Oxide Systems Research Articles

Metabolic Adaptation in Epilepsy: From Acute Response to Chronic Impairment.

Epilepsy is characterized by hypersynchronous neuronal discharges, which are associated with an increased cerebral metabolic rate of oxygen and ATP demand. Uncontrolled seizure activity (status epilepticus) results in mitochondrial exhaustion and ATP depletion, which potentially generate energy mismatch and neuronal loss. Many cells can adapt to increased energy demand by increasing metabolic capacities. However, acute metabolic adaptation during epileptic activity and its relationship to chronic epilepsy remains poorly understood. We elicited seizure-like events (SLEs) in an in vitro model of status epilepticus for eight hours. Electrophysiological recording and tissue oxygen partial pressure recordings were performed. After eight hours of ongoing SLEs, we used proteomics-based kinetic modeling to evaluate changes in metabolic capacities. We compared our findings regarding acute metabolic adaptation to published proteomic and transcriptomic data from chronic epilepsy patients. Epileptic tissue acutely responded to uninterrupted SLEs by upregulating ATP production capacity. This was achieved by a coordinated increase in the abundance of proteins from the respiratory chain and oxidative phosphorylation system. In contrast, chronic epileptic tissue shows a 25-40% decrease in ATP production capacity. In summary, our study reveals that epilepsy leads to dynamic metabolic changes. Acute epileptic activity boosts ATP production, while chronic epilepsy reduces it significantly.

Green Solvent γ-Valerolactone (GVL) Facilitates Photocatalytic C-H Bond Activation.

Solvents can significantly influence chemical reactions in condensed phases. Their critical properties are increasingly recognized in various research domains such as organic synthesis and biomass valorization. However, in semiconductor photocatalysis, solvents are primarily viewed as mediums for dissolving and diffusing substances, with their potential beneficial effects on photocatalytic conversions often overlooked. Additionally, common photocatalysis solvents like acetonitrile (ACN) pose serious safety and environmental concerns. In this study, we demonstrate that novel and safe green solvents, such as γ-valerolactone (GVL), can significantly enhance the performance of semiconductor photocatalysis for C-H bond activation. Non-specific solvent-solute interactions are the primary contributors to increased photocatalytic activity in the self-coupling of benzylic compounds. Specifically, GVL's large dielectric constant and high refractive index lower the energy barrier for the rate-determining C-H bond activation step, facilitating a faster coupling reaction. The versatility of GVL is further demonstrated in reactions with multiple reagents and in various oxidation and reduction photocatalytic systems beyond classic C-H bond activation. This work not only pioneers the use of green solvents but also provides comprehensive insights for proper solvent selection in semiconductor photocatalysis.

Green Solvent γ‐Valerolactone (GVL) Facilitates Photocatalytic C−H Bond Activation

AbstractSolvents can significantly influence chemical reactions in condensed phases. Their critical properties are increasingly recognized in various research domains such as organic synthesis and biomass valorization. However, in semiconductor photocatalysis, solvents are primarily viewed as mediums for dissolving and diffusing substances, with their potential beneficial effects on photocatalytic conversions often overlooked. Additionally, common photocatalysis solvents like acetonitrile (ACN) pose serious safety and environmental concerns. In this study, we demonstrate that novel and safe green solvents, such as γ‐valerolactone (GVL), can significantly enhance the performance of semiconductor photocatalysis for C−H bond activation. Non‐specific solvent‐solute interactions are the primary contributors to increased photocatalytic activity in the self‐coupling of benzylic compounds. Specifically, GVL′s large dielectric constant and high refractive index lower the energy barrier for the rate‐determining C−H bond activation step, facilitating a faster coupling reaction. The versatility of GVL is further demonstrated in reactions with multiple reagents and in various oxidation and reduction photocatalytic systems beyond classic C−H bond activation. This work not only pioneers the use of green solvents but also provides comprehensive insights for proper solvent selection in semiconductor photocatalysis.

Effects of different housing systems on the oxidative defence system, heterophil functions, cellular immune response and cytokines in laying hens.

1. This study investigated the effects of different housing systems on oxidative defence mechanisms, heterophil functions, cellular immune response and cytokine production in laying hens. One hundred and twenty laying hens were allocated into one of four groups: conventional cages, furnished cages, deep litter, and free range.2. Housing system did not affect malondialdehyde concentrations and enzymatic antioxidant status. Ascorbic acid values were higher in deep-litter hens than in those in conventional cages and free range.3. Phagocytic and chemotactic activities tended to rise in the deep-litter system, and oxidative burst was higher than in furnished cages. Cytotoxic T cells were decreased in furnished cages, both cytotoxic and helper T cells decreased in deep litter compared to free range.4. The IL-2 and IL-13 expression was higher in deep litter than in conventional cages, and IL-6 expression was higher in furnished cages than in free range.5. Housing system had no significant effects on the oxidative defence system; however, they affected heterophil functions, cellular defence mechanisms and cytokine production. The results suggested that breeders need to consider the housing system's potential effects on immune defence responses while applying a breeding strategy appropriate for animal welfare and consumer demand.

Construction of heterojunction superparamagnetic Cd0.5Ag0.5Fe2O4 nanocomposite photocatalyst for simulated textile effluent oxidation

Abstract Significant attempts have been recently made regarding nanomaterials due to their several environmental applications especially in wastewater treatment technologies. Among the available nanoparticles, ferrite based substances are gaining a special interest since their superior characteristics such as their magnetic nature, high adsorption capacity and large specific surface area. In this regard, Cd0.5Ag0.5Fe2O4 was prepared using the green simple co-precipitation route. Then, the sample is characterized via Scanning Electron Microscopy (SEM) that proved the produced material’s surface morphology. The substance is then employed as a catalyst source for Fenton reaction to oxidize textile effluent solution containing Rhodamine B (Rh-B 6G) dye. The oxidation experiment conducted under ultraviolet (UV) light with the ferrite-based Fenton catalyst supplemented with hydrogen peroxide showed an exceptional removal rate of up to 94% removals. Notably, the oxidation system is significantly impacted by the operational variables. The oxidation efficiency of the dye was maximized at pH 3.0 and 50 mg/L and 1600 mg/L for ferrite-based Fenton catalyst and H2O2, respectively. The impacts of the operational factors, i.e. initial pH value, initial dye concentration, catalyst, and H2O2 concentrations were also investigated. This perspective introduces the role of a superparamagnetic material to be a recyclable sustained catalyst.

Metal-Organic Frameworks (MIL-100 (Fe)) for commercial reactive dye effluent oxidation

Abstract Urgent calls for scientists’ contributions towards saving eco-system from toxic deterioration. Such ever increasing urgency is leading the researchers for searching for ever greening technologies such as advanced oxidation processes for aqueous effluent elimination. This current investigation is leading to investigate the metal organic frameworks, role, MIL-100(Fe) that is prepared from reacting H3BTC and ferric nitrate nona hydrate Also, Scanning electron microscopy is applied to set the surface shape of the synthesized substance. Subsequently, the prepared MIL-100(Fe) is used to oxidize Procion Blue MX-7RX in aqueous solution as a model textile wastewater effluent. The catalyst is applying in Fenton’s oxidation system and operating parameters that affecting the system is evaluated. The experimental results revealed that 40 and 800 mgL−1 for MIL-100(Fe) and hydrogen peroxide, respectively at pH 3.0 that is leading to a complete dye removal (100%). Furthermore, the dye loading affects the reaction rate since its increase reduces the removal efficacy. Finally, thermodynamic parameters and kinetics of the reaction is evaluated for the scale-up purposes for real applications.

Oxidative stress and aging: synergies for age related diseases.

Aging is characterized by a progressive decline in physiological function and underlies several disabilities, including the increased sensitivity of cells and tissues to undergo pathological oxidative stress. In recent years, efforts have been made to better understand the relationship between age and oxidative stress and further develop therapeutic strategies to minimize the impact of both events on age-related diseases. In this work, we review the impact of the oxidant and antioxidant systems during aging and disease development and discuss the crosstalk of oxidative stress and other aging processes, with a focus on studies conducted in elderly populations.

So, you cannot vent: A deep dive into other explosion protection methods

AbstractCurrent NFPA standards for managing combustible dust hazards require equipment with an explosion hazard to be protected from the effects of deflagration. These protections include deflagration venting in accordance with NFPA 68, Standard on Explosion Protection by Deflagration Venting, 2023 Edition, or oxidant concentration reduction, combustible concentration reduction, deflagration suppression, or deflagration pressure containment, via NFPA 69, Standard on Explosion Protection Systems, 2024 Edition. It makes sense to choose the simplest and most cost‐effective option based on the inherent design and risk of the operation. Implementation of any one of these protection methods requires an understanding of the method and all the associated requirements. While the basic methods are fairly straightforward, the associated requirements are often less understood. In this paper, the author will introduce the basic methods and take a deep dive into the associated requirements of each method of protection. Based on the author's experience in evaluating protection systems, common misunderstandings will be highlighted, for example, the margin of safety to apply and the operational limits required to be developed and documented for oxidant concentration reduction systems.

Prognostic significance of a 3-gene ferroptosis-related signature in lung cancer via LASSO analysis and cellular functions of UBE2Z

Ferroptosis is a newly identified form of non-apoptotic programmed cell death resulting from iron-dependent lipid peroxidation. It is controlled by integrated oxidation and antioxidant systems. Ferroptosis exerts a crucial effect on the carcinogenesis of several cancers, including pulmonary cancer. Herein, a ferroptosis-associated gene signature for lung cancer prognosis and diagnosis was identified using integrative bioinformatics analyses. From the FerrDB database, 256 ferroptotic regulators and markers were identified. Of these, 25 exhibited differential expression between lung cancer and non-cancerous samples, as evidenced by the GSE19804 and GSE7670 datasets from the GEO database. Utilizing LASSO Cox regression analysis on TCGA-LUAD data, a potent 3-gene risk signature comprising CAV1, RRM2, and EGFR was established. This signature adeptly differentiates various survival outcomes in lung cancer patients, including overall survival and disease-specific intervals. Based on the 3-gene risk signature, lung cancer patients were categorized into high-risk and low-risk groups. Comparative analysis revealed 69 differentially expressed genes between these groups, with UBE2Z significantly associated with overall survival in TCGA-LUAD. UBE2Z was found to be upregulated in LUAD tissues and cells compared to normal controls. Functionally, the knockdown of UBE2Z curtailed aggressive behaviors in LUAD cells, including viability, migration, and invasion. Moreover, this knockdown led to a decrease in the mesenchymal marker vimentin while elevating the epithelial marker E-cadherin within LUAD cell lines. In conclusion, the ferroptosis-associated 3-gene risk signature effectively differentiates prognosis and clinical features in patients with lung cancer. UBE2Z was identified through this model, and it is upregulated in LUAD samples. Its knockdown inhibits aggressive cellular behaviors, suggesting UBE2Z's potential as a therapeutic target for lung cancer treatment.

Sunitinib-induced endocapillary proliferative glomerulonephritis with IgA2 deposit in addition to thrombotic microangiopathy: a case report

BackgroundSunitinib, a multi-targeted tyrosine kinase inhibitor, is used as a second-line therapy for gastrointestinal stromal tumors (GIST) resistant to imatinib. However, its impact on the vascular endothelial growth factor (VEGF) pathway can lead to significant toxicities, including hypertension and thrombotic microangiopathy (TMA).Case presentationThis case report describes a unique instance of a patient with metastatic GIST who developed endocapillary proliferative glomerulonephritis (EPGN) with IgA2 deposits and TMA following sunitinib treatment. The patient presented with severe hypertension, nephrotic syndrome, and acute kidney injury. Renal biopsy confirmed the diagnosis, revealing IgA2 deposits, which are not commonly associated with TMA. Discontinuation of sunitinib led to a rapid improvement in renal function and proteinuria. The potential mechanisms underlying sunitinib-induced glomerular injury may involve the blockade of VEGFR-1, affecting immune cell recruitment and function, and the disruption of the nitric oxide and endothelin systems, leading to endothelial damage and immune dysregulation. Management of these toxicities requires a personalized approach, with options ranging from symptomatic relief to drug discontinuation. The use of endothelin receptor antagonists and other therapeutic alternatives for GIST management is discussed.ConclusionsThis case highlights the complex interplay between the therapeutic effects of sunitinib and its potential renal and cardiovascular toxicities, emphasizing the need for close monitoring and effective management strategies to optimize patient outcomes.

Macrophage targeted graphene oxide nanosystem synergize antibiotic killing and host immune defense for Tuberculosis Therapy

Tuberculosis (TB), a deadly disease caused by Mycobacterium tuberculosis (Mtb) infection, remains one of the top killers among infectious diseases worldwide. How to increase targeting effects of current anti-TB chemotherapeutics and enhance anti-TB immunological responses remains a big challenge in TB and drug-resistant TB treatment. Here, mannose functionalized and polyetherimide protected graphene oxide system (GO-PEI-MAN) was designed for macrophage-targeted antibiotic (rifampicin) and autophagy inducer (carbamazepine) delivery to achieve more effective Mtb killings by combining targeted drug killing and host immunological clearance. GO-PEI-MAN system demonstrated selective uptake by in vitro macrophages and ex vivo macrophages from macaques. The endocytosed GO-PEI-MAN system would be transported into lysosomes, where the drug loaded Rif@Car@GO-PEI-MAN system would undergo accelerated drug release in acidic lysosomal conditions. Rif@Car@GO-PEI-MAN could significantly promote autophagy and apoptosis in Mtb infected macrophages, as well as induce anti-bacterial M1 polarization of Mtb infected macrophages to increase anti-bacterial IFN-γ and nitric oxide production. Collectively, Rif@Car@GO-PEI-MAN demonstrated effectively enhanced intracellular Mtb killing effects than rifampicin, carbamazepine or GO-PEI-MAN alone in Mtb infected macrophages, and could significantly reduce mycobacterial burdens in the lung of infected mice with alleviated pathology and inflammation without systemic toxicity. This macrophage targeted nanosystem synergizing increased drug killing efficiency and enhanced host immunological defense may be served as more effective therapeutics against TB and drug-resistant TB.

Defective post-transcriptional modification of tRNA disrupts mitochondrial homeostasis in Leber’s hereditary optic neuropathy

Leber's Hereditary Optic Neuropathy (LHON) is a rare, maternally inherited eye disease, predominantly due to the degeneration of retinal ganglion cells (RGCs). It is associated with a mitochondrial DNA (mtDNA) point mutation. Our previous study identified that the m.15927G>A homoplasmic mutation damaged the highly conserved basepairing (28C-42G) in anticodon stem of tRNAThr, caused deficient t6A modification and significantly decreased efficiency in aminoacylation and steady-state levels of tRNAThr, and led to mitochondrial dysfunction. Meanwhile, mechanisms underlying mtDNA mutations regulate intracellular signaling related to the mitochondrial and cellular integrity are less explored. Here, we manifested that defective nucleotide modification induced by the m.15927G>A mutation interfered with the expression of nuclear genes involved in cytoplasmic proteins essential for oxidative phosphorylation system (OXPHOS), thereby impacting the assemble and integrity of OXPHOS complexes. As a result of these mitochondrial dysfunctions, there was an imbalance in mitochondrial dynamics, particularly distinguished by an increased occurrence of mitochondrial fission. Excessive fission compromised the autophagy process, including initiation phase, formation and maturation of autophagosome. Both Parkin-mediated mitophagy and receptor-dependent mitophagy were significantly impaired in cybrids haboring the m.15927G>A mutation. These changes facilitated intrinsic apoptosis, as indicated by increased cytochrome c release and elevated levels of apoptosis-associated proteins (e.g., BAK, BAX, cleaved caspase 9, cleaved caspase 3, and cleaved PARP) in the mutant cybrids. This study demonstrates that the m.15927G>A mutation contributes to LHON by dysregulating OXPHOS biogenesis, aberrant quality control, increased autophagy, inhibited mitophagy, and abnormal apoptosis.

Physically hybrid Zr(OH)4 + CuO catalyzed selective aniline oxidation: A new Ph‐N˙OH mediated mechanism

AbstractDeveloping the sustainable and cost‐effective heterogeneous catalytic system for controlling chemoselectivity holds substantial importance in fine organic chemicals. Herein we construct a unique Zr(OH)4 + CuO physically hybrid system for selective oxidation of anilines. Zr(OH)4 alone leads to azoxybenzene formation, and Zr(OH)4 + CuO shifts the reaction favorably toward nitrosobenzene. The proximity study indicates Zr(OH)4 + CuO outperforms its counterparts synthesized through methods like ball‐milling, loading, and coprecipitation, because the closer proximity exhibits stronger chemical interaction, restricting the activity of Zr‐OH hydroxyl sites. Through mechanistic experiments, in situ DRIFT‐IR and DFT calculations, a new Ph‐OH intermediate mechanism is firstly proposed. Two Ph‐OH self‐condensate to form azoxybenzene for only Zr(OH)4, whereas Zr(OH)4 + CuO could promote rapid transformation of Ph‐OH to nitrosobenzene on CuO through a hydrogen transfer process. Moreover, Zr(OH)4 + CuO displays good recyclability and robust scalability. This is the first report demonstrating the utilization of a physically hybrid catalyst to adjust the selectivity of the aniline oxidation reaction.

Visible light-improved periodate activation of natural chalcopyrite particles towards effective pollutants degradation

The aim of this paper is to build an efficient and low-cost advanced oxidation system to realize the antibiotic wastewater purification which has a realistic significance. In this study, a system of visible light-assisted activation of natural chalcopyrite to activate periodate was adopted to remove tetracycline (TC). The results demonstrated that the removal efficiency (100% within 60 min) of TC in the CuFeS2/PI/Light system was significantly improved compared to the CuFeS2/PI system or PI process alone, and the degradation rates of TC in the CuFeS2/PI/Light system were 5.9 times higher than that of the CuFeS2/PI system, which is attributed to the synergistic effect of light and PI activation. The system also effectively degraded sodium butyl xanthate (SBX), chlortetracycline hydrochloride (CTC) and Rhodamine B (RhB), while the efficiency of the degradation process was further enhanced in natural water quality. The active substance quenching experiment revealed that [Formula: see text]OH, 1O2, O2[Formula: see text], IO4[Formula: see text] and IO3[Formula: see text] were the main substances to achieve effective TC removal. In addition, the cycling of Cu[Formula: see text]/Cu[Formula: see text] and Fe[Formula: see text]/Fe[Formula: see text] present in chalcopyrite improved the activation of PI. Further, visible light is conducive to the valence cycling of Cu and Fe, as well as the production of more oxidizing free radicals, thus achieving the synergy of photocatalysis and PI activation. This study provides a low-cost and efficient advanced oxidation pathway for the removal of antibiotic wastewater.

Efficient degradation and mineralization of polyethylene terephthalate microplastics by the synergy of sulfate and hydroxyl radicals in a heterogeneous electro-Fenton-activated persulfate oxidation system

The presence of polyethylene terephthalate (PET) microplastics (MPs) in waters has posed considerable threats to the environment and humans. In this work, a heterogeneous electro-Fenton-activated persulfate oxidation system with the FeS2-modified carbon felt as the cathode (abbreviated as EF-SR) was proposed for the efficient degradation of PET MPs. The results showed that i) the EF-SR system removed 91.3 ± 0.9 % of 100 mg/L PET after 12 h at the expense of trace loss (< 0.07 %) of [Fe] and that ii) dissolved organics and nanoplastics were first formed and accumulated and then quickly consumed in the EF-SR system. In addition to the destruction of the surface morphology, considerable changes in the surface structure of PET were noted after EF-SR treatment. On top of the emergence of the O-H bond, the ratio of C-O/C=O to C-C increased from 0.25 to 0.35, proving the rupture of the backbone of PET and the formation of oxygen-containing groups on the PET surface. With the verified involvement and contributions of SO4•- and •OH, three possible paths were proposed to describe the degradation of PET towards complete mineralization through chain cleavage and oxidation in the EF-SR system.

Hormonal orchestra: mastering mitochondria's role in health and disease.

Mitochondria is a subcellular organelle involved in the pathogenesis of cellular stress, immune responses, differentiation, metabolic disorders, aging, and death by regulating process of fission, fusion, mitophagy, and transport. However, an increased interest in mitochondria as powerhouse for ATP production, the mechanisms of mitochondria-mediated cellular dysfunction in response to hormonal interaction remains unknown. Mitochondrial matrix contains chaperones and proteases that regulate intrinsic apoptosis pathway through pro-apoptotic Bcl-2 family's proteins Bax/Bak, and Cyt C release, and induces caspase-dependent and independent cells death. Energy and growth regulators such as thyroid hormones have profound effect on mitochondrial inner membrane protein and lipid compositions, ATP production by regulating oxidative phosphorylation system. Mitochondria contain cholesterol side-chain cleavage enzyme, P450scc, ferredoxin, and ferredoxin reductase providing an essential site for steroid hormones biosynthesis. In line with this, neurohormones such as oxytocin, vasopressin, and melatonin are correlated with mitochondrial integrity, displaying therapeutic implications for inflammatory and immune responses. Melatonin's also displayed protective role against oxidative stress and mitochondrial synthesis of ROS, suggesting a defense mechanism against aging-related diseases. An imbalance in mitochondrial bioenergetics can cause neurodegenerative disorders, cardiovascular diseases, and cancers. Hormone-induced PGC-1α stimulates mitochondrial biogenesis via activation of NRF1 and NRF2, which in turn triggers mtTFA in brown adipose and cardiac myocytes. Mitochondria can be transferred through cells merging, exosome-mediated transfer, and tunneling through nanotubes. By delineating the underlying molecular mechanism of hormonal mitochondrial interaction, this study reviews the dynamics mechanisms of mitochondria and its effects on cellular level, health, diseases, and therapeutic strategies targeting mitochondrial diseases.

The Oxidation of Electron-Rich Arenes Using a H2O2-Proline System.

This study introduces a novel proline-catalyzed oxidation system employing hydrogen peroxide to synthesize quinones from a diverse range of substrates, including hydroquinones, phenols, resorcinols, aldehydes, and polycyclic aromatics. This approach is well-aligned with green chemistry principles, offering a more environmentally benign approach than earlier studies. Notably, this approach uses cost-effective reagents, proline as a readily available organocatalyst, reduced equivalents of H2O2, metal-free conditions, and notably short reaction times to achieve moderate-to-high yields. This promising approach encourages further exploration of the H2O2-proline system in oxidation reactions. This study's innovative approach and good results set a strong foundation for future research to expand the scope and efficiency of green oxidation processes.

Catalytic selectivity in activating peroxymonocarbonate with a commercial MnO2 catalyst for water remediation

An in-situ formed peroxymonocarbonate (HCO4-) oxidation system from an equilibrium reaction of H2O2 and HCO3– was established for eliminating organic pollutants in water. With H2O2 as a co-existing oxidant in the system, a commercial available MnO2 was able to proceed selective catalytic activation of HCO4- to generate reactive oxygen species (ROS), such as ·OH and CO3·-. The MnO2 catalyst showed excellent catalytic ability, structural stability and recyclability in the HCO4--based oxidation system for removing tetracycline. The degradation rate of tetracycline reached 86 % and the catalyst could be used for at least 5 times with only 0.1 ppm of Mn leached in water. The kinetic study indicated that HCO4- was more related to the degradation of tetracycline and generated ·OH at the yield of 40 % was primarily from HCO4- activation rather than H2O2 activation. The evaluation of ROS and quenching experiments suggested that CO3·- was most responsible for tetracycline degradation. The catalyst characterization and reaction mechanism indicated that low-valent Mn species were the active sites for HCO4- activation. Finally, in-situ formation of HCO4- derived from CO2 in Na2CO3 solution with H2O2 was preliminarily explored, presenting a MnO2-catalzyed CO2-derived HCO4--based oxidation system for pollutant control. This study shows that a cheap and commercial available MnO2 can be an efficient, facile and selective catalyst for in-situ formed HCO4--based advanced oxidation processes.

Biallelic variants in DAP3 result in reduced assembly of the mitoribosomal small subunit with altered intrinsic and extrinsic apoptosis and a Perrault syndrome-spectrum phenotype.

The mitoribosome synthesizes 13 protein subunits of the oxidative phosphorylation system encoded by the mitochondrial genome. The mitoribosome is composed of 12S rRNA, 16S rRNA and 82 mitoribosomal proteins encoded by nuclear genes. To date, variants in 12 genes encoding mitoribosomal proteins are associated with rare monogenic disorders, and frequently show combined oxidative phosphorylation deficiency. Here, we describe five unrelated individuals with biallelic variants in the DAP3 nuclear gene encoding mitoribosomal small subunit 29 (MRPS29), with variable clinical presentations ranging from Perrault syndrome (sensorineural hearing loss and ovarian insufficiency) to an early childhood neurometabolic phenotype. Assessment of respiratory chain function and proteomic profiling of fibroblasts from affected individuals demonstrated reduced MRPS29 protein levels, and consequently decreased levels of additional protein components of the mitoribosomal small subunit, associated with a combined complex I and IV deficiency. Lentiviral transduction of fibroblasts from affected individuals with wild-type DAP3 cDNA increased DAP3 mRNA expression, and partially rescued protein levels of MRPS7, MRPS9 and complex I and IV subunits, demonstrating the pathogenicity of the DAP3 variants. Protein modelling suggested that DAP3 disease-associated missense variants can impact ADP binding, and in vitro assays demonstrated DAP3 variants can consequently reduce both intrinsic and extrinsic apoptotic sensitivity, DAP3 thermal stability and DAP3 GTPase activity. Our study presents genetic and functional evidence that biallelic variants in DAP3 result in a multisystem disorder of combined oxidative phosphorylation deficiency with pleiotropic presentations, consistent with mitochondrial dysfunction.

Hydrogen technologies for decarbonization of ferrous metallurgy: scientific background and technical capabilities

To date, the use of ferrous hydrogen in metallurgy is considered from several positions. First, the partial or complete replacement of carbon with hydrogen reduces greenhouse gas emissions of CO2. Secondly, due to the different chemical affinity for oxygen, it becomes possible to selectively (selectively) extract components from complex complex ores and man-made materials. Thirdly, the absence of carbon in the reaction zone will make it possible to obtain a metal with a low content of carbides, which in many cases increases the demand for the product. Thermodynamic calculations of a multicomponent oxide system (Ni, Fe, P, Zn, Mn, V, Cr, O) in the presence of various reducing agents: carbon, hydrogen are performed using the TERRA software package, and their combined effects are considered. It is shown that the type of reducing agent affects the temperature of the beginning of the reduction of metals and the degree of their metallization. Thus, by selecting the type of reducing agent, its quantity, and the temperature of the process, it is possible to selectively extract some metals, and leave others in an oxidized state. Ilmenite concentrate in the form of a briquette was used in laboratory tests. The analysis of the iron reduction process using carbon or hydrogen as a reducing agent has been carried out. In comparison with the results obtained using carbon, the kinetics of iron reduction with hydrogen is higher. At the same time, the reduction proceeds with the formation of metallic iron, rutile (TiO2) and residual ilmenite. Whereas, in a carbothermic process occurring at higher temperatures (1000–1300 ℃), the reduction products are iron and iron ditanate (FeTi2O5). In addition, an important advantage of selective reduction by gas is the possibility of subsequent separation of the metallic and oxide phases by melting, without fear of secondary reduction due to solid carbon residues.