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World Journal of Gastroenterology Jul 2023Glycogen storage diseases (GSDs), also referred to as glycogenoses, are inherited metabolic disorders of glycogen metabolism caused by deficiency of enzymes or... (Review)
Review
Glycogen storage diseases (GSDs), also referred to as glycogenoses, are inherited metabolic disorders of glycogen metabolism caused by deficiency of enzymes or transporters involved in the synthesis or degradation of glycogen leading to aberrant storage and/or utilization. The overall estimated GSD incidence is 1 case per 20000-43000 live births. There are over 20 types of GSD including the subtypes. This heterogeneous group of rare diseases represents inborn errors of carbohydrate metabolism and are classified based on the deficient enzyme and affected tissues. GSDs primarily affect liver or muscle or both as glycogen is particularly abundant in these tissues. However, besides liver and skeletal muscle, depending on the affected enzyme and its expression in various tissues, multiorgan involvement including heart, kidney and/or brain may be seen. Although GSDs share similar clinical features to some extent, there is a wide spectrum of clinical phenotypes. Currently, the goal of treatment is to maintain glucose homeostasis by dietary management and the use of uncooked cornstarch. In addition to nutritional interventions, pharmacological treatment, physical and supportive therapies, enzyme replacement therapy (ERT) and organ transplantation are other treatment approaches for both disease manifestations and long-term complications. The lack of a specific therapy for GSDs has prompted efforts to develop new treatment strategies like gene therapy. Since early diagnosis and aggressive treatment are related to better prognosis, physicians should be aware of these conditions and include GSDs in the differential diagnosis of patients with relevant manifestations including fasting hypoglycemia, hepatomegaly, hypertransaminasemia, hyperlipidemia, exercise intolerance, muscle cramps/pain, rhabdomyolysis, and muscle weakness. Here, we aim to provide a comprehensive review of GSDs. This review provides general characteristics of all types of GSDs with a focus on those with liver involvement.
Topics: Humans; Glycogen Storage Disease; Liver; Glycogen; Muscle, Skeletal; Genetic Therapy
PubMed: 37476587
DOI: 10.3748/wjg.v29.i25.3932 -
Open Research Europe 2024The changes in the energy sector after the Paris agreement and the establishment of the Green Deal, pressed the governments to embrace new measures to reduce greenhouse... (Review)
Review
The changes in the energy sector after the Paris agreement and the establishment of the Green Deal, pressed the governments to embrace new measures to reduce greenhouse gas emissions. Among them, is the replacement of fossil fuels by renewable energy sources or carbon-neutral alternative means, such as green hydrogen. As the European Commission approved green hydrogen as a clean fuel, the interest in investments and dedicated action plans related to its production and storage has significantly increased. Hydrogen storage is feasible in aboveground infrastructures as well as in underground constructions. Proper geological environments for underground hydrogen storage are porous media and rock cavities. Porous media are classified into depleted hydrocarbon reservoirs and aquifers, while rock cavities are subdivided into hard rock caverns, salt caverns, and abandoned mines. Depending on the storage option, various technological requirements are mandatory, influencing the required capital cost. Although the selection of the optimum storage technology is site depending, the techno-economical appraisal of the available underground storage options featured the porous media as the most economically attractive option. Depleted hydrocarbon reservoirs were of high interest as site characterisation and cavern mining are omitted due to pre-existing infrastructure, followed by aquifers, where hydrogen storage requires a much simpler construction. Research on data analytics and machine learning tools will open avenues for consolidated knowledge of geological storage technologies.
PubMed: 38764756
DOI: 10.12688/openreseurope.16974.1 -
Innovation (Cambridge (Mass.)) Nov 2023Carbon neutrality calls for renewable energies, and the efficient use of renewable energies requires energy storage mediums that enable the storage of excess energy and... (Review)
Review
Carbon neutrality calls for renewable energies, and the efficient use of renewable energies requires energy storage mediums that enable the storage of excess energy and reuse after spatiotemporal reallocation. Compared with traditional energy storage technologies, mobile energy storage technologies have the merits of low cost and high energy conversion efficiency, can be flexibly located, and cover a large range from miniature to large systems and from high energy density to high power density, although most of them still face challenges or technical bottlenecks. In this review, we provide an overview of the opportunities and challenges of these emerging energy storage technologies (including rechargeable batteries, fuel cells, and electrochemical and dielectric capacitors). Innovative materials, strategies, and technologies are highlighted. Finally, the future directions are envisioned. We hope this review will advance the development of mobile energy storage technologies and boost carbon neutrality.
PubMed: 37841885
DOI: 10.1016/j.xinn.2023.100518 -
Polymers Nov 2023Facing the era of information explosion and the advent of artificial intelligence, there is a growing demand for information technologies with huge storage capacity and... (Review)
Review
Facing the era of information explosion and the advent of artificial intelligence, there is a growing demand for information technologies with huge storage capacity and efficient computer processing. However, traditional silicon-based storage and computing technology will reach their limits and cannot meet the post-Moore information storage requirements of ultrasmall size, ultrahigh density, flexibility, biocompatibility, and recyclability. As a response to these concerns, polymer-based resistive memory materials have emerged as promising candidates for next-generation information storage and neuromorphic computing applications, with the advantages of easy molecular design, volatile and non-volatile storage, flexibility, and facile fabrication. Herein, we first summarize the memory device structures, memory effects, and memory mechanisms of polymers. Then, the recent advances in polymer resistive switching materials, including single-component polymers, polymer mixtures, 2D covalent polymers, and biomacromolecules for resistive memory devices, are highlighted. Finally, the challenges and future prospects of polymer memory materials and devices are discussed. Advances in polymer-based memristors will open new avenues in the design and integration of high-performance switching devices and facilitate their application in future information technology.
PubMed: 38006098
DOI: 10.3390/polym15224374 -
Microbiome Aug 2023There is an increasing interest in investigating the human gut virome for its influence on the gut bacterial community and its putative influence on the trajectory...
BACKGROUND
There is an increasing interest in investigating the human gut virome for its influence on the gut bacterial community and its putative influence on the trajectory towards health or disease. Most gut virome studies are based on sequencing of stored fecal samples. However, relatively little is known about how conventional storage buffers and storage conditions affect the infectivity of bacteriophages and influence the downstream metavirome sequencing.
RESULTS
We demonstrate that the infectivity and genome recovery rate of different spiked bacteriophages (T4, c2 and Phi X174) are variable and highly dependent on storage buffers. Regardless of the storage temperature and timespan, all tested phages immediately lost 100% (DNA/RNA Shield) or more than 90% (StayRNA and RNAlater) of their infectivity. Generally, in SM buffer at 4 °C phage infectivity was preserved for up to 30 days and phage DNA integrity was maintained for up to 100 days. While in CANVAX, the most effective buffer, all spiked phage genomes were preserved for at least 100 days. Prolonged storage time (500 days) at - 80 °C impacted viral diversity differently in the different buffers. Samples stored in CANVAX or DNA/RNA Shield buffer had the least shifts in metavirome composition, after prolonged storage, but they yielded more contigs classified as "uncharacterised". Moreover, in contrast to the SM buffer, these storage buffers yielded a higher fraction of bacterial DNA in metavirome-sequencing libraries. We demonstrated that the latter was due to inactivation of the DNases employed to remove extra-cellular DNA during virome extraction. The latter could be partly avoided by employing additional washing steps prior to virome extraction.
CONCLUSION
Fecal sample storage buffers and storage conditions (time and temperature) strongly influence bacteriophage infectivity and viral composition as determined by plaque assay and metavirome sequencing. The choice of buffer had a larger effect than storage temperature and storage time on the quality of the viral sequences and analyses. Based on these results, we recommend storage of fecal virome samples at in SM buffer at 4 °C for the isolation of viruses and at - 80 °C for metagenomic applications if practically feasible (i.e., access to cold storage). For fecal samples stored in other buffers, samples should be cleared of these buffers before viral extraction and sequencing. Video Abstract.
Topics: Humans; Bacteriophages; DNA, Bacterial; Feces; Metagenome; RNA
PubMed: 37635262
DOI: 10.1186/s40168-023-01632-9 -
Trends in Biotechnology Feb 2024DNA is an intelligent data storage medium due to its stability and high density. It has been used by nature for over 3.5 billion years. Compared with traditional... (Review)
Review
DNA is an intelligent data storage medium due to its stability and high density. It has been used by nature for over 3.5 billion years. Compared with traditional methods, DNA offers better compression and physical density. DNA can retain information for thousands of years. However, challenges exist in scalability, standardization, metadata gathering, biocybersecurity, and specialized tools. Addressing these challenges is crucial for widespread implementation. Collaboration among experts, as well as keeping the future in mind, is needed to unlock the full potential of DNA data storage, which promises low energy costs, high-density storage, and long-term stability.
Topics: Information Storage and Retrieval; DNA
PubMed: 37673693
DOI: 10.1016/j.tibtech.2023.08.001 -
Molecules (Basel, Switzerland) Sep 2023Currently, energy storage systems are of great importance in daily life due to our dependence on portable electronic devices and hybrid electric vehicles. Among these... (Review)
Review
Currently, energy storage systems are of great importance in daily life due to our dependence on portable electronic devices and hybrid electric vehicles. Among these energy storage systems, hybrid supercapacitor devices, constructed from a battery-type positive electrode and a capacitor-type negative electrode, have attracted widespread interest due to their potential applications. In general, they have a high energy density, a long cycling life, high safety, and environmental friendliness. This review first addresses the recent developments in state-of-the-art electrode materials, the structural design of electrodes, and the optimization of electrode performance. Then we summarize the possible classification of hybrid supercapacitor devices, and their potential applications. Finally, the fundamental theoretical aspects, charge-storage mechanism, and future developing trends are discussed. This review is intended to provide future research directions for the next generation of high-performance energy storage devices.
PubMed: 37687261
DOI: 10.3390/molecules28176432 -
Materials (Basel, Switzerland) Feb 2024Nowadays, high-pressure hydrogen storage is the most commercially used technology owing to its high hydrogen purity, rapid charging/discharging of hydrogen, and low-cost... (Review)
Review
Nowadays, high-pressure hydrogen storage is the most commercially used technology owing to its high hydrogen purity, rapid charging/discharging of hydrogen, and low-cost manufacturing. Despite numerous reviews on hydrogen storage technologies, there is a relative scarcity of comprehensive examinations specifically focused on high-pressure gaseous hydrogen storage and its associated materials. This article systematically presents the manufacturing processes and materials used for a variety of high-pressure hydrogen storage containers, including metal cylinders, carbon fiber composite cylinders, and emerging glass material-based hydrogen storage containers. Furthermore, it introduces the relevant principles and theoretical studies, showcasing their advantages and disadvantages compared to conventional high-pressure hydrogen storage containers. Finally, this article provides an outlook on the future development of high-pressure hydrogen storage containers.
PubMed: 38591616
DOI: 10.3390/ma17030721 -
Annual Review of Chemical and... Jun 2023Scientific and engineering capabilities in hydrocarbon supply chains developed over decades in international oil and gas companies (IOCs) uniquely position these... (Review)
Review
Scientific and engineering capabilities in hydrocarbon supply chains developed over decades in international oil and gas companies (IOCs) uniquely position these companies to drive rapid scale-up and transition to a net-zero emission economy. Flexible large-scale production of energy carriers such as hydrogen, ammonia, methanol, and other synthetic fuels produced with low- or zero-emission renewable power, nuclear energy, or hydrogen derived from natural gas with carbon capture and storage will enable long-distance transport and permanent storage options for clean energy. Use of energy carriers can overcome the inherent constraints of a fully electrified energy system by providing the energy and power densities, as well as transport and storage capacity, required to achieve energy supply and security in a net-zero emission economy, and over time allow optimization to the lowest cost for a consumer anywhere on the globe.
Topics: Natural Gas; Methanol; Nuclear Energy; Physical Phenomena; Hydrogen
PubMed: 36944251
DOI: 10.1146/annurev-chembioeng-092220-030446