoluble thiosulfate and polythionate complex species on the surfaces of all tested MXenes. Furthermore, the selective adsorption of lithium and sulfur, and the extent of conversion of the adsorbed species on MXenes varied based on their chemistry. For instance, Ti2CTx exhibits a strong tendency to adsorb lithium ions, while Mo2TiC2Tx is effective in trapping sulfur by forming long‐chain polythionates. The latter demonstrates a significant conversion of intermediate polysulfides into low‐order species. This study offers valuable guidance for the informed selection of MXenes in various functional components benefiting the future development of high‐performance LSBs. [ABSTRACT FROM AUTHOR]

In this study, the effects of Al2O3 and Y2O3 passivation layers on Ge substrates are investigated to enhance the thermal stability of Ge-based devices. Using X-ray photoelectron spectroscopy, we analyze the growth characteristics and chemical composition of Al2O3, Y2O3, and ZrO2 on Ge. The changes in the crystallinity of ZrO2 on different substrates of Ge, Al2O3/Ge, and Y2O3/Ge configurations are observed via X-ray diffraction. Material properties, including capacitance, flat band voltage shift (ΔVFB), oxide charge trap (Not), interface defect density (Dit), and leakage current, are analyzed using the metal–oxide–semiconductor capacitor, with a particular focus on their electrical characteristics. Additionally, we investigate whether the passivation mechanisms of each material are more suitable for enhancing thermal stability. Overall, this study provides insight into the role of passivation layers in improving the interface and thermal stability of Ge-based devices, offering valuable contributions to the advancement of semiconductor technology. [ABSTRACT FROM AUTHOR]

Metal‐organic frameworks (MOFs) are nanomaterials with engineered chemical structures, offering remarkable properties. However, their limited film‐formation capability hinders their integration into triboelectric nanogenerators (TENGs). This study proposes a simple yet effective solution to overcome this challenge by employing electrospinning techniques to integrate the zeolitic imidazolate framework (ZIF‐8) into an easy‐to‐use nanofibrous mat. ZIF‐8 has high surface potential, a unique cubical structure, and an easy fabrication process that makes it an ideal material for TENGs. By incorporating ZIF‐8 into the electrospinning solution, significant improvements are achieved in the electropositivity of the resulting nanofibers. It leads to notable changes in the shape, morphology, and roughness of electrospun fibers, consequently enhancing the overall performance of the TENG. The results indicate that utilizing the ZIF‐based electrospun mat as a tribo‐positive material can increase transferred charges between electrodes by more than 100%. Utilizing the MOF‐based nanofibrous mat, this study also introduces a novel rotary TENG that works based on a mode of TENG operation called rolling mode. The reliable charge generation by the proposed rolling system reveals that this mode of TENG operation could be a superb alternative for traditional TENG modes, like contact/separation or sliding, which cause high levels of mechanical stress due to harsh physical impact or friction. [ABSTRACT FROM AUTHOR]

In an era marked by increasing environmental challenges affecting human well‐being, traditional acoustic materials struggle to effectively handle the diverse and multi‐frequency nature of harmful environmental noises. This has spurred a demand for innovative acoustic metamaterial solutions by utilizing sustainable design strategies. This research introduces tunable Schwarz metamaterial capable of transforming into a soft meta‐foam to solve the complex problems of varying environmental noises. This study primarily focuses on adjusting single to multiple sound‐blocking bandgaps mechanism using a multi‐layered approach, incorporating the Schwarz P‐type triply periodic minimal surface (TPMS) and its elective soft foam counterpart, known as tunable Schwarz meta‐foams (TSMF‐x). The tunable design parameters of the unit cell, multi‐layered TPMS, and soft programmable TSMF‐lichen version are comprehensively explored including a fire‐safety test. The results demonstrate these enhanced flame retardant meta‐foam families have the potential to be used for mid‐to‐high‐frequency environmental noises in industrial equipment and smart homes for sustainable architecture and environmental health applications. [ABSTRACT FROM AUTHOR]

Lithium metal batteries (LMBs) and anode‐free LMBs (AFLMBs) present a solution to the need for batteries with a significantly superior theoretical energy density. However, their adoption is hindered by low Coulombic efficiency (CE) and rapid capacity fading, primarily due to the formation of unstable solid electrolyte interphase (SEI) layer and Li dendrite growth as a result of uneven Li plating. Here, we report on the use of a stoichiometric Ti3C2Tx (S‐Ti3C2Tx) MXene coating on the copper current collector to enhance the cyclic stability of an anode‐free lithium metal battery. The S‐Ti3C2Tx coating provides abundant nucleation sites, thereby lowering the overpotential for Li nucleation, and promoting uniform Li plating. Additionally, the fluorine (−F) termination of S‐Ti3C2Tx participates in the SEI formation, producing a LiF‐rich SEI layer, vital for stabilizing the SEI and improving cycle life. Batteries equipped with S‐Ti3C2Tx@Cu current collectors displayed reduced Li consumption during stable SEI formation, resulting in a significant decrease in capacity loss. AFLMBs with S‐Ti3C2Tx@Cu current collectors achieved a high initial capacity density of 4.2 mAh cm−2, 70.9% capacity retention after 50 cycles, and an average CE of 98.19% in 100 cycles. This innovative application of MXenes in the energy field offers a promising strategy to enhance the performance of AFLMBs and could potentially accelerate their commercial adoption. [ABSTRACT FROM AUTHOR]

Considerable research has been conducted on the application of functional nano‐fillers to enhance the power generation capabilities of triboelectric nanogenerators (TENGs). However, these additives often exhibit a decrease in output power at higher concentration. Here, a Janus cobalt metal–organic framework‐SEBS (JCMS) membrane is reported as a dual‐purpose dielectric layer capable of efficiently capturing and blocking charges for high‐performance TENGs. The JCMS is produced asymmetrically through gravitational sedimentation, employing spherical CoMOFs within a diluted SEBS solution. Beyond its dual dielectric characteristics, the JCMS showcases exceptional mechanical durability, displaying notable stretchability of up to 475% and remarkable resilience when subjected to diverse mechanical pressures. Consequently, the JCMS‐TENG produces a maximum peak‐to‐peak voltage of 936 V, a current of 42.8 µA, and a power density of 10.89 W m−2 when exposed to an external force of 10 N at a 5 Hz frequency. This investigation highlights the potential of JCMS‐TENGs with unique structures, known for their exceptional energy harvesting capabilities, mechanical strength, and flexibility. Additionally, the promising prospects of easily produced asymmetric structures is emphasized with bifunctionalities for developing efficient and flexible MOFs‐based TENGs. These advancements are well‐suited for self‐powered wearables, rehabilitation devices, and energy harvesters. [ABSTRACT FROM AUTHOR]