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Myotonic dystrophy type 1 (DM1), the most frequent inherited muscular dystrophy in adults, is caused by the CTG repeat expansion in the 3’UTR of the DMPK gene. Mutant DMPK RNA accumulates in nuclear foci altering diverse cellular functions including alternative splicing regulation. DM1 is a multisystemic condition, with debilitating central nervous system alterations. Although a defective neuroglia communication has been described as a contributor of the brain pathology in DM1, the specific cellular and molecular events potentially affected in glia cells have not been totally recognized. Thus, to study the effects of DM1 mutation on glial physiology, in this work, we have established an inducible DM1 model derived from the MIO-M1 cell line expressing 648 CUG repeats. This new model recreated the molecular hallmarks of DM1 elicited by a toxic RNA gain-of-function mechanism accumulation of RNA foci colocalized with MBNL proteins and dysregulation of alternative splicing. By applying a microarray whole-transcriptome approach, we identified several gene changes associated with DM1 mutation in MIO-M1 cells, including the immune mediators CXCL10, CCL5, CXCL8, TNFAIP3, and TNFRSF9, as well as the microRNAs miR-222, miR-448, among others, as potential regulators. A gene ontology enrichment analyses revealed that inflammation and immune response emerged as major cellular deregulated processes in the MIO-M1 DM1 cells. Our findings indicate the involvement of an altered immune response in glia cells, opening new windows for the study of glia as potential contributor of the CNS symptoms in DM1.The influence of the repair process on the electrical properties of the normally off p-GaN high-electron-mobility transistor (HEMT) is studied in detail in this paper. We find that the etching process will cause the two-dimensional electron gas (2DEG) and the mobility of the p-GaN HEMT to decrease. However, the repair process will gradually recover the electrical properties. We study different repair methods and different repair conditions, propose the best repair conditions, and further fabricate the p-GaN HEMTs devices. The threshold voltage of the fabricated device is 1.6 V, the maximum gate voltage is 7 V, and the on-resistance is 23 Ω·mm. The device has a good performance, which proves that the repair conditions can be successfully applied to the fabricate of the p-GaN HEMT devices.Hybrid organic-inorganic perovskites, while well examined for photovoltaic applications, remain almost completely unexplored in the terahertz (THz) range. These low-cost hybrid materials are extremely attractive for THz applications because their optoelectronic properties can be chemically engineered with relative ease. Here, we experimentally demonstrate the first attempt to apply solution-processed polycrystalline films of hybrid perovskites for the development of photoconductive terahertz emitters. By using the widely studied methylammonium-based perovskites MAPbI3 and MAPbBr3, we fabricate and characterize large-aperture photoconductive antennas. The work presented here examines polycrystalline perovskite films excited both above and below the bandgap, as well as the scaling of THz emission with the applied bias field and the optical excitation fluence. The combination of ultrafast time-resolved spectroscopy and terahertz emission experiments allows us to determine the still-debated room temperature carrier lifetime and mobility of charge carriers in halide perovskites using an alternative noninvasive method. Our results demonstrate the applicability of hybrid perovskites for the development of scalable THz photoconductive devices, making these materials competitive with conventional semiconductors for THz emission.Microsatellite instability (MSI) is a hallmark of Lynch syndrome (LS)-related tumors but is not specific to it, as approximately 80% of MSI/mismatch repair-deficient (dMMR) tumors are sporadic. Methods leading to the diagnosis of LS have considerably evolved in recent years and so have tumoral tests for LS screening and for the discrimination of LS-related to MSI-sporadic tumors. In this review, we address the hallmarks of LS, including the clinical, histopathological, and molecular features. We present recent advances in diagnostic and screening strategies to identify LS patients. We also discuss the pitfalls associated with the current strategies, which should be taken into account to improve the diagnosis of LS and avoid inappropriate clinical management.Some studies have described that when the hemoglobin levels of chronic kidney disease (CKD) patients change, especially in those taking erythropoiesis-stimulating agents (ESA), they are associated with unfavorable outcomes such as increased morbidity and mortality, mainly due to cardiovascular events. This prospective cohort study included patients with end-stage renal disease currently undergoing hemodialysis. The initial 6-month clinical evaluation provided data of the variability in hemoglobin, associated blood parameters, and the use of erythropoietin. Subsequently, the patients were followed up for 78 months to evaluate mortality-associated factors. Fluorescein-5-isothiocyanate in vitro In total, 133 patients completed the 6-month follow-up with a mean age of 47.1 (±13.2) years. The majority were women (51.9%). Six-month hemoglobin levels were as follows always low (18.0%), intermediate/target (1.5%), always high (0.8%), low-amplitude fluctuation/Hb low (n = 37; 27.8%), low-amplitude fluctuation/Hb high (13.53%), and high-amplitude fluctuation (38.6%), among end-stage renal disease patients. At the end of 78 months, 50 (37.6%) patients died; 70% of deaths were attributed to cardiovascular etiologies. A high variability was observed in hemoglobin levels, which was not associated with mortality. Among all the variables evaluated, age, erythropoietin dose, and transferrin saturation were associated with a higher mortality. Thus, this study suggests that greater attention to erythropoietin doses and transferrin saturation levels may improve the survival of dialysis patients.Oculo-auriculo-vertebral-spectrum (OAVS; OMIM 164210) is a rare disorder originating from abnormal development of the first and second branchial arch. The clinical phenotype is extremely heterogeneous with ear anomalies, hemifacial microsomia, ocular defects, and vertebral malformations being the main features. MYT1, AMIGO2, and ZYG11B gene variants were reported in a few OAVS patients, but the etiology remains largely unknown. A multifactorial origin has been proposed, including the involvement of environmental and epigenetic mechanisms. link2 To identify the epigenetic mechanisms contributing to OAVS, we evaluated the DNA-methylation profiles of 41 OAVS unrelated affected individuals by using a genome-wide microarray-based methylation approach. The analysis was first carried out comparing OAVS patients with controls at the group level. It revealed a moderate epigenetic variation in a large number of genes implicated in basic chromatin dynamics such as DNA packaging and protein-DNA organization. link3 The alternative analysis in individual profiles based on the searching for Stochastic Epigenetic Variants (SEV) identified an increased number of SEVs in OAVS patients compared to controls. Although no recurrent deregulated enriched regions were found, isolated patients harboring suggestive epigenetic deregulations were identified. The recognition of a different DNA methylation pattern in the OAVS cohort and the identification of isolated patients with suggestive epigenetic variations provide consistent evidence for the contribution of epigenetic mechanisms to the etiology of this complex and heterogeneous disorder.Many studies have suggested a prognostic value of one or several positron emission tomography (PET) parameters in patients with small cell lung cancer (SCLC). However, studies are often small, and there is a considerable interstudy disagreement about which PET parameters have a prognostic value. The objective of this study was to perform a review and meta-analysis to identify the most promising PET parameter for prognostication. PubMed®, Cochrane, and Embase® were searched for papers addressing the prognostic value of any PET parameter at any treatment phase with any endpoint in patients with SCLC. Pooled hazard ratios (HRs) were calculated by a random effects model for the prognostic value of the baseline maximum standardized uptake value (SUVmax) and metabolic tumor volume (MTV). The qualitative analysis included 38 studies, of these, 19 studies were included in the meta-analyses. The pooled results showed that high baseline MTV was prognostic for overall survival (OS) (HR 2.83 (95% confidence interval [CI] 2.00-4.01) and progression-free survival (PFS) (HR 3.11 (95% CI 1.99-4.90)). The prognostic value of SUVmax was less pronounced (OS HR 1.50 (95% CI 1.17-1.91); PFS HR 1.24 (95% CI 0.94-1.63)). Baseline MTV is a strong prognosticator for OS and PFS in patients with SCLC. MTV has a prognostic value superior to those of other PET parameters, but whether MTV is superior to other prognosticators of tumor burden needs further investigation.Amphiphilic copolymers containing polydimethylsiloxane (PDMS) and polyethylene glycol methyl ether (MPEG) were obtained via an azide-alkyne cycloaddition reaction between alkyne-functionalized copolymer of MPEG methacrylate and azide-functionalized PDMS. “Click” reactions were carried out with an efficiency of 33-47% increasing grafting degrees. The grafted copolymers were able to carry out the micellization and encapsulation of active substances, such as vitamin C (VitC), ferulic acid (FA) and arginine (ARG) with drug loading content (DLC) in the range of 2-68% (VitC), and 51-89% (FA or ARG). In vitro release studies (phosphate buffer saline, PBS; pH = 7.4 or 5.5) demonstrated that the maximum release of active substances was mainly after 1-2 h. The permeability of released active substances through membrane mimicking skin evaluated by transdermal tests in Franz diffusion cells indicated slight diffusion into the solution (2-16%) and their remaining in the membrane. Studies on the selected carrier with FA showed no negative effect on cell viability, proliferation capacity or senescence, as well as cell apoptosis/necrosis differences or cell cycle interruption in comparison with control cells. These results indicated that the presented micellar systems are good candidates for carriers of cosmetic substances according to physicochemical characterization and biological studies.Stainless steel, widely present in the food industry, is frequently exposed to bacterial colonization with possible consequences on consumers’ health. 288 stainless steel disks with different roughness (0.25, 0.5 and 1 μm) were challenged with four Gram-negative (Escherichia coli ATCC 25922, Salmonella typhimurium ATCC 1402, Yersinia enterocolitica ATCC 9610 and Pseudomonas aeruginosa ATCC 27588) and four Gram-positive bacteria (Staphylococcus aureus ATCC 6538, Enterococcus faecalis ATCC 29212, Bacillus cereus ATCC 14579 and Listeria monocytogenes NCTT 10888) and underwent three different sanitizing treatments (UVC, alcohol 70% v/v and Gold lotion). Moreover, the same procedure was carried out onto the same surfaces after a nanotechnological surface coating (nanoXHAM® D). A significant bactericidal effect was exerted by all of the sanitizing treatments against all bacterial strains regardless of roughness and surface coating. The nanoXHAM® D coating itself induced an overall bactericidal effect as well as in synergy with all sanitizing treatments regardless of roughness.