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1.
BMC Plant Biol ; 24(1): 807, 2024 Aug 27.
Artículo en Inglés | MEDLINE | ID: mdl-39187785

RESUMEN

Cadmium (Cd) is a biologically non-essential heavy metal, a major soil pollutant, and extremely harmful to plants. The phytohormone methyl jasmonate (MeJA) plays an important role in plant heavy-metal resistance. However, the understanding of the effects of MeJA supply level on alleviating Cd toxicity in plants is limited. Here, we investigated how MeJA regulated the development of physiological processes and cell wall modification in Cosmos bipinnatus. We found that low concentrations of MeJA increased the dry weight of seedlings under 120 µM Cd stress by reducing the transport of Cd from roots to shoots. Moreover, a threshold concentration of exogenous MeJA increased the activities of superoxide dismutase (SOD), peroxidase (POD), and catalase (CAT) in plant roots, the concentration of Cd in the root cell wall, and the contents of pectin and hemicellulose 1 polysaccharides, through converting Cd into pectin-bound forms. These results suggested that MeJA mitigated Cd toxicity by modulating root cell wall polysaccharide and functional group composition, especially through pectin polysaccharides binding to Cd, with effects on Cd transport capacity, specific chemical forms of Cd, and homeostatic antioxidant systems in C. bipinnatus.


Asunto(s)
Acetatos , Cadmio , Ciclopentanos , Oxilipinas , Reguladores del Crecimiento de las Plantas , Oxilipinas/metabolismo , Ciclopentanos/metabolismo , Acetatos/farmacología , Cadmio/toxicidad , Reguladores del Crecimiento de las Plantas/metabolismo , Raíces de Plantas/efectos de los fármacos , Raíces de Plantas/crecimiento & desarrollo , Raíces de Plantas/metabolismo , Contaminantes del Suelo/toxicidad , Pared Celular/metabolismo , Pared Celular/efectos de los fármacos , Plantones/efectos de los fármacos , Plantones/crecimiento & desarrollo , Plantones/metabolismo , Antioxidantes/metabolismo , Superóxido Dismutasa/metabolismo
2.
Environ Sci Technol ; 58(28): 12598-12608, 2024 Jul 16.
Artículo en Inglés | MEDLINE | ID: mdl-38904976

RESUMEN

Understanding the transport of 137Cs emitted during the Fukushima accident is challenging because the critical emissions that produced the high-deposition area are not adequately resolved in existing source terms. This paper presents an objective inverse reconstruction of these emissions by fusing atmospheric concentrations with a-priori emissions extracted from total depositions. This extraction, previously considered impossible for complex real-world accidents, is achieved by identifying the critical temporal formation process of depositions in the high-deposition area and estimating the corresponding emissions by using an atmospheric transport model. The reconstructed source term reveals two emission peaks from 10:00-11:00 and 14:00-15:00 on March 15, which agree with the in situ pressure measurements and accident analysis, suggesting that they came from pressure drops in the primary containment vessels of Units 3 and 2, respectively. This finding explains the environmental observations of spherical 137Cs particles. The source term also objectively and independently confirms the widely used reverse estimate. The corresponding 137Cs transport simulations better match the various observations than those produced by other source terms, proving that the two-peak emission creates a high-deposition area. The proposed method outperforms the direct fusion of deposition and atmospheric concentration observations, providing a robust tool for multiobservation fusion.


Asunto(s)
Contaminantes Radiactivos del Aire , Atmósfera , Radioisótopos de Cesio , Accidente Nuclear de Fukushima , Monitoreo de Radiación , Radioisótopos de Cesio/análisis , Contaminantes Radiactivos del Aire/análisis , Atmósfera/química , Japón , Modelos Teóricos
3.
Angew Chem Int Ed Engl ; 63(13): e202401074, 2024 Mar 22.
Artículo en Inglés | MEDLINE | ID: mdl-38311965

RESUMEN

The electronic structure of metal complexes plays key roles in determining their catalytic features. However, controlling electronic structures to regulate reaction mechanisms is of fundamental interest but has been rarely presented. Herein, we report electronic tuning of Cu porphyrins to switch pathways of the hydrogen evolution reaction (HER). Through controllable and regioselective ß-oxidation of Cu porphyrin 1, we synthesized analogues 2-4 with one or two ß-lactone groups in either a cis or trans configuration. Complexes 1-4 have the same Cu-N4 core site but different electronic structures. Although ß-oxidation led to large anodic shifts of reductions, 1-4 displayed similar HER activities in terms of close overpotentials. With electrochemical, chemical and theoretical results, we show that the catalytically active species switches from a CuI species for 1 to a Cu0 species for 4. This work is thus significant to present mechanism-controllable HER via electronic tuning of catalysts.

4.
Angew Chem Int Ed Engl ; : e202414104, 2024 Aug 15.
Artículo en Inglés | MEDLINE | ID: mdl-39145688

RESUMEN

Improving proton transfer is vital for electrocatalysis with porous materials. Although several strategies are reported to assist proton transfer in channels, few studies are dedicated to improving proton transfer at the local environments of active sites in porous materials. Herein, we report on new Co-corrole-based porous organic polymers (POPs) with improved proton transfer for electrocatalytic oxygen reduction reaction (ORR) and oxygen evolution reaction (OER). By tuning the pore sizes and installing proton relays at Co corrole sites, we designed and synthesized POP-2-OH with improved proton transfer both in channels and at local Co active sites. This POP shows remarkable activity for both electrocatalytic ORR with E1/2 = 0.91 V vs RHE and OER with h10 = 255 mV. Therefore, this work is significant to present a strategy to improve active site local proton transfer in porous materials and highlight the key role of such structural functionalization in boosting oxygen electrocatalysis.

5.
Clin Immunol ; 257: 109811, 2023 12.
Artículo en Inglés | MEDLINE | ID: mdl-37858752

RESUMEN

BACKGROUND: To explore the specific marker of CD8+ T cell subsets which are closely related to the prognosis and immunotherapy of patients with colon cancer. METHODS: 18 kinds of immune cell expression profile data sets were obtained from GEO database. Compared with other immune cell types, the specific markers of CD8 (+) T cells (TI-CD8) in colorectal cancer were screened. Regression analyses were used to further screen prognostic related genes and construct a prognostic evaluation model. The patients were stratified and analyzed according to the risk scores, KRAS mutation status, stage, lymphatic infiltration and other indicators. The landscape of infiltration level, mutation and copy number variation of immune subsets in high and low TI-CD8Sig score groups were compared and analyzed. The difference of drug response between high and low TI-CD8Sig score groups was analyzed. Differential expression of the model genes was verified by the HPA database. RESULTS: Six prognostic-related CD8T cell-specific gene targets were further screened, and the prognostic evaluation model was constructed. The AUC value of the model is >0.75. FAT3 and UNC13C showed a high mutation state in the low-risk group, while USH2A, MUC5B et al. specifically showed a high mutation state in the high-risk group. Compared with the low-risk group, the high-risk group had lower effective rate of drug response. The expression of PD-1 gene was positively correlated with the level of TI-CD8Sig score. CONCLUSION: The risk assessment model based on CD8T cell-specific marker genes can effectively predict the prognosis and the drug response of patients with CRC.


Asunto(s)
Neoplasias del Colon , Variaciones en el Número de Copia de ADN , Humanos , Neoplasias del Colon/genética , Neoplasias del Colon/terapia , Linfocitos T CD8-positivos , Pronóstico , Inmunoterapia , Aprendizaje Automático , Microambiente Tumoral
6.
Thromb J ; 21(1): 31, 2023 Mar 20.
Artículo en Inglés | MEDLINE | ID: mdl-36941692

RESUMEN

BACKGROUND: Platelet indices are blood-based parameters reflecting the activation of platelets. Previous studies have identified an association between platelet indices and blood pressure (BP). However, causal inferences are prone to bias by confounding effects and reverse causation. We performed a Mendelian randomization (MR) study to compare the causal roles between genetically determined platelet indices and BP levels. METHODS: Single-nucleotide polymorphisms (SNPs) associated with platelet count (PLT), plateletcrit (PCT), mean platelet volume (MPV), platelet distribution width (PDW), and BP at the level of genome-wide significance (p < 5 × 10- 8) in the UK Biobank were used as instrumental variables. In bidirectional univariable MR analyses, inverse variance-weighted (IVW), MR‒Egger, and weighted median methods were used to obtain estimates for individual causal power. In addition, heterogeneity and sensitivity analyses were performed to examine the pleiotropy of effect estimates. Finally, multivariable MR analyses were undertaken to disentangle the comparative effects of four platelet indices on BP. RESULTS: In the univariable MR analyses, increased levels of PLT and PCT were associated with higher BP, and PDW was associated with higher DBP alone. In the reverse direction, SBP had a minor influence on PLT and PCT. In multivariable MR analysis, PDW and PLT revealed an independent effect, whereas the association for PCT and MPV was insignificant after colinear correction. CONCLUSION: These findings suggest that platelets and BP may affect each other. PDW and PLT are independent platelet indices influencing BP. Increased platelet activation and aggregation may be involved in the pathogenesis of hypertension, which may provide insights into evaluating thromboembolic events in people with high BP. The necessity of initiating antiplatelet therapy among hypertension groups needs further investigation.

7.
Neurol Sci ; 43(12): 6719-6730, 2022 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-35982362

RESUMEN

BACKGROUND: Cognitive impairment can seriously affect the quality of life of Parkinson's disease (PD) patients. Although numerous studies showed that N200, P300 latency and amplitude are correlated with cognitive functions, there is a sufficient amount of controversial results. Therefore, it is necessary to conduct a meta-analysis of N200, P300 latency and amplitude data of event-related potential (ERP) in PD. METHODS: We systematically searched on PubMed and Web of Science for PD-related ERP studies published before December 2021. Standard mean difference (SMD) and 95% confidence interval (CI) estimates of N200 and P300 components were compared among PD patients, PD dementia (PDD) patients, PD non-dementia (PDND) patient, and healthy control (HC). RESULTS: Our meta-analysis showed prolonged N200 latency at the Fz, Cz electrode sites, prolonged P300 latency at the Fz sites in PD patients, compared to HC; prolonged N200 latency at the Cz, Pz electrode sites in PDND patients, compared to HC; prolonged P300 latency at the Cz site in PDD patients, compared to PDND patients; and reduced P300 amplitude at the Fz electrode site in PDND patients, compared to HC. CONCLUSIONS: N200 and P300 component may be potential electrophysiological biomarkers of early cognitive impairment in PD patients. Future studies are needed to confirm this conclusion. Estimates of N200 and P300 component can be a valuable support for clinicians in diagnosis of early cognitive impairment in PD patients due to the simplicity and non-invasiveness of the procedure.


Asunto(s)
Disfunción Cognitiva , Enfermedad de Parkinson , Humanos , Disfunción Cognitiva/diagnóstico , Disfunción Cognitiva/etiología , Potenciales Relacionados con Evento P300/fisiología , Potenciales Evocados/fisiología , Calidad de Vida
8.
BMC Oral Health ; 21(1): 596, 2021 11 22.
Artículo en Inglés | MEDLINE | ID: mdl-34809636

RESUMEN

OBJECTIVE: To provide a simplified treatment strategy for patients with maxillary transverse deficiency. We investigated and compared the fracture mechanics and stress distribution of a midline palatal suture under dynamic loads during surgically-assisted rapid palatal expansion. METHODS: Based on the cone-beam computed tomography (CBCT) data of a 21-year-old female volunteer, a three-dimensional model of the cranio-maxillofacial complex (including the palatal suture) was constructed. A finite element analysis model was constructed based on meshwork. After the yield strength of the palatal suture was set, an increasing expansion force (0-500 N) was applied within 140 ms to calculate the time-load curve, which mimicked nonsurgical bone expansion (model A). The same method was used to evaluate the fracture process, time and stress distribution of the palatal suture in maxillary lateral osteotomy-assisted (model B) and LeFort osteomy I (LFIO)-assisted expansion of the maxillary arch (model C). RESULTS: Compared with model A, the palatal suture of model B and model C showed a faster stress accumulation rate and shorter fracture time, and the fracture time of model B and model C was almost identical. Compared with model A, we discovered that model B and model C showed greater lateral extension of the maxilla, and the difference was reflected mainly in the lower part of the maxilla, and there was no difference between model B and model C in lateral extension of the maxilla. CONCLUSIONS: Compared with arch expansion using nonsurgical assistance (model A), arch expansion using maxillary lateral wall-osteotomy (model B) or LFIO had a faster rate of stress accumulation, shorter time of fracture of the palatal suture and increased lateral displacement of the maxilla. Compared with arch expansion using LFIO (model C), arch expansion using lateral osteotomy (model B) had a similar duration of palatal suture rupture and lateral maxillary extension. In view of the trauma and serious complications associated with LFIO, maxillary lateral wall-osteotomy could be considered a substitute for LFIO.


Asunto(s)
Maxilar , Técnica de Expansión Palatina , Adulto , Femenino , Análisis de Elementos Finitos , Humanos , Maxilar/diagnóstico por imagen , Maxilar/cirugía , Osteotomía Maxilar , Hueso Paladar , Adulto Joven
9.
Sci Total Environ ; 920: 170955, 2024 Apr 10.
Artículo en Inglés | MEDLINE | ID: mdl-38354805

RESUMEN

Examining the rationality of zoning designations and management measures in the initial establishment of national parks in China is of great significance for supporting decision-making regarding habitat conservation. There exists a research gap in exploring the threshold effects of both environmental and human-related factors on habitat distribution in the context of national parks. However, it may be a challenge because of the limited species distribution data. Our study aims to put forward an analytical framework that integrates species distribution models (SDMs) with interpretable machine learning methods. A case study was performed in the Sichuan region of the Giant Panda National Park (GPNP). We constructed a SDM based on the Random Forest algorithm and made use of accessible remote sensing and big data to predict the distribution of giant panda habitat (GPH) in 2020. Interpretable machine learning methods, namely Partial dependence plots (PDPs) and SHapley Additive exPlanations (SHAP), were utilized to uncover the underlying mechanisms of environmental and anthropogenic variables influencing the GPH distribution. Through GIS overlay analysis, areas where conflicts between human settlements, transportation infrastructure, and GPH exist were identified. Our findings indicated a potential 28.44 % decrease in GPH from 2014 to 2020. Environmental factors such as temperature, topography, and vegetation type, as well as anthropogenic factors including distance to built-up areas and transportation infrastructure, notably distance to national roads, provincial roads and city arterial roads, influenced the GPH distribution with threshold effects significantly. The overlay analysis revealed escalated conflicts between human settlements, transportation infrastructure, and GPH in 2020 compared to 2014. Currently, the Sichuan region of the GPNP implements two zones: a core protection zone and a general control zone, covering 63.71 % of the GPH, while 36.29 % remains outside the management scope. Drawing from the analysis above, this study provided suggestions for the adjustment of zoning designations and management measures in the GPNP.


Asunto(s)
Conservación de los Recursos Naturales , Ursidae , Animales , Humanos , Conservación de los Recursos Naturales/métodos , Parques Recreativos , Ecosistema , China
10.
Mar Pollut Bull ; 205: 116563, 2024 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-38861800

RESUMEN

This paper combines the meta-frontier analysis framework with the SBM-DEA model based on undesirable outputs to thoroughly reveal the mechanism of ecological efficiency differences in China's mariculture. This paper accurately identifies the weak links in ecological efficiency in various regions, thereby achieving a precise evaluation of mariculture ecological efficiency within the framework of technological heterogeneity. The results indicate that under the meta-frontier, the annual average ecological efficiency of mariculture in coastal areas of China during the sample period is 0.586, with a 41.4 % efficiency improvement space. However, under the group frontier, the annual average ecological efficiency is 0.783. The MEI in the BR economic zone and the YRD economic zone respectively stem from MI and TI, whereas in the PRD economic zone, the difference in the proportions of management inefficiency and technological inefficiency is not significant. Finally, this paper proposes four types of improvement paths.


Asunto(s)
Acuicultura , Conservación de los Recursos Naturales , China , Conservación de los Recursos Naturales/métodos , Ecosistema , Ecología
11.
Zookeys ; 1192: 213-235, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38433760

RESUMEN

A new bush frog species is described from Yunnan, China, based on phylogenetic analyses, species delimitation analyses, and morphological comparisons. Raorchesteshekouensissp. nov. is distinguished from all other congeners by a combination of 11 morphological characters. The new species brings the current number of Raorchestes species in China to ten, nine of which are distributed in Yunnan. Molecular analyses supported an unnamed lineage previously recorded as "Raorchestesgryllus" in northern Vietnam. Further studies including additional samples are necessary to clarify the species diversity and boundaries of Raorchestes in China and Indochina.

12.
Heliyon ; 10(14): e33944, 2024 Jul 30.
Artículo en Inglés | MEDLINE | ID: mdl-39114005

RESUMEN

It is challenging to accurately model the overall uncertainty of the power system when it is connected to large-scale intermittent generation sources such as wind and photovoltaic generation due to the inherent volatility, uncertainty, and indivisibility of renewable energy. Deep reinforcement learning (DRL) algorithms are introduced as a solution to avoid modeling the complex uncertainties and to adapt the fluctuation of uncertainty by interacting with the environment and using feedback to continuously improve their strategies. However, the large-scale nature and uncertainty of the system lead to the sparse reward problem and high-dimensional space issue in DRL. A hierarchical deep reinforcement learning (HDRL) scheme is designed to decompose the process of solving this problem into two stages, using the reinforcement learning (RL) agent in the global stage and the heuristic algorithm in the local stage to find optimal dispatching decisions for power systems under uncertainty. Simulation studies have shown that the proposed HDRL scheme is efficient in solving power system economic dispatch problems under both deterministic and uncertain scenarios thanks to its adaptation system uncertainty, and coping with the volatility of uncertain factors while significantly improving the speed of online decision-making.

13.
Sci Total Environ ; 917: 170287, 2024 Mar 20.
Artículo en Inglés | MEDLINE | ID: mdl-38266728

RESUMEN

Wet scavenging was critical in the atmospheric transport of 137Cs aerosols following the Fukushima accident. The aerosol size diversity and related microphysical processes produce complex behaviors during wet scavenging. Such behaviors are difficult to investigate using traditional simplified size distributions, resulting in inaccurate modeling. This study establishes an improved size-resolved wet scavenging model that considers the activation process. Using this model, five monodisperse simulations with five representative observed diameters with realistic solubility setting are performed to investigate the spatiotemporal wet scavenging behaviors of 137Cs aerosols. One polydisperse simulation with an empirical size distribution is also validated against the observation. The results reveal that 137Cs aerosols with diameters of 0.6 and 2.0 µm are mainly subject to below-cloud scavenging, which makes a significant contribution to low-deposition areas (<300 kBq/m2). For 137Cs aerosols with diameters of 6.4, 15, and 30 µm, in-cloud scavenging dominates, and the resulting depositions make significant contributions in high-deposition areas. The polydisperse results satisfy the criteria for good performance and better agree with the size, and deposition observations than the five monodisperse simulations, whereas for the concentration, the results show a similar RANK2 with the best mono1 and mono2 cases and reach the satisfactory criteria. These findings reveal the complex behavior and wet scavenging process of multi-mode 137Cs aerosols, improving our understanding and modeling.

14.
J Environ Radioact ; 272: 107363, 2024 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-38160503

RESUMEN

Release rate estimation is a vital means of revealing the emission process of radionuclides and assessing the environmental consequences in an emergency. Inverse modeling is widely used in emergency cases, but is vulnerable to plume biases in atmospheric dispersion modeling. One promising solution is a model called "Simultaneously Estimates the Release rate And Corrects both the plume range and Transport pattern" (SERACT). This study investigates the feasibility and behavior of SERACT based on four wind tunnel experiments replicating complex dispersion scenarios with both dense buildings and heterogeneous topography. SERACT's performance is compared with that of Tikhonov-regularized inversion and its predecessor. The results demonstrate that SERACT successfully corrects the modeled plume biases and simultaneously improves the release rate estimations in all four complex local-scale scenarios. The release rates retrieved by SERACT provide better agreement with the true release rates than those given by the other methods for all scenarios, with an average deviation of only 5.83%. After correction, the simulated plume reproduces the concentrations at all sites and achieves the best Pearson correlation coefficient (1.00) and fraction of simulations within a factor of 2 of the measurements (1.00); these values are 7.33 and 2.09 times higher, respectively, than those of simulations using release rates obtained using Tikhonov-regularized inversion.


Asunto(s)
Contaminantes Radiactivos del Aire , Monitoreo de Radiación , Modelos Teóricos , Monitoreo de Radiación/métodos , Contaminantes Radiactivos del Aire/análisis , Radioisótopos
15.
Ecol Evol ; 14(1): e10853, 2024 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-38259957

RESUMEN

The invasion of alien plant species threatens the composition and diversity of native communities. However, the invasiveness of alien plants and the resilience of native communities are dependent on the interactions between biotic and abiotic factors, such as natural enemies and nutrient availability. In our study, we simulated the invasion of nine invasive plant species into native plant communities using two levels of nutrient availability and suppression of natural enemies. We evaluated the effect of biotic and abiotic factors on the response of alien target species and the resistance of native communities to invasion. The results showed that the presence of enemies (enemy release) increased the biomass proportion of alien plants while decreasing that of native communities in the absence of nutrient addition. Furthermore, we also found that the negative effect of enemy suppression on the evenness of the native community and the root-to-shoot ratio of alien target species was greatest under nutrient addition. Therefore, nutrient-poor and natural enemies might promote the invasive success of alien species in native communities, whereas nutrient addition and enemy suppression can better enhance the resistance of native plant communities to invasion.

16.
Neuromolecular Med ; 26(1): 17, 2024 Apr 29.
Artículo en Inglés | MEDLINE | ID: mdl-38684592

RESUMEN

Post-stroke neuroinflammation affects the damage and recovery of neurological functions. T cells including CD8+ T cells were present in the ipsilateral hemisphere in the subacute and late phases of ischemic stroke. However, the potential roles of CD8+ T cell subsets in the progression of neuroinflammation have not been characterized. In the current mouse transient middle cerebral artery occlusion model, we investigated the existence of CD8+ T cell subsets in the ipsilateral hemisphere in the subacute and late phases of stroke. We found that ipsilateral CD8+ T cells were present on post-stroke day 3 and increased on post-stroke day 30. The day-3 ipsilateral CD8+ T cells predominantly produced interferon-γ (IFN-γ), while the day-30 ipsilateral CD8+ T cells co-expressed IFN-γ and interleukin-17A (IL-17A). In addition, evaluation of cytokines and transcription factors of the day-30 ipsilateral CD8+ T cells revealed the presence of T cytotoxic 1 (Tc1), T cytotoxic 17 (Tc17), and T cytotoxic 17/1 (Tc17/1) cells. Furthermore, based on the expression of a series of chemokine/cytokine receptors, viable ipsilateral Tc1, Tc17, and Tc17.1 cells were identified and enriched from the day-30 ipsilateral CD8+ T cells, respectively. Co-culture of microglia with ipsilateral Tc1, Tc17, or Tc17.1 cells indicated that the three CD8+ T cell subsets up-regulated the expression of pro-inflammatory mediators by microglia, with Tc17.1 cells being the most potent cell in doing so. Collectively, this study sheds light on the contributions of Tc1, Tc17, and Tc17.1 cells to long-term neuroinflammation after ischemic stroke.


Asunto(s)
Infarto de la Arteria Cerebral Media , Interleucina-17 , Ratones Endogámicos C57BL , Microglía , Enfermedades Neuroinflamatorias , Linfocitos T Citotóxicos , Animales , Microglía/metabolismo , Ratones , Masculino , Infarto de la Arteria Cerebral Media/inmunología , Infarto de la Arteria Cerebral Media/patología , Linfocitos T Citotóxicos/inmunología , Enfermedades Neuroinflamatorias/etiología , Accidente Cerebrovascular Isquémico/inmunología , Interferón gamma/biosíntesis , Encéfalo , Células Th17/inmunología , Modelos Animales de Enfermedad , Linfocitos T CD8-positivos , Técnicas de Cocultivo , Células Cultivadas
17.
Front Genet ; 15: 1410381, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39139823

RESUMEN

Spastic paraplegia type 4 (SPG4), the predominant form of Autosomal Dominant Hereditary spastic paraplegia (AD-HSP), is characterized by variants in the SPAST gene. This study reports a unique case of a late-onset SPG4 in a Han Chinese male, manifesting primarily as gait disturbances from lower extremity spasticity. Uncovered through whole-genome sequencing, a previously undocumented frameshift variant, c.1545dupA in exon 14 of the SPAST gene, was identified. Notably, this variant was absent in asymptomatic parents with confirmed paternity and maternity status, suggesting a de novo variant occurrence. This discovery emphasizes the potential of de novo variants to exhibit a late-onset pure pattern, extending the SPG4 variant spectrum, and consideration of such variants should be given in HSP patients with a negative family history.

18.
J Hazard Mater ; 466: 133632, 2024 Mar 15.
Artículo en Inglés | MEDLINE | ID: mdl-38309164

RESUMEN

Due to the capacity to offer abundant catalytic sites within porous solids featuring high surface areas, metal-organic frameworks (MOFs) and their derivatives have garnered considerable attention as prospective catalysts in environmental catalysis. To promote the industrial application of MOFs, there is an urgent need for an effective and environmental-friendly preparation approach. Breaking through the limitation of the traditional two-step preparation method that Pd was introduced to the already prepared Ce-BTC (Pd/Ce-BTC, BTC = 1, 3, 5 benzenetricarboxylate), in this work, we present a novel one-pot solvothermal method for synthesizing the Pd material supported by Ce-BTC (Pd@Ce-BTC). After pyrolysis in N2 flow or air flow, Pd-CeO2 catalysts derived from Pd@Ce-BTC exhibited much higher CO oxidation activity than those from Pd/Ce-BTC. Moreover, Pd/Ce-BTC and Pd@Ce-BTC pyrolyzed in N2 flow (Pd/Ce-BTC-N and Pd@Ce-BTC-N) could better catalyze the oxidation of CO than Pd/Ce-BTC and Pd@Ce-BTC pyrolyzed in air flow (Pd/Ce-BTC-A and Pd@Ce-BTC-A). Further characterizations revealed that the abundant surface Ce3+ species, rich surface adsorbed oxygen species and superior redox properties were the main reasons for the superior CO oxidation activity of Pd@Ce-BTC-N.

19.
J Hazard Mater ; 447: 130786, 2023 04 05.
Artículo en Inglés | MEDLINE | ID: mdl-36669410

RESUMEN

Eleven years after the Fukushima accident, independent objective estimates of the atmospheric 137Cs release still suffer from discontinuities such as negative release terms, oscillations, and temporal gaps, leading to noticeable differences from the subjective estimate. This paper describes an objective method that handles these artifacts and promotes the continuity of releases at fine resolutions. The proposed method uses the joint estimation model to reduce the oscillations induced by the model-observation discrepancies, and employs total variation regularization to recover the missing releases caused by insufficient observations. Adaptive parameterization is used to correct negative values. The application of this method to the Fukushima accident produces a source term that accurately approximates continuous releases at a fine temporal resolution of 1 h, providing a better match with the recognized subjective source term than nine published estimates, with a Pearson's correlation coefficient of 0.923 and an index of agreement of 0.872. This source term agrees with the timing of on-site gamma dose rate peaks, significantly improving the air concentration and deposition simulations, with FAC10 values of 0.564 and 0.990, respectively. The estimation error varies smoothly in a limited range with different regularization parameters, enabling automatic parameterization and demonstrating the potential for operational inversions.


Asunto(s)
Contaminantes Radiactivos del Aire , Accidente Nuclear de Fukushima , Monitoreo de Radiación , Monitoreo de Radiación/métodos , Radioisótopos de Cesio , Contaminantes Radiactivos del Aire/análisis , Japón
20.
Sci Total Environ ; 872: 162165, 2023 May 10.
Artículo en Inglés | MEDLINE | ID: mdl-36775160

RESUMEN

Wet deposition remains an important source of uncertainty in modeling of the atmospheric transport of 137Cs following the Fukushima Daiichi Nuclear Power Plant accident. Its behavior is often difficult to investigate owing to the limited resolution of meteorological field data and inconsistent model implementations. This study investigated the detailed behavior of 25 combinations of in- and below-cloud wet scavenging models using high-resolution (1 km × 1 km) meteorological input. These combinations were all implemented in the Weather Research and Forecasting-Chemistry model, thereby enabling consistent evaluation. The 1-km-resolution simulations were compared with simulations obtained previously using 3-km-resolution meteorological field data. Results revealed that rainfall of <1 mm/h is critical for simulation accuracy. The 1-km results revealed better representation of rainfall than that revealed by the 3-km results, but with spatiotemporal variability in accuracy. Owing to their sensitivity to rainfall, single-parameter wet deposition models showed improvements in performance in the 1-km simulations relative to that in the 3-km simulations. The multiparameter models showed more robust performance in terms of both simulations, and the Roselle-Mircea model presented the best performance among the 25 models considered. Wind transport showed substantial influence on the removal of atmospheric 137Cs, and it was nonnegligible even during periods in which wet deposition was dominant. The 1-km-resolution simulations effectively reproduced local-scale 137Cs concentrations but with deviations in timing, mainly because of biased wind direction. These findings indicate the necessity for a refined wind and dispersion model for local-scale simulation of 137Cs concentration.

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