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Natural gas (NG), known for its eco-friendly energy attributes, faces challenges such as low volumetric density, leakage susceptibility, and explosion risks during storage and transport. To narrow this gap, this study reports on the potential of bio-based adsorbents from cashew nut shells (CNS) and avocado seeds (AVS) as an alternative approach for the onboard storage of NG. CNS and AVS were characterized to determine moisture content, volatile matter content, ash content, and fixed carbon content. Additionally, the study determined the optimal loading of activating agents to produce activated carbons (ACs). ACs were produced through a dual process involving simultaneous chemical activation and carbonization. In the chemical activation phase, a sample of either cashew nut shells (CNS) or avocado seeds (AVS) was impregnated with phosphoric acid at various ratios. Subsequently, the properties of the ACs, including surface area, pore volume, and X-ray diffraction (XRD), were evaluated. The NG adsorption capacity of the ACs was assessed, and Freundlich and Langmuir isotherm adsorption equations were derived to elucidate the NG adsorption behavior on the ACs. The results revealed that the proximate analysis of CNS and AVS showed low moisture content (6.48% and 8.91%), high volatile matter (80.21% and 73.09%), low ash content (1.48% and 4.71%), and high fixed carbon (13.65% and 15.59%), indicating suitability for AC formulations. During synthesis, adding phosphoric acid to AVS or CNS in a 1:1 ratio resulted in significantly larger surface areas and pore volumes in the produced ACs. X-ray diffraction analysis of the ACs produced from CNS or AVS with phosphoric acid added at a 1:1 ratio indicated a broad diffraction background and a predominantly amorphous structure. The performance of the ACs in the adsorption of NG demonstrated that ACs produced from 1:1 mixtures of CNS with phosphoric acid and AVS with phosphoric acid exhibited higher adsorption capacities, 0.038 g/g and 0.031 g/g, respectively, at a low pressure of 5 bar, showcasing a novelty of this study. Furthermore, the Freundlich equation test yielded a constant R² exceeding 0.9, with positive adsorption intensity and adsorption capacity values, indicating a favorable fit for the model. In contrast, the Langmuir equation test resulted in a constant R² exceeding 0.9, but the adsorption capacity exhibited negative values, leading to inconclusive explanations. These findings affirm that bio-wastes can be an effective source of adsorbents for onboard natural gas storage , suggesting a means to reduce the take-dispose model and promote a circular economy in the energy sector.

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Hereditary erythrocytosis is a rare hematologic disorder characterized by an excess of red blood cell production. Here we describe a European collaborative study involving a collection of 2,160 patients with erythrocytosis sequenced in ten different laboratories. We focused our study on the EGLN1 gene and identified 39 germline missense variants including one gene deletion in 47 probands. EGLN1 encodes the PHD2 prolyl 4-hydroxylase, a major inhibitor of hypoxia-inducible factor. We performed a comprehensive study to evaluate the causal role of the identified PHD2 variants: (i) in silico studies of localization, conservation, and deleterious effects; (ii) analysis of hematologic parameters of carriers identified in the UK Biobank; (iii) functional studies of the protein activity and stability; and (iv) a comprehensive study of PHD2 splicing. Altogether, these studies allowed the classification of 16 pathogenic or likely pathogenic mutants in a total of 48 patients and relatives. The in silico studies extended to the variants described in the literature showed that a minority of PHD2 variants can be classified as pathogenic (36/96), without any differences from the variants of unknown significance regarding the severity of the developed disease (hematologic parameters and complications). Here, we demonstrated the great value of federating laboratories working on such rare disorders in order to implement the criteria required for genetic classification, a strategy that should be extended to all hereditary hematologic diseases.

Heme, iron protoporphyrin IX, is one of life’s most central molecules. Hence, availability of the enzymatic machinery necessary for its synthesis is crucial for every cell. Consequently, inborn errors of porphyrin metabolism that compromise normal synthesis, namely the family of porphyrias, undermine normal cellular metabolism given that heme has functions in catalytic centers, signal transduction and functional regulation and its synthesis is fully integrated into the center of intermediary metabolism. Very often, diagnosis of porphyrias is difficult and therefore delayed. Therapy can be as complicated. Over the last 50 years, several strategies have been developed: because of its integration with other parts of intermediary metabolism, the infusion of glucose (glucose effect) was one of the first attempts to counterbalance the dysregulation of porphyrin synthesis in porphyrias. Since heme synthesis is impaired, infusional replacement of heme was the next important therapeutic step. Recently, siRNA technology has been introduced in order to downregulate 5-ALA-synthase 1, which contributes to the patho-physiology of these diseases. Moreover, other novel therapies using enzyme protein replacement, mRNA techniques or proteostasis regulators are being developed.

A comparative study of the processes of metal-stimulated formation of silicon nanocrystals in SiOx–Sm structures was carried out. Samples with identical oxide matrix stoichiometry and Sm impurity content were formed using two methods: layer-by-layer deposition and coevaporation of SiO and Sm. Studies of PL and IR absorption spectra found that the admixture of samarium in samples of both types accelerates the thermally stimulated decomposition of samarium-doped SiOx films into Si and SiO2 and reduces the transition temperature of silicon nanoparticles from an amorphous state to a crystalline state. However, in (SiOx/Sm)10 multilayers, this effect is more pronounced than in single-layer films obtained using the co-evaporation method. Studying the Raman spectra of (SiOx/Sm)10 multilayers annealed at 750 °C determined that the relative volume fraction of nanocrystals in the silicon phase of the sample is ~ 38%. No line corresponding to the crystalline nc-Si was observed in the Raman spectra of samples deposited by co-evaporation and annealed under the same conditions. For these samples, a narrow line in the Raman spectrum corresponding to crystalline nc-Si appears only after annealing at 970 °C. In this case, the relative fraction of Si nanocrystals in the silicon phase of the sample was ~ 17%, 2.2 times less than for a similar multilayer annealed at 750 °C. This also indicates that, in multilayer (SiOx/Sm)10 samples, the thermally stimulated decomposition of SiOx, as well as the formation and crystallization of silicon nanoparticles, occurs at lower temperatures than in similar samples obtained by coevaporation. A possible mechanism for lowering the crystallization temperature of nc-Si in (SiOx/Sm)10 multilayers is discussed. [ABSTRACT FROM AUTHOR]