Targeted nanoliposomes to improve enzyme replacement therapy of Fabry disease.

The central nervous system represents a major target tissue for therapeutic approach of numerous lysosomal storage disorders. Fabry disease arises from the lack or dysfunction of the lysosomal alpha-galactosidase A (GLA) enzyme, resulting in substrate accumulation and multisystemic clinical manifestations. Current enzyme replacement therapies (ERTs) face limited effectiveness due to poor enzyme biodistribution in target tissues and inability to reach the brain.

Effects of left ventrolateral prefrontal stimulation on forming and maintaining deep and shallow episodic traces.

The levels-of-processing framework, proposing that deep encoding enhances retention, plays a crucial role in episodic memory research. Neuroimaging evidence highlights that increased activity of the left ventrolateral prefrontal cortex during deep encoding predicts subsequent memory success. However, cognitive mechanisms underlying this region's involvement in establishing and consolidating deep and shallow traces remain unclear.

Total Synthesis and Structural Reassignment of the Molt-Inhibiting Marine Alkaloid Erebusinone.

The marine alkaloid erebusinone is a secondary metabolite isolated from the Antarctic sponge . Initial biological assays have shown that erebusinone increases amphipod mortality, probably by inhibition of the biosynthesis of molting hormone (ecdysone). Herein, we report the first total synthesis of the proposed structure of erebusinone and a structural revision.

Semisynthetic Studies Establish a Role for Conjugate Halide Exchange in the Formation of Chlorinated Pyrroloiminoquinones and Related Alkaloids.

Two novel pyrroloiminoquinone alkaloids, 6-chlorodamirone A and 6-bromodamirone A, have been identified for the first time from the marine sponge sp. (order: Poecilosclerida: family Latrunculiidae), sourced from Western Australia. Alongside these new compounds, seven previously known metabolites were also isolated.

Did H.M. exhibit accelerated long-term forgetting? Measuring forgetting in amnesia.

The early investigations of patient H.M. inaugurated the modern era of memory research.

Principles and regulation of mechanosensing.

Research over the past two decades has highlighted that mechanical signaling is a crucial component in regulating biological processes. Although many processes and proteins are termed 'mechanosensitive', the underlying mechanisms involved in mechanosensing can vary greatly. Recent studies have also identified mechanosensing behaviors that can be regulated independently of applied force.

Proper Frequency of Perinatal Retinal Waves Is Essential for the Precise Wiring of Visual Axons in Nonimage-Forming Nuclei.

The development of the visual system is a complex and multistep process characterized by the precise wiring of retinal ganglion cell (RGC) axon terminals with their corresponding neurons in the visual nuclei of the brain. Upon reaching primary image-forming nuclei (IFN), such as the superior colliculus and the lateral geniculate nucleus, RGC axons undergo extensive arborization that refines over the first few postnatal weeks. The molecular mechanisms driving this activity-dependent remodeling process, which is influenced by waves of spontaneous activity in the developing retina, are still not well understood.

Prenatal origin of NUTM1 gene rearrangement in infant B-cell precursor acute lymphoblastic leukaemia.

Rearrangement of NUTM1 gene (NUTM1r) is one of the most frequent aberrations occurring in infants (younger than 1 year at diagnosis) with B-cell precursor Acute Lymphoblastic Leukaemia (BCP-ALL). In this study we had the unique opportunity to analyze the umbilical cord blood (UCB) sample from one infant patient with NUTM1r BCP-ALL. Herein we reported for the first time that NUTM1r infant ALL arise prenatally, as both the patient-specific CUX1::NUTM1 fusion gene, as well as two IG/TR leukaemic markers were already present and detectable in the patient's UCB at birth.

Zinc speciation in fly ash from MSWI using XAS - novel insights and implications.

The chemical forms of zinc in fly ash from municipal solid waste incineration (MSWI) crucially affect ash management, influencing both material recovery options and the risk of unwanted leaching into ecosystems. The zinc speciation was investigated in fly ash samples sourced from full-scale MSWI plants, including four grate fired boilers (GB) and one fluidized bed boiler (FB). We applied X-ray Absorption Spectroscopy (XAS), and the spectra were analyzed against a unique library of over 30 relevant compounds, tailored to the nuances of zinc chemistry of fly ash.

Distinctive clinical traits of lupus-related myocarditis: a multicentre retrospective study.

Objectives: Cardiovascular involvement in systemic lupus erythematosus (SLE) is frequent but little is known about possible distinctive traits of SLE-related myocarditis (myoSLE) in comparison to patients with SLE (onlySLE) or myocarditis alone (onlyMyo).

Methods: A retrospective analysis was performed comparing patients with myoSLE (n = 25) from three centres with consecutive patients with onlySLE (n = 279) and onlyMyo (n = 88). SLE patients were dichotomised by disease duration ≤1 vs >1 year into recent onlySLE/early myoSLE vs longstanding onlySLE/late myoSLE.

Non-muscle myosin 2 can incorporate into established filaments in cells without an assembly competence domain.

Myosin 2 dynamically assembles into filaments that exert force on the actin cytoskeleton. To form filaments, myosin 2 monomers transition between folded and unfolded states. Monomer unfolding exposes an extended coiled-coil that interacts with other monomers in parallel and antiparallel fashions, enabling bipolar filament formation.

Influence of degree of learning on rate of forgetting of tonal sequences.

Initial performance is frequently equated in studies that compare forgetting rates across groups. However, since the encoding capacity of different groups can be different, some procedures to match initial degree of learning need to be implemented, adding confounding variables such as longer exposures to the material, which would create memories of a different age. Slamecka and McElree Journal of Experimental Psychology: Learning, Memory, and Cognition, 9, 384-397, (1983) and our previous work found that the rate of forgetting was independent from initial degree of learning using verbal material.

Effect of levels-of-processing on rates of forgetting.

The levels-of-processing (LOP) framework, proposing that deep processing yields superior retention, has provided an important paradigm for memory research and a practical means of improving learning. However, the available levels-of-processing literature focuses on immediate memory performance. It is assumed within the LOP framework that deep processing will lead to slower forgetting than will shallow processing.

The ion channel TRPM8 is a direct target of the immunosuppressant rapamycin in primary sensory neurons.

Background And Purpose: The mechanistic target of rapamycin (mTOR) signalling pathway is a key regulator of cell growth and metabolism. Its deregulation is implicated in several diseases. The macrolide rapamycin, a specific inhibitor of mTOR, has immunosuppressive, anti-inflammatory and antiproliferative properties.

Endomyocardial biopsy: safety and prognostic utility in paediatric and adult myocarditis in the European Society of Cardiology EURObservational Research Programme Cardiomyopathy and Myocarditis Long-Term Registry.

Background And Aims: Contemporary multicentre data on clinical and diagnostic spectrum and outcome in myocarditis are limited. Study aims were to describe baseline features, 1-year follow-up, and baseline predictors of outcome in clinically suspected or biopsy-proven myocarditis (2013 European Society of Cardiology criteria) in adult and paediatric patients from the EURObservational Research Programme Cardiomyopathy and Myocarditis Long-Term Registry.

Methods: Five hundred eighty-one (68.

Evaluating the Environmental Sustainability of Alternative Ways to Produce Benzene, Toluene, and Xylene.

The petrochemical industry can reduce its environmental impacts by moving from fossil resources to alternative carbon feedstocks. Biomass and plastic waste-based production pathways have recently been developed for benzene, toluene, and xylene (BTX). This study evaluates the environmental impacts of these novel BTX pathways at a commercial and future (2050) scale, combining traditional life cycle assessment with absolute environmental sustainability assessment using the planetary boundary concept.

Pentraxin 3 in Myocarditis: Proof-of-Principle Assessment as a Diagnostic and Prognostic Biomarker.

Pentraxin 3 (PTX3) is an acute phase protein produced in various tissues in response to microbial and sterile stimuli, which regulates the inflammation outcomes. PTX3 has not been investigated in myocarditis. Our aim was to assess circulating and cardiac tissue expression of PTX3 in 55 patients with myocarditis proven by magnetic resonance and/or endomyocardial biopsy.

Life cycle thinking for the assessment of waste and circular economy policy: status and perspectives from the EU example.

Life cycle thinking methods such as life cycle assessment (LCA) and costing (LCC) were originally developed to assess the performance of products and services (business-making decisions). However, they are increasingly deployed to support policy-making along the entire policy cycle, including via impact assessment (IA) of different policy options. These applications are associated with a number of challenges, mainly related to the dynamic and prospective nature of policy IA, typically forward-looking into 10-20 years ahead.

Characterization and Future Distribution Prospects of "" Landrace for Its In Situ Conservation.

"Carciofo di Malegno" is a little-known landrace of subsp. cultivated in Camonica Valley (northern Italy). The morphological and phytochemical characteristics of this landrace were investigated; furthermore, a species distribution model (MaxEnt algorithm) was used to explore its ecological niche and the geographical area where it could be grown in the future.