Search Results (55)

Background/Objectives: this paper investigates the local vs. global visual processing preference in typically developing (TD) children, youth with Down syndrome (DS), and youth with Williams syndrome (WS). In particular, the global precedence effect (GPE) and the global interference effect (GI) have recently been described as two distinct and at least partially independent effects. Methods: in this study, 50 participants (TD = 25, DS = 13, WS = 12) completed two experiments requiring the identification of either the global or local level of hierarchical stimuli, which consisted of letters and schematic faces. For each stimulus type, two separate blocks were conducted, one with the task to focus on the local elements and the other with the task to focus on the global shape. Results: our results indicate that TD children demonstrate a global precedence effect for letters but not for schematic faces, suggesting a developmental modulation of configural processing. In contrast, both DS and WS groups showed a global processing bias for schematic faces and a significant global interference effect in both conditions, likely reflecting deficits in inhibitory control. Conclusions: these findings challenge the notion that DS and WS individuals can be classified strictly as global or local processors, respectively, emphasizing the influence of stimulus type and cognitive demands. Implications for neurodevelopmental research and clinical interventions are discussed. Full article

The incorporation of digital music into the reform of education has become one of the primary methods to improve educational outcomes, increase creativity, and innovate the practices in the classroom. This combination, together with the close industry–academia cooperation, presents the possibilities to integrate educational strategies in accordance with the technological and creative demands of the contemporary world. Nevertheless, uncertainty, reluctance, symmetry, and subjectivity in expert ratings are significant problems to cope with when considering multi-criteria decision-making (MCDM). To resolve them, this paper suggests a Picture Fuzzy Faire Un Choix Adequat (PF-FUCA) decision support model, where fifteen options will be rated by seven criteria, depending on the contribution of four professional decision-makers. These findings indicate that the PF-FUCA framework is effective and superior to the current PF-MCDM models, as illustrated by sensitivity and comparison analysis. The identified best strategies based on the framework are blockchain-based music copyright education and integrated music–STEM platforms that, when combined, offer a viable policy instrument to policymakers, educators, and industry stakeholders. Full article

Mangrove ecosystems underpin coastal livelihoods and biodiversity in Mozambique, yet gendered patterns of resource use and their implications for management remain underexplored. This study explores how artisanal fishing and shellfish collection differ between men and women on Inhaca Island (Maputo Bay), focusing on how these gender-specific practices shape livelihood outcomes, spatial use of mangroves, and perceptions of ecological change. To address this question, we combined structured interviews (n = 35; 51.4% men, 48.6% women) and camera-trap monitoring in two mangrove areas during September 2024 to document fishing practices, catch characteristics, spatial patterns, and ecological perceptions. We found pronounced gendered divisions of labor and space use: men, using boats and nets, harvested a median of 15 kg of fish per day for commercial sale, generating cash income, whereas women collected a median of 3 kg of shellfish by hand, primarily for household consumption. Camera traps confirmed pronounced spatial segregation in mangrove use: women foraged in targeted areas, and men traversed broader zones, both synchronizing their activities with tidal and daylight cycles. By integrating social and ecological data, the study revealed nuanced gender roles and resource pressures, with 82.9% of participants reporting declines in fish and shellfish stocks, emphasizing mangroves’ critical role in livelihoods, biodiversity, and climate resilience. Our findings highlight the value of mixed-method approaches for understanding socio-ecological dynamics and advocate for gender-sensitive conservation policies, strengthened Community Fisheries Councils, and infrastructure investments to regulate resource use, enhance mangrove management, and promote equitable livelihoods in Mozambique’s coastal communities. Full article

In the current digital landscape, where platforms process AI-generated content and intelligent network traffic on a large scale, it is the duty of such platforms to continuously measure the reliability, trustworthiness, and security of various data streams. Driven by this practical challenge, this research develops an effective decision-support mechanism in intelligent decision-making in big-data AI-generated content and network systems. The decision problem has considered several uncertainties, including content authenticity, processing efficiency, user trust, cybersecurity, system scalability, privacy protection, and cost of computing. The multidimensional uncertainty of AI-generated information and trends in network behavior are challenging to capture in traditional crisp and fuzzy decision-making models. To fill that gap, a new Picture Fuzzy Faire Un Choix Adequat (PF-FUCA) methodology is proposed, based on multi-perspective expert assessment and better computational aggregation to improve the accuracy of rankings, symmetry, and uncertainty treatment. A case scenario comprising fifteen different alternative intelligent decision strategies and seven evaluation criteria are examined under the evaluation of four decision-makers. The PF-FUCA model successfully prioritizes the best strategies to control AI-based content and network activities to generate a stable and realistic ranking. The comparative and sensitivity analysis show higher robustness, accuracy, and flexibility levels than the existing MCDM techniques. The results indicate that PF-FUCA is specifically beneficial in settings where a large amount of data has to flow, a high uncertainty rate exists, and the variables of decision are dynamic. The research introduces a scalable and credible methodological conception that can be used to facilitate high levels of intelligent computing applications to content governance and network optimization. Full article

The global automotive industry faces significant challenges with respect to its supply chain, particularly component scarcity and the increasing complexity of modern vehicles, which have severely impacted the production of high-tech harnesses. This study addresses the issues faced by a leading automotive harness manufacturing company in Ciudad Obregón, Mexico (an international company that requested confidentiality), which has suffered considerable economic losses (over USD 2870) and production downtime due to component scarcity and delivery delays in component deliveries, affecting “Crew Grande” harness production. This proposal aims to develop a technological solution with a graphical interface to support decision-making in the face of this scarcity. The methodology employed system dynamics to model the supply chain’s complexity, using software such as Stella^® Architect for Forrester diagrams and equations and Vensim^® PLE for causal diagrams. The model was validated with a relative error, confirming its reliability. Multicriteria decision-making (MCDM) was performed using the Technique for Order of Preference by Similarity to Ideal Solution (TOPSIS) and Faire Un Choix Adequate (FUCA) methods to evaluate 15 scenarios (normal, pessimistic, and optimistic), identifying the four most favorable scenarios for optimizing operational performance. The results demonstrate these solutions’ potential to mitigate losses, improve operational efficiency, and strengthen the company’s position against market and demand fluctuations, especially for its main client, Ford Motor Company, using a graphical user interface (GUI) to support analysis and decision-making. Full article

Fucose-containing glycoproteins and glycolipids broadly occur in humans as well as in many other species and are essential for a wide range of physiological processes, such as cell adhesion, fertilization, and tumor development. In humans, the cellular degradation of various fucosylated glycoconjugates depends on the FUCA1-encoded lysosomal tissue α-L-fucosidase (FUCA1). The crucial role of FUCA1 is reflected by the severe lysosomal storage disease fucosidosis, which causes a massive accumulation of fucosylated glycans, glycolipids, and α(1,6)-fucosylated glycoasparagines. Therefore, it is reasonable to assume that FUCA1 is predominantly responsible for the degradation of fucosylated glycoconjugates, although a second, functionally uncharacterized α-L-fucosidase, the plasma α-L-fucosidase (FUCA2), is known. To investigate the impact of both fucosidases in more detail, we generated two different monoclonal antibodies as useful tools for the detection of human and murine FUCA1 and utilized a FUCA2-specific antibody to demonstrate that FUCA2 is a bona fide lysosomal protein that is sorted in a mannose 6-phosphate (M6P)-dependent manner. We then compared FUCA1 and FUCA2 upon ectopic expression and evaluated their enzyme activity profiles under various conditions. Untagged and differently tagged versions of FUCA1 exhibited α-L-fucosidase activity, while various FUCA2 derivatives, even after affinity purification, did not show any fucosidase activity against commonly used pseudo-substrates. Our findings suggest that FUCA1 and not FUCA2 is exclusively responsible for the lysosomal de-fucosylation of glycoconjugates. Full article

Treatment-free remission (TFR) is a key therapeutic goal for chronic myeloid leukemia (CML) patients in deep molecular response (DMR). While predicting patient outcome remains challenging, different NK cell populations seem crucial. We conducted an immunological sub-study from the Argentina Stop Trial (AST), including 46 patients in 2019 (AST I) and 35 new patients between 2022 and 2023 (AST II). To characterize NK cell subsets in patients attempting TFR, peripheral blood mononuclear cell samples were collected before stopping treatment and phenotype and functional characteristics were assessed by flow cytometry. Non-relapsing patients from AST I exhibited NK cell subpopulations with cytomegalovirus-related memory features, high expression of cytotoxicity markers, and robust functionality. Remarkably, though clinical variables were very similar between cohorts, significant immune differences were observed. NK cell percentage and CD16 and CD57 receptor expression levels were significantly reduced in AST II (p = 0.0051; p = 0.0222; p = 0.0033, respectively), whereas NKp46, NKp44 and PD-1 expression levels were significantly increased (p = 0.0081; p < 0.0001; p < 0.0001, respectively). NK cells from AST II patients demonstrated higher overall functionality and more memory-like subpopulations, characterized mainly by the expression of CD57, NKG2C, NKp30 and NKp46 receptors among CD56^dim NK cells, also with enhanced functional performance. However, in AST II, we were unable to report an association with clinical outcome. Given the enrollment time of both cohorts and that they appear to be clinically homogeneous, we consider that COVID could be impacting the immune landscape; accordingly, serum samples from AST II, but not AST I, confirmed the presence of anti-SARS-CoV-2 IgG. The influence of the COVID pandemic and the different vaccine platforms on NK cells cannot be underestimated when evaluating the role of the immune system in cancer. Full article

Background/Objectives: Instrumental Activities of Daily Living (IADL) are the key indicators of the autonomy and functional ability in older adults with neurocognitive disorders (NCDs). However, the specific predictors of IADL performance across the NCD spectrum remains insufficiently characterized. This study aimed to identify the cognitive, motor, and caregiver-related determinants of the IADL in individuals with minor and major NCDs. Methods: A cross-sectional study was conducted involving 117 participants referred from a university-based dementia clinic. Standardized tools were administered to evaluate their IADL performance (Lawton IADL Scale), cognition [Addenbrooke’s Cognitive Examination III (ACE-III)], Functional Cognitive Assessment Scale (FUCAS)], motor functions, balance, and mobility [Tinetti Test, Timed Up and Go (TUG)], emotional status [Geriatric Depression Scale (GDS)], neuropsychiatric symptoms [Neuropsychiatric Inventory (NPI)], and caregiver burden [Zarit Burden Interview (ZBI)]. Multiple regression analyses were performed to identify the significant predictors of IADL performance. Results: In the total sample (n = 117), the IADL performance was significantly predicted via ACE-III, FUCAS, and Tinetti-balance (adjusted R² = 0.729). In the minor NCD group (n = 41), the significant predictors included sex, FUCAS, GDS, Tinetti-balance, and TUG (adjusted R² = 0.725). In the major NCD group (n = 76), ACE-III, FUCAS, and Tinetti-balance remained the significant predictors (adjusted R² = 0.634). Female sex and a worse profile on the other variables were associated with lower IADL scores. Conclusions: Global cognitive decline, executive dysfunction in everyday problem-solving situations, and balance impairment are the key determinants of IADL performance across both minor and major NCDs. Female sex and depressive symptoms further predicted the IADL performance in the minor NCD group. These findings highlight the need for multidisciplinary assessment and intervention strategies to promote health and autonomy and preserve the functional independence in older adults with NCDs. Full article

Fucosidosis is a rare lysosomal storage disease caused by α-L-fucosidase deficiency following a mutation in the FUCA1 gene. This enzyme is responsible for breaking down fucose-containing glycoproteins, glycolipids, and oligosaccharides within the lysosome. Mutations in FUCA1 result in either reduced enzyme activity or complete loss of function, leading to the accumulation of fucose-rich substrates in lysosomes. Lysosomes become engorged with undigested substrates, which leads to secondary storage defects affecting other metabolic pathways. The central nervous system is particularly vulnerable, with lysosomal dysfunction causing microglial activation, inflammation, and neuronal loss, leading to the neurodegenerative symptoms of fucosidosis. Neuroinflammation contributes to secondary damage, including neuronal apoptosis, axonal degeneration, and synaptic dysfunction, exacerbating the disease process. Chronic neuroinflammation impairs synaptic plasticity and neuronal survival, leading to progressive intellectual disability, learning difficulties, and loss of previously acquired skills. Inflammatory cytokines and lysosomal burden in motor neurons and associated pathways contribute to ataxia, spasticity, and hypotonia, which are common motor symptoms in fucosidosis. Elevated neuroinflammatory markers can increase neuronal excitability, leading to the frequent occurrence of epilepsy in affected individuals. So, fucosidosis is characterized by rapid mental and motor loss, along with growth retardation, coarse facial features, hepatosplenomegaly, telangiectasis or angiokeratomas, epilepsy, inguinal hernia, and dysostosis multiplex. Patients usually die at an early age. Treatment of fucosidosis is a great challenge, and there is currently no definitive effective treatment. Hematopoietic cell transplantation studies are ongoing in the treatment of fucosidosis. However, early diagnosis of this disease and treatment can be effective. In addition, the body’s immune system decreases due to chemotherapy applied after transplantation, leaving the body vulnerable to microbes and infections, and the risk of death is high with this treatment. In another treatment method, gene therapy, the use of retroviral vectors, is promising due to their easy integration, high cell efficiency, and safety. In another treatment approach, enzyme replacement therapy, preclinical studies are ongoing for fucosidosis, but the blood–brain barrier is a major obstacle in lysosomal storage diseases affecting the central nervous system. Early diagnosis is important in fucosidosis, a rare disease, due to the delay in the diagnosis of patients identified so far and the rapid progression of the disease. In addition, enzyme replacement therapy, which carries fewer risks, is promising. Full article

This study examines the Logistics Performance Index (LPI) rankings developed by the World Bank from a methodological perspective and proposes an alternative decision support framework. LPI serves as an interactive tool that helps countries identify challenges, innovative solutions, and opportunities in their trade and logistics sectors. In this study, the efficiency of logistics operations in 118 countries was evaluated using an integrated multi-criteria decision-making (MCDM) model objectively weighted by the Entropy method. Countries were ranked using the MCRAT, SAW, TOPSIS, and FUCA methods. According to the findings, large datasets provide more robust insights for sensitivity analyses, and wider weighting coefficient combinations make the data more meaningful. In addition, it is suggested to use low-compensation methods instead of classical additive methods for LPI. Unlike other studies in literature, this research applied an innovative sensitivity analysis to test the robustness of the model and comprehensively examined the effects of weighting techniques based on over 2500 different MCDM results. The findings suggest that the FUCA method should be recommended to decision-makers for calculating LPI rankings due to its simplicity, practicality, low compensatory power, and low sensitivity. This study offers methodological improvements when evaluating logistics performance and provides significant contributions to decision-making processes. The findings are expected to provide a valuable resource for policymakers and businesses in understanding a country’s position in global competition, as well as serving as a reference for researchers evaluating the logistics performance of countries. Full article

Background: Olive leaves are a significant source of biophenols, which have a beneficial impact on cognitive performance. Objective: To examine, for the first time, in humans the effect of the daily consumption of a beverage containing olive leaf extract (OLE) versus a Mediterranean diet (MeDi) on patients diagnosed with mild Alzheimer’s Disease (AD), in addition to their regular treatment. Methods: A randomized clinical trial compared OLE’s effects on cognitive and functional performance in 55 mild AD patients. Each participant was randomly assigned to two groups: (1) Group 1 was given olive leaves for making a daily beverage and MeDi instructions through monthly diet programs; (2) Group 2 received only the MeDi instructions. After six months, all participants underwent a second neuropsychological evaluation. Results: Group 1 participants had statistically significantly higher MMSE scores compared to Group 2 with a p-value of 0.0135. Specifically, the mean MMSE difference in patients receiving OLE was close to 0, indicating no memory deterioration, whereas in controls it was −4.1, indicative of cognitive decline. The remaining neuropsychological assessments (FRSSD, FUCAS, ADAS-Cog, CDR, GDS, and NPI) revealed better results in the OLE group, except for GDS, which showed no change, but without statistically significant differences between the two groups. Full article

Autism spectrum disorder (ASD) is a neurodevelopmental disorder. Investigations of gut microbiota (GM) play an important role in deciphering disease severity and symptoms. Overall, we stratified 70 ASD patients by neuropsychological assessment, based on Calibrated Severity Scores (CSSs) of the Autism Diagnostic Observation Schedule-Second edition (ADOS-2), Child Behavior Checklist (CBCL) and intelligent quotient/developmental quotient (IQ/DQ) parameters. Hence, metataxonomy and PICRUSt-based KEGG predictions of fecal GM were assessed for each clinical subset. Here, 60% of ASD patients showed mild to moderate autism, while the remaining 40% showed severe symptoms; 23% showed no clinical symptoms, 21% had a risk of behavior problems and 56% had clinical symptoms based on the CBCL, which assesses internalizing problems; further, 52% had no clinical symptoms, 21% showed risk, and 26% had clinical symptoms classified by CBCL externalizing problems. Considering the total CBCL index, 34% showed no clinical symptoms, 13% showed risk, and 52% had clinical symptoms. Here, 70% of ASD patients showed cognitive impairment/developmental delay (CI/DD). The GM of ASDs with severe autism was characterized by an increase in Veillonella, a decrease in Monoglobus pectinilyticus and a higher microbial dysbiosis index (MDI) when compared to mild-moderate ASDs. Patients at risk for behavior problems and showing clinical symptoms were characterized by a GM with an increase of Clostridium, Eggerthella, Blautia, Intestinibacter, Coprococcus, Ruminococcus, Onthenecus and Bariatricus, respectively. Peptidoglycan biosynthesis and biofilm formation KEGGs characterized patients with clinical symptoms, while potential microbiota-activated PPAR-γ-signaling was seen in CI/DD patients. This evidence derived from GM profiling may be used to further improve ASD understanding, leasing to a better comprehension of the neurological phenotype. Full article

The role of oxidative stress in health and homeostasis has generated interest in the scientific community due to its association with cardiovascular and neurodegenerative diseases, cancer, and other diseases. Therefore, extensive research seeks to identify new exogenous antioxidant compounds for supplementation. Polysaccharides are recognized for their antioxidant properties. However, polysaccharide chemical modifications are often necessary to enhance these properties. Therefore, dextran was conjugated with gallic acid (Dex-Gal) and later combined with fucoidan A (FucA) to formulate blends aimed at achieving superior antioxidant activity compared to individual polysaccharides. A factorial design was employed to combine FucA and Dex-Gal in different proportions, resulting in five blends (BLD1, BLD2, BLD3, BLD4, and BLD5). An analysis of surface graphs from in vitro antioxidant tests, including total antioxidant capacity (TAC), reducing power, and hydroxyl radical scavenging, guided the selection of BLD4 as the optimal formulation. Tests on 3T3 fibroblasts under various conditions of oxidative stress induced by hydrogen peroxide revealed that BLD4 provided enhanced protection compared to its isolated components. The BLD4 formulation, resulting from the combination of Dex-Gal and FucA, showed promise as an antioxidant strategy, outperforming its individual components and suggesting its potential as a supplement to mitigate oxidative stress in adverse health conditions. Full article

From the most ancient RNAs, which followed an RNY pattern and folded into small hairpins, modern RNA molecules evolved by two different pathways, dubbed Extended Genetic Code 1 and 2, finally conforming to the current standard genetic code. Herein, we describe the evolutionary path of the RNAome based on these evolutionary routes. In general, all the RNA molecules analysed contain portions encoded by both genetic codes, but crucial features seem to be better recovered by Extended 2 triplets. In particular, the whole Peptidyl Transferase Centre, anti-Shine–Dalgarno motif, and a characteristic quadruplet of the RNA moiety of RNAse-P are clearly unveiled. Differences between bacteria and archaea are also detected; in most cases, the biological sequences are more stable than their controls. We then describe an evolutionary trajectory of the RNAome formation, based on two complementary evolutionary routes: one leading to the formation of essentials, while the other complemented the molecules, with the cooperative assembly of their constituents giving rise to modern RNAs. Full article

Currently, therapy for early-stage human epidermal growth factor receptor 2-positive (HER2+) breast cancer (BC) is based on the combination of trastuzumab and pertuzumab plus chemotherapy in a neoadjuvant regimen. The INMUNOHER study aimed to detect immunological markers in peripheral blood and their association with treatment response. Sixty-two HER2+ BC patients were recruited. Pre-treatment samples were obtained before the start of treatment, while post-treatment samples were obtained after completing therapy and before surgery and were analyzed by flow cytometry. The pathologic complete response (pCR) rate achieved was 82.3%. The expression of the NKp30, PD-1, and TIM-3 receptors was reduced in the Natural Killer (NK)-CD56dim subset of patients who did not achieve pCR. Following therapy, many changes were found in leukocytes, including alterations in T cell lymphocyte proportions. Also, the percentage of NK cells decreased, and several phenotypic changes were observed in this population. After treatment, IFN-γ production by NK cells against HER2+-cells with or without trastuzumab was significantly reduced. HER2-targeted therapy plus chemotherapy demonstrated high efficacy in most patients, reducing the statistical power for finding immunological markers. However, NK subset phenotypes correlated better with response groups, and numerous changes in the percentage of leukocytes and T and NK cells, as well as changes in the functionality of NK cells, were observed in most patients after treatment, encouraging further research into these immune populations. Full article