Expanding the clinical and immunological phenotypes of PAX1-deficient SCID and CID patients.

Paired box 1 (PAX1) deficiency has been reported in a small number of patients diagnosed with otofaciocervical syndrome type 2 (OFCS2). We described six new patients who demonstrated variable clinical penetrance. Reduced transcriptional activity of pathogenic variants confirmed partial or complete PAX1 deficiency.

Integrated assessment of eutrophication in the southern Black Sea waters, using the Nested Environmental Status Assessment Tool.

This research deals with the land-based pressures on the southern Black Sea coast (Turkey), with the objective of assessing their eutrophication impacts. In this context, reference values of some of the eutrophication indicators were calculated, and eutrophication assessment was carried out for this area using the holistic approach NEAT (Nested Environmental Status Assessment Tool). In addition, correlations between NEAT results of coastal regions and pressure-impact analysis methods were investigated.

Quantum-Classical Protocol for Efficient Characterization of Absorption Lineshape and Fluorescence Quenching upon Aggregation: The Case of Zinc Phthalocyanine Dyes.

A quantum-classical protocol that incorporates Jahn-Teller vibronic coupling effects and cluster analysis of molecular dynamics simulations is reported, providing a tool for simulations of absorption spectra and ultrafast nonadiabatic dynamics in large molecular photosystems undergoing aggregation in solution. Employing zinc phthalocyanine dyes as target systems, we demonstrated that the proposed protocol provided fundamental information on vibronic, electronic couplings and thermal dynamical effects that mostly contribute to the absorption spectra lineshape and the fluorescence quenching processes upon dye aggregation. Decomposing the various effects arising upon dimer formation, the structure-property relations associated with their optical responses have been deciphered at atomistic resolution.

Mesenchymal stem cells differentiate to retinal ganglion-like cells in rat glaucoma model induced by polystyrene microspheres.

Aim: The study aimed to evaluate the differentiation ability of intravitreally injected rat bone marrow-derived mesenchymal stem cells (rBM-MSCs) to retinal ganglion-like cells in a polystyrene microsphere induced rat glaucoma model.

Materials And Methods: The glaucoma rat model was generated via intracameral injection of 7 microliter polystyrene microspheres. Green fluorescence protein-labeled (GFP) rBM-MSCs were transplanted intravitreally at or after induction of ocular hypertension (OHT), depending on the groups.

Therapeutic modalities and clinical outcomes in a large cohort with LRBA deficiency and CTLA4 insufficiency.

Background: LPS-responsive beige-like anchor (LRBA) deficiency (LRBA) and cytotoxic T-lymphocyte-associated antigen-4 (CTLA4) insufficiency (CTLA4) are mechanistically overlapped diseases presenting with recurrent infections and autoimmunity. The effectiveness of different treatment regimens remains unknown.

Objective: Our aim was to determine the comparative efficacy and long-term outcome of therapy with immunosuppressants, CTLA4-immunoglobulin (abatacept), and hematopoietic stem cell transplantation (HSCT) in a single-country multicenter cohort of 98 patients with a 5-year median follow-up.

Therapeutic Effect of C-Vx Substance in K18-hACE2 Transgenic Mice Infected with SARS-CoV-2.

C-Vx is a bioprotective product designed to boost the immune system. This study aimed to determine the antiviral activity of the C-Vx substance against SARS-CoV-2 infection. The effect of C-Vx in K18-hACE2 transgenic mice against the SARS-CoV-2 virus was investigated.

Cryogenic X-ray crystallographic studies of biomacromolecules at Turkish Light Source "".

X-ray crystallography is a robust and powerful structural biology technique that provides high-resolution atomic structures of biomacromolecules. Scientists use this technique to unravel mechanistic and structural details of biological macromolecules (e.g.

Recent Developments in CRISPR/Cas9 Genome-Editing Technology Related to Plant Disease Resistance and Abiotic Stress Tolerance.

The revolutionary CRISPR/Cas9 genome-editing technology has emerged as a powerful tool for plant improvement, offering unprecedented precision and efficiency in making targeted gene modifications. This powerful and practical approach to genome editing offers tremendous opportunities for crop improvement, surpassing the capabilities of conventional breeding techniques. This article provides an overview of recent advancements and challenges associated with the application of CRISPR/Cas9 in plant improvement.

Prolinamides containing 2-(2-aminocyclohexyl)phenols as highly enantioselective organocatalysts for aldol reactions.

Effective synthesis of prolinamides of 2-(2-aminocyclohexyl)phenols has been accomplished. The novel prolinamides are demonstrated to catalyze the direct aldol reaction between ketones and aldehydes with high stereoselectivity, thus affording up to 99 : 1 / diastereomeric and 99 : 1 enantiomeric ratio. Experimental results as well as computational investigations have revealed that the electrophile ( aldehyde) is activated by dual hydrogen bonding with the amide NH and phenolic OH group of the catalyst.

Metabarcoding of colonic cleansing fluid reveals unique bacterial members of mucosal microbiota associated with Inflammatory Bowel Disease.

Background: Inflammatory Bowel Disease (IBD) is a group of chronic idiopathic inflammatory diseases of the gastrointestinal (GI) tract associated with the dysbiosis of gut microbiota. Metabarcoding-based profiling of the gut microbiota of IBD patients is generally based on the stool samples collected from individual patients which rarely represent the mucosa-associated microbiota. The ideal sampling strategy for routine monitoring of the mucosal component of IBD has yet to be determined.

Investigation of simultaneous effects of noise barriers on near-road noise and air pollutants.

Noise barriers are one of the common solutions to control road traffic noise. Many studies have also shown that noise barriers cause reductions in near-road air pollutant concentrations. In this study, the simultaneous effects of a specific noise barrier application on near-road noise and air pollution at a specific location were investigated.

Electrochemical aptasensors for pathogenic detection toward point-of-care diagnostics.

A biosensor system refers to a biomedical device, which detects biological, chemical, or biochemical components by converting those signals to an electrical signal by utilizing and uniting physical or chemical transducer with biorecognition elements. An electrochemical biosensor is generally based on the reaction of either production or consumption of electrons under a three-electrode system. Biosensor systems are exploited in a wide range of areas, such as medicine, agriculture, husbandry, food, industry, environment protection, quality control, waste disposal, and the military.

Rapid and efficient ambient temperature X-ray crystal structure determination at Turkish Light Source.

High-resolution biomacromolecular structure determination is essential to better understand protein function and dynamics. Serial crystallography is an emerging structural biology technique which has fundamental limitations due to either sample volume requirements or immediate access to the competitive X-ray beamtime. Obtaining a high volume of well-diffracting, sufficient-size crystals while mitigating radiation damage remains a critical bottleneck of serial crystallography.

Investigation of the Antiviral Effects of Saussurea lappa Root Extract against SARS-CoV-2 Virus.

Scope: The purpose of this study was to look into the antiviral activity of a plant extract derived from the roots of the Saussurea lappa as a food supplement against SARS-CoV-2 infection.

Methods And Results: Vero E6 cells are employed in the study to test the neutralizing effect of Saussurea lappa extract against the SARS-CoV-2 virus. For anti-viral activity detection, a sensitive real-time cell analyzer (xCELLigence RTCA) with a high repetition rate is used.

Chewable tablet with herbal extracts and propolis arrests Wuhan and Omicron variants of SARS-CoV-2 virus.

Prevention of COVID-19 is of paramount importance for public health. Some natural extracts might have the potential to suppress COVID-19 infection. Therefore, this study aimed to design a standardised, efficient, and safe chewable tablet formulation (with propolis and three herbal extracts) for possible prevention against two variants (Wuhan B.

Silk-derived nitrogen-doped porous carbon electrodes with enhanced ionic conductivity for high-performance supercapacitors.

Supercapacitors are attracting extensive attention in energy storage fields thanks to their high safety, cost-effectiveness, and environmental friendliness. The carbon materials, especially for the porous carbon materials derived from renewable biomass materials, are important electrode materials with cost-effective feature for supercapacitors. However, the inferior ionic conductivity of biomass materials inhibits their electrochemical performance in energy storage devices.

Dried Fruits: Bioactives, Effects on Gut Microbiota, and Possible Health Benefits-An Update.

Dried fruits contain many bioactive compounds broadly classified as phytochemicals including phenolics, flavonoids, carotenoids, proanthocyanidins, stilbenes, chalcones/dihydrochalcones, and phytoestrogens. These compounds have antioxidant effects that may benefit health. Dried fruits are also a diverse group of foods with varying fibre contents.

The impact of livestock activities and geochemical processes on groundwater quality of fractured volcanic rock aquifer: Lake Çıldır watershed (NE Turkey).

This paper presents the impact of livestock activities and geochemical processes on the water quality of a fractured volcanic rock aquifer in the Lake Çıldır watershed, located at the northeastern part of Turkey. The existence of a high livestock population and animal grazing activities in meadow and pasturelands of the watershed during the short summer period poses serious stress on both surface and groundwater resources being the only drinking water supply for the local communities. Therefore, understanding the effect of grazing and livestock breeding activities occurring in the recharge areas of the fractured volcanic rock aquifer is vital to take precautions in order to protect limited water supplies at the watershed and vulnerable lake ecosystem as well.

One-Pot Synthesis of Melamine Formaldehyde Resin-Derived -Doped Porous Carbon for CO Capture Application.

The design and synthesis of porous carbons for CO adsorption have attracted tremendous interest owing to the ever-soaring concerns regarding climate change and global warming. Herein, for the first time, nitrogen-rich porous carbon was prepared with chemical activation (KOH) of commercial melamine formaldehyde resin (MF) in a single step. It has been shown that the porosity parameters of the as-prepared carbons were successfully tuned by controlling the activating temperature and adjusting the amount of KOH.

Facile synthesis of NiTe-CoTe@rGO nanocomposite for high-performance hybrid supercapacitor.

The design of bimetallic tellurides that exhibit excellent electrochemical properties remains a huge challenge for high-performance supercapacitors. In the present study, tellurium is consolidated on CoNi@rGO for the first time, to synthesize NiTe-CoTe@rGO nanocomposite by using a facile hydrothermal method. As-prepared NiTe-CoTe@rGO nanocomposite was characterized by EDS, TEM, FESEM, Raman, BET, XRD, and XPS techniques to prove the structural transformation.

Fabrication of a Dual-Drug-Loaded Smart Niosome-g-Chitosan Polymeric Platform for Lung Cancer Treatment.

Changes in weather conditions and lifestyle lead to an annual increase in the amount of lung cancer, and therefore it is one of the three most common types of cancer, making it important to find an appropriate treatment method. This research aims to introduce a new smart nano-drug delivery system with antibacterial and anticancer capabilities that could be applied for the treatment of lung cancer. It is composed of a niosomal carrier containing curcumin as an anticancer drug and is coated with a chitosan polymeric shell, alongside Rose Bengal (RB) as a photosensitizer with an antibacterial feature.

A comprehensive assessment, source input determination and distribution of persistent organic pollutants (POPs) along with heavy metals (HMs) in reservoir lake sediments from Çanakkale province, Türkiye.

This study presents the distribution of persistent organic pollutants (POPs), polycyclic aromatic hydrocarbons (PAHs) and heavy metals (HMs) in the sediments from four dams and two ponds in the Çanakkale city, Türkiye. The total concentration varied from 41.47 to 145.