bluuebunny/crossref_metadata_2025_split huggingface dataset:
Hereditary spastic paraplegia type 35 in a Turkish girl with fatty acid hydroxylase-associated neurodegeneration
Authors: Engin Erdal Ayşenur , Yürek Burak , Kıreker Köylü Oya , Ceylan Ahmet Cevdet , Çıtak Kurt Ayşegül Neşe , Kasapkara Çiğdem Seher 
Published: February 2024

Abstract

Objectives
The fatty acid 2-hydroxylase gene (FA2H) compound heterozygous or homozygous variants that cause spastic paraplegia type 35 (SPG35) (OMIM # 612319) are autosomal recessive HSPs. FA2H gene variants in humans have been shown to be associated with not only SPG35 but also leukodystrophy and neurodegeneration with brain iron accumulation.

Case presentation
A patient with a spastic gait since age seven was admitted to the paediatric metabolism department. She was born to consanguineous, healthy Turkish parents and had no family history of neurological disease. She had normal developmental milestones and was able to walk at 11 months. At age seven, she developed a progressive gait disorder with increased muscle tone in her lower limbs, bilateral ankle clonus and dysdiadochokinesis. She had frequent falls and deteriorating school performance. Despite physiotherapy, her spastic paraplegia was progressive. Whole exome sequencing (WES) identified a homozygous NM_024306.5:c.460C>T missense variant in the FA2H gene, of which her parents were heterozygous carriers. A brain MRI showed a slight reduction in the cerebellar volume with no iron deposits.

Conclusions
Pathogenic variants of the FA2H gene have been linked to neurodegeneration with iron accumulation in the brain, leukodystrophy and SPG35. When patients developed progressive gait deterioration since early childhood even if not exhibited hypointensity in the basal ganglia detected by neuroimaging, FA2H-related neurodegeneration with brain iron accumulation should be ruled out. FA2H/SPG35 disease is characterised by notable clinical and imaging variability, as well as phenotypic diversity.
Coagulation assessment of severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) infected pregnant women and their offspring by using rotational thromboelastometry (ROTEM)
Authors: Mitsiakos Georgios , Gialamprinou Dimitra , Kontovazainitis Christos-Georgios , Moraitis Athanasios , Katsaras Georgios , Pouliakis Abraham , Diamanti Elissavet 
Published: February 2024

Abstract

Objectives
During pregnancy, severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) infection may intensify the gestational procoagulant state. Coronavirus disease 2019 (COVID-19) associated coagulopathy (CAC) constitutes an exacerbated immunothrombosis response. There is limited data regarding the coagulation profile of SARS-CoV2-infected pregnant women, especially those with CAC, and the effect on their offspring. This prospective study aimed to compare the hemostatic profile of those women and their neonates with healthy mother–neonate pairs.

Methods
Conventional coagulation tests (CCTs) and rotational thromboelastometry (ROTEM) were employed to evaluate the hemostatic profiles. Neonates were assessed at birth and on the fourth day of life.

Results
We enrolled 46 SARS-CoV2-infected pregnant women and 22 healthy controls who gave birth to 47 and 22 neonates, respectively. CAC was present in 10 participants. SARS-CoV2-infected pregnant women manifested slightly prolonged APTT and higher fibrinogen levels. Regarding ROTEM, we noted decreased FIBTEM CFT, with higher A10, A-angle, and MCF. The CAC group presented lower platelet count, increased fibrinogen levels, and higher FIBTEM A10 and MCF. PT was slightly prolonged at birth in neonates born to SARS-CoV2-infected mothers. During the fourth day of life, D-dimers were significantly increased. Concerning ROTEM, neonates born to SARS-CoV2-infected mothers showed lower FIBTEM CT at birth.

Conclusions
SARS-CoV2-infected pregnant women present a hypercoagulable profile. Hypercoagulability with elevated fibrinolysis and lower platelet count is observed in participants with CAC. The coagulation profile of neonates born to SARS-CoV2 mothers seems unaffected. Elevated D-dimers on the fourth day may reflect a neonatal inflammatory response to maternal SARS-CoV2.
Public Reason, Coercion, and Overlapping Consensus
Authors: Spector Ezequiel 
Published: February 2024

Abstract
The idea of public reason involves a standard of legitimacy that requires that laws and institutions be acceptable to all reasonable people, regardless of their conceptions of the good. Many philosophers have argued that public reason should be understood as an answer to the question of how to justify state coercion. However, some authors have criticized this traditional account because it overlooks noncoercive state actions that seem appropriate topics of public reason. More recently, some philosophers have defended the traditional account against that objection. In this paper, I argue that these approaches cannot effectively deal with that objection and offer a different version of the traditional account that can do so. This version rests on the ideas of overlapping consensus and stability. According to this version, the point of public reason is preserving an overlapping consensus on a coercive system of laws and institutions and achieving a stable society.
Quantifying robustness against sharp bending in an integrated topological interface of valley photonic crystals
Authors: Guo Chao-Heng , Tang Guo-Jing , Li Meng-Yu , Chen Wen-Jie , He Xin-Tao , Dong Jian-Wen 
Published: February 2024

Abstract
Sharp-bending waveguide is a key element for ultra-compact and densely integrated photonic devices, which is promising to enlarge the capability of modern information processing in a single chip. Topological photonics manifest the nature of robust propagation against sharp bending and such robustness has been experimentally demonstrated in topological integrated interfaces. It is important to quantify the bending loss of topological interface but has remained exclusive. In this work, we report on the characterization of sharp-bending robustness in the integrated topological interface of valley photonic crystals (VPCs) by experimentally quantifying the ultralow bending loss. The VPCs are designed on a standard silicon-on-insulator platform with the inversion-symmetry broken in honeycomb lattice, and four types of topological interfaces can be constructed by two topologically-distinct VPCs. As one of the representative cases, zigzag-AA interface is applied to demonstrate the robust propagation along sharp bending. In experiment, we fabricate a series of VPC interfaces with different turn number and the same transmission distance, which perform the ultralow bending loss less than 0.02 dB per 120-deg turning. Furthermore, we experimentally characterize the propagation loss in the integrated interfaces. Our approach not only shows the ability of VPC topological interfaces to suppress backscattering stemming from sharp bending, but also paves the way for topological nanophotonic dense integration.
Crystal structure of bis(3,5-diisopropyl-1H-pyrazol-4-ammonium) tetrafluoroterephthalate, 2[C9H18N3][C8F4O4]
Authors: Fujisawa Kiyoshi , Harakuni Seigo , Iwai Kako , Ageishi Keigo , Tiekink Edward R. T. 
Published: February 2024

Abstract
2[C9H18N3][C8F4O4], monoclinic, I2/a (no. 15), a = 10.2282(4) Å, b = 11.9366(5) Å, c = 24.5548(12) Å, β = 98.948(4)°, V = 2961.4(2) Å3, Z = 4, Rgt
(F) = 0.0456, wRref
(F
2) = 0.1217, T = 178 K.
The crystal structure of t-butyl 7-[4-(4-fluorophenyl)-2-[(methanesulfonyl)(methyl)amino]-6-(propan-2-yl)pyrimidin-5-yl]-3,5-dihydroxyhept-6-enoate, C26H36FN3O6S
Authors: Zou Jing , Sheng Lv-Qing , Xu Xin-Xin , Xu Rou-Han , Jiang Cheng-Jun 
Published: February 2024

Abstract
C26H36FN3O6S, monoclinic, P21 (no. 4), a = 10.6615(4) Å, b = 8.7470(3) Å, c = 29.4342(11) Å, α = 9°, β = 95.6130(10)°, γ = 90°, V = 2731.76(17) Å3, Z = 4,

R
gt

${R}_{\text{gt}}$

(F) = 0.0439,

w

R
ref

$\mathrm{w}{R}_{\text{ref}}$

(F
2) = 0.1083, T = 170 K.
MTT assay of human anti-breast cancer cells (MCF-7) in vitro potentials and phytochemicals screening of the root bark extracts from Cassia sieberiana
Authors: Magashi Labaran Abdulkadir 
Published: February 2024

Abstract
Medicinal plant Cassia sieberiana root bark was investigated for phytochemicals and Anti-Breast Cancer cells (MCF-7) properties, using Chemical Separations and MTT assay in vitro. The C. sieberiana root bark was extracted with soxhlet extractor using different solvents based on polarity guided method and the respective extracts were concentrated under reduced pressure. These extracts were screened for their phytochemicals qualitatively using standard methods. Percentage Yields and Physico-Chemical Evaluation of the Extracts from the various fractions were recorded. The results of phytochemicals screening revealed the presence of some secondary metabolites of pharmacological significance in the Methyl acetate, MeOH and 70 % MeOH root bark extracts including Saponins, Quinones, Phenolic, Steroids, Tannins, Flavounoids, Terpenoids, Anthraquinones, Cardiac-glycosides, Alkaloids, Carbohydrates, Glycosides and Coumarins. Results obtained from MTT assay revealed that Extracts of MeOH and 70 % MeOH samples shown a certain degree of inhibition towards MCF-7 cell line. IC50 were calculated using Graph Pad Prism 6 and results shown that IC50 of 245.3 μg/ml and 239.6 μg/ml dose dependent inhibitions in MCF-7 cells respectively, compared to Oxaliplatin IC50 of 38.04 μg/ml. Therefore, 70 % MeOH sample with IC50 of 239.6 μg/ml have shown more potential of inhibiting breast cancer cell, MCF-7 cells line. The inhibition of the root bark is attributed to some of the phytochemicals present in the plant.
Evaluating sustainability of Bhuj aquifer system, Western India using nuclear dating techniques
Authors: Roy Annadasankar , Sinha Uday Kumar , Hathi Bharatkumar , Chatterjee Sitangshu , Pant Diksha , Mohokar Hemant , Jaryal Ajay , Pant Harish Jagat , Saha Dipankar , Jain Ratan , Keesari Tirumalesh 
Published: February 2024

Abstract
Increasing population, food demands and climatic stressors pose an imminent threat to groundwater sustainability in regional aquifer systems globally. Limited availability of surface water and erratic/uncertain rainfall necessitates preservation of groundwater resources, which form reliable fresh water reserves in most of the arid regions. Bhuj Sandstone aquifer is one such fresh groundwater systems in Western India catering the domestic/irrigational needs for over 2 million population. Rapid groundwater depletion in this area warranted a mandatory conservation of groundwater resources for future sustainability. Nuclear techniques using 3H and 14C provide estimates of groundwater age that help in planning sustainable groundwater management. In this study, sustainability of deep groundwater was assessed using environmental radioactive isotopes (3H, 14C). Results indicate that 14C activity of the groundwater samples varies from 26 to 73.6 pMC (percent modern carbon). Considering the various biases and uncertainties present, multiple correction models were applied to obtain representative groundwater ages by incorporating stable (13C) isotope and hydrogeochemical data. The corrected and representative ages are found to range from 5.8 to 8.6 ka BP (thousand years before present). From the study it can be inferred that central Bhuj aquifer hosts paleo-groundwater while the western part is recently recharged. Therefore, over-exploitation of deep groundwater in central Bhuj aquifer may further lower the water levels and this would have long-term impact on the socio-economic development of Kutch region.
“Brain–breath” interactions: respiration-timing–dependent impact on functional brain networks and beyond
Authors: Nakamura Nozomu H. , Oku Yoshitaka , Fukunaga Masaki 
Published: February 2024

Abstract
Breathing is a natural daily action that one cannot do without, and it sensitively and intensely changes under various situations. What if this essential act of breathing can impact our overall well-being? Recent studies have demonstrated that breathing oscillations couple with higher brain functions, i.e., perception, motor actions, and cognition. Moreover, the timing of breathing, a phase transition from exhalation to inhalation, modulates specific cortical activity and accuracy in cognitive tasks. To determine possible respiratory roles in attentional and memory processes and functional neural networks, we discussed how breathing interacts with the brain that are measured by electrophysiology and functional neuroimaging: (i) respiration-dependent modulation of mental health and cognition; (ii) respiratory rhythm generation and respiratory pontomedullary networks in the brainstem; (iii) respiration-dependent effects on specific brainstem regions and functional neural networks (e.g., glutamatergic PreBötzinger complex neurons, GABAergic parafacial neurons, adrenergic C1 neurons, parabrachial nucleus, locus coeruleus, temporoparietal junction, default-mode network, ventral attention network, and cingulo-opercular salience network); and (iv) a potential application of breathing manipulation in mental health care. These outlines and considerations of “brain–breath” interactions lead to a better understanding of the interoceptive and cognitive mechanisms that underlie brain–body interactions in health conditions and in stress-related and neuropsychiatric disorders.
In vivo C6 glioma models: an update and a guide toward a more effective preclinical evaluation of potential anti-glioblastoma drugs
Authors: Pournajaf Safura , Afsordeh Nastaran , Pourgholami Mohammad Hossein 
Published: February 2024

Abstract
Glioblastoma multiform (GBM) is the most common primary brain tumor with a poor prognosis and few therapeutic choices. In vivo, tumor models are useful for enhancing knowledge of underlying GBM pathology and developing more effective therapies/agents at the preclinical level, as they recapitulate human brain tumors. The C6 glioma cell line has been one of the most widely used cell lines in neuro-oncology research as they produce tumors that share the most similarities with human GBM regarding genetic, invasion, and expansion profiles and characteristics. This review provides an overview of the distinctive features and the different animal models produced by the C6 cell line. We also highlight specific applications of various C6 in vivo models according to the purpose of the study and offer some technical notes for more convenient/repeatable modeling. This work also includes novel findings discovered in our laboratory, which would further enhance the feasibility of the model in preclinical GBM investigations.
Verum, focus and evidentiality in Conchucos Quechua
Authors: Bendezú-Araujo Raúl 
Published: February 2024

AbstractFor most Quechuan languages, the discourse enclitic=mihas been described as a (contrastive) focus marker and/or as a marker of direct evidentiality. In this paper, I argue that these claims do not seem to apply to Conchucos Quechua (Ancash, Peru). To challenge the association between=miand focus, I offer a quantitative analysis that strongly suggests that this connection is not categorical in nature. To contest the association between=miand direct evidentiality, I offer a qualitative analysis based on aquestion-under-discussion(QUD) model of discourse structure, using naturalistic data. I will argue that this enclitic is better characterised as an assertion operator, whose function is to bring closure to the current QUD (be it explicit or implicit). In this sense, the use of=miis reminiscent of what has been calledverumin the literature, and I thus explore how this characterisation fits within this discussion.
Polar solvation molecular dynamics, quantum reactivity (ELF, HOMO–LUMO, NBO) studies, spectroscopy (FT-IR, UV), and the antibiotic potential of carbazide derivative via in-silico molecular docking
Authors: Mujafarkani Nagoor , Adindu Eze A. , Godfrey Obinna C. , Agurokpon Daniel C. , Alawa John A. , Odey Michael O. , Gber Terkumbur E. , Owen Aniekan E. , Jafar Ahamed Abdul Gafoor , Benjamin Innocent , Louis Hitler 
Published: February 2024

Abstract
Recently, the treatment of bacterial infection has been very worrisome as a decline in antibiotic sensitivity is hitting a majority of the world population. Among many bacterial infection’s causing agents, Enterococcus species and Mycoplasma pneumonia are highly threatening because of their resistance to powerful antibiotics such as vancomycin, erythromycin, and azithromycin. Herein, effect of polar (DMSO, EtOH, MeOH, H2O) solvation on the quantum chemical parameters, molecular structure, spectroscopy, and the antimicrobial potential of p-phenylenediamine-thiosemicarbazide-formaldehyde (PTSF) terpolymer is presented within the framework of density functional theory (DFT), solvation molecular dynamics, and molecular docking approach. Herein, ethanol was keenly observed with the most insightful properties across all analyses specifically by its high energy gap (4.6344 eV) which accounted for the stability of compound. The molecular docking revealed the binding affinities for PSTF with respect to the best docking modes are −4.6 kcal/mol with LYS 288 and bond distance of 2.82 Å for 5V2M and −5.1 kcal/mol with ASN 770 at 2.27 Å, GLU 767 at 2.61 Å, ARG 777 at 3.69 Å, GLU 775 at 2.57 Å, and ALA 763 at 2.86 Å for 6rj1. From the result obtained, the studied compound has higher binding affinity at the active site of 6RJ1 than that of 5V2M. This suggests that PSTF has greater efficacy for inhibiting the growth of M. pneumoniae than vancomycin resistant Enterococcus faecalis. Hence, further in vitro and in vivo studies should delve into the more exploration of the reported ligand.
Exploring the dynamics of halogen and hydrogen bonds in halogenated coumarins
Authors: Varghese Mebin , Thomas Jisha Mary , Alzahrani Abdullah Y. , Thomas Renjith 
Published: February 2024

Abstract
Halogen bonds find application in supramolecular chemistry, DNA Holliday junction, drug design, organic catalysis and various other fields. Coumarin derivatives are high in demand due to their application in photochemotherapy, drugs and other cancer treatments. Halogenated coumarins are widely known for their biological activities. There exists a competition between the halogen bond and hydrogen bond in singly hydrated halogenated coumarins. The competition between hydrogen and halogen bonding interactions in 3-halogenated 4-hydroxyl coumarin [coumarin derivative; halogen, X = F (A), Cl (B), Br (C), I (D)] with water molecule in the corresponding binding regions C3–X and C2=O4 is studied. This study was conducted using PBE0 D3BJ with augmented correlation consistent basis set in order to include the diffuse functions. Improved findings for non-bonded distances and much more distinct intramolecular effects were obtained using BJ-damping. In the singly-hydrated systems, the water molecule forms a hydrogen bond with C2=O4 in all the halogenated molecules, whereas halogen bonding between the water oxygen and C2–X exists only in the case of X = Br (C) and I (D). The absence of a halogen-bonded structure in singly-hydrated chlorine and fluorine substituted coumarin derivative is therefore attributed to the competing hydrogen-bonding interaction with C2=O4. RDG scatter plot as well QTAIM analysis implied that halogen bond exists between water molecule and the coumarin derivative. Further, the most modern local energy decomposition (LED) analysis of intermolecular interaction was also studied using DLPNO-CCSD(T). Finally, ab initio molecular dynamics was also performed.
Los servicios digitales foráneos consumidos por personas naturales y el Impuesto General a las Ventas
Authors: Bravo Padilla Jorge Andres 
Published: February 2024

Objetivo: Fue determinar si los servicios digitales foráneos consumidos por personas naturales y el Impuesto General a las Ventas (IGV) están relacionados, y establecer si la legislación peruana del mencionado impuesto grava dichos servicios. Métodos: De tipo correlacional y enfoque cuantitativo-cualitativo; las variables de investigación son: Los servicios digitales foráneos consumidos por personas naturales y el IGV; la hipótesis de investigación es: Existe una relación significativa entre los servicios digitales foráneos consumidos por personas naturales y el IGV. Resultados: Se resuelve rechazar la hipótesis de investigación; y de la revisión documental a la legislación del IGV se resuelve que dicho impuesto sí grava los mencionados servicios, a pesar de que no los incluye expresamente ni establece un mecanismo para su recaudación. Conclusiones: Los servicios digitales foráneos consumidos por personas naturales y el IGV no están relacionados; y la legislación peruana del IGV debe actualizarse para que las empresas prestadoras de los mencionados servicios se inscriban ante la Superintendencia Nacional de Aduanas y de Administración Tributaria (SUNAT) para que sean sujetos del impuesto y agentes de retención, caso contrario, los sujetos del impuesto serán los consumidores de dichos servicios y los agentes de retención serán las empresas financieras por medio de las cuales se realiza el pago.
Marketing relacional y propuesta de valor al cliente. Análisis y reflexión teórica
Authors: Choque Lozano Guido Jesús 
Published: February 2024

El marketing relacional y la propuesta de valor son componentes esenciales en la gestión de relaciones con los clientes y son fundamentales para establecer vínculos de fidelización a largo plazo. En un mercado cada vez más competitivo, las empresas buscan maneras de destacar y obtener ventajas competitivas. En este sentido, el presente artículo tuvo como objetivo conocer en base a un análisis y reflexión teórica, si el marketing relacional y una idónea propuesta de valor contribuyen a tener relaciones redituables con los clientes a largo plazo. El estudio se basó en la selección de 26 referencias utilizando la metodología de revisión sistemática PRISMA. Luego de realizar búsquedas en bases de datos como Scopus, WOS y Scielo, se definieron criterios de inclusión y exclusión, lo que condujo a la identificación de 13 estudios más relevantes realizados entre los años 2016 a 2021 para su posterior análisis, interpretación de resultados y discusión. Los hallazgos resaltan la importancia de las estrategias de marketing enfocadas en el cliente y el impacto positivo de una propuesta de valor adecuada en la construcción de relaciones duraderas. Se concluye que una gestión efectiva del marketing relacional y de la propuesta de valor tiene un efecto favorable en la retención y la toma de decisiones de compra por parte del cliente.
Kaunas Tatar Community and Organization in 1918–1940: Structure and Work
Authors: Aleksandravičiūtė Ina 
Published: February 2024

After World War I Tatars in Lithuania had to reorganize and institutionalize their parishes. Though Kaunas Tatars didn’t accomplish much in spheres of culture and education, they managed to build a new mosque in Kaunas this way leaving their mark. The main goal of Kaunas Tatar community was creating religious central institution. In 1925 in Vilnius polish Tatar community established institution of Mufti. Kaunas Tatars tried to establish similar organizations thorough all interwar period. Sadly, the conflicts between Muslim parishes in Lithuania constantly stopped this process. Kaunas Tatar Association tried to become dominating institution in Lithuanian Muslim community. This initiative was supported by Lithuanian Government but Tatars of Raižiai and Vinkšnupiai parishes wanted to function independently.