bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical and allelic heterogeneity in dystrophic epidermolysis bullosa- lessons from an Indian cohort
Authors: Gupta Divya , Jayashankar Charitha , Srinivas Manoj , Baraka Vishwanathan Gurudatta , Reddy Kristipati Raghavendra , Kubba Asha , Batrani Meenakshi , Hiremagalore Ravi 
Published: August 2023

BackgroundDystrophic epidermolysis bullosa (DEB) is due to variation in theCOL7A1gene. The clinical phenotype and severity depends on the type of variation and domain of the affected protein.ObjectivesTo characterize the spectrum ofCOL7A1variations in a cohort of DEB patients from India, to correlate these findings with clinical phenotypes and to establish a genotype-phenotype correlation.MethodsThis was a retrospective, observational study involving patients with DEB diagnosed on the basis of clinical manifestations, Immuno-fluorescence antigen mapping (IFM) and genetic analysis. A genotype-phenotype correlation was attempted and observations were further explained using IFM on skin biopsies and molecular dynamic simulations. Descriptive statistics were performed using SPSS version 20.0 with P values of <0.05 considered significant.ResultsWe report 68 unrelated Indian DEB patients classified as RDEB-Intermediate (RDEB-I), RDEB-Severe (RDEB-S) or DDEB based on the EB diagnostic matrix, immunofluorescence antigen mapping and genetic data. Of 68 DEB patients, 59 (86.76%) were inherited in a recessive pattern (RDEB) and 9 (13.24%) in a dominant pattern (DDEB). Limbal stem cell deficiency was seen in four cases of RDEB-S very early in the course of the disease. A total of 88 variants were detected of which 66 were novel. There were no hotspots and recurrent variations were seen in a very small group of patients. We found a high frequency of compound heterozygotes (CH) in RDEB patients born out of non-consanguineous marriage. RDEB patients older than two years who had oral mucosal involvement, and/or deformities, were more likely to have esophageal involvement. Genotype phenotype correlation showed a higher frequency of extracutaneous manifestations and deformities in patients with Premature Termination Codons (PTCs) than in patients with other variations. Molecular simulation studies in patients with missense mutations showed severe phenotype when they were localized in interrupted regions of GLY-X-Y repeats.ConclusionThis large study of DEB patients in South Asia adds to the continually expanding genetic database of this condition. This study has direct implications on management as this group of patients can be screened early and managed appropriately.
Combination therapy with budesonide and acetylcysteine alleviates LPS-induced acute lung injury via the miR-381/NLRP3 molecular axis
Authors: Yu Huimin , Lv Meifen , Zhang Shiying , Zou Kaiwen , Qian Yan , Lv Shaokun 
Published: August 2023

BackgroundAcute lung injury (ALI) usually has a high morbidity and mortality rate, but the current treatment is relatively scarce. Both budesonide (Bud) and N-acetylcysteine (NAC) exhibit protective effects in ALI, so we further investigated whether they have a synergistic effect on ALI when used together.MethodsEstablishment of a rat model of ALI with Lipopolysaccharide (LPS). Bud and NAC were administered by nebulized inhalation alone or in combination. Subsequently, HE staining was performed to observe the pathological changes in lungs of rat. Evans blue staining was implemented to assess alveolar permeability, and the pulmonary edema was assessed by measuring the ratio of wet to dry weight of the lung. Moreover, a TUNEL kit was served to test apoptosis in lung tissues. Western blot and immunohistochemistry were analyzed for expression of scorch-related proteins and NLRP3 in lung tissue, respectively. ELISA was implemented to detect inflammatory factor levels in BALF. and RT–qPCR was utilized to assess the expression level of miR-381. After stable transfection of miR-381 inhibitor or OE-NLRP3 in BEAS-2B treated with LPS, Bud and NAC, miR-381 expression was assessed by RT–qPCR, scorch death-related protein expression was measured by western blot, cell proliferation/viability was assayed by CCK-8, apoptosis was measured by flow cytometry, and ELISA was implemented to assess inflammatory factor levels. Furthermore, the Dual-luciferase assay was used to verify the targeting relationship.ResultsBud and NAC treatment alone or in combination with nebulized inhalation attenuated the increased alveolar permeability, pulmonary edema, inflammatory response and scorching in LPS-induced ALI rats, and combined treatment with Bud and NAC was the most effective. In addition, combined treatment with Bud and NAC upregulated miR-381 expression and inhibited NLRP3 expression in cellular models and LPS-induced ALI rats. Transfection of the miR-381 inhibitor and OE-NLRP3 partially reversed the protective effects of Bud and NAC combination treatment on BEAS-2B cell proliferation inhibition, apoptosis, focal death and the inflammatory response.ConclusionCombined Bud and NAC nebulization therapy alleviates LPS-induced ALI by modulating the miR-381/NLRP3 molecular axis.
PERFORMATIVE POLITICS AND DIGITAL POPULISM IN INDONESIA
Authors: Nadzir Ibnu 
Published: August 2023

Dalam beberapa tahun terakhir, gerakan-gerakan populis telah menjadi salah satu kekuatan berpengaruh dalam politik Indonesia. Kemunculan cepat gerakan-gerakan ini tidak dapat dilepaskan dari integrasi media digital ke dalam politik, khususnya untuk memobilisasi dan menyebarkan gagasan – gagasan politik mereka. Meskipun demikian, kajian yang berupaya memahami hubungan antara populisme dan media digital masih relatif sedikit. Dalam konteks tersebut, artikel ini mengkaji pertautan antara teknologi dan perkembangan populisme di Indonesia. Berangkat dari perspektif populisme sebagai langgam politik, artikel ini berargumen bahwa media digital terpadu dalam terbentuknya gerakan-gerakan populis di Indonesia. Fitur-fitur spesifik dari media digital menyediakan ruang yang ideal bagi politik performatif yang menjadi landasan penting bagi populisme. Untuk mengelaborasikan proposisi tersebut, artikel ini membandingkan dua gerakan populis di Indonesia: gerakan nasionalis dan Islamis. Kedua gerakan tersebut menggunakan media digital untuk memeperluas pengaruh mereka dalam politik Indonesia. Meskipun demikian, pengaruh tersebut tetap dibatasi pada kemampuan mereka untuk mengontrol institusi politik yang telah mapan. Dalam konteks tersebut, populisme sulit untuk dapat dipisahkan dari institusi-institusi politik formal yang kemudian berpengaruh pada pelemahan demokrasi di Indonesia. Kata Kunci: digital, populisme, demokrasi, Indonesia, politik performatif
STRATEGI KEPEMIMPINAN KRISIS INDONESIA DALAM MENGHADAPI TANTANGAN GLOBAL UNTUK PEMULIHAN EKONOMI
Authors: Said Moch. Fauzie 
Published: August 2023

Ketidakpasitian kondisi global saat ini dirasakan oleh setiap negara di dunia. Kondisi yang berubah-ubah dipicu oleh beberapa tantangan yang sedang terjadi saat ini dan membawa pengaruh besar bagi sikap pemimpin sebuah negara. Dengan melalukan analisis pemberitaan online, artikel ini ingin mengetahui bagaimana kepemimpinan krisis di Indonesia digunakan untuk menghadapi tantangan global dalam pemulihan ekonomi. Artikel ini melakukan telaah teks media online yang terkait dengan fenomena yang diteliti, serta melakukan komparasi antara gaya kepemimpinan transaksional dan tranformasional yang dikaji dari beberapa literatur online (melalui pemberitaan media online). Kajian ini menemukan bahwa, Presiden Jokowi menggunakan dua gaya kepemimpinan dalam menghadapi tantangan global pemulihan ekonomi, yaitu gaya kepemimpinan transaksional pada aspek (1) reaksi pemimpin; (2) pemberdayaan budaya dan (3) kinerja manajemen, sedangkan pada aspek lainnya, yaitu: (1) dorongan motivasi kepada pengikut dan (2) mekanisme pemecahan masalah, Presiden Jokowi lebih menggunakan gaya trasnformasional. Dari hasil kajian ini disimpulkan bahwa gaya kepemimpinan Presiden Jokowi bersifat adaptif terhadap perubahan yang terjadi. Kata Kunci: kepemimpinan krisis, Presiden Jokowi, pemulihan ekonomi, tantangan global
REVIEW BUKU KONSERVATISME ISLAM: POLITIK IDENTITAS DAN KELOMPOK ISLAMIS DI INDONESIA
Authors: Zulfadli Zulfadli 
Published: August 2023

Setelah jatuhnya rezim Orde Baru telah terjadi pergeseran dinamika Islam dalam masyarakat muslim di Indonesia. Salah satu fenomena yang muncul sejak dua dasawarsa terakhir adalah menguatnya konservatisme Islam dalam berbagai dimensi kehidupan masyarakat baik secara teologis, sosiologis-politik. Konservatisme Islam tidak hanya menunjukkan pengaruh yang cukup kuat dalam kehidupan masyarakat akan tetapi juga merupakan tantangan serius terhadap Islam arus utama Indonesia yang mengedepankan karakteristik moderatisme Islam. Pergeseran ini, sangat berbeda dengan persepsi Islam Indonesia yang dikenal selama ini sebagai salah satu entitas Islam yang distingtif, ramah, karakteristik moderat, inklusif, toleran, pluralistik menunjukkan wajah yang senyum mengalami perubahan menuju arus konservatisme agama. Buku ini merupakan studi terbaru daam menggambarkan bagaimana dinamika Islam Indonesia mengalami penguatan ke arah konservatisme agama. Kehadiran kelompok keagamaan konservatif secara aktif merespon isu-isu sosial politik dan keagamaan dan mempromosikan sikap dan pemahaman konservatisme agama. Aktivitas kelompok konservatisme Islam telah mengubah wajah Islam Indonesia dari yang bercorak inklusif-moderat ke arah orientasi ekslusif-literal. Buku ini diakhiri dengan prediksi bahwa Islamisme konservatif masih menjadi pengaruh signifikan bagi perkembangan politik dan sosial-ekonomi di Indonesia untuk masa depan. Kata Kunci: Konservatisme Islam, Kelompok Keagamaan, Politik Identitas, Demokrasi
The role of microbes in the inhibition of the atmospheric corrosion of steel caused by air pollutants
Authors: Oshin Temitope Alaba , Fakinle Bamidele Sunday , Oyewole Olamide 
Published: August 2023

Abstract
Due to the presence of corrosive contaminants in the air, metals naturally corrode when exposed to the environment. Air pollution, in conjunction with climate conditions, may significantly deteriorate outdoor materials, especially metals and hence, the need for corrosion control. Using inhibitors is a powerful strategy that is frequently employed for corrosion prevention and control. Chemical inhibitors are often used. However, due to their low effectiveness and stringent environmental regulations, the majority of chemical corrosion inhibition techniques are becoming less desirable. For this reason, there is an increasing interest in and focus on biological inhibition approaches, which most recently have included the use of microbes. Microbiologically-influenced corrosion inhibition (MICI) is apparently far more complex than traditional corrosion inhibition procedures. A current overview of the mechanisms that have been used or may be efficient for MICI technologies is important in order to facilitate the advancement of MICI and its practical industrial applications, especially for atmospheric corrosion caused by air pollutants, for which there is little information in the reviewed literature. Therefore, this review addresses the role of microbes, like Pseudomonas putida, in the inhibition of atmospheric corrosion of metals and brings the reader up-to-date on the few literatures existing on the subject. The review describes and characterizes MICI for atmospheric corrosion as a developing field still in need of enthusiastic researchers to further investigate the area in order to establish useful methodologies, procedures, and technologies for later adoption in industrial terrains and applications.
Effect of chemical composition and heat treatment on intergranular corrosion and strength of AlMgSiCu alloys
Authors: Çelik Osman Halil , Yücel Onuralp 
Published: August 2023

Abstract
The use of aluminum in chassis, bumper, and crash boxes has increased in the last 10 years with an increase in the production of electric vehicles in the automotive industry. The extrusion process has also gained importance because it allows mass production. While basic 6xxx series aluminum alloys such as 6060 and 6063 were used in the early stages of the process, later on, 6005A and 6082 alloys, which provide higher strength, have been used. Alloys with higher strength and crash ability are needed with an increase in safety requirements in automotive. In this study, the effect of chemical composition and heat treatment on the intergranular corrosion strength of 6056 alloys was examined. Another aim of this study is not only to produce high strength and ductility alloy but also to provide good corrosion resistance as automotives are used in different environments for several decades. The 6056 alloys are potential candidate materials for the new-generation electrical vehicles in the automobile industry due to their high strength, weldability, machinability, and impact resistance. Therefore, in our work, we produced 6056 alloy samples in a billet form using the direct chill casting method. Then they were homogenized, and billets were extruded as a box profile. Experimental studies were carried out on 6056 alloys with two different chemical compositions and three different heat treatment conditions (T42, T62, and T76) using Method B of EN ISO 11846 standard for corrosion testing. Crack sizes of metallographic sections from corroded areas were calculated g using a scanning electron microscope. As a result, we found that the addition of Mg to 6056 alloys improves corrosion resistance, while copper reduces it. When Zn is added to the alloys, Mg starts to react with it and forms MgZn2, which increases the corrosion progress. Moreover, when heat treatment is applied at T76 conditions, the alloys demonstrate high corrosion resistance.
Adaptive centralized energy management algorithm for islanded bipolar DC microgrid
Authors: Dodda Satish Reddy , Sandepudi Srinivasa Rao 
Published: August 2023

Abstract
In this paper, an adaptive centralized energy management algorithm is developed for an islanded bipolar DC microgrid (BDCMG). The proposed microgrid consists of a solar photovoltaic (SPV) system, a wind energy conversion system (WECS), and a battery energy storage system (BESS). The SPV and WECS are operated in maximum power point tracking mode to extract maximum power. The adaptive control algorithm focus on power sharing at a bipolar DC bus based on variations in wind speed, solar irradiance, battery state of charge (SoC), and load. Furthermore, this algorithm maintains voltage balance and voltage regulation on a bipolar DC bus without the use of a communication link and provides effective energy management through coordinated control of renewable energy sources and BESS. The efficacy of the proposed bipolar DC microgrid is simulated and validated on a lab scale prototype to verify its feasibility, and it is observed that the results are found to be satisfactory.
Alkbh5 plays indispensable roles in maintaining self-renewal of hematopoietic stem cells
Authors: Yang Bijie , Liu Yuanyuan , Xiao Feifei , Liu Zhilong , Chen Zhe , Li Zhigang , Zhou Chengfang , Kuang Mei , Shu Yi , Liu Shan , Zou Lin 
Published: August 2023

Abstract

Alkbh5 is one of the primary demethylases responsible for reversing N6-methyladenosine (m6A) modifications on mRNAs, and it plays a crucial role in many physiological and pathological processes. Previous studies have shown that Alkbh5 is required for maintaining the function of leukemia stem cells but is dispensable for normal hematopoiesis. In this study, we found that Alkbh5 deletion led to a moderate increase in the number of multiple progenitor cell populations while compromising the long-term self-renewal capacity of hematopoietic stem cells (HSCs). Here, we used RNA-seq and m6A-seq strategies to explore the underlying molecular mechanism. At the molecular level, Alkbh5 may regulate hematopoiesis by reducing m6A modification of Cebpa and maintaining gene expression levels. Overall, our study unveiled an essential role for Alkbh5 in regulating HSC homeostasis and provides a reference for future research in this area.
The crystal structure of (4-hydroxybenzenesulfonate)-k1
O-6,6′-((1E,1′E)- (ethane-1,2-diylbis(azaneylylidene))bis(methaneylylidene)) bis(2-methoxyphenol)-κ2
N,N,μ
2
O,O,κ2
O, O)-(methanol)-cobalt(II) sodium(I), C25H27CoN2NaO9S
Authors: Zhang Shaoliang , Wang Yanlan , Li Shanshan 
Published: August 2023

Abstract
C25H27CoN2NaO9S, monoclinic, P21/c (no. 14), a = 12.8169(11) Å, b = 7.9965(7) Å, c = 25.597(2) Å, β = 91.530(2)°, V = 2622.5(4) Å3, Z = 4, R
gt(F) = 0.0611, wR
ref(F
2) = 0.1596, T = 298 K.
Analytical Lens for Investigating CSOs and State Relations: The Contributions of Coproduction and Institutional Logics Perspectives
Authors: Holanda Bruna de Morais , Mendonça Patricia Maria Emerenciano de 
Published: August 2023

AbstractThe 1990s saw what Della Porta (2020. “Building Bridges: Social Movements and Civil Society in Times of Crisis.”Voluntas31 (5): 938–48) calls the renaissance of civil society, from which studies on the relations between Civil Society Organizations (CSOs) and the state grew exponentially. Based on this context, this theoretical essay aims to demonstrate how the theories of coproduction and institutional logics can be used in a complementary way to understand these relations. We use a qualitative methodology of extensive literature review and study of the proposed concepts. We identify six contributions of this complementary use: (i) CSOs-state coproduction processes take place on institutional fields, (ii) Coproduction encompasses the mobilization of institutional logics’ material and symbolic resources, (iii) Structural mechanisms influence coproduction, (iv) Institutional logics bring both collaboration and conflict dimensions into coproduction processes, (v) Coproduction helps operationalize studies in institutional logics, and (vi) Institutional logics influence coproduced results. We hope that our theoretical–analytical contributions can be applied in empirical studies and improved in the future.
Microstructure and Pitting Corrosion Characteristics of Tig Welded Joints of Super 304HCu Austenitic Stainless Steel
Authors: Vinoth Kumar M. , Rajendran C. , Balasubramanian V. 
Published: August 2023

Abstract
Super 304HCu is an advanced ultra-super critical (A-USC) boiler grade austenitic stainless steel with the distinct addition of 3 wt.-% of Copper. A-USC power plants intended to operate in chloride rich environments (sea shore, feed water residues, etc.) are susceptible to chloride assisted corrosion failures. In this study, the pitting corrosion behaviour of the Super 304HCu parent material and tungsten inert gas weld joints was studied using a potentiodynamic cyclic polarization test in 3.5 % NaCl solution at three different pH levels (pH = 3, pH = 7, and pH = 11). The Epit values of the parent material is found to be much nobler than that of the weld joints. The micrographs of the pitted weld joints and the oxalic acid etched structure of Super 304HCu joints are presented. From the micrographs it is revealed that the heat affected zone is the most susceptible region to pitting corrosion.
Catalytic membrane reactors for alkane dehydrogenation applications: an integration of catalysis and separation process
Authors: Pati Subhasis , Dewangan Nikita , Jangam Ashok , Kawi Sibudjing 
Published: August 2023

Abstract
Catalytic dehydrogenation of saturated hydrocarbons to corresponding alkenes by the release of the stoichiometric amount of hydrogen is the paramount solution for safe storage of hydrogen. The utilization of a catalytic membrane reactor for this process enhances the reaction yield beyond thermodynamic equilibrium by selectively and simultaneously removing the produced H2 during the reaction. To this end, the present review is focused on the integration of H2 permeable membranes with the catalysts for dehydrogenation of lighter alkanes for coproduction of olefins and high-purity hydrogen in a single step. Besides, this review also covers dehydrogenation of liquid organic hydrogen carriers for safe storage of hydrogen. Herein, different types of H2 perm-selective membranes used for the dehydrogenation reaction are highlighted and the effect of hydrocarbon on H2 permeation through these membranes are discussed in detail. Furthermore, the simulation studies along with the experimental investigation performed on the membrane reactors for dehydrogenation of linear and cyclic alkanes are critically reviewed to find the coherence between simulation and experimental findings. Systematic discussion is done on the different types of alkane dehydrogenation reactions and the parameters affecting the reaction performance. Finally, directions are provided to prepare a cheaper and large industrial scale membrane reactor for dehydrogenation reaction. The concept of coupling an exothermic reaction with the endothermic dehydrogenation reaction is provided as a future direction study to enhance the overall yield and energy efficiency of the integrated membrane reactor.
“For Us, There Are No More Back Doors”
Authors: Bobadilla Eladio B. 
Published: August 2023

This article argues that the nativist-led referendum known as Proposition 187, which in 1994 sought to deny undocumented immigrants and their families access to many of the state’s social services, represents a set of paradoxes about Latino immigration to California and the United States whose roots, consequences, and implications are not yet fully understood. First, Prop 187 revealed the enduring place of undocumented people as “essential but unwanted.” Second, it represented both a victory and a defeat for the immigrants’ rights struggle. And finally, it represented both an end and a beginning in U.S. immigration history.
By interpreting Prop 187 in this way, historians and other scholars should see the moment in more complex and elucidating ways than we have so far, not merely as a “turn” against undocumented immigrants characterized by a new form of nativism fueled by fears about demographic and cultural change, not merely as a moment that spurred action and a search for power among both undocumented immigrants and Latino U.S. citizens, and not merely as something that “foreshadowed” attempts to bring immigration policy under local control.
Instead, a more holistic look, and one that takes a longer view of this story, stretching both farther back in time and closer to the present, reveals that Prop 187 was never an issue of importance only to California, and that while there was much that was “new” about this form of nativism, there was also much more that simply made Prop 187 the logical conclusion. Neither did California merely foreshadow other states’ attempts to take immigration into their own hands. Rather, California’s Prop 187 was a direct cause of their doing so.