Clinical and Pathological Characteristics of Four Korean Patients with Limb-Girdle Muscular Dystrophy type 2B

Limb-girdle muscular dystrophy type 2B (LGMD2B), a subtype of autosomal recessive limb-girdle muscular dystrophy (ARLGMD), is characterized by a relatively late onset and slow progressive course. LGMD2B is known to be caused by the loss of the dysferlin protein at sarcolemma in muscle fibers. In this study, the clinical and pathological characteristics of Korean LGMD2B patients were investigated. Seventeen patients with ARLGMD underwent muscle biopsy and the histochemical examination was performed. For the immunocytochemistry, a set of antibodies against dystrophin, α, β, γ, δ-sarcoglycans, dysferlin, caveolin-3, and β-dystroglycan was used. Four patients (24%) showed selective loss of immunoreactivity against dysferlin at the sarcolemma on the muscle specimens. Therefore, they were classified into the LGMD2B category. The age at the onset of disease ranged from 9 yr to 33 yr, and none of the patients was wheelchair bound at the neurological examination. The serum creatine kinase (CK) was high in all the patients (4010-5310 IU/L). The pathologic examination showed mild to moderate dystrophic features. These are the first Korean LGMD2B cases with a dysferlin deficiency confirmed by immunocytochemistry. The clinical, pathological, and immunocytochemical findings of the patients with LGMD2B in this study were in accordance with those of other previous reports

Comparative Interactomes of VRK1 and VRK3 with Their Distinct Roles in the Cell Cycle of Liver Cancer

Vaccinia-related kinase 1 (VRK1) and VRK3 are members of the VRK family of serine/threonine kinases and are principally localized in the nucleus. Despite the crucial roles of VRK1/VRK3 in physiology and disease, the molecular and functional interactions of VRK1/VRK3 are poorly understood. Here, we identified over 200 unreported VRK1/VRK3-interacting candidate proteins by affinity purification and LC-MS/MS. The networks of VRK1 and VRK3 interactomes were found to be associated with important biological processes such as the cell cycle, DNA repair, chromatin assembly, and RNA processing. Interactions of interacting proteins with VRK1/VRK3 were confirmed by biochemical assays. We also found that phosphorylations of XRCC5 were regulated by both VRK1/VRK3, and that of CCNB1 was regulated by VRK3. In liver cancer cells and tissues, VRK1/VRK3 were highly upregulated and its depletion affected cell cycle progression in the different phases. VRK3 seemed to affect S phase progression and G2 or M phase entry and exit, whereas VRK1 affects G1/S transition in the liver cancer, which could be explained by different interacting candidate proteins. Thus, this study not only provides a resource for investigating the unidentified functions of VRK1/VRK3, but also an insight into the regulatory roles of VRK1/VRK3 in biological processes.11Ysciescopuskc

Perceptions of health status, physical fitness, and participation in physical activity among adolescents before and after COVID-19: analysis of 2019‑2021 data from the Republic of Korea national sports survey

Research has examined the detrimental effects of the coronavirus disease (COVID-19) pandemic on health and physical fitness in adolescents; however, studies comparing these parameters before and after the COVID-19 outbreak have been scarce. Therefore, this study investigated differences in perceived health status, perceived physical fitness, and participation in physical activity among adolescents in the Republic of Korea before and after the COVID-19 outbreak. We chose a sample of data from 2102 adolescents aged 14–19, collected as part of a national survey by the Republic of Korea Ministry of Culture, Sports, and Tourism from 2019 to 2021. We focused on five items from the survey related to health awareness and physical activity. Although perceived health status was lower in 2021 than in 2019 or 2020, there were no differences in perceived physical fitness during the three years of the study. Regular participation in physical activity was less common in 2020 than in 2019 or 2021. The proportion of adolescents reporting sufficient rest and sleep was lower in 2021 than in 2020. In addition, fewer adolescents reported eating regular meals and engaging in nutritional supplementation in 2021 than in 2019 and 2020. Rates of abstinence from alcohol and smoking cessation were higher in 2021 than in 2019 or 2020. For all three years, adolescents reported the following as the primary reasons for engaging in regular physical activity (in order): “maintenance of mental health”, “maintenance of physical health”, “help in daily life”, and “reduction of medical expenses”. In preparation for the post-COVID-19 era, these results highlight the need to prepare measures and countermeasures to promote health and physical activity among adolescents in the Republic of Korea

A Study on Group Key Agreement in Sensor Network Environments Using Two-Dimensional Arrays

These days, with the emergence of the concept of ubiquitous computing, sensor networks that collect, analyze and process all the information through the sensors have become of huge interest. However, sensor network technology fundamentally has wireless communication infrastructure as its foundation and thus has security weakness and limitations such as low computing capacity, power supply limitations and price. In this paper, and considering the characteristics of the sensor network environment, we propose a group key agreement method using a keyset pre-distribution of two-dimension arrays that should minimize the exposure of key and personal information. The key collision problems are resolved by utilizing a polygonal shape’s center of gravity. The method shows that calculating a polygonal shape’s center of gravity only requires a very small amount of calculations from the users. The simple calculation not only increases the group key generation efficiency, but also enhances the sense of security by protecting information between nodes

The Korean Mistletoe (Viscum album coloratum) Extract Has an Antiobesity Effect and Protects against Hepatic Steatosis in Mice with High-Fat Diet-Induced Obesity

This study investigates the inhibitory effects of Korean mistletoe extract (KME) on adipogenic factors in 3T3-L1 cells and obesity and nonalcoholic fatty liver disease (NAFLD) in mice fed a high-fat diet. Male C57Bl/6 mice fed a high-fat diet were treated with KME (3 g/kg/day) for 15 weeks for the antiobesity and NAFLD experiments. Body weight and daily food intake were measured regularly during the experimental period. The epididymal pad was measured and liver histology was observed. The effects of KME on thermogenesis and endurance capacity were measured. The effects of KME on adipogenic factors were examined in 3T3-L1 cells. Body and epididymal fat pad weights were reduced in KME-treated mice, and histological examination showed an amelioration of fatty liver in KME-treated mice, without an effect on food consumption. KME potently induces mitochondrial activity by activating thermogenesis and improving endurance capacity. KME also inhibited adipogenic factors in vitro. These results demonstrate the inhibitory effects of KME on obesity and NAFLD in mice fed a high-fat diet. The effects appear to be mediated through an enhanced mitochondrial activity. Therefore, KME may be an effective therapeutic candidate for treating obesity and fatty liver caused by a high-fat diet

Autism-like behavior caused by deletion of vaccinia-related kinase 3 is improved by TrkB stimulation

Vaccinia-related kinases (VRKs) are multifaceted serine/threonine kinases that play essential roles in various aspects of cell signaling, cell cycle progression, apoptosis, and neuronal development and differentiation. However, the neuronal function of VRK3 is still unknown despite its etiological potential in human autism spectrum disorder (ASD). Here, we report that VRK3-deficient mice exhibit typical symptoms of autism-like behavior, including hyperactivity, stereotyped behaviors, reduced social interaction, and impaired context-dependent spatial memory. A significant decrease in dendritic spine number and arborization were identified in the hippocampus CA1 of VRK3-deficient mice. These mice also exhibited a reduced rectification of AMPA receptor-mediated current and changes in expression of synaptic and signaling proteins, including tyrosine receptor kinase B (TrkB), Arc, and CaMKII alpha. Notably, TrkB stimulation with 7,8-dihydroxyflavone reversed the altered synaptic structure and function and successfully restored autism-like behavior in VRK3-deficient mice. These results reveal that VRK3 plays a critical role in neurodevelopmental disorders and suggest a potential therapeutic strategy for ASD.112Ysciescopu

Non-Dioxin-Like Polychlorinated Biphenyls Inhibit G-Protein Coupled Receptor-Mediated Ca2+ Signaling by Blocking Store-Operated Ca2+ Entry

Polychlorinated biphenyls (PCBs) are ubiquitous pollutants which accumulate in the food chain. Recently, several molecular mechanisms by which non-dioxin-like (NDL) PCBs mediate neurodevelopmental and neurobehavioral toxicity have been elucidated. However, although the G-protein coupled receptor (GPCR) is a significant target for neurobehavioral disturbance, our understanding of the effects of PCBs on GPCR signaling remains unclear. In this study, we investigated the effects of NDL-PCBs on GPCR-mediated Ca2+ signaling in PC12 cells. We found that ortho-substituted 2,2', 6-trichlorinated biphenyl (PCB19) caused a rapid decline in the Ca2+ signaling of bradykinin, a typical Gq-and phospholipase C beta-coupled GPCR, without any effect on its inositol 1,4,5-trisphosphate production. PCB19 reduced thapsigargin-induced sustained cytosolic Ca2+ levels, suggesting that PCB19 inhibits SOCE. The abilities of other NDL-PCBs to inhibit store-operated Ca2+ entry (SOCE) were also examined and found to be of similar potencies to that of PCB19. PCB19 also showed a manner equivalent to that of known SOCE inhibitors. PCB19-mediated SOCE inhibition was confirmed by demonstrating the ability of PCB19 to inhibit the SOCE current and thapsigargin-induced Mn2+ influx. These results imply that one of the molecular mechanism by which NDL-PCBs cause neurobehavioral disturbances involves NDL-PCB-mediated inhibition of SOCE, thereby interfering with GPCR-mediated Ca2+ signaling.1142Ysciescopu

Successful Radiofrequency Catheter Ablation for Wolff-Parkinson-White Syndrome Within the Neck of a Coronary Sinus Diverticulum

Posteroseptal accessory pathways are often associated with coronary sinus diverticula. These diverticula contain myocardial coats which serve as a bypass tract. We report a 54-year-old woman who underwent radiofrequency (RF) catheter ablation for Wolff-Parkinson-White (WPW) syndrome. The surface electrocardiography (ECG) demonstrated pre-excitation, indicating a posteroseptal accessory pathway. A catheter ablation via a transaortic approach failed to ablate the accessory pathway. Coronary sinus venography revealed the presence of a diverticulum near the ostium. An electrogram in the neck of the diverticulum showed the coronary sinus myocardial extension potential, which was successfully ablated by delivery of RF energy