Argentine program of home infusions with enzyme replacement therapy for lysosomal diseases: Results in safety, quality of life and adherence.

With over 25 years of experience, enzyme replacement therapies have proven to be safe and effective for patients with Fabry, Gaucher, Pompe, Mucopolysaccharidosis I (MPS I) diseases. As with many chronic conditions, one of the keys to achieving therapeutic goals is to avoid interruptions in infusions to ensure proper adherence (AD). Treatment interruptions are associated with the recurrence of previously resolved clinical manifestations depending on the length of the interruption, and the reaccumulation of substrate at the cellular level.

Structural and Functional Glycosylation of the Abdala COVID-19 Vaccine.

Abdala is a COVID-19 vaccine produced in Pichia pastoris and is based on the receptor-binding domain (RBD) of the SARS-CoV-2 spike. Abdala is currently approved for use in multiple countries with clinical trials confirming its safety and efficacy in preventing severe illness and death. Although P.

Revalorisation of brewer's spent grain for biotechnological production of hydrogen with .

Introduction: Agro-industrial wastes are generated in huge amounts triggering damages to the environment and human health. Therefore, there is an urgent necessity for its revalorisation into high-value compounds, including biofuels. One such wastes is the brewer's spent grain (BSG), a by-product of the beer industry, which is produced in vast quantities worldwide.

Control of myeloid-derived suppressor cell dynamics potentiates vaccine protection in multiple mouse models of infection.

To date, there is no licensed vaccine against the protozoan parasite (, the etiological agent of Chagas Disease. has evolved numerous mechanisms to evade and manipulate the host immune system. Among the subversive strategies employed by the parasite, marked increases in CD11b+ Gr-1+ myeloid-derived suppressor cells (MDSCs) in several organs have been described.

Liquid Organic Hydrogen Carrier Concepts and Catalysts for Hydrogenation and Dehydrogenation Reactions.

Background: The issue of renewable energy (RE) source intermittency, such as wind and solar, along with the geographically uneven distribution of the global RE potential, makes it imperative to establish an energy transport medium to balance the energy demand and supply areas. A promising energy vector to address this situation is hydrogen, which is considered a clean energy carrier for various mobile and portable applications. Unfortunately, at standard pressure and temperature, its energy content per volume is very low (0.

Bee Venom Reduces Early Inflammation and Oxidative Stress Associated with Lipopolysaccharide-induced Alpha-synuclein in the Substantia Nigra-striatum Axis.

Neuroinflammation and oxidative stress are important features in the pathogenesis and development of synucleinopathies, the glial activation and upregulation of pro-inflammatory and oxidative mediators induce alpha-synuclein (α-syn) accumulation. Recent studies have shown that bee venom (BV) has beneficial effects on symptoms of these neurodegenerative diseases. BV is known to exert anti-inflammatory and anti-oxidative effects.

Multiple-Point Metamaterial-Inspired Microwave Sensors for Early-Stage Brain Tumor Diagnosis.

Simple, instantaneous, contactless, multiple-point metamaterial-inspired microwave sensors, composed of multi-band, low-profile metamaterial-inspired antennas, were developed to detect and identify meningioma tumors, the most common primary brain tumors. Based on a typical meningioma tumor size of 5-20 mm, a higher operating frequency, where the wavelength is similar or smaller than the tumor target, is crucial. The sensors, designed for the microwave Ku band range (12-18 GHz), where the electromagnetic property values of tumors are available, were implemented in this study.

Chelator boosted tumor-retention and pharmacokinetic properties: development of Cu labeled radiopharmaceuticals targeting neurotensin receptor.

Purpose: Accumulating evidence suggests that neurotensin (NTS) and neurotensin receptors (NTSRs) play key roles in lung cancer progression by triggering multiple oncogenic signaling pathways. This study aims to develop Cu-labeled neurotensin receptor 1 (NTSR1)-targeting agents with the potential for both imaging and therapeutic applications.

Method: A series of neurotensin receptor antagonists (NRAs) with variable propylamine (PA) linker length and different chelators were synthesized, including [Cu]Cu-CB-TE2A-iPA-NRA ([Cu]Cu-4a-c, i = 1, 2, 3), [Cu]Cu-NOTA-2PA-NRA ([Cu]Cu-4d), [Cu]Cu-DOTA-2PA-NRA ([Cu]Cu-4e, also known as [Cu]Cu-3BP-227), and [Cu]Cu-DOTA-VS-2PA-NRA ([Cu]Cu-4f).

Sensitivity of Daphnia spinulata Birabén, 1917 to glyphosate at different salinity levels.

Daphnia spinulata Birabén, 1917 is an endemic cladoceran species, frequent in the zooplankton communities of the shallow lakes of the Pampean region of Argentina. These lakes have varying salinity levels and, being located in agricultural areas, are frequently subject to pesticide pollution. This study aimed to determine the effects of the herbicide glyphosate (Panzer Gold®) in combination with different salinity levels on the biological parameters of D.

Argentinian Rose Petals as a Source of Antioxidant and Antimicrobial Compounds.

The total phenolic, flavonoid, and anthocyanin contents were evaluated in 11 cultivars of Argentinian roses of different colors. HPLC-ESI-QTOF/MS was used to identify the components where ellagic and quinic acids, quercetin, and kaempferol glycosylated derivatives were found. The phenolic contents ranged from 78.

Simple protocol for measuring CD11b+ GR-1+ (Ly6C+/Ly6G+) myeloid cells from a minimum volume of mouse peripheral blood.

Myeloid-derived suppressor cells (MDSCs) comprise a heterogeneous population of myeloid origin and immature state, whose hallmark is the capacity to suppress T cells and other immune populations. In mice, the first approach to identify MDSCs relies in the measurement of their phenotypical markers: CD11b and GR-1. In addition, two main subtypes of MDSCs have been defined based on the expression of the following markers: CD11b+ Ly6G- Ly6C+ (monocytic-MDSCs, M-MDSCs) and CD11b+ Ly6G+ Ly6C (polymorphonuclear-MDSCs, PMN-MDSCs).

Calcium complexation by steroids involved in the steroidogenesis.

Steroids that take part in the pathways of human steroidogenesis are involved in many biological mechanisms where they interact with calcium. In the present work, the binding selectivities and affinities for calcium of progestagens, mineralocorticoids, androstagens, and estrogens were studied by Electrospray Ionization-Mass Spectrometry (ESI-MS). The adduct profile of each steroid was characterized by high resolution and tandem mass spectrometry.

Montmorillonite Exfoliation in LLDPE and Factors Affecting Its Orientation: From Monolayer to Multi-Nano-Layer Polymer Films.

The motivations of the present work are to investigate the exfoliation of montmorillonite within a linear low-density polyethylene matrix and to control its orientation during the cast extrusion process. The first part is focused on the exfoliation of the montmorillonite through the melt extrusion process. The accuracy and relevance of each method used to determine the exfoliation state of montmorillonite have been examined, thanks to X-ray diffraction, transmission electronic microscopy, and rheology.

Host macrocyclic acylcarnitines mediate symbiotic interactions between frogs and their skin microbiome.

The host-microbiome associations occurring on the skin of vertebrates significantly influence hosts' health. However, the factors mediating their interactions remain largely unknown. Herein, we used integrated technical and ecological frameworks to investigate the skin metabolites sustaining a beneficial symbiosis between tree frogs and bacteria.

Optimization of the 2-arylquinazoline-4(3)one scaffold for a selective and potent antitrypanosomal agent: modulation of the mechanism of action through chemical functionalization.

We sought to identify a potent and selective antitrypanosomal agent through modulation of the mechanism of action of a 2-arylquinazoline scaffold as an antitrypanosomal agent chemical functionalization at the 4-position. We wished to use the: (i) susceptibility of trypanosomatids towards nitric oxide (NO) and reactive oxygen species (ROS); (ii) capacity of the 4-substituted quinazoline system to act as an antifolate agent. Three quinazolin-based moieties that differed from each other by having at the 4-position key pharmacophores targeting the induction of NO and ROS production were evaluated against and parasites and their modes of action were explored.

A Novel NOX Inhibitor Alleviates Parkinson's Disease Pathology in PFF-Injected Mice.

Oxidative stress-mediated damage is often a downstream result of Parkinson's disease (PD), which is marked by sharp decline in dopaminergic neurons within the nigrostriatal regions of the brain, accounting for the symptomatic motor deficits in patients. Regulating the level of oxidative stress may present a beneficial approach in preventing PD pathology. Here, we assessed the efficacy of a nicotinamide adenine phosphate (NADPH) oxidase (NOX) inhibitor, an exogenous reactive oxygen species (ROS) regulator synthesized by Aptabio therapeutics with the specificity to NOX-1, 2 and 4.

Artificial microRNA suppresses C9ORF72 variants and decreases toxic dipeptide repeat proteins in vivo.

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease that affects motor neurons, causing progressive muscle weakness and respiratory failure. The presence of an expanded hexanucleotide repeat in chromosome 9 open reading frame 72 (C9ORF72) is the most frequent mutation causing familial ALS and frontotemporal dementia (FTD). To determine if suppressing expression of C9ORF72 gene products can reduce toxicity, we designed a set of artificial microRNAs (amiRNA) targeting the human C9ORF72 gene.

Tautomerism and Rotamerism of Favipiravir and Halogenated Analogues in Solution and in the Solid State.

Favipiravir is an important selective antiviral against RNA-based viruses, and currently, it is being repurposed as a potential drug for the treatment of COVID-19. This type of chemical system presents different carboxamide-rotameric and hydroxyl-tautomeric states, which could be essential for interpreting its selective antiviral activity. Herein, the tautomeric 3-hydroxypyrazine/3-pyrazinone pair of favipiravir and its 6-substituted analogues, 6-Cl, 6-Br, 6-I, and 6-H, were fully investigated in solution and in the solid state through ultraviolet-visible, H nuclear magnetic resonance, infrared spectroscopy, and X-ray diffraction techniques.

Zoonotic Cryptosporidium meleagridis in urban invasive monk parakeets.

Zoonotic Cryptosporidium meleagridis was identified in invasive monk parakeets (Myiopsitta monachus) from Santiago metropolis in Chile. Oocysts were isolated from nestlings' faeces, and a fragment of 18S ribosomal RNA gene was amplified and sequenced. This finding emphasizes the importance of permanent pathogen monitoring in synanthropic species with wide global distribution.

Exploring binding chemistry of alkali/alkaline earth cations in solution through modulation of intramolecular charge-transfer in an excited ambidentate organic fluorophore.

Studying the metal-ligand monoligation of alkali/alkaline earth metals (AMs) in solution represents a significant challenge due to the low stabilization of their complexes and the absence of an effective strategy to identify this type of weak binding. Herein, we show that the modulation of the intramolecular charge-transfer (ICT) in an excited ambidentate organic fluorophore is a convenient strategy to characterize the binding chemistry of AM cations in solution through simple steady-state fluorescence and fluorescence lifetime measurements. The key points of the fluorophore as a metal-binding probe were the location of diverse coordination functionalities with different binding abilities (ionic-, pseudo-covalent- and non-covalent-probes) along the donor-acceptor (D-A) chain and the occurrence of an intramolecular charge-transfer (ICT) mechanism upon excitation.

Effect of the Drying Method and Optimization of Extraction on Antioxidant Activity and Phenolic of Rose Petals.

The effect of freeze and hot air drying methods on the retention of total phenolics, antioxidant activity (AA), and color of different cultivars of rose petals was analyzed. Both methods similarly preserved the phenolic content and AA, while freeze drying showed better red color retention. Furthermore, the conditions of total phenolics and AA extraction from two rose cultivars, and , were optimized by response surface methodology through a Box-Behnken design.