bluuebunny/crossref_metadata_2025_split huggingface dataset:
ATXN7-Related Cone-Rod Dystrophy
Authors: Nassisi Marco , Coarelli Giulia , Blanchard Benoit , Dubec-Fleury Charlotte , Drine Karima , Kitic Nicolas , Sancho Serge , Hilab Rania , Tezenas du Montcel Sophie , Junge Candice , Lane Roger , Arnold H. Moore , Durr Alexandra , Audo Isabelle 
Published: February 2024

ImportanceReliable biomarkers with diagnostic and prognostic values are needed for upcoming gene therapy trials for spinocerebellar ataxias.ObjectiveTo identify ophthalmological biomarkers in a sample of spinocerebellar ataxia type 7 (SCA7) carriers.Design, Setting, and ParticipantsThis article presents baseline data from a cross-sectional natural history study conducted in Paris, France, reference centers for rare diseases from May 2020 to April 2021. Data were analyzed from September to December 2022. Fifteen adult ATXN7 pathogenic expansion carriers (9 with preataxia and 6 with ataxia) were included, all with a Scale for the Assessment and Rating of Ataxia (SARA) score of 15 of 40 or lower. Patients were recruited at the Paris Brain Institute, and all contacted patients accepted to participate in the study.Main Outcomes and MeasuresThree visits (baseline, 6 months, and 12 months) were planned, including neurological examination (SARA and Composite Cerebellar Functional Severity Score), ophthalmological examination (best-corrected visual acuity, microperimetry, full-field electroretinogram, optical coherence tomography, and fundus autofluorescence imaging), and neurofilament light chain (NfL) measurements. Here we report the baseline ophthalmic data from the cohort and determine whether there is a correlation between disease scores and ophthalmic results.ResultsAmong the 15 included SCA7 carriers (median [range] age, 38 [18-60] years; 8 women and 7 men), 12 displayed cone or cone-rod dystrophy, with the number of CAG repeats correlating with disease severity (ρ, 0.73, 95% CI, 0.34 to 0.90; P &amp;lt; .001). Two patients with cone-rod dystrophy exhibited higher repeat numbers and greater ataxia scores (median [range] SARA score, 9 [7-15]) compared to those with only cone dystrophy (median [range] SARA score, 2 [0-5]). A correlation emerged for outer nuclear layer thickness with SARA score (ρ, −0.88; 95% CI, −0.96 to −0.59; P &amp;lt; .001) and NfL levels (ρ, −0.87; 95% CI, −0.86 to 0.96; P &amp;lt; .001). Moreover, ataxia severity was correlated with visual acuity (ρ: 0.89; 95% CI, 0.68 to 0.96; P &amp;lt; .001) and retinal sensitivity (ρ, −0.88; 95% CI, −0.96 to 0.59; P &amp;lt; .001).Conclusions and RelevanceIn this cross-sectional study, retinal abnormalities were found at preataxic stages of the disease. Most of the carriers presented with cone dystrophy and preserved rod function. The outer nuclear layer thickness correlated with SARA score and plasma NfL levels suggesting nuclear layer thickness to be a biomarker of disease severity. These findings contribute to understanding the dynamics of SCA7-related retinal dystrophy and may help lay the groundwork for future therapeutic intervention monitoring and clinical trials.Trial RegistrationClinicalTrials.gov Identifier: NCT04288128
Stem End Rot Infection of Avocado (
Persea
Americana
)
Authors: Fostvedt Jessica E. , Dicks Lynn , Wagstaff Carol 
Published: February 2024

Abstract

Stem end rot (SER) is the name given to a post‐harvest rot disorder that manifests as internal browning and degradation of the tissues inside the fruit of avocado. It has commercial significance, causing up to 30% of a grower's yield to be negated. Symptom development is often only apparent when the fruit are opened by the consumer, resulting in complaints to the retailer, and consequent lost revenue along the supply chain. The disorder is proposed to be the consequence of infection by a number of pathogens including,
Colletotrichum
,
Botryosphaeria
, and
Fusarium
species and is exacerbated by orchard location, management, and environmental conditions. The exact mechanism and timing of infection is not clear although there is evidence for infection at flowering, during fruit development, and at harvest. An understanding of the infection routes of pathogens causing SER could guide effective mitigation strategies and this article explores how greater mechanistic insight into the route of infection of causative pathogens might be determined. Improvements that could be made to orchard management and at harvest/post‐harvest handling of the fruit to reduce SER development are also discussed.
Exploring the Impact of TLR‐2 Signaling on miRNA Dysregulation in Intervertebral Disc Degeneration
Authors: Cazzanelli Petra , Lamoca Mikkael , Hausmann Oliver Nic , Mesfin Addisu , Puvanesarajah Varun , Hitzl Wolfgang , Haglund Lisbet , Wuertz‐Kozak Karin 
Published: February 2024

AbstractToll‐like receptors (TLRs) are key mediators of inflammation in intervertebral disc (IVD) degeneration. TLR‐2 activation contributes to the degenerative process by increasing the expression of extracellular matrix‐degrading enzymes, pro‐inflammatory cytokines, and neurotrophins. As potent post‐transcriptional regulators, microRNAs can modulate intracellular mechanisms, and their dysregulation is known to contribute to numerous pathologies. This study aims to investigate the impact of TLR‐2 signaling on miRNA dysregulation in the context of IVD degeneration. Small‐RNA sequencing of degenerated IVD cells shows the dysregulation of ten miRNAs following TLR‐2 activation by PAM2CSK4. The miR‐155‐5p is most significantly upregulated in degenerated and non‐degenerated annulus fibrosus and nucleus pulposus cells. Sequence‐based target and pathway prediction shows the involvement of miR‐155‐5p in inflammation‐ and cell fate‐related pathways and TLR‐2‐induced miR‐155‐5p expression leads to the downregulation of its target c‐FOS. Furthermore, changes specific to the activation of TLR‐2 through fragmented fibronectin are seen in miR‐484 and miR‐487. Lastly, miR‐100‐3p, miR‐320b, and miR‐181a‐3p expression exhibit degeneration‐dependent changes. These results show that TLR‐2 signaling leads to the dysregulation of miRNAs in IVD cells as well as their possible downstream effects on inflammation and degeneration. The identified miRNAs provide important opportunities as potential therapeutic targets for IVD degeneration and low back pain.
Biomimetic and Durably Superhydrophobic Nanofibrous Membranes for High‐Performance Waterproof and Breathable Textiles
Authors: Gong Xiaobao , Ji Yu , Liu Xiaoyan , Yu Jianyong , Zhang Shichao , Ding Bin 
Published: February 2024

AbstractMan‐made superhydrophobic membranes are of interest for various applications in energy, environment, and medical fields. However, simultaneously achieving durable superhydrophobicity, robust waterproofness, and high breathability of the membranes is still challenging. Herein, an efficient and powerful strategy is reported to create superhydrophobic membranes with perdurable liquid repellency and robust breathability via combining humidity‐induced electrospinning with coat‐crosslinking technology. The micro/nano rough structure is in situ constructed based on manipulating the phase separation of electrospun jets; meanwhile, the waterborne acrylic resins are bridged to the surface of nanofiber to endow the membranes with stable low surface energy via coating and crosslinking treatment. Eventually, the resultant membranes present superhydrophobicity with a water contact angle of ≈154°, good self‐cleaning and anti‐icing properties, robust waterproofness of 83.4 kPa, and high breathability of 3.71 kg m−2 d−1. More interestingly, the nanomaterials can maintain durable superhydrophobicity even after exposure to various severe conditions. The successful creation of high‐performance superhydrophobic membranes opens a new avenue for synthesizing advanced functional materials for application in a variety of fields.
Gradient‐Tuned VO4 Vacancies in BiVO4 Photoanode for Boosting Bulk Hole Transport and Oxygen Evolution Reaction Performance
Authors: Pei Hao , Peng Lingling , Jiang Zhuo , Zhang Yuexing , Li Renjie , Peng Tianyou 
Published: February 2024

AbstractAlthough bismuth vanadate (BiVO4) photoanode has been widely used, the solar‐driven oxygen evolution reaction (OER) performance is constrained due to the substantial bulk recombination and poor mobility of charge carriers. Herein, a facile solvothermal post‐treatment approach that employs N,N‐dimethylformamide (DMF) is developed to induce a VO4 vacancy gradient from bulk to surface of BiVO4 film, which not only improves the charge diffusion but also establishes an advantageous upward band gradient to promote the bulk hole transport. Under Air Mass 1.5 Global (AM1.5G) simulated solar illumination, the optimized BiVO4 photoanode with a VO4 vacancy gradient (denoted as B(VO)1‐δ) exhibits excellent OER performance with a charge separation efficiency of 74.4% at 1.23 V versus reversible hydrogen electrode (RHE) and incident‐photon‐to‐current conversion efficiency of 30.7% at 445 nm, 1.2 and 1.4 times higher than that of the pristine BiVO4, respectively. After loading nickel‐iron hydroxyl oxide (NiFeOOH) as a cocatalyst, the photocurrent density of B(VO)1‐δ escalates to 5.92 mA cm−2 in a hole scavenger (Na2SO3) solution and 5.07 mA cm−2 in a potassium borate buffer solution at 1.23 V versus RHE, far superior to the pristine BiVO4. This work highlights that the gradient‐tuned VO4 vacancies can effectively modulate the bulk band structure and charge transfer in BiVO4 photoanode, providing a new strategy for boosting solar water splitting performance.
Flat‐Knit, Flexible, Textile Metasurfaces
Authors: Carter Michael J. , Resneck Leah , Ra'di Younes , Yu Nanfang 
Published: February 2024

AbstractLightweight, low‐cost metasurfaces and reflectarrays that are easy to stow and deploy are desirable for many terrestrial and space‐based communications and sensing applications. This work demonstrates a lightweight, flexible metasurface platform based on flat‐knit textiles operating in the cm‐wave spectral range. By using a colorwork knitting approach called float‐jacquard knitting to directly integrate an array of resonant metallic antennas into a textile, two textile reflectarray devices, a metasurface lens (metalens), and a vortex‐beam generator are realized. Operating as a receiving antenna, the metalens focuses a collimated normal‐incidence beam to a diffraction‐limited, off‐broadside focal spot. Operating as a transmitting antenna, the metalens converts the divergent emission from a horn antenna into a collimated beam with peak measured directivity, gain, and efficiency of 21.30, 15.30, and −6.00 dB (25.12%), respectively. The vortex‐beam generating metasurface produces a focused vortex beam with a topological charge of m = 1 over a wide frequency range of 4.1–5.8 GHz. Strong specular reflection is observed for the textile reflectarrays, caused by wavy yarn floats on the backside of the float‐jacquard textiles. This work demonstrates a novel approach for the scalable production of flexible metasurfaces by leveraging commercially available yarns and well‐established knitting machinery and techniques.
Reduction of 5‐Hydroxymethylfurfural to 2,5‐Bis(hydroxymethyl)Furan at High Current Density using a Ga‐Doped AgCu:Cationomer Hybrid Electrocatalyst
Authors: Tian Cong , Yu Jiaqi , Zhou Daojin , Ze Huajie , Liu Hengzhou , Chen Yuanjun , Xia Rong , Ou Pengfei , Ni Weiyan , Xie Ke , Sargent Edward H. 
Published: February 2024

AbstractHydrogenation of biomass‐derived chemicals is of interest for the production of biofuels and valorized chemicals. Thermochemical processes for biomass reduction typically employ hydrogen as the reductant at elevated temperatures and pressures. Here, the authors investigate the direct electrified reduction of 5‐hydroxymethylfurfural (HMF) to a precursor to bio‐polymers, 2,5‐bis(hydroxymethyl)furan (BHMF). Noting a limited current density in prior reports of this transformation, a hybrid catalyst consisting of ternary metal nanodendrites mixed with a cationic ionomer, the latter purposed to increase local pH and facilitate surface proton diffusion, is investigated. This approach, when implemented using Ga‐doped Ag‐Cu electrocatalysts designed for p–d orbital hybridization, steered selectivity to BHMF, achieving a faradaic efficiency (FE) of 58% at 100 mA cm−2 and a production rate of 1 mmol cm−2 h−1, the latter a doubling in rate compared to the best prior reports.
BF3 ⋅ OEt2 and Visible Light‐Controlled [3,3]‐ or [1,3]‐Rearrangement of Quinazolinone N−O Aryl Moieties
Authors: Nie Shu‐Min , Cheng Lu‐Min , Chen Chun‐Hua , Liang Cui , Pan Cheng‐Xue , Mo Dong‐Liang 
Published: February 2024

AbstractWe described a base‐promoted O‐arylation of N3‐hydroxyl quinazolinones with diaryliodonium salts and sequential BF3 ⋅ OEt2 and visible light‐controlled [3,3]‐ or [1,3]‐rearrangements of quinazolinone N−O aryl moieties to prepare a variety of 2‐(quinazolin‐4‐yloxy)phenols and atropisomeric 3‐(2‐hydroxyphenyl)quinazolin‐4‐ones in 44%‐75% yields and 28%‐70% yields, respectively. Mechanistic studies showed that HBF4 generated in situ from BF3 ⋅ Et2O and water served as the catalyst in this process and aryloxyquinazolinium salts are vital intermediates promoting the [3,3]‐ or [1,3]‐rearrangements. The N−O aryl moieties of aryloxyquinazolinium salts were found to undergo solely [3,3]‐rearrangement with heating whereas [1,3]‐rearrangement occurred via a radical process under irradiation of visible light. This method highlights the formation of aryloxyquinazolinium salts based on the use of BF3 ⋅ OEt2 and visible light to prompt [1,3]‐rearrangement generating atropisomeric quinazolinones.
Cleaving DNA with DNA: Cooperative Tuning of Structure and Reactivity Driven by Copper Ions
Authors: Dantu Sarath Chandra , Khalil Mahdi , Bria Marc , Saint‐Pierre Christine , Orio Maylis , Gasparutto Didier , Sicoli Giuseppe 
Published: February 2024

AbstractA copper‐dependent self‐cleaving DNA (DNAzyme or deoyxyribozyme) previously isolated by in vitro selection has been analyzed by a combination of Molecular Dynamics (MD) simulations and advanced Electron Paramagnetic Resonance (Electron Spin Resonance) EPR/ESR spectroscopy, providing insights on the structural and mechanistic features of the cleavage reaction. The modeled 46‐nucleotide deoxyribozyme in MD simulations forms duplex and triplex sub‐structures that flank a highly conserved catalytic core. The DNA self‐cleaving construct can also form a bimolecular complex that has a distinct substrate and enzyme domains. The highly dynamic structure combined with an oxidative site‐specific cleavage of the substrate are two key‐aspects to elucidate. By combining EPR/ESR spectroscopy with selectively isotopically labeled nucleotides it has been possible to overcome the major drawback related to the “metal‐soup” scenario, also known as “super‐stoichiometric” ratios of cofactors versus substrate, conventionally required for the DNA cleavage reaction within those nucleic acids‐based enzymes. The focus on the endogenous paramagnetic center (Cu2+) here described paves the way for analysis on mixtures where several different cofactors are involved. Furthermore, the insertion of cleavage reaction within more complex architectures is now a realistic perspective towards the applicability of EPR/ESR spectroscopic studies.
2D Graphene Oxide Membrane Nanoreactors for Rapid Directional Flow Ring‐Opening Reactions with Dominant Same‐Configuration Products
Authors: Fu Jiangwei , Pang Shuai , Zhang Yuhui , Li Xiang , Song Bo , Peng Daoling , Zhang Xiqi , Jiang Lei 
Published: February 2024

AbstractNanoconfinement within enzymes can increase reaction rate and improve selectivity under mild conditions. However, it remains a great challenge to achieve chemical reactions imitating enzymes with directional molecular motion, short reaction time, ≈100% conversion, and chiral conversion in artificial nanoconfined systems. Here, directional flow ring‐opening reactions of styrene oxide and alcohols are demonstrated with ≈100% conversion in <120 s at 22 °C using graphene oxide membrane nanoreactors. Dominant products have the same configuration as chiral styrene oxide in confined reactions, which is dramatically opposed to bulk reactions. The unique chiral conversion mechanism is caused by spatial confinement, limiting the inversion of benzylic chiral carbon. Moreover, the enantiomeric excess of same‐configuration products increased with higher alkyl charge in confined reactions. This work provides a new route to achieve rapid flow ring‐opening reactions with specific chiral conversion within 2D nanoconfined channels, and insights into the impact of nanoconfinement on ring‐opening reaction mechanisms.
Ink‐Extrusion 3D Printing and Silicide Coating of HfNbTaTiZr Refractory High‐Entropy Alloy for Extreme Temperature Applications
Authors: Zhang Dingchang , Hsu Ya‐Chu , Dunand David C. 
Published: February 2024

AbstractAn oxygen‐resistant refractory high‐entropy alloy is synthesized in microlattice or bulk form by 3D ink‐extrusion printing, interdiffusion, and silicide coating. Additive manufacturing of equiatomic HfNbTaTiZr is implemented by extruding inks containing hydride powders, de‐binding under H2, and sintering under vacuum. The sequential decomposition of hydride powders (HfH2+NbH+TaH0.5+TiH2+ZrH2) is followed by in situ X‐ray diffraction. Upon sintering at 1400 °C for 18 h, a nearly fully densified, equiatomic HfNbTaTiZr alloy is synthesized; on slow cooling, both α‐HCP and β‐BCC phases are formed, but on quenching, a metastable single β‐BCC phase is obtained. Printed and sintered HfNbTaTiZr alloys with ≈1 wt.% O shows excellent mechanical properties at high temperatures. Oxidation resistance is achieved by silicide coating via pack cementation. A small‐size lattice‐core sandwich is fabricated and tested with high‐temperature flames to demonstrate the versatility of this sequential approach (printing, sintering, and siliconizing) for high‐temperature, high‐stress applications of refractory high‐entropy alloys.
Novel Biomimetic “Spider Web” Robust, Super‐Contractile Liquid Crystal Elastomer Active Yarn Soft Actuator
Authors: Wu Dingsheng , Li Xin , Zhang Yuxin , Cheng Xinyue , Long Zhiwen , Ren Lingyun , Xia Xin , Wang Qingqing , Li Jie , Lv Pengfei , Feng Quan , Wei Qufu 
Published: February 2024

AbstractIn nature, spider web is an interwoven network with high stability and elasticity from silk threads secreted by spider. Inspired by the structure of spider webs, light‐driven liquid crystal elastomer (LCE) active yarn is designed with super‐contractile and robust weavability. Herein, a novel biomimetic gold nanorods (AuNRs) @LCE yarn soft actuator with hierarchical structure is fabricated by a facile electrospinning and subsequent photocrosslinking strategies. Meanwhile, the inherent mechanism and actuation performances of the as‐prepared yarn actuator with interleaving network are systematically analyzed. Results demonstrate that thanks to the unique “like‐spider webs” structure between fibers, high molecular orientation within the LCE microfibers and good flexibility, they can generate super actuation strain (≈81%) and stable actuation performances. Importantly, benefit from the robust covalent bonding at the organic–inorganic interface, photopolymerizable AuNRs molecules are uniformly introduced into the polymer backbone of electrospun LCE yarn to achieve tailorable shape‐morphing under different light intensity stimulation. As a proof‐of‐concept illustration, light‐driven artificial muscles, micro swimmers, and hemostatic bandages are successfully constructed. The research disclosed herein can offer new insights into continuous production and development of LCE‐derived yarn actuator that are of paramount significance for many applications from smart fabrics to flexible wearable devices.
Upgrading Cycling Stability and Capability of Hybrid Na‐CO2 Batteries via Tailoring Reaction Environment for Efficient Conversion CO2 to HCOOH
Authors: Yang Xiecheng , Zhang Dantong , Zhao Lanqing , Peng Chao , Ren Kun , Xu Changfan , Liu Pan , Zhou Yingjie , Lei Yong , Yang Bin , Xue Dongfeng , Liang Feng 
Published: February 2024

AbstractRechargeable Na‐CO2 batteries are considered to be an effective way to address the energy crisis and greenhouse effect due to their dual functions of CO2 fixation/utilization and energy storage. However, the insolubility and irreversibility of solid discharge products lead to poor discharge capacity and poor cycle performance. Herein, a novel strategy is proposed to enhance the electrochemical performance of hybrid Na‐CO2 batteries, using water‐in‐salt electrolyte (WiSE) to establish an optimal reaction environment, regulate the CO2 reduction pathway, and ultimately convert the discharge product of the battery from Na2CO3 to formic acid (HCOOH). This strategy effectively resolves the issue of poor reversibility, allowing the battery to exhibit excellent cycle performance (over 1200 cycles at 30 °C), especially under low‐temperature conditions (2534 cycles at −20 °C). Furthermore, density functional theory (DFT) calculations and experiments indicate that by adjusting the relative concentration of H/O atoms at the electrolyte/catalyst interface, the CO2 reduction pathway in the battery can be regulated, thus effectively enhancing CO2 capture capability and consequently achieving an ultra‐high discharge specific capacity of 148.1 mAh cm−2. This work effectively promotes the practical application of hybrid Na‐CO2 batteries and shall provide a guidance for converting CO2 into products with high‐value‐added chemicals.
Synthesis of Trifluoromethyl‐Containing 2,3‐Dihydrobenzofurans via a Formal [4+1] Annulation of In‐situ Generated o‐Quinone Methides and CF3−Imidoyl Sulfoxonium Ylides
Authors: Sun Yue , Ling Sihao , Chen Zhengkai 
Published: February 2024

AbstractA metal‐free [4+1] annulation of in‐situ generated ortho‐quinone methides (o‐QMs) and CF3−imidoyl sulfoxonium ylides (TFISYs) has been achieved under mild conditions, which provides a facile and straightforward access to a variety of trifluoromethyl‐containing 2,3‐dihydrobenzofurans in moderate to excellent yields.
Redesigned Nylon 6 Variants with Enhanced Recyclability, Ductility, and Transparency
Authors: Tian Jun‐Jie , Liu Xiaoyang , Ye Liwei , Zhang Zhen , Quinn Ethan C. , Shi Changxia , Broadbelt Linda J. , Marks Tobin J. , Chen Eugene Y.‐X. 
Published: February 2024

AbstractGeminal (gem−) disubstitution in heterocyclic monomers is an effective strategy to enhance polymer chemical recyclability by lowering their ceiling temperatures. However, the effects of specific substitution patterns on the monomer's reactivity and the resulting polymer's properties are largely unexplored. Here we show that, by systematically installing gem‐dimethyl groups onto ϵ‐caprolactam (monomer of nylon 6) from the α to ϵ positions, both the redesigned lactam monomer's reactivity and the resulting gem‐nylon 6’s properties are highly sensitive to the substitution position, with the monomers ranging from non‐polymerizable to polymerizable and the gem‐nylon properties ranging from inferior to far superior to the parent nylon 6. Remarkably, the nylon 6 with the gem‐dimethyls substituted at the γ position is amorphous and optically transparent, with a higher Tg (by 30 °C), yield stress (by 1.5 MPa), ductility (by 3×), and lower depolymerization temperature (by 60 °C) than conventional nylon 6.