Objective
Spinal and bulbar muscular atrophy (SBMA) is characterized by slow, progressive bulbar and limb muscle weakness; however, the pattern of progression of muscle fat infiltration remains ...unclear. We assessed the progression of muscle involvement in 81 patients with SBMA using whole‐body muscle magnetic resonance imaging (MRI), alongside clinical and laboratory findings.
Methods
This prospective study included patients with genetically confirmed SBMA who underwent whole‐body muscle MRI. We analyzed muscle fat infiltration and the pattern of involved muscles using cluster analysis, visualizing the sequential progression of fat infiltration. Muscle clusters demonstrated correlation with clinical scales and laboratory findings. Additionally, linear regression analysis was performed to identify the MRI section most strongly associated with 6‐minute walk test (6MWT).
Results
We included 81 patients with SBMA (age = 54.3 years). After categorizing the patients into 6 clusters based on the pattern of muscle fat infiltration, we observed that muscle involvement began in the posterior calf and progressed to the posterior thigh, pelvis, trunk, anterior thigh, medial thigh, anterior calf, and upper extremity muscles. These muscle clusters correlated significantly with disease duration (τ = 0.47, p < 0.001), 6MWT (τ = −0.49, p < 0.001), and serum creatinine level (τ = −0.46, p < 0.001). The whole‐body MRI indicated the thigh as the section most significantly correlated with 6MWT.
Interpretation
We used whole‐body muscle MRI to determine the sequential progression of the fat infiltration in SBMA. Our findings may enable the identification of objective and reliable imaging outcome measures in the study of the natural history or future clinical trials of SBMA. ANN NEUROL 2024;95:596–606
Microvilli on T cells have been proposed to survey surfaces of antigen-presenting cells (APC) or facilitate adhesion under flow; however, whether they serve essential functions during T cell ...activation remains unclear. Here we show that antigen-specific T cells deposit membrane particles derived from microvilli onto the surface of cognate antigen-bearing APCs. Microvilli carry T cell receptors (TCR) at all stages of T cell activation and are released as large TCR-enriched, T cell microvilli particles (TMP) in a process of trogocytosis. These microvilli exclusively contain protein arrestin-domain-containing protein 1, which is directly involved in membrane budding and, in combination with vacuolar protein-sorting-associated protein 4, transforms large TMPs into smaller, exosome-sized TMPs. Notably, TMPs from CD4
T cells are enriched with LFA-2/CD2 and various cytokines involved in activating dendritic cells. Collectively, these results demonstrate that T cell microvilli constitute "immunological synaptosomes" that carry T cell messages to APCs.
Oxidative stress caused by free radicals has been implicated in the pathogenesis of amyotrophic lateral sclerosis (ALS). Edaravone (also known as MCI-186), a free radical scavenger, was approved as ...an ALS treatment in 2015 in Japan. However, the therapeutic effects of edaravone on patients with ALS outside of Japan are not yet reported. This study aims to investigate effects of edaravone on ALS patients in the Korean population. The study included 22 patients with ALS who were treated with edaravone. Of the 16 patients who finished six cycles of treatment, a mean decline of ALSFRS-R after the treatments was 5.75 ± 6.07 points and the average change of FVC was − 8.7 ± 17.0%. Patients experienced only minor adverse events. This study reports on the open-label study of edaravone on patients in Korea for ALS patients, which showed a modest effect of edaravone in this population of ALS patients.
Electrospinning has emerged as one of the potential techniques for producing nanofibers. The use of electrospun nanofibers in drug delivery has increased rapidly over recent years due to their ...valuable properties, which include a large surface area, high porosity, small pore size, superior mechanical properties, and ease of surface modification. A drug loaded nanofiber membrane can be prepared via electrospinning using a model drug and polymer solution; however, the release of the drug from the nanofiber membrane in a safe and controlled way is challenging as a result of the initial burst release. Employing a core-sheath design provides a promising solution for controlling the initial burst release. Numerous studies have reported on the preparation of core-sheath nanofibers by coaxial electrospinning for drug delivery applications. This paper summarizes the physical phenomena, the effects of various parameters in coaxial electrospinning, and the usefulness of core-sheath nanofibers in drug delivery. Furthermore, this report also highlights the future challenges involved in utilizing core-sheath nanofibers for drug delivery applications.
Objectives
Myasthenia gravis (MG) is an autoimmune disease characterized by muscle weakness and fatigue. Our objective was to investigate the incidence of MG using the National Health Insurance ...database of South Korea.
Materials and methods
We conducted a retrospective cohort analysis of patients with the G70.0 code designated as MG and administered with MG medications for >3 months from 2007 to 2018 using nationwide data from South Korea.
Results
A total of 8,376 patients with MG during the period of 2010–2018 were identified. There were 3,862 (46.1%) male and 4,517 (53.9%) female patients. The standardized incidence rate was 1.18/100,000 in 2010, and increased to 1.81/100,000 in 2018. The standardized prevalence was 7.50/100,000 in 2010, and changed to 11.15/100,000 in 2018. Pyridostigmine was used to treat 82.3 ± 1.2% of patients with MG during 2010–2018. Among MG patients, 85.7 ± 0.9% used steroids, 31.6 ± 4.8% used azathioprine, 12.9 ± 9.5% used tacrolimus, 7.2 ± 2.1% used cyclosporine, 6.2 ± 1.8% used mycophenolate mofetil, and 0.4 ± 0.1% used methotrexate. Thymectomy was performed in 1,130 MG patients, and the time from MG diagnosis to thymectomy decreased from 2010 to 2018.
Conclusion
Based on the national registry data from 2010 to 2018, the incidence and prevalence rate in South Korea has increased. Whereas the use of IVIG has remained stable, thymectomy is performed earlier than before, and the distribution of immunosuppressant therapies has changed over the years with an increase in tacrolimus and mycophenolate mofetil. We expect that this study will serve as a basis for future South Korean MG epidemiological studies.
Polymer foams have low density, good heat insulation, good sound insulation effects, high specific strength, and high corrosion resistance, and are widely used in civil and industrial applications. ...In this paper, the classification of polymer foams, principles of the foaming process, types of blowing agents, and raw materials of polymer foams are reviewed. The research progress of various foaming methods and the current problems and possible solutions are discussed in detail.
Molybdenum trioxide (MoO3) has significant potential as an attractive semiconductor material owing to its low cost, nontoxicity, high electrochemical activity, and environmentally benign nature ...compared to other metal oxides. Nevertheless, its potential for photocatalytic application has been hindered due to the high recombination rate of photogenerated electron–hole pairs, resulting in lower photocatalytic performance. In this work, a facile method was developed to synthesize SnS2/MoO3 hollow nanotubes. The as-prepared samples exhibit enhanced hydrogen peroxide production performance. A series of characterization experiments were conducted and the results prove that SnS2 nanosheets are uniformly dispersed on the surface of the hollow MoO3 nanotubes. Three-dimensional hetero-SnS2/MoO3 exhibited high charge transfer ability owing to its hollow structure and high surface area, which provides more active sites and improves the mobility of the radicals. Meanwhile, a synergistic effect between MoO3 and SnS2 is found to yield optimal hydrogen peroxide production performance. The as-prepared SnS2/MoO3 hollow nanotubes can exhibit best hydrogen peroxide production performance with good stability (95% after four cycles).
Recently, MXenes, which are 2D early transition metal carbides and carbonitrides, have attracted wide attention because of their excellent conductivities. Here, the electrode applications of ...Ti2C(OH)xFy, one member of the MXene family, in WSe2 and MoS2 field effect transistors (FETs) are assessed. Kelvin probe force microscopy analysis is performed to determine its work function, which is estimated to be ≈4.98 eV. Devices based on WSe2/Ti2C(OH)xFy and MoS2/Ti2C(OH)xFy heterostructures are fabricated with the mechanical transfer method and their electronic performances evaluated. The temperature‐dependent current–voltage transfer characteristics of the devices are determined to extract their Schottky barrier heights. The hole barrier between WSe2 and Ti2C(OH)xFy is estimated to be ≈0.23 eV and the electron barrier between the MoS2 band and Ti2C(OH)xFy is ≈0.19 eV, which indicates that the pinning effect occurs at the MoS2/Ti2C(OH)xFy interface but not at the WSe2/Ti2C(OH)xFy interface; this difference arises because of the difference between the band structures of WSe2 and MoS2. A complementary metal–oxide–semiconductor inverter based on these electrode properties of Ti2C(OH)xFy with MoS2 (n‐channel) and WSe2 (p‐channel) is fabricated, which demonstrates that Ti2C(OH)xFy is a promising electrode for future nanoelectronics applications.
By using Ti2C(OH)xFy as an electrode for the integration of 2D p‐FET (WSe2) and n‐FET (MoS2), the heights of the hole barriers of WSe2/Ti2C(OH)xFy and MoS2/Ti2C(OH)xFy are determined to be 0.23 eV and 0.19 eV, respectively. A complementary metal–oxide–semiconductor inverter is fabricated, which demonstrates that Ti2C(OH)xFy is a promising electrode for future nanoelectronics applications.
► We investigate formation, evolution, and impact of co-opetition and develop a multi-level conceptual framework. ► Our study shows that co-opetition is challenging yet very helpful for firms to ...address technological challenges, create mutual benefits, and generate innovation. ► Co-opetition between large players causes subsequent co-opetition among other firms. ► Co-opetition capabilities of firms are important in enhancing common benefits and in gaining larger share of the benefits.
We investigate why and how co-opetition (simultaneous pursuit of collaboration and competition) between large firms occurs, evolves, and impacts the participating firms and the industry. We develop a multi-level conceptual framework by combining literature-based conceptual arguments and insights from an in-depth study of an exemplar case of co-opetition between Samsung Electronics and Sony Corporation. Our study demonstrates that co-opetition is challenging yet very helpful for firms to address major technological challenges, to create benefits for partnering firms, and to advance technological innovation. We also show that co-opetition between giants causes subsequent co-opetition among other firms and results in advanced technological development. Moreover, co-opetition capabilities of firms play an important role in enhancing common benefits as well as in gaining proportionately larger share of the benefits.
With the continuous development and progress of materials science, increasingly more attention has been paid to the new technology of powder synthesis and material preparation. The hydrothermal ...method is a promising liquid phase preparation technology that has developed rapidly during recent years. It is widely used in many fields, such as the piezoelectric, ferroelectric, ceramic powder, and oxide film fields. The hydrothermal method has resulted in many new methods during the long-term research process, such as adding other force fields to the hydrothermal condition reaction system. These force fields mainly include direct current, electric, magnetic (autoclaves composed of non-ferroelectric materials), and microwave fields. Among them, the microwave hydrothermal method, as an extension of the hydrothermal reaction, cleverly uses the microwave temperature to compensate for the lack of temperature in the hydrothermal method, allowing better practical application. This paper reviews the development of the hydrothermal and microwave hydrothermal methods, introduces their reaction mechanisms, and focuses on the practical application of the two methods.
PDF
