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Journal of Inherited Metabolic Disease Sep 2023Lysosomal Storage Disorders (LSDs) are a diverse group of inherited, monogenic diseases caused by functional defects in specific lysosomal proteins. The lysosome is a... (Review)
Review
Lysosomal Storage Disorders (LSDs) are a diverse group of inherited, monogenic diseases caused by functional defects in specific lysosomal proteins. The lysosome is a cellular organelle that plays a critical role in catabolism of waste products and recycling of macromolecules in the body. Disruption to the normal function of the lysosome can result in the toxic accumulation of storage products, often leading to irreparable cellular damage and organ dysfunction followed by premature death. The majority of LSDs have no curative treatment, with many clinical subtypes presenting in early infancy and childhood. Over two-thirds of LSDs present with progressive neurodegeneration, often in combination with other debilitating peripheral symptoms. Consequently, there is a pressing unmet clinical need to develop new therapeutic interventions to treat these conditions. The blood-brain barrier is a crucial hurdle that needs to be overcome in order to effectively treat the central nervous system (CNS), adding considerable complexity to therapeutic design and delivery. Enzyme replacement therapy (ERT) treatments aimed at either direct injection into the brain, or using blood-brain barrier constructs are discussed, alongside more conventional substrate reduction and other drug-related therapies. Other promising strategies developed in recent years, include gene therapy technologies specifically tailored for more effectively targeting treatment to the CNS. Here, we discuss the most recent advances in CNS-targeted treatments for neurological LSDs with a particular emphasis on gene therapy-based modalities, such as Adeno-Associated Virus and haematopoietic stem cell gene therapy approaches that encouragingly, at the time of writing are being evaluated in LSD clinical trials in increasing numbers. If safety, efficacy and improved quality of life can be demonstrated, these therapies have the potential to be the new standard of care treatments for LSD patients.
Topics: Humans; Child; Quality of Life; Lysosomal Storage Diseases; Genetic Therapy; Brain; Lysosomes; Enzyme Replacement Therapy
PubMed: 37078180
DOI: 10.1002/jimd.12615 -
Transfusion Medicine and Hemotherapy :... Apr 2024Transfusion of platelets is a life-saving medical strategy used worldwide to treat patients with thrombocytopenia as well as platelet function disorders. (Review)
Review
BACKGROUND
Transfusion of platelets is a life-saving medical strategy used worldwide to treat patients with thrombocytopenia as well as platelet function disorders.
SUMMARY
Until the end of 1960s, platelets were stored in the cold because of their superior hemostatic functionality. Cold storage of platelets was then abandoned due to better posttransfusion recovery and survival of room temperature (RT)-stored platelets, demonstrated by radioactive labeling studies. Based on these findings, RT became the standard condition to store platelets for clinical applications. Evidence shows that RT storage increases the risk of septic transfusion reactions associated with bacterial contamination. Therefore, the storage time is currently limited to 4-7 days, according to the national guidelines, causing a constant challenge to cover the clinical request. Despite the enormous efforts made to optimize storage conditions of platelets, the quality and efficacy of platelets still decrease during the short storage time at RT. In this context, during the last years, cold storage has seen a renaissance due to the better hemostatic functionality, reduced risk of bacterial contamination, and potentially longer storage time.
KEY MESSAGES
In this review, we will focus on the impact of cold storage on the in vitro platelet functions as promising alternative storage temperature for future medical applications.
PubMed: 38584694
DOI: 10.1159/000533735 -
Heliyon Aug 2023To achieve the Net Zero Carbon Emissions (NZCE) target by 2050, Carbon Capture, Utilization, and Storage (CCUS) is a major method. Gathering and injecting CO into shale... (Review)
Review
To achieve the Net Zero Carbon Emissions (NZCE) target by 2050, Carbon Capture, Utilization, and Storage (CCUS) is a major method. Gathering and injecting CO into shale reservoirs is an effective way to reduce the CO amount in the air and thus, release the greenhouse effect. CO injection into organic-rich shales could provide dual benefits of incremental oil or gas recovery and secure CO storage. When planning the CO injection project, the most important question is how much gas could be stored in the reservoir. Methods for calculating CO storage potential in shale reservoirs have been studied by many researchers. However, few researchers put those methods together and make comparisons to each other. This paper summarized five methods for evaluating CO storage potential in five shale reservoirs by using the literature published in recent years. This paper aims to discuss and evaluate the technical aspects related to gas storage. Those geomechanical properties, petrophysical properties, and construction parameters were discussed. Among those parameters, CO injection rate, skin factor, and Knudsen diffusion could significantly affect CO storage potential evaluation results. Also, if well integrity, especially cement quality, and permeability could be taken into consideration, CO storage simulation models' results will be more realistic. The significances of this study are: (1) served as guidance in calculating CO storage capacity in shale oil plays; (2) provides analyses in evaluating nowadays methods' limitations; (3) gives recommendations to researchers on how to improve those methods or create a new one.
PubMed: 37560675
DOI: 10.1016/j.heliyon.2023.e18458 -
Biochemical Society Transactions Oct 2023Lysosomal storage diseases are inborn errors of metabolism that arise due to loss of function mutations in genes encoding lysosomal enzymes, protein co-factors or... (Review)
Review
Lysosomal storage diseases are inborn errors of metabolism that arise due to loss of function mutations in genes encoding lysosomal enzymes, protein co-factors or lysosomal membrane proteins. As a consequence of the genetic defect, lysosomal function is impaired and substrates build up in the lysosome leading to 'storage'. A sub group of these disorders are the sphingolipidoses in which sphingolipids accumulate in the lysosome. In this review, I will discuss how the study of these rare lysosomal disorders reveals unanticipated links to other rare and common human diseases using Niemann-Pick disease type C as an example.
Topics: Humans; Niemann-Pick Disease, Type C; Sphingolipids; Lysosomal Storage Diseases; Sphingolipidoses; Lysosomes
PubMed: 37844193
DOI: 10.1042/BST20220711 -
Materials (Basel, Switzerland) Sep 2023Hydrogen has been widely considered to hold promise for solving challenges associated with the increasing demand for green energy. While many chemical and biochemical... (Review)
Review
Hydrogen has been widely considered to hold promise for solving challenges associated with the increasing demand for green energy. While many chemical and biochemical processes produce molecular hydrogen as byproducts, electrochemical approaches using water electrolysis are considered to be a predominant method for clean and green hydrogen production. We discuss the current state-of-the-art in molecular hydrogen production and storage and, more significantly, the increasing role of computational modeling in predictively designing and deriving insights for enhancing hydrogen storage efficiency in current and future materials of interest. One of the key takeaways of this review lies in the continued development and implementation of large-scale atomistic simulations to enable the use of designer electrolyzer-electrocatalysts operating under targeted thermophysical conditions for increasing green hydrogen production and improving hydrogen storage in advanced materials, with limited tradeoffs for storage efficiency.
PubMed: 37687742
DOI: 10.3390/ma16176050 -
Discover Nano Jan 2024This comprehensive review explores the transformative role of nanomaterials in advancing the frontier of hydrogen energy, specifically in the realms of storage,... (Review)
Review
This comprehensive review explores the transformative role of nanomaterials in advancing the frontier of hydrogen energy, specifically in the realms of storage, production, and transport. Focusing on key nanomaterials like metallic nanoparticles, metal-organic frameworks, carbon nanotubes, and graphene, the article delves into their unique properties. It scrutinizes the application of nanomaterials in hydrogen storage, elucidating both challenges and advantages. The review meticulously evaluates diverse strategies employed to overcome limitations in traditional storage methods and highlights recent breakthroughs in nanomaterial-centric hydrogen storage. Additionally, the article investigates the utilization of nanomaterials to enhance hydrogen production, emphasizing their role as efficient nanocatalysts in boosting hydrogen fuel cell efficiency. It provides a comprehensive overview of various nanocatalysts and their potential applications in fuel cells. The exploration extends to the realm of hydrogen transport and delivery, specifically in storage tanks and pipelines, offering insights into the nanomaterials investigated for this purpose and recent advancements in the field. In conclusion, the review underscores the immense potential of nanomaterials in propelling the hydrogen energy frontier. It emphasizes the imperative for continued research aimed at optimizing the properties and performance of existing nanomaterials while advocating for the development of novel nanomaterials with superior attributes for hydrogen storage, production, and transport. This article serves as a roadmap, shedding light on the pivotal role nanomaterials can play in advancing the development of clean and sustainable hydrogen energy technologies.
PubMed: 38169021
DOI: 10.1186/s11671-023-03949-8 -
Heliyon Dec 2023The CO trap mechanisms during carbon capture and storage (CCS) are classified into structural, residual, solution, and mineral traps. The latter is considered as the... (Review)
Review
The CO trap mechanisms during carbon capture and storage (CCS) are classified into structural, residual, solution, and mineral traps. The latter is considered as the most permanent and stable storage mechanism as the injected CO is stored in solid form by the carbon mineralization. In this study, the carbon mineralization process in geological CO storage in basalt, sandstone, carbonate, and shale are reviewed. In addition, relevant studies related to the carbon mineralization mechanisms, and suggestions for future research directions are proposed. The carbon mineralization is defined as the conversion of CO into stable carbon minerals by reacting with divalent cations such as Ca, Mg, or Fe. The process is mainly affected by rock types, temperature, fluid composition, injected CO phase, competing reaction, and nucleation. Rock properties such as permeability, porosity, and rock strength can be altered by the carbon mineralization. Since changes of the properties are directly related to injectivity, storage capacity, and stability during the geological CO storage, the carbon mineralization mechanism should be considered for an optimal CCS design.
PubMed: 38149201
DOI: 10.1016/j.heliyon.2023.e23135 -
Food Research International (Ottawa,... Oct 2023Storage is a key factor controlling the quality of Jiangxiangxing baijiu, and storage time and the type of storage container play crucial roles in shaping the baijiu's...
Storage is a key factor controlling the quality of Jiangxiangxing baijiu, and storage time and the type of storage container play crucial roles in shaping the baijiu's distinct flavor. To investigate the influence of storage containers on the flavor characteristics of Jiangxiangxing baijiu, the sensory qualities, flavor components, and metal ions of Jiangxiangxing baijiu were measured during 24 months of storage in a pottery jar or a stainless steel tank. The results showed that Jiangxiangxing baijiu preserved in a pottery jar was superior to that stored in a stainless steel tank. A total of 96 flavor substances were detected, and 17 key flavor characteristic substances were screened by combining the results of odor activity values (OAV) and orthogonal partial least squares-discriminant analysis (OPLS-DA). A correlation heat map and redundancy analysis (RDA) showed that aluminum, cadmium, iron, cobalt, magnesium, potassium, and copper ions promoted the formation of key characteristic substances including diethoxymethane, lactic acid, 2,3-dimethyl-5-ethylpyrazine, 1-hexanol, and 2-methyl-1-propanol. Overall, the results show that 24-month pottery jar storage can promote the flavor quality of Jiangxiangxing baijiu. This study established a theoretical foundation to select the appropriate storage conditions and control the flavor quality of Jiangxiangxing baijiu.
Topics: Stainless Steel; Aluminum; Cadmium; Copper; Hot Temperature
PubMed: 37689951
DOI: 10.1016/j.foodres.2023.113196 -
Materials (Basel, Switzerland) May 2024Materials offering high energy density are currently desired to meet the increasing demand for energy storage applications, such as pulsed power devices, electric... (Review)
Review
Materials offering high energy density are currently desired to meet the increasing demand for energy storage applications, such as pulsed power devices, electric vehicles, high-frequency inverters, and so on. Particularly, ceramic-based dielectric materials have received significant attention for energy storage capacitor applications due to their outstanding properties of high power density, fast charge-discharge capabilities, and excellent temperature stability relative to batteries, electrochemical capacitors, and dielectric polymers. In this paper, we present fundamental concepts for energy storage in dielectrics, key parameters, and influence factors to enhance the energy storage performance, and we also summarize the recent progress of dielectrics, such as bulk ceramics (linear dielectrics, ferroelectrics, relaxor ferroelectrics, and anti-ferroelectrics), ceramic films, and multilayer ceramic capacitors. In addition, various strategies, such as chemical modification, grain refinement/microstructure, defect engineering, phase, local structure, domain evolution, layer thickness, stability, and electrical homogeneity, are focused on the structure-property relationship on the multiscale, which has been thoroughly addressed. Moreover, this review addresses the challenges and opportunities for future dielectric materials in energy storage capacitor applications. Overall, this review provides readers with a deeper understanding of the chemical composition, physical properties, and energy storage performance in this field of energy storage ceramic materials.
PubMed: 38793340
DOI: 10.3390/ma17102277 -
Sensors (Basel, Switzerland) Oct 2023This research paper investigates the integration of blockchain technology to enhance the security of Android mobile app data storage. Blockchain holds the potential to... (Review)
Review
This research paper investigates the integration of blockchain technology to enhance the security of Android mobile app data storage. Blockchain holds the potential to significantly improve data security and reliability, yet faces notable challenges such as scalability, performance, cost, and complexity. In this study, we begin by providing a thorough review of prior research and identifying critical research gaps in the field. Android's dominant position in the mobile market justifies our focus on this platform. Additionally, we delve into the historical evolution of blockchain and its relevance to modern mobile app security in a dedicated section. Our examination of encryption techniques and the effectiveness of blockchain in securing mobile app data storage yields important insights. We discuss the advantages of blockchain over traditional encryption methods and their practical implications. The central contribution of this paper is the Blockchain-based Secure Android Data Storage (BSADS) framework, now consisting of six comprehensive layers. We address challenges related to data storage costs, scalability, performance, and mobile-specific constraints, proposing technical optimization strategies to overcome these obstacles effectively. To maintain transparency and provide a holistic perspective, we acknowledge the limitations of our study. Furthermore, we outline future directions, stressing the importance of leveraging lightweight nodes, tackling scalability issues, integrating emerging technologies, and enhancing user experiences while adhering to regulatory requirements.
PubMed: 37960449
DOI: 10.3390/s23218749