The ongoing COVID-19 pandemic, caused by SARS-CoV-2, continues to pose significant global health challenges. The results demonstrated that GB-2 at 200 μg/mL effectively increased the population of 293T-ACE2 cells with low RBD binding for both SARS...
Invasive neonatal GBS infections constitute a major cause of sepsis and meningitis in Western countries. Vaginal/rectal GBS colonization during pregnancy is the main risk factor for the development of early-onset infections (GBS-EOD) in newborn by ve...
(1) Background: We aimed to explore the feasibility of collecting intrapartum maternal Group B Streptococcus (GBS) colonization and immediate post-birth neonatal GBS colonization cultures for use in a larger trial and to identify cases of residual GB...
Cotton is an economically critical crop worldwide, and drought stress strongly affects its growth and development. Ubiquitination modifies protein activity and is crucial in numerous biological processes. Ubiquitin-conjugating enzymes serve as interm...
Shiga toxin (Stx) is released by enterohemorrhagic Escherichia coli (EHEC) into the human intestinal lumen and transferred across the colon epithelium to the circulation. Stx-mediated damage of human kidney and brain endothelial cells and renal epith...
In this paper, we present an area-efficient noise-optimized programmable 4 × 25-to-28.9 Gb/s optical receiver. Both high- and low-power modes are available for the receiver to meet different requirements. Emitter degeneration provides the input...
Fabry disease, the second most common lysosomal storage disorder, is caused by a deficiency of α-galactosidase A (α-Gal A), which leads to an accumulation of glycosphingolipids (GSL), mainly globotriaosylceramide (also known as Gb3). This...
We have shown previously that neurons in the mouse spinal cord express Gb3. We show in this article that distribution of anti-Gb3-Ab reactivity occurs in many different types of neurons of different areas of the central nervous system (CNS). The immu...
Glucosylsphingosine (lyso-Gb1) serves as a biomarker for evaluating disease activity in Gaucher disease (GD). While treatment-related changes are documented, the dynamics of lyso-Gb1 during untreated states remain poorly understood. This retrospectiv...
Lysosomal storage diseases are caused by defective lysosomal function, such as impaired lysosomal enzyme activities, which include more than 70 different diseases. Although biomarkers and therapies have been developed to date for some of them, many o...
Background/Objectives: Gaucher disease (GD) is a lysosomal disorder caused by a deficiency of β-glucosidase. Disease-modifying therapies (DMTs) include enzyme replacement therapy (ERT) and substrate reduction therapy (SRT). Glucosylsphingosine (...
Fabry disease is a lysosomal storage disease with an X-linked heritage caused by absent or decreased activity of lysosomal enzymes named alpha-galactosidase A (α-gal A). Among the various manifestations of Fabry disease, Fabry nephropathy signi...
Blood vessels in vertebrates are established and genetically controlled in an evolutionarily-conserved manner during embryogenesis. Disruption of vascular growth by chemical compounds or environmental hormones may cause developmental defects. This st...
Glucosylsphingosine (lyso-Gb1), the deacylated form of glucocerebroside, was shown to be the most specific and sensitive biomarker for diagnosing Gaucher disease (GD). The aim of this study is to assess the contribution of lyso-Gb1 at the time of dia...
Gaucher disease (GD) is a lysosomal storage disorder caused by mutations in the glucocerebrosidase gene (GBA1), leading to acid β-glucosidase deficiency and the accumulation of glucosylceramide-derived glycosphingolipids. Its three phenotypes (n...
Glycosphingolipids are known to play a role in developing and maintaining the integrity of various organs and tissues. Among glycosphingolipids, there are several reports on the involvement of gangliosides in bone metabolism. However, there have been...
The role of glucosylsphingosine (lyso-Gb1), a downstream metabolic product of glucosylceramide, for monitoring treated and untreated children with Gaucher disease (GD) has not yet been studied. We reviewed the clinical charts of 81 children (<18 y...
Boron neutron capture therapy (BNCT) combines preferential tumor uptake of 10B compounds and neutron irradiation. Electroporation induces an increase in the permeability of the cell membrane. We previously demonstrated the optimization of boron biodi...
The challenges in the diagnosis, prognosis, and monitoring of Gaucher disease (GD), an autosomal recessive inborn error of glycosphingolipid metabolism, can negatively impact clinical outcomes. This systematic literature review evaluated the value of...
A major problem with anti-cancer drug treatment is the development of acquired multidrug resistance (MDR) of the tumor cells. Verotoxin-1 (VT-1) exerts its cytotoxicity by targeting the globotriaosylceramide membrane receptor (Gb3), a glycolipid asso...
Protease-activated receptor 2 (PAR2) is a G-protein-coupled receptor (GPCR) activated by proteolytic cleavage of its N-terminal domain. Once activated, PAR2 is rapidly desensitized and internalized by phosphorylation and β-arrestin recruitment....
Historically, disease burden and treatment responses in patients with Gaucher disease (GD) was assessed by monitoring clinical data, laboratory, imaging, chitotriosidase (CHITO), and other biomarkers; however, these biomarkers lack specificity and CH...
Anderson-Fabry disease (FD) is a rare, progressive, multisystem storage disorder caused by the partial or total deficit of the lysosomal enzyme α-galactosidase A (α-Gal A). It is an X-linked, lysosomal enzymopathy due to mutations in the...
Background/Objectives: Group B Streptococcus (GBS) is a significant cause of perinatal infection in neonates and infants. Complications could include neonatal sepsis and meningitis, preterm birth, stillbirth, or death. Though no GBS vaccine is curren...
Streptococcus agalactiae (Group B Streptococcus, GBS) is an opportunistic pathogen, which asymptomatically colonizes the gastrointestinal and genitourinary tract of up to one third of healthy adults. Nevertheless, GBS carriage in pregnant women may l...
Objectives: Flavonoids are a class of widely distributed secondary metabolites in plants. Ginkgo biloba leaves are rich in flavonoids and thus are utilized for extracting medicinal components to treat and prevent cardiovascular and cerebrovascular di...
Ground-based synthetic aperture radar interferometry (GB-InSAR) enables the continuous monitoring of areal deformation and can thus provide near-real-time control of the overall deformation state of dam surfaces. In the continuous small-scale deforma...
The novel plant hormone strigolactones (SL) are involved significantly in plant growth and development. Its key members SMXL6, 7, 8 can modulate SL signal reception and response negatively and can regulate plant branching remarkably. There are relati...
Ground-based interferometric synthetic aperture radar (GB-InSAR) technology can be applied to generate a digital elevation model (DEM) with high spatial resolution and high accuracy. Phase unwrapping is a critical procedure, and unwrapping errors can...
For years, the gold standard for diagnosing Gaucher disease (GD) has been detecting reduced β-glucocerebrosidase (GCase) activity in peripheral blood cells combined with GBA1 mutation analysis. The use of dried blood spot (DBS) specimens offers...
Ground-based synthetic aperture radar (GB-SAR) is a useful tool to simulate advanced SAR systems with its flexibility on RF system and SAR configuration. This paper reports an indoor experiment of bistatic/multistatic GB-SAR operated in Ku-band with...
The BIM-based building energy simulation plays an important role in sustainable design on the track of achieving the net-zero carbon building stock by 2050. However, the issues on BIM-BEM interoperability make the design process inefficient and less...
Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by α-galactosidase A gene (GLA) mutations, resulting in loss of activity of the lysosomal hydrolase, α-galactosidase A (α-Gal A). As a result, the main glycosph...
Phase analysis based on high-quality pixel (HQP) is crucial to ensure the measurement accuracy of ground-based SAR (GB-SAR). The amplitude dispersion (ADI) criterion has been widely applied to identify pixels with high amplitude stability, i.e., perm...
The soaring demand for higher speeds in datacenters to address the relentless growth of the global IP traffic places optical interconnects in the spotlight. In this manuscript, we present a high-speed optical transceiver for intra-datacenter connecti...
Fabry disease is a rare X-linked lysosomal storage disorder characterized by progressive glycosphingolipid accumulation and multisystem organ involvement. Globotriaosylsphingosine (Lyso-Gb3) has emerged as a promising biomarker of disease burden; how...
In the hexaploid wheat genome, there are three Gα genes, three Gβ and twelve Gγ genes, but the function of Gβ in wheat has not been explored. In this study, we obtained the overexpression of TaGB1 Arabidopsis plants through infl...
In the past two decades, ground-based synthetic aperture radars (GB-SARs) have developed rapidly, providing a large amount of SAR data in minutes or even seconds. However, the real-time processing of big data is a challenge for the existing GB-SAR in...
Background/Objectives: Oral donepezil, an acetylcholinesterase (AChE) inhibitor for Alzheimer’s disease, faces adherence challenges. Long-acting injectable (LAI) formulations like GB-5001 aim to enhance adherence by reducing dosing frequency. T...
Sea Island cotton (Gossypium barbadense L.) is a premium raw material for high-end textiles due to its excellent fiber quality. The AP2/ERF transcription factor family plays critical roles in plant growth and hormone signaling. Here, 161 GbERF family...
Ground-Based Interferometric Synthetic Aperture Radar (GB-InSAR) is widely used for geohazard and infrastructure health assessment because it enables high-precision deformation monitoring. However, long-term time series observations often contain pha...
Hyperimmune globulin (HIG) has shown efficacy against human cytomegalovirus (HCMV) for both transplant and congenital transmission indications. Replicating that activity with a monoclonal antibody (mAb) offers the potential for improved consistency i...
Fabry disease is a rare lysosomal storage disorder resulting from the lack of α-Gal A gene activity. Globotriaosylceramide (GB3, ceramide trihexoside) is a novel endogenous biomarker which predicts the incidence of Fabry disease. At the early s...
Anthocyanidin reductase (ANR) is an important regulator of flavonoid metabolism, and proanthocyanidins, the secondary metabolites of flavonoids, play an important role in the response of plants to pathogenic stress. Therefore, in this study, the expr...
Streptococcus agalactiae (Group B Streptococci, GBS) can cause severe neonatal sepsis. The recto-vaginal GBS screening of pregnant women and intrapartum antibiotic prophylaxis (IAP) to positive ones is one of the main preventive options. However, suc...
Background: Podocyturia in Fabry nephropathy leads to glomerulosclerosis and kidney disease progression. Integrins are involved in podocyte attachment to the glomerular basement membrane. We hypothesized that in Fabry nephropathy, lyso-Gb3 could modu...
Gaucher disease (GD) is a lysosomal storage disorder that responds well to enzyme replacement therapy (ERT). Certain laboratory parameters, including blood concentration of glucosylsphingosine (Lyso-Gb1), the lyso-derivate of the common glycolipid gl...
This article presents a methodology for the monitoring of tall structures based on the joint use of a terrestrial laser scanner (TLS), configured in line scanner mode, and a ground-based real aperture radar (GB-RAR) interferometer. The methodology pr...
Background: Gaucher disease (GD) is a lysosomal storage disorder caused by mutations in the GBA1 gene, leading to β-glucocerebrosidase deficiency and glucosylceramide accumulation. Methods: We analyzed short- and long-term dynamics of lyso-gluco...
We present a Molecular Dynamics study of mixtures of charged Gay-Berne (GB) ellipsoids and spherical Lennard-Jones (LJ) particles as models of ionic liquids and ionic liquid crystals. The GB system is highly anisotropic (GB(4.4,20.0,1,1)) and we obse...
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