Impaired Cardiomyocyte Maturation Leading to DCM: A Case Report and Literature Review

Background: The maturation of cardiomyocytes is a rapidly evolving area of research within the field of cardiovascular medicine. Understanding the molecular mechanisms underlying cardiomyocyte maturation is essential to advancing our knowledge of the underlying causes of cardiovascular disease. Impaired maturation can lead to the development of cardiomyopathy, particularly dilated cardiomyopathy (DCM). Recent studies have confirmed the involvement of the ACTN2 and RYR2 genes in the maturation process, facilitating the functional maturation of the sarcomere and calcium handling. Defective sarcomere and electrophysiological maturation have been linked to severe forms of cardiomyopathy. This report presents a rare case of DCM with myocardial non-compaction, probably resulting from allelic collapse of both the ACTN2 and RYR2 genes. Case Presentation: The proband in this case was a four-year-old male child who presented with a recurrent and aggressive reduction in activity tolerance, decreased ingestion volume, and profuse sweating. Electrocardiography revealed significant ST-T segment depression (II, III, aVF V3-V6 ST segment depression >0.05 mV with inverted T-waves). Echocardiography showed an enlarged left ventricle and marked myocardial non-compaction. Cardiac magnetic resonance imaging revealed increased left ventricular trabeculae, an enlarged left ventricle, and a reduced ejection fraction. Whole exome sequencing revealed a restricted genomic depletion in the 1q43 region (chr1:236,686,454-237,833,988/Hg38), encompassing the coding genes ACTN2, MTR, and RYR2. The identified variant resulted in heterozygous variations in these three genes, with the ACTN2 g.236,686,454-236,764,631_del and RYR2 g.237,402,134-237,833,988_del variants being the dominant contributors to the induction of cardiomyopathy. The patient was finally diagnosed with DCM and left ventricular myocardial non-compaction. Conclusions: This study reports a rare case of DCM with myocardial non-compaction caused by the allelic collapse of the ACTN2 and RYR2 genes. This case provides the first human validation of the critical role of cardiomyocyte maturation in maintaining cardiac function and stability and confirms the key findings of previous experimental research conducted by our group. This report emphasizes the connection between genes involved in regulating the maturation of cardiomyocytes and the development of cardiomyopathy

Expression and information hierarchical authorization of BDS reference station observation data based on states and residual

BDS reference stations typically store and transmit raw observations directly and make them available to users, which can lead to two disadvantages:first, there is no hierarchical authorization mechanism for users. Any user can solve the precise spatial, time and atmospheric datum information of the reference station through the original observation measurement, which poses a potential threat to the safety of the reference station, second, the strong correlation between the original observation measurement leads to a large amount of data, in BDS receiver observation appears network, uninterrupted, high sampling and multi-band trend, its observation data is a massive explosion, to the data storage and transmission have brought great pressure. Therefore, this paper proposes to use state and residual to express the raw observation data of BDS reference station. Compared with the common data services based on international protocols such as RINEX, Compact RINEX and RTCM, the advantage of this method is that it cannot only provide hierarchical authorization service of datum information for users, technically guarantee the security of data services, but also significantly reduce the amount of transmission data and user-side computation. At the same time, the new expression is equivalent to the original data and does not reduce the accuracy of the final calculation results. This method can be used to establish BDS observation data storage and transmission protocol with independent intellectual property rights, and provide technical support for satellite-based broadcasting services of high-precision data

Room-Temperature-Processed Flexible Amorphous InGaZnO Thin Film Transistor

A room-temperature flexible amorphous indium–gallium–zinc oxide thin film transistor (a-IGZO TFT) technology is developed on plastic substrates, in which both the gate dielectric and passivation layers of the TFTs are formed by an anodic oxidation (anodization) technique. While the gate dielectric Al₂O₃ is grown with a conventional anodization on an Al:Nd gate electrode, the channel passivation layer Al₂O₃ is formed using a localized anodization technique. The anodized Al₂O₃ passivation layer shows a superior passivation effect to that of PECVD SiO₂. The room-temperature-processed flexible a-IGZO TFT exhibits a field-effect mobility of 7.5 cm²/V·s, a subthreshold swing of 0.44 V/dec, an on–off ratio of 3.1 × 10⁸, and an acceptable gate-bias stability with threshold voltage shifts of 2.65 and −1.09 V under positive gate-bias stress and negative gate-bias stress, respectively. Bending and fatigue tests confirm that the flexible a-IGZO TFT also has a good mechanical reliability, with electrical performances remaining consistent up to a strain of 0.76% as well as after 1200 cycles of fatigue testing

An Overview of Light-Mediated Impact of Graphene Oxide on Algae: Photo-Transform, Toxicity and Mechanism

Due to the unique chemical and physical properties, graphene-based nanomaterials are increasingly being introduced into various scientific fields. They all play very important roles in different fields and are widely used. Graphene oxide (GO) is one of the most popular and representative carbon nanomaterials; scientists have great research interest in it. When carbon nanomaterials such as GO are released into the aquatic environment, their physicochemical properties will be influenced by natural light, resulting in the potential change in toxic effects on aquatic organisms. Algae, as a typical aquatic organism, is extensively regarded as a model microorganism to assess the biotoxicity of nanomaterials. In this review, we overview the light-mediated impact of GO on algae. We summarize the photo-transformation of GO under different illumination conditions and the effect of illumination on the physicochemical properties of GO. Then, we combined metabolomics, genotoxicity, and proteomics with standard toxicity assays (cell division, membrane permeability, oxidative stress, photosynthesis, cellular ultrastructure, and so on) to compare native and environmentally transformed GO induction toxicological mechanisms. By correlating lights, physicochemical properties, and biotoxicity, this review is valuable for environmental fate assessments on graphene-based nanoparticles, providing a theoretical basis and support for evaluating the potential ecological health and environmental risks of graphene-based nanoparticles in real natural water environments

Room-Temperature-Processed Flexible Amorphous InGaZnO Thin Film Transistor

A room-temperature flexible amorphous indium–gallium–zinc oxide thin film transistor (a-IGZO TFT) technology is developed on plastic substrates, in which both the gate dielectric and passivation layers of the TFTs are formed by an anodic oxidation (anodization) technique. While the gate dielectric Al₂O₃ is grown with a conventional anodization on an Al:Nd gate electrode, the channel passivation layer Al₂O₃ is formed using a localized anodization technique. The anodized Al₂O₃ passivation layer shows a superior passivation effect to that of PECVD SiO₂. The room-temperature-processed flexible a-IGZO TFT exhibits a field-effect mobility of 7.5 cm²/V·s, a subthreshold swing of 0.44 V/dec, an on–off ratio of 3.1 × 10⁸, and an acceptable gate-bias stability with threshold voltage shifts of 2.65 and −1.09 V under positive gate-bias stress and negative gate-bias stress, respectively. Bending and fatigue tests confirm that the flexible a-IGZO TFT also has a good mechanical reliability, with electrical performances remaining consistent up to a strain of 0.76% as well as after 1200 cycles of fatigue testing