Characteristics of Retinitis Pigmentosa Associated with ADGRV1 and Comparison with USH2A in Patients from a Multicentric Usher Syndrome Study Treatrush

In contrast to USH2A, variants in ADGRV1 are a minor cause of Usher syndrome type 2, and the associated phenotype is less known. The purpose of the study was to characterize the retinal phenotype of 18 ADGRV1 patients (9 male, 9 female; median age 52 years) and compare it with that of 204 USH2A patients (111 male, 93 female; median age 43 years) in terms of nyctalopia onset, best corrected visual acuity (BCVA), fundus autofluorescence (FAF), and optical coherence tomography (OCT) features. There was no statistical difference in the median age at onset (30 and 18 years; Mann–Whitney U test, p = 0.13); the mean age when 50% of the patients reached legal blindness (≥1.0 log MAR) based on visual acuity (64 years for both groups; log-rank, p = 0.3); the risk of developing advanced retinal degeneration (patch or atrophy) with age (multiple logistic regression, p = 0.8); or the frequency of cystoid macular edema (31% vs. 26%, Fisher’s exact test, p = 0.4). ADGRV1 and USH2A retinopathy were indistinguishable in all major functional and structural characteristics, suggesting that the loss of function of the corresponding proteins produces similar effects in the retina. The results are important for counseling ADGRV1 patients, who represent the minor patient subgroup.     

耐候钢在2.0%NaCl中性溶液中的腐蚀过程

采用电化学阻抗谱技术研究了耐候钢Q400NQR1和09CuPCrNi在2.0％NaCl中性溶液中的腐蚀行为,建立了其腐蚀等效电路模型并分析了其相关电化学参数随时间的演化规律,结果表明,两种耐候钢的溶液腐蚀过程均可以分为四个阶段,即点蚀诱导期、点蚀发展期、腐蚀中期和腐蚀后期;Q400NQR1较09CuPCrNi具有较高的抗溶液腐蚀能力,前者在腐蚀的中期生成保护性较强的内层锈层,而后者形成的内层锈层难以稳定且易于出现均匀腐蚀的现象。     

COMPOSITION AND QUALITY OF THE GASOLINE OBTAINED FROM SYNGAS ON Cr2O3-ZnO/ZSM5 CATALYSTS

The kinetic equations for the formation of the lumps of the gasoline produced (C₅⁺ fraction), paraffins (C₅-C₈), xylenes (o-xylene, m-xylene and p-xylene) and aromatics (benzene, toluene, C₉-C₁₁) in the transformation of syngas on Cr₂O₃-ZnO/ZSM5 bifunctional catalyst have been established as a function of the concentration of both reactants (CO and H₂) and CO₂ byproduct. The effect of the operating conditions on the RON and on the molecular weight of the gasoline has been studied by experimentation in an integral fixed bed reactor in the range between 10 and 50atm and between 300 and 425°C. The octane index increases with Cr/Zn atomic ratio of the Cr₂O₃-ZnO metallic function, with Si/Al ratio of the HZSM5 zeolite, with space time and with the CO/H₂ molar ratio in the feed, in that order, whereas it passes through a maximum with pressure (at 20 atm) and with temperature (at 375°C). On the other hand, the molecular weight of the gasoline increases with the Cr/Zn atomic ratio, pressure, CO/H₂ molar ratio and space time, and decreases with the Si/Al ratio and temperature.     

Current and future experimental approaches in the study of grape and wine polyphenols interacting gut microbiota

Interactions between polyphenols and gut microbiota are indeed a major issue of current interest in food science research. Knowledge in this subject is progressing as the experimental procedures and analysis techniques do. The aim of this article is to critically review the more leading-edge approaches that have been applied so far in the study of the interactions between grape/wine polyphenols and gut microbiota. This is the case of in vitro dynamic gastrointestinal simulation models that try to mitigate the limitations of simple static models (batch culture fermentations). More complex approaches include the experimentation with animals (mice, rats, pigs, lambs and chicks) and nutritional intervention studies in humans. Main advantages and limitations as well as the most relevant findings achieved by each approach in the study of how grape/wine polyphenols can modulate the composition and/or functionality of gut microbiota, are detailed. Also, common findings obtained by the three approaches (in vitro, animal models and human nutritional interventions) such as the fact that the Firmicutes/Bacteroidetes ratio tends to decrease after the feed/intake/consumption of grape/wine polyphenols are highlighted. Additionally, a nematode (Caenorhabditis elegans) model, previously used for investigating the mechanisms of processes such as aging, neurodegeneration, oxidative stress and inflammation, is presented as an emerging approach for the study of polyphenols interacting gut microbiota. © 2020 Society of Chemical Industry     

Structural evaluation of copolymers of ethylene and 1‐octadecene by using the temperature rising elution fractionation technique

Three samples of ethylene‐octadecene copolymers having different quantitative composition were analyzed structurally and in terms of their thermal behavior. The samples were fractionated by temperature rising elution fractionation, presenting different chemical composition distributions (CCD) that are essentially the result of the proportion of incorporated octadecene. The CCD profiles were relatively wide for samples generated by metallocene catalysts. The analyses of the fractions showed that the melting and crystallization temperatures decrease with increasing comonomer incorporation, but this relation is affected by the average molecular weight of the chains. The melting thermograms of those fractions having higher proportions of octadecene may be divided into two characteristic regions: the first one, at a higher temperature, originates from the melting of the least modified chains, which crystallize more perfectly. The second one is formed by the melting of chains having a high degree of comonomer incorporation, which melt in a diffuse manner over a wide range of temperatures. It is probable that the morphology of the crystals formed in this region does not follow the folded chain model, and are better represented by a model involving the alignment of chain segments (bundling). © 2000 John Wiley & Sons, Inc. J Appl Polym Sci 79: 221–227, 2001     

Dimensioning a Cyclone Separator for Circulating Fluidized Bed Gasifier: Validation of a Procedure

Abstract

The transformation process of biomass through gasification may produce a large amount of solid particles of different granulometry. In order to avoid equipment damage, these particles must be separated from the producer gas before it is utilized in other processes. This separation can be performed through a cyclone separator. This equipment is part of the circulating fluidized bed gasifier and is responsible for particulate separation and producer gas partial cleanup. Cyclone dimensioning requires careful attention and must be done according to the conditions this device is going to be subject. Thus, this work aims to validate and analyze a dimensioning methodology and apply it to a gasifier. Three cyclone geometries were considered (Stairmand, Swift and Lapple) and they were compared in terms of collection efficiency and pressure drop. A parameter sensibility study was performed. Variations of dimension, efficiency and pressure drop for a producer gas volumetric flow range were tested. It was found that an increase of the volumetric flow rate provoked the rise of separation efficiency and pressure drop. From the tested geometries, Swift presented the highest collection efficiency and pressure drop.     

Decline in Constitutive Proliferative Activity in the Zebrafish Retina with Ageing

It is largely assumed that the teleost retina shows continuous and active proliferative and neurogenic activity throughout life. However, when delving into the teleost literature, one finds that assumptions about a highly active and continuous proliferation in the adult retina are based on studies in which proliferation was not quantified in a comparative way at the different life stages or was mainly studied in juveniles/young adults. Here, we performed a systematic and comparative study of the constitutive proliferative activity of the retina from early developing (2 days post-fertilisation) to aged (up to 3–4 years post-fertilisation) zebrafish. The mitotic activity and cell cycle progression were analysed by using immunofluorescence against pH3 and PCNA, respectively. We observed a decline in the cell proliferation in the retina with ageing despite the occurrence of a wave of secondary proliferation during sexual maturation. During this wave of secondary proliferation, the distribution of proliferating and mitotic cells changes from the inner to the outer nuclear layer in the central retina. Importantly, in aged zebrafish, there is a virtual disappearance of mitotic activity. Our results showing a decline in the proliferative activity of the zebrafish retina with ageing are of crucial importance since it is generally assumed that the fish retina has continuous proliferative activity throughout life.