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Natural history study of hepatic glycogen storage disease type IV and comparison to Gbe1ys/ys model.JCI Insight May 2024BackgroundGlycogen storage disease type IV (GSD IV) is an ultrarare autosomal recessive disorder that causes deficiency of functional glycogen branching enzyme and... (Comparative Study)
Comparative Study
BackgroundGlycogen storage disease type IV (GSD IV) is an ultrarare autosomal recessive disorder that causes deficiency of functional glycogen branching enzyme and formation of abnormally structured glycogen termed polyglucosan. GSD IV has traditionally been categorized based on primary hepatic or neuromuscular involvement, with hepatic GSD IV subclassified as discrete subtypes: classic (progressive) and nonprogressive.MethodsTo better understand the progression of liver disease in GSD IV, we present clinical and histopathology data from 23 patients from around the world and characterized the liver involvement in the Gbe1ys/ys knockin mouse model.ResultsWe propose an alternative to the established subtype-based terminology for characterizing liver disease in GSD IV and recognize 3 tiers of disease severity: (i) "severe progressive" liver disease, (ii) "intermediate progressive" liver disease, and (iii) "attenuated" liver disease. Analysis of liver pathology revealed that risk for liver failure cannot be predicted from liver biopsy findings alone in individuals affected by GSD IV. Moreover, analysis of postmortem liver pathology from an individual who died over 40 years after being diagnosed with nonprogressive hepatic GSD IV in childhood verified that liver fibrosis did not regress. Last, characterization of the liver involvement in a mouse model known to recapitulate the adult-onset neurodegenerative form of GSD IV (Gbe1ys/ys mouse model) demonstrated hepatic disease.ConclusionOur findings challenge the established subtype-based view of GSD IV and suggest that liver disease severity among patients with GSD IV represents a disease continuum.Trial registrationClinicalTrials.gov NCT02683512FundingNone.
Topics: Adolescent; Adult; Animals; Child; Child, Preschool; Female; Humans; Infant; Male; Mice; Middle Aged; Young Adult; Disease Models, Animal; Disease Progression; Glycogen Debranching Enzyme System; Glycogen Storage Disease Type IV; Liver; Liver Diseases
PubMed: 38912588
DOI: 10.1172/jci.insight.177722 -
Heliyon Jun 2024Functional bioactive ingredients isolated from microalgae as sustainable sources have become a new subject of pharmacology and functional foods. Thus, the work aims to...
Characterization of C-phycocyanin antioxidant, anti-inflammatory, anti-tumour, and anti-HCoV-229E activities and encapsulation for implementation in an innovative functional yogurt.
Functional bioactive ingredients isolated from microalgae as sustainable sources have become a new subject of pharmacology and functional foods. Thus, the work aims to produce crude phycocyanin (C-PC), define it, and investigate its pharmacological effects before warping it in a nanophytosome. Subsequently, the physicochemical properties of nanoparticles were evaluated. Both free and nanophytosomes of C-PC were incorporated into cow milk fermented with the probiotic KU985435 to make functional yoghurt and the stability of C-PC of both phytosomes was assessed. The amino acid content of C-PC revealed the presence of eight of nine essential amino acids and eight of eleven non-essential amino acids. C-PC has a medium molecular weight (82.992 kDa). Some pharmacological effects like reducing inflammation (98.76 % ± 0.065), fighting free radicals (99.12 % ± 0.027), and being able to inhibit the human coronavirus 229 E with a selective index of 27.9 were observed. The maximum viral inhibitory activity was detected during the adsorption stage. Anti-human liver and colon carcinomas that exceeded Doxorubicin with very low cytotoxicity against normal cell lines were detected. C-PC is an unstable protein that could be degraded in the yoghurt during storage. Therefore, phytosome encapsulation can effectively stabilize C-PC (particle size 44.50 ± 12 nm and zeta-potential -32.4 ± 5 mV) and protect it from the acidic environment of the yoghurt. The produced yoghurt showed the desired physicochemical and functional properties and overall acceptance. The results prove that C-PC from spirulina algae is a renewable source of dyes. The encapsulation process using phytosomes gave it high stability against environmental influences, and therefore, it can be applied in the food and pharmaceutical industries in the future.
PubMed: 38912514
DOI: 10.1016/j.heliyon.2024.e31642 -
Heliyon Jun 2024The increasing penetration of high-volatility renewable energy sources in the power system presents higher demands for flexibility from coal-fired power plant (CFPP). To...
The increasing penetration of high-volatility renewable energy sources in the power system presents higher demands for flexibility from coal-fired power plant (CFPP). To enhance the flexibility of CFPPs, researchers have conducted a significant amount of thermal-system-level research in recent years on increasing system peak shaving depth. However, the load ramp rate of CFPPs under deep peak shaving is rarely discussed, despite its significance to the overall flexibility performance of CFPPs. This paper proposes a steam accumulator storage system integrating to the turbine's bypass system. The steam accumulator charges directly with working fluid from the live steam or reheat systems and discharge to the turbine, responding quickly to power ramp commands. A steady state model and a dynamic model of the proposed system were built and validated, and the calculation shows that the proposed scheme provides a load change of +2.13 % Pe and -8.3%Pe during a round-trip with a power efficiency of 63.6 % at a unit load of 40 % THA. The unit's load increase rate under coordinated control was enhanced by 1.5 % Pe/min, reaching 3 % Pe/min, using the proposed steam accumulator without revising the original controls, and the load decrease rate reached at least 5 % Pe/min. The results indicate that the proposed system provides a straightforward, easy-to-implement, and efficient solution for enhancing the load ramp rate of CFPPs at low loads.
PubMed: 38912492
DOI: 10.1016/j.heliyon.2024.e32412 -
Heliyon Jun 2024This paper evaluates GHG emissions and energy usage in "short" and "long" cold chains for oranges, table grapes, and apples transported from South Africa to a retail...
This paper evaluates GHG emissions and energy usage in "short" and "long" cold chains for oranges, table grapes, and apples transported from South Africa to a retail store in Scotland. Novel formulae assess energy usage and emissions based on cold chain duration. "Short" chains show carbon footprints between 0.87 and 1.28 kg COe/kg of saleable fruit, contrasting starkly with extended cold chains. Extending storage durations increases emissions; a one-month extension results in 24-27 % emissions for oranges and grapes and 16 % for apples. Six months of CA storage of apples increases emissions by 96 % compared to "short" cold chains. Energy consumption follows a similar trend as emissions. This research informs policymakers and consumers, emphasising the need for sustainable and "short" cold chains. This is also the first paper that comprehensively assesses both the energy requirements and emissions outputs in a fruit supply chain based on the combined transport and storage duration of the cold chain from tree to retail markets.
PubMed: 38912466
DOI: 10.1016/j.heliyon.2024.e32507 -
ACS Applied Nano Materials Feb 2024The transcendence toward smarter technologies and the rapid expansion of the Internet of Things requires miniaturized energy storage systems, which may also be...
The transcendence toward smarter technologies and the rapid expansion of the Internet of Things requires miniaturized energy storage systems, which may also be shape-conformable, such as microflexible supercapacitors. Their fabrication must be compatible with emerging manufacturing platforms with regard to scalability and sustainability. Here, we modify a laser-based method we recently developed for simultaneously synthesizing and transferring graphene onto a selected substrate. The modification of the method lies in the tuning of two key parameters, namely, the inclination of the laser beam and the distance between the precursor material and the acceptor substrate. A proper combination of these parameters enables the displacement of the trace of the transmitted laser beam from the deposited graphene film area. This mitigates the negative effects that arise from the laser-induced ablation of graphene on heat-sensitive substrates and significantly improves the electrical conductivity of the graphene films. The optimized graphene exhibits very high C/O (36) and sp/sp (13) ratios. Post-transport irradiation was used to transform the continuous graphene films to interdigitated electrodes. The capacitance of the microflexible supercapacitor was measured to be among the highest reported ones in relation to interdigitated supercapacitors with electrodes based on laser-grown graphene. The device shows good cycling stability, retaining 91% of its capacitance after 10,000 cycles, showing no substantial degradation after applying bending conditions. This promising laser-based approach emerges as a viable alternative for the fabrication of microflexible interdigitated supercapacitors for paper electronics and smart textiles.
PubMed: 38912400
DOI: 10.1021/acsanm.3c05387 -
Frontiers in Plant Science 2024Blackheart is one of the most common physiological diseases in potatoes during storage. In the initial stage, black spots only occur in tissues near the potato core and...
INTRODUCTION
Blackheart is one of the most common physiological diseases in potatoes during storage. In the initial stage, black spots only occur in tissues near the potato core and cannot be detected from an outward appearance. If not identified and removed in time, the disease will seriously undermine the quality and sale of theentire batch of potatoes. There is an urgent need to develop a method for early detection of blackheart in potatoes.
METHODS
This paper used visible-near infrared (Vis/NIR) spectroscopy to conduct online discriminant analysis on potatoes with varying degrees of blackheart and healthy potatoes to achieve real-time detection. An efficient and lightweight detection model was developed for detecting different degrees of blackheart in potatoes by introducing the depthwise convolution, pointwise convolution, and efficient channel attention modules into the ResNet model. Two discriminative models, the support vector machine (SVM) and the ResNet model were compared with the modified ResNet model.
RESULTS AND DISCUSSION
The prediction accuracy for blackheart and healthy potatoes test sets reached 0.971 using the original spectrum combined with a modified ResNet model. Moreover, the modified ResNet model significantly reduced the number of parameters to 1434052, achieving a substantial 62.71% reduction in model complexity. Meanwhile, its performance was evidenced by a 4.18% improvement in accuracy. The Grad-CAM++ visualizations provided a qualitative assessment of the model's focus across different severity grades of blackheart condition, highlighting the importance of different wavelengths in the analysis. In these visualizations, the most significant features were predominantly found in the 650-750 nm range, with a notable peak near 700 nm. This peak was speculated to be associated with the vibrational activities of the C-H bond, specifically the fourth overtone of the C-H functional group, within the molecular structure of the potato components. This research demonstrated that the modified ResNet model combined with Vis/NIR could assist in the detection of different degrees of black in potatoes.
PubMed: 38911981
DOI: 10.3389/fpls.2024.1403713 -
Frontiers in Plant Science 2024Both subsoiling tillage (ST) and ridge and furrow rainfall harvesting (RF) are widely implemented and play an important role in boosting wheat productivity. However,...
Combined subsoiling and ridge-furrow rainfall harvesting during the summer fallow season improves wheat yield, water and nutrient use efficiency, and quality and reduces soil nitrate-N residue in the dryland summer fallow-winter wheat rotation.
Both subsoiling tillage (ST) and ridge and furrow rainfall harvesting (RF) are widely implemented and play an important role in boosting wheat productivity. However, information about the effects of ST coupled with RF during the summer fallow season on wheat productivity and environmental issues remains limited. This study aims to explore the effects of ST coupled with RF on water harvesting, wheat productivity-yield traits, water and nutrient use efficiency and quality, and soil nitrate-N residue in dryland winter wheat-summer fallow rotation at the intersection of southern Loess Plateau and western Huang-Huai-Hai Plain in China in 2018-2022. Three tillage practices-deep plowing with straw turnover (PTST), subsoiling with straw mulching (STSM), and STSM coupled with RF (SRFSM)-are conducted during the summer fallow season. The results indicated that tillage practices during the summer fallow season significantly impacted wheat productivity and soil nitrate-N residue. Compared to PTST, STSM significantly enhanced rainfall fallow efficiency and water use efficiency by 7.0% and 14.2%, respectively, as well as N, P, and K uptake efficiency by 16.9%, 16.2%, and 15.3%, and thus increased grain yield by 14.3% and improved most parameters of protein components and processing quality, albeit with an increase in nitrate-N residue in the 0- to 300-cm soil depth by 12.5%. SRFSM, in turn, led to a further increase in water storage at sowing, resulting in an increase of water use efficiency by 6.8%, as well as N, P, and K uptake efficiency and K internal efficiency by 11.8%, 10.4%, 8.8%, and 4.7%, thereby significantly promoting grain yield by 10.2%, and improving the contents of all the protein components and enhancing the processing quality in grain, and simultaneously reducing the nitrate-N residue in the 0- to 300-cm soil layer by 16.1%, compared to STSM. In essence, this study posits that employing subsoiling coupled with ridge-furrow rainfall harvesting (SRFSM) during the summer fallow season is a promising strategy for enhancing wheat yield, efficiency, and quality, and simultaneously reducing soil nitrate-N residue within the dryland summer fallow-winter wheat rotation system.
PubMed: 38911975
DOI: 10.3389/fpls.2024.1401287 -
RSC Advances Jun 2024The high theoretical energy density of Li-S batteries makes them a viable option for energy storage systems in the near future. Considering the challenges associated... (Review)
Review
The high theoretical energy density of Li-S batteries makes them a viable option for energy storage systems in the near future. Considering the challenges associated with sulfur's dielectric properties and the synthesis of soluble polysulfides during Li-S battery cycling, the exceptional ability of MXene materials to overcome these challenges has led to a recent surge in the usage of these materials as anodes in Li-S batteries. The methods for enhancing anode performance in Li-S batteries the use of MXene interfaces are thoroughly investigated in this study. This study covers a wide range of techniques such as surface functionalization, heteroatom doping, and composite structure design for enhancing MXene interfaces. Examining challenges and potential downsides of MXene-based anodes offers a thorough overview of the current state of the field. This review encompasses recent findings and provides a thorough analysis of advantages and disadvantages of adding MXene interfaces to improve anode performance to assist researchers and practitioners working in this field. This review contributes significantly to ongoing efforts for the development of reliable and effective energy storage solutions for the future.
PubMed: 38911835
DOI: 10.1039/d4ra02704c -
ACS Omega Jun 2024is the most significant oilseed nutritious legume crop in agricultural trade across the world. It is recognized as a valued crop for its contributions to nourishing... (Review)
Review
is the most significant oilseed nutritious legume crop in agricultural trade across the world. It is recognized as a valued crop for its contributions to nourishing food, as a cooking oil, and for meeting the protein needs of people who are unable to afford animal protein. Currently, its production, marketability, and consumption are hindered because of species infection that consequently contaminates the kernels with aflatoxins. Regarding health concerns, humans and animals are affected by acute and chronic aflatoxin toxicity and millions of people are at high risk of chronic levels. Most methods used to store peanuts are traditional and serve effectively for short-term storage. Now the question for long-term storage has been raised, and this promptly finds potential approaches to the issue. It is imperative to reduce the aflatoxin levels in peanuts to a permissible level by introducing detoxifying innovations. Most of the detoxification reports mention physical, chemical, and biological techniques. However, many current approaches are impractical because of time consumption, loss of nutritional quality, or weak detoxifying efficiency. Therefore, it is crucial to investigate practical, economical, and green methods to control that address current global food security problems. Herein, a green and economically revolutionary way is a nanotechnology that has demonstrated its potential to connect farmers to markets, elevate international marketability, improve human and animal health conditions, and enhance food quality and safety by the management of fungal diseases. Due to the antimicrobial potential of nanoparticles, they act as nanofungicides and have an incredible role in the control of aflatoxins. Nanoparticles have ultrasmall sizes and therefore penetrate the fungal body and invade the pathogen machinery, leading to fungal cell death by ROS production, mutation in DNA, disruption of organelles, and membrane leakage. This is the first mechanistic overview that unveils a comprehensive insight into aflatoxin contamination in peanuts, its prevalence, health effects, and management in addition to nanotechnological interventions that serve as a triple defense approach to detoxify aflatoxins. The optimum use of nanofungicides ensures food safety and the development of goals, especially "zero hunger".
PubMed: 38911815
DOI: 10.1021/acsomega.4c01316 -
ACS Omega Jun 2024The main goal of traditional methods for sweetening natural gas (NG) is to remove hydrogen sulfide (HS) and significantly lower carbon dioxide (CO). However, when NG...
The main goal of traditional methods for sweetening natural gas (NG) is to remove hydrogen sulfide (HS) and significantly lower carbon dioxide (CO). However, when NG processes are integrated into the carbon capture and storage (CCS) framework, there is potential for synergy between these two technologies. A steady-state model utilizing a hybrid solvent consisting of -methyl-2-pyrrolidone (NMP) and monoethanolamine (MEA) has been developed to successfully anticipate the CO and HS capture process from NG. The model was tested against important variables affecting process performance. This article specifically explores the impact of operational parameters such as lean amine temperature, absorber pressure, and amine flow rate on the concentrations of CO and HS in the sweet gas and reboiler duty. The result shows that hybrid solvents (MEA + NMP) perform better in removing acid gases and reducing reboiler duty than conventional chemical solvent MEA. The primary purpose is to meet product requirements while consuming the least energy possible, which is in line with any process plant's efficiency goals.
PubMed: 38911790
DOI: 10.1021/acsomega.3c09100