bluuebunny/crossref_metadata_2025_split huggingface dataset:
Therapy of X‐linked adrenoleukodystrophy: Prognosis based upon age and MRI abnormality and plans for placebo‐controlled trials
Authors: Moser H. W. , Bezman L. , Lu S. E. , Raymond G. V. 
Published: December 2002

AbstractEvaluation of the therapy of X‐linked adrenoleukodystrophy (X‐ALD) is hampered by its rarity and by the striking and unpredictable variation in phenotypic expression. We present two approaches that may facilitate therapy evaluation. (1) We have analysed data on 377 X‐ALD patients who have been followed at the Kennedy Krieger Institute for a mean period of 38 months and have subdivided them into 18 subgroups on the basis of age and the degree of abnormality in brain magnetic resonance imaging (MRI) as assessed by the Loes score (Am. J. Neuroradiol 1994; 15: 1761). We find that grouping on the basis of age and MRI score provides information that is of significant prognostic value. (2) We present plans for the development of a placebo‐controlled multicentre international study that will have sufficient biostatistical power to provide objective evaluation of new therapeutic interventions.
Phenylketonuria: Tyrosine beyond the phenylalanine‐restricted diet
Authors: van Spronsen F. J. , Smit P. G. A. , Koch R. 
Published: December 2002

AbstractControversies exist on the role of tyrosine in the pathogenesis of phenylketonuria (PKU) and, consequently, on the therapeutic role of tyrosine. This review examines data and theoretical considerations on the role of tyrosine in the pathogenesis and treatment of PKU. It is concluded that treatment with tyrosine alone to replace the phenylalanine‐restricted diet cannot be justified. A treatment with large neutral amino acids (LNAA) including tyrosine to restore the balance in the transport of phenylalanine and other LNAA across the blood–brain barrier deserves further investigation. Such studies should prove the safety and the efficacy of such a treatment, finding the optimal dose of all LNAA, disclosing the correct age to start and the way to monitor treatment biochemically.
Treatment of late‐onset nonketotic hyperglycinaemia: Effectiveness of imipramine and benzoate
Authors: Wiltshire E. J. , Poplawski N. K. , Harrison J. R. , Fletcher J. M. 
Published: December 2002

AbstractWe report a patient with late‐onset nonketotic hyperglycinaemia managed with a sequential approach to drug therapy in placebo‐controlled therapeutic trials. Partial response to low‐protein diet and sodium benzoate and dramatic response to imipramine are demonstrated, with parental scores on the Developmental Behavioural Checklist falling from the 86th centile before treatment to normal with combined benzoate and imipramine therapy.
Adaptive trajectory tracking control for VTOL UAVs with unknown time‐varying mass using an extended I&I estimator
Authors: Han Qi , Liu Zhitao , Su Hongye , Liu Xiangbin 
Published: January 2024

SummaryThis article presents an adaptive control scheme for vertical takeoff and landing unmanned aerial vehicles subject to time‐varying mass based on the immersion and invariance scheme in an extended form. For the varying mass parameter in the position control, a generalized I&I adaptive law is proposed with higher dimension to estimate the mass and its derivatives. The stability of the resulting estimation error manifold, which is in a cascade form, is guaranteed by introducing smooth saturation functions in the position control law. The attitude control is designed by employing a smooth function with positive integrable time‐varying function to compensate unknown time‐varying moment of inertia to achieve asymptotic attitude tracking. Simulation and experimental results verify the effectiveness of the proposed method under different cases, and the robustness to time‐varying parameter is assured.
1‐Naphthaleneacetic Acid Improved the In Vitro Cell Culturing by Inhibiting Apoptosis
Authors: Wang Zhongyi , Li Fengqi , Feng Chunjing , Zheng Dongpeng , Pang Zhaojun , Ma Yue , Xu Ying , Yang Ce , Li Xueren , Peng Shouchun , Liu Zichuan , Mu Xin 
Published: January 2024

AbstractIn vitro cell culturing witnessed its applications in scientific research and industrial activities. Attempts to shorten the doubling time of cultured cells have never ceased. In plants, auxin is applied to promote plant growth, the synthetic derivative 1‐Naphthaleneacetic acid (NAA) is a good example. Despite the auxin's naturally occurring receptors are not present in mammalian cells, studies suggested they may affect cell culturing. Yet the effects and mechanisms are still unclear. Here, an up to 2‐fold increase in the yield of in vitro cultured human cells is observed. Different types of human cell lines and primary cells are tested and found that NAA is effective in all the cells tested. The PI staining followed by FACS suggested that NAA do not affect the cell cycling. Apoptosis‐specific dye staining analysis implicated that NAA rescued cell death. Further bulk RNA sequencing is done and it is identified that the lipid metabolism‐engaging and anti‐apoptosis gene, ANGPTL4, is enhanced in expression upon NAA treatment. Studies on ANGPTL4 knockout cells indicated that ANGPTL4 is required for NAA‐mediated response. Thus, the data identified a beneficial role of NAA in human cell culturing and highlighted its potency in in vitro cell culturing.
Highly Circularly Polarized Light‐Sensitive Chiral One‐Dimensional Perovskites Enabled by Antisolvent Engineering
Authors: Yang Seongyeon , Jang Gyumin , Lee Chan Uk , Son Jaehyun , Lee Junwoo , Jeong Wooyong , Roe Dong Gue , Cho Jeong Ho , Moon Jooho 
Published: January 2024

AbstractChiral perovskites have emerged as promising next‐generation materials for polarization detection due to their excellent circularly polarized light (CPL) detection capabilities. However, they suffer from a low chiroptical response when fabricated as a polycrystalline thin film, limiting their potential range of applications. Herein, it is demonstrated that antisolvent dripping during the spin‐coating process can effectively improve the chiroptical properties of polycrystalline chiral perovskite thin films. Systematic analysis with different antisolvents reveals that the highly polar antisolvent chloroform interacts with dimethyl sulfoxide molecules via hydrogen bonding. This strong hydrogen bonding suppresses the formation of intermediate and secondary phases and accelerates the crystallization of chiral 1D perovskites, thus reducing the density of the iodine vacancies inside the perovskite thin films. The lower density of iodine vacancies also intensifies the asymmetric tilting inorganic distortion of PbI6 octahedrons, enhancing the chiroptical response of the fabricated chiral perovskite material. Photodetectors based on the chloroform‐treated chiral perovskite films achieve a remarkably high distinguishability of 0.31, outperforming previously reported photodetectors based on the chiral perovskites. The fabricated photodetectors also exhibit outstanding responsivity and detectivity with enhanced operational stability.
Selenoprotein‐Regulated Hydrogel for Ultrasound‐Controlled Microenvironment Remodeling to Promote Bone Defect Repair
Authors: Xu Renhao , You Yuanyuan , Zheng Wenyi , Ma Li , Chang Yanzhou , Pan Shuya , He Yanni , Zhou Meijun , Xu Zhili , Chen Tianfeng , Liu Hongmei 
Published: January 2024

AbstractAbnormal levels of reactive oxygen species (ROS) and the hypoxic microenvironment within bone defects are important factors that impede bone repair processes. Herein, an innovative ultrasound‐modulatable hydrogel platform with selenoprotein‐mediated antioxidant effects to promote bone injury repair is presented. This hydrogel platform encapsulates oxygen‐enriched selene‐incorporated thin‐shell silicon within methacrylate gelatin (O2‐PSSG). The resultant construct orchestrates the modulation of the bone‐defect microenvironment, thereby expediting the course of bone regeneration. Ultrasound (US) is used to regulate the pore size of the hydrogel to release selenium‐containing nanoparticles and promote the in situ synthesis of efficient intracellular selenoproteins and hydrogen peroxide consumption. As expected, O2‐PSSG rapidly releases selenocystine ([Sec]2) under US control to scavenge reactive oxygen species and maintain the homeostasis of bone marrow mesenchymal stem cells (BMSCs). Over time, the action of the system by selenoprotein increases the activation of Wnt/β‐catenin pathways and promotes the differentiation of BMSCs. Consequently, O2‐PSSG potentiates the antioxidant proficiency of BMSCs both in vitro and in vivo, alleviates hypoxic environments, promotes osteogenic differentiation, and expedites cranial bone repair in rat models. In summary, this study suggests that the designed and constructed US‐responsive antioxidant hydrogel is a promising prospective strategy for addressing bone defects and fostering bone regeneration.
Hydrogen‐Bonding Organocatalysis Enabled Photocatalytic Intramolecular [2+2]‐Cycloaddition Reaction
Authors: Perulli Stefania , Desai Om , Ardevines Sandra , Uygur Mustafa , Delgado‐Hernández Samuel , García Mancheño Olga 
Published: January 2024

AbstractThe combination of organocatalytic activation and photocatalysis for enabling the intramolecular [2+2]‐cycloaddition of enone‐ene substrates bearing one Lewis base binding site is reported. While in a variety of solvents a poor conversion or no reaction takes place in the absence of a hydrogen bonding catalyst, the corresponding ring‐fused cyclobutane products could be built in moderate to good yields using a synergistic dual iridium‐urea co‐catalytic system. Control and mechanistic studies supported the postulated interaction between the organocatalyst and the substrate, which proved essential for an efficient energy transfer from the photosensitizer.
An Algorithm of Complete Coverage Path Planning for Deep‐Sea Mining Vehicle Clusters Based on Reinforcement Learning
Authors: Xing Bowen , Wang Xiao , Liu Zhenchong 
Published: January 2024

AbstractThis paper proposes a deep reinforcement learning algorithm to achieve complete coverage path planning for deep‐sea mining vehicle clusters. First, the mining vehicles and the deep‐sea mining environment are modeled. Then, this paper implements a series of algorithm designs and optimizations based on Deep Q Networks (DQN). The map fusion mechanism can integrate the grid matrix data from multiple mining vehicles to get the state matrix of the complete environment. In this paper, a preprocessing method for the state matrix is also designed to provide suitable training data for the neural network. The reward function and action selection mechanism of the algorithm are also optimized according to the requirements of cluster cooperative operation. Furthermore, the algorithm uses distance constraints to prevent the entanglement of underwater hoses. To improve the training efficiency of the neural network, the algorithm filters and extracts training samples for training through the sample quality score. Considering the requirement of cluster complete coverage mission, this paper introduces Long Short‐Term Memory (LSTM) based on the neural network to achieve a better training effect. After completing the above optimization and design, the algorithm proposed in this paper is verified through simulation experiments.
Single‐Component Dual‐Functional Autoboost Strategy by Dual Photodynamic and Cyclooxygenase‐2 Inhibition for Lung Cancer and Spinal Metastasis
Authors: Wang Ben , Lu Zhen‐Ni , Song Meng‐Xiong , He Xiao‐Wen , Hu Zhi‐Chao , Liang Hai‐Feng , Lu Hong‐Wei , Chen Qing , Liang Bing , Yi Tao , Wei Peng , Jiang Li‐Bo , Dong Jian 
Published: January 2024

AbstractColoading adjuvant drugs or biomacromolecules with photosensitizers into nanoparticles to enhance the efficiency of photodynamic therapy (PDT) is a common strategy. However, it is difficult to load positively charged photosensitizers and negatively charged adjuvants into the same nanomaterial and further regulate drug release simultaneously. Herein, a single‐component dual‐functional prodrug strategy is reported for tumor treatment specifically activated by tumor microenvironment (TME)‐generated HOCl. A representative prodrug (DHU‐CBA2) is constructed using indomethacin grafted with methylene blue (MB). DHU‐CBA2 exhibited high sensitivity toward HOCl and achieved simultaneous release of dual drugs in vitro and in vivo. DHU‐CBA2 shows effective antitumor activity against lung cancer and spinal metastases via PDT and cyclooxygenase‐2 (COX‐2) inhibition. Mechanistically, PDT induces immunogenic cell death but stimulates the gene encoding COX‐2. Downstream prostaglandins E2 and Indoleamine 2,3 dioxygenase 1 (IDO1) mediate immune escape in the TME, which is rescued by the simultaneous release of indomethacin. DHU‐CBA2 promotes infiltration and function of CD8+ T cells, thus inducing a robust antitumor immune response. This work provides an autoboost strategy for a single‐component dual‐functional prodrug activated by TME‐specific HOCl, thereby achieving favorable tumor treatment via the synergistic therapy of PDT and a COX‐2 inhibitor.
Effect of waterlogging on photosynthesis and growth of finger millet (Eleusine coracana)
Authors: Maai Eri 
Published: January 2024

AbstractFinger millet (Eleusine coracana (L.) Gaertn.) is an important cereal crop grown in most parts of Asia and Africa owing to its ability to adapt to stressful environments. The objective of this study was to examine the effect of waterlogging on photosynthesis and growth of finger millet. Plants were subjected to waterlogging by keeping pots filled with water from June 16 (transplanting) until November 12 (harvesting) in 2021 in Tokyo. After being subject to 13 days of waterlogging, net photosynthetic rate (Pn) decreased by 10.1% due to reduced stomatal conductance (gs), transpiration rate, and relative chlorophyll content (SPAD). From July to September, long‐term waterlogging increased Pn in the range of 4.9%–26.3%. The alleviation of high temperature, increase in SPAD and gs, and other nonstomatal components were implicated as the cause of increased Pn in summer. These findings suggest that the effect of waterlogging on photosynthesis varies with the duration of waterlogging or the growing season. Plant height was significantly reduced by 27.9% during the first 13 days of waterlogging and remained lower throughout the waterlogging treatment than in irrigated conditions. Waterlogging also caused other morphological changes, such as a significant increase in the number of tillers (63.2%) and a 16.7% increase in the number of panicles. Grain yield decreased by 13.6%, but the reduction was not significant. Overall, finger millet has the potential to tolerate waterlogging and is a promising crop for both paddy and field farming.
Mapping nut‐cracking in a new population of wild capuchin monkeys (Sapajus libidinosus) at Ubajara National Park, Brazil
Authors: Falótico Tiago , Valença Tatiane , Verderane Michele P. , Santana Beatriz C. , Sirianni Giulia 
Published: January 2024

AbstractPopulations of bearded capuchin monkeys (Sapajus libidinosus) vary in their tool use behaviors, with some of this divergence regarded as culturally determined. The use of stone tools, primarily to crack open encased foods, is widespread among bearded capuchins living in dry habitats (Caatinga and Cerrado). Significant diversity in targets, processed foods, material, and size of tools is observed across populations. However, so far, only a few sites have been systematically studied, and we are still distant from a representative picture of the range of variation in capuchins’ culture. In this study, we did a systematic assessment of stone tool use sites in the Ubajara National Park (UNP), in the Caatinga region of Ceará, Brazil, recording and measuring stone tools, processed foods, and available lithic resources as part of an extensive comparative research, the CapCult project. We found indirect and direct evidence that capuchin monkeys at UNP customarily use hammerstones and anvils to process at least two species of palm nuts, macauba (Acrocomia aculeata) and the harder babaçu (Attalea speciosa). Most of the anvils were rock surfaces and had leftovers of only one palm nut species. The hammerstones used to process both palm nuts were not significantly different in weight, although the ones used for Ac. aculeata were longer. We found a higher frequency of nut‐cracking sites in the drier lowland area of the park, reflecting differences in the density of the most common palm species, Ac. aculeata, and availability of raw stone material. The stone tool use observed in UNP is within the scope of previously reported in savannah capuchin populations. Our study widens the knowledge of stone tool‐use diversity in wild capuchin monkeys, which could contribute to shaping conservation policy, including cultural traits.
Regulating Crystallinity Mismatch Between Donor and Acceptor to Improve Exciton/Charge Transport in Efficient Organic Solar Cells
Authors: Liao Xunfan , Liu Mingtao , Pei Hongqiao , Zhu Peipei , Xia Xinxin , Chen Zeng , Zhang Yihan , Wu Zhongyuan , Cui Yongjie , Xu Guodong , Gao Mengyuan , Ye Long , Ma Ruijie , Liu Tao , Lu Xinhui , Zhu Haiming , Chen Yiwang 
Published: January 2024

AbstractAchieving a more balanced charge transport by morphological control is crucial in reducing bimolecular and trap‐assisted recombination and enhancing the critical parameters for efficient organic solar cells (OSCs). Hence, a facile strategy is proposed to reduce the crystallinity difference between donor and acceptor by incorporating a novel multifunctional liquid crystal small molecule (LCSM) BDTPF4‐C6 into the binary blend. BDTPF4‐C6 is the first LCSM based on a tetrafluorobenzene unit and features a low liquid crystal phase transition temperature and strong self‐assembly ability, conducive to regulating the active layer morphology. When BDTPF4‐C6 is introduced as a guest molecule into the PM6 : Y6 binary, it exhibits better compatibility with the donor PM6 and primarily resides within the PM6 phase because of the similarity‐intermiscibility principle. Moreover, systematic studies revealed that BDTPF4‐C6 could be used as a seeding agent for PM6 to enhance its crystallinity, thereby forming a more balanced and favourable charge transport with suppressed charge recombination. Intriguingly, dual Förster resonance energy transfer was observed between the guest molecule and the host donor and acceptor, resulting in an improved current density. This study demonstrates a facile approach to balance the charge mobilities and offers new insights into boosting the efficiency of single‐junction OSCs beyond 20 %.
Epigenetic Regulatory Axis MIR22‐TET3‐MTRNR2L2 Represses Fibroblast‐Like Synoviocyte–Mediated Inflammation in Rheumatoid Arthritis
Authors: Fang Yilong , Huang Wei , Zhu Xiangling , Wang Xinming , Wu Xuming , Wang Huihui , Hong Wenming , Yan Shangxue , Zhang Lingling , Deng Yujie , Wei Wei , Tu Jiajie , Zhu Chen 
Published: January 2024

ObjectiveThe specific role of fibroblast‐like synoviocytes (FLSs) in the pathogenesis of rheumatoid arthritis (RA) is still not fully elucidated. This study aimed to explore the molecular mechanisms of epigenetic pathways, including three epigenetic factors, microRNA (miRNA)‐22 (MIR22), ten‐eleven translocation methylcytosine dioxygenase 3 (TET3), and MT‐RNR2 like 2 (MTRNR2L2), in RA‐FLSs.MethodsThe expression of MIR22, TET3, and MTRNR2L2 in the synovium of patients with RA and arthritic mice were determined by fluorescence in situ hybridization, quantitative polymerase chain reaction (qPCR), immunohistochemistry, and Western blot. Mir22−/− and Tet3+/− mice were used to establish a collagen antibody–induced arthritis (CAIA) model. Mir22 angomir and Tet3 small interfering RNA (siRNA) were used to illustrate the therapeutic effects on arthritis using a collagen‐induced (CIA) model. Bioinformatics, luciferase reporter assay, 5‐hydroxymethylcytosine (5hmC) dot blotting, chromatin immunoprecipitation–qPCR, and hydroxymethylated DNA immunoprecipitation were conducted to show the direct repression of MIR22 on the TET3 and transcriptional activation of TET3 on MTRNR2L2.ResultsThe Mir22−/− CAIA model and RA‐FLS–related in vitro experiments demonstrated the inhibitory effect of MIR22 on inflammation. MIR22 can directly inhibit the translation of TET3 in RA‐FLSs by binding to its 3′ untranslated region in TET3. The Tet3+/− mice–established CAIA model showed less severe symptoms of arthritis in vivo. In vitro experiments further confirmed the proinflammatory effect of TET3 in RA. In addition, the CIA model was used to validate the therapeutic effects of Mir22 angomir and Tet3 siRNA. Finally, TET3 exerts its proinflammatory effect by promoting 5hmC production in the promoter of its target MTRNR2L2 in RA‐FLSs.ConclusionThe key role of the MIR22‐TET3‐MTRNR2L2 pathway in RA‐FLSs provided an experimental basis for further studies into the pathogenesis and related targets of RA from the perspective of FLSs.
Sustainability isomorphism in buyer–supplier relationships: The impact of supply chain leadership
Authors: Ahmadi‐Gh Zahra , Bello‐Pintado Alejandro 
Published: January 2024

AbstractWithin the framework of institutional theory, this study explores how the proactiveness of focal (or buying) manufacturing firms, demonstrated through the adoption of various sustainability practices, influences the sustainability performance of suppliers. Additionally, it examines how the leadership capability of buying firms, particularly in terms of supply chain leadership, can play a crucial role in this link. Through empirical analysis of a sample taken from the fourth round of the High‐Performance Management (HPM) project, our study reveals compelling evidence indicating that buying firms, by adopting diverse sustainability practices, such as internal initiatives, monitoring efforts and collaborative approaches with suppliers, are able to create distinct institutional pressures. These pressures serve as a conduit for the diffusion of a shared set of sustainability goals, values and norms among suppliers, ultimately contributing to the development of sustainability competences and improving their overall sustainability performance. Furthermore, our findings suggest that when buying firms undertake a leading position, they can effectively translate isomorphism pressures into sustainability improvements on the supplier side. Overall, this study sheds light on important and understudied aspects of sustainability practices in buyer–supplier relationships and underscores the critical role that supply chain leadership can play in promoting sustainable practices across the entire supply chain.