Background: Fabry disease (FD) is an X-linked lysosomal storage disorder caused by mutations in the GLA gene, resulting in α-galactosidase A (α-Gal A) deficiency and progressive accumulation of globotriaosylceramide (Gb3). Current therapi...
One mechanism to regulate pathological vascular calcification (VC) is its active inhibition. Loss or inactivation of endogenic inhibitors is a major inductor of VC. Such inhibitors are proteins rich in gamma-glutamyl residues (Gla-proteins), whose fu...
Fabry disease (FD) is an X-linked recessive inheritance lysosomal storage disorder caused by pathogenic mutations in the GLA gene leading to a deficiency of the enzyme alpha-galactosidase A (α-Gal A). Multiple organ systems are implicated in FD...
Fabry disease (FD) exhibits a spectrum of clinical manifestations ranging from mild to severe, posing a diagnostic challenge, particularly in non-classic subtypes. Genetic testing remains a gold standard for a precise diagnosis of FD and is pivotal i...
The use of polyunsaturated fatty acids in Attention-Deficit/Hyperactivity Disorder (ADHD) and developmental disorders has been gaining interest with preparations containing different dosages and combinations. Gamma-linolenic acid (GLA) is an ω-...
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...
Objectives: The pathophysiology of osteoarthritis is mainly unknown. Matrix Gla protein (MGP) and Gla-rich protein (GRP) are both vitamin-K-dependent mineralization inhibitors. In this study, we aimed to compare the levels of MGP and GRP in the synov...
Anderson–Fabry disease is a hereditary, progressive, multisystemic lysosomal storage disorder caused by a functional deficiency of the enzyme α-galactosidase A (α-GalA). This defect is due to mutations in the GLA gene, located in th...
Fabry disease (FD) is a rare X-linked disorder of glycosphingolipid metabolism caused by pathogenic variants within the alpha-galactosidase A (GLA) gene, often leading to neurological manifestations including stroke. Multiple screening programs seeki...
Background: Matrix Gla protein (MGP) is an extrahepatic protein that is dependent on glutamate carboxylation, a vitamin K-dependent process. Its dysfunctional form, desphospho-uncarboxylated-MGP, has been associated with increased arterial calcificat...
Objectives: Identifying reliable biomarkers to predict mortality in critically ill patients is crucial for optimizing management in intensive care units (ICUs). Inflammatory and metabolic markers are increasingly recognized for their prognostic value...
Fabry disease is an X-linked lysosomal storage disorder caused by pathogenic variants in the GLA gene, encoding α-galactosidase A. Enzyme deficiency leads to progressive globotriaosylceramide (Gb3) accumulation and multisystemic involvement. He...
Fabry disease (FD) is an X-linked inborn metabolic disorder due to partial or complete lysosomal α-galactosidase A deficiency. FD is characterized by progressive renal insufficiency and cardio- and cerebrovascular involvement. Restricted access...
Matrix Gla Protein (MGP), a small Gla vitamin K-dependent protein, is the most powerful natural occurring inhibitor of calcification in the human body. To become biologically active, MGP must undergo vitamin K-dependent carboxylation and phosphorylat...
Anderson Fabry disease (AFD) is an X-linked hereditary lysosomal abnormality that causes the accumulation of glycosphingolipids in body fluids and tissues, leading to progressive organ damage and a shortened life span. More than 1000 mutations in the...
Anderson–Fabry (or Fabry) disease is a rare lysosomal storage disorder caused by a functional deficiency of the enzyme alpha-galactosidase A. The partial or total defect of this lysosomal enzyme, which is caused by variants in the GLA gene, lea...
Specific patient cohorts are at increased risk of vascular calcification. Functional matrix-gla protein (MGP), a tissue-derived vitamin K dependent protein, is reported to be an important inhibitor of vascular calcification and may have clinical pote...
Background/Objectives: Ulcerative colitis (UC) is a chronic inflammatory bowel disease with limited effective treatments, prompting the need for investigation of novel therapeutic approaches. Eicosapentaenoic acid (EPA) and gamma-linolenic acid (GLA)...
Background/Objectives: Vascular calcification (VC) is a crucial risk factor for cardiovascular diseases (CVD), particularly in chronic kidney disease (CKD) populations. However, the specific relationship between VC and end-stage renal disease (ESRD)...
Chronic inflammation is a major driver of chronic inflammatory diseases (CIDs), with a tremendous impact worldwide. Besides its function as a pathological calcification inhibitor, vitamin K-dependent protein Gla-rich protein (GRP) was shown to act as...
Background. Colorectal cancer is a real public health issue, with high morbidity and severe impact on quality of life. Although mortality from this type of cancer is decreasing due to modern diagnostic and treatment methods, the understanding of its...
Microbial cultures repurposing organic industrial residues for value-added metabolite production is pivotal for sustainable resource use. Highlighting polyunsaturated fatty acids (PUFAs), particularly gamma-linolenic acid (GLA), renowned for their nu...
Coronary artery disease (CAD) is widely recognized as one of the most important clinical entities. In recent years, a large body of accumulated data suggest that coronary artery calcification, a process highly prevalent in patients with CAD, occurs v...
Neurodegenerative diseases are characterized by neuroinflammation, neuronal depletion and oxidative stress. They coincide with subtle chronic or flaring inflammation, sometimes escalating with infiltrations of the immune system cells in the inflamed...
Vascular calcification contributes to the pathogenesis of coronary artery disease while matrix Gla protein (MGP) was recently identified as a potent inhibitor of vascular calcification. MGP fractions, such as dephosphorylated-uncarboxylated MGP (dp-u...
Anderson–Fabry disease (AFD) is an X-linked multisystemic disorder with a heterogeneous phenotype, resulting from deficiency of the lysosomal enzyme α-galactosidase A (α-Gal A) and leading to globotriaosylceramide systemic accumulat...
This article explores how Charlotte Glas, a founding member of the Austrian Social Democratic Party and a leading figure in the public sphere during the late imperial period, attempted to advance the cause of workers’ rights and women’s emancipation....
The major cause of ovarian cancer treatment failure in cancer patients is inherent or acquired during treatment drug resistance of cancer. Matrix Gla protein (MGP) is a secreted, non-collagenous extracellular matrix protein involved in inhibition of...
Visceral leishmaniasis (VL) is the most severe clinical form of leishmaniasis, fatal if untreated. Vaccination is the most cost-effective approach to disease control; however, to date, no vaccines against human VL have been made available. This work...
Accelerated and premature cardiovascular calcification is a hallmark of chronic kidney disease (CKD) patients. Valvular calcification (VC) is a critical indicator of cardiovascular disease and all-cause mortality in this population, lacking validated...
Gla-rich protein (GRP), also known as UCMA, is a vitamin K-dependent protein that has emerged as an important regulator of pathological calcification and inflammation. Vascular calcification is a major complication of chronic kidney disease and cardi...
The “Can Rapid risk stratification of Unstable angina patients Suppress ADverse outcomes with Early implementation of the ACC/AHA guidelines” (CRUSADE) score emerged as a predictor of major bleeding in patients presenting with the acute coronary synd...
This study explores ICESat/GLAS waveform data in Thuringian Forest, a low mountain range located in central Germany. Lidar remote sensing has been proven to directly derive tree height as a key variable of forest structure. The GLAS signal is, howeve...
Background: Systemic sclerosis (SSc) patients face an elevated risk of cardiovascular disease (CVD), even when classic cardiovascular risk factors are considered. Plasma dephosphorylated-uncarboxylated Matrix Gla-protein (dp-ucMGP), an inactive form...
Background/Objectives: Vascular calcification and arterial stiffness are common in chronic kidney disease (CKD). Gla-rich protein (GRP) is a vitamin K-dependent protein implicated in mineral biology, but clinical evidence across CKD stages is limited...
Forest biomass is an important parameter for quantifying and understanding biological and physical processes on the Earth’s surface. Rapid, reliable, and objective estimations of forest biomass are essential to terrestrial ecosystem research. The Geo...
Glycosylation plays a critical role in various biological processes, yet identifying specific glycosyltransferase substrates remains a challenge due to the complexity of glycosylation. Here, we employ proximity labeling with biotin ligases BASU and T...
Forest aboveground biomass (AGB) was mapped throughout China using large footprint LiDAR waveform data from the Geoscience Laser Altimeter System (GLAS) onboard NASA’s Ice, Cloud, and land Elevation Satellite (ICESat), Moderate Resolution Imaging Spe...
Objectives: Gla-rich protein (GRP), a vitamin K-dependent protein, has been increasingly recognized for its dual role in modulating inflammation and inhibiting pathological calcification. Despite its emerging importance in chronic conditions, limited...
Vascular calcification (VC) is one of the strongest predictors of cardiovascular risk in chronic kidney disease (CKD) patients. New diagnostic/prognostic tools are required for early detection of VC allowing interventional strategies. Gla-rich protei...
Background: Chronic kidney disease–mineral and bone disorder contributes to vascular calcification in kidney transplant recipients. However, the relationship between circulating bone turnover markers, Gla-rich protein (GRP), pelvic arterial cal...
Indonesian peatlands are one of the largest near-surface pools of terrestrial organic carbon. Persistent logging, drainage and recurrent fires lead to huge emission of carbon each year. Since tropical peatlands are highly inaccessible, few measuremen...
Background: The GLA:D® program is an evidence-based intervention widely used in Western countries to improve knee function and exercise adherence among individuals with osteoarthritis. However, its application in postoperative total knee arthropl...
Mapping the magnitude and spatial distribution of forest aboveground biomass (AGB, in Mg·ha−1) is crucial to improve our understanding of the terrestrial carbon cycle. Landsat/TM (Thematic Mapper) and ICESat/GLAS (Ice, Cloud, and land Elevation Satel...
Vascular calcification (VC) is a complex process involving vascular smooth muscle cell (VSMC) osteogenic differentiation, inflammation, and extracellular vesicle (EV) calcification and communication networks. Gla rich protein (GRP) is a calcification...
Vegetation structure, including forest canopy height, is an important input variable to fire behavior modeling systems for simulating wildfire behavior. As such, forest canopy height is one of a nationwide suite of products generated by the LANDFIRE...
Shuttle Radar Topography Mission (SRTM) digital elevation model (DEM) inherently suffers from various errors. Many previous works employed Geoscience Laser Altimeter System onboard the Ice, Cloud, and land Elevation Satellite (ICESat/GLAS) data to as...
Clumping index (CI) is a canopy structural variable important for modeling the terrestrial biosphere, but its retrieval from remote sensing data remains one of the least reliable. The majority of regional or global CI products available so far were g...
The vertical foliage profile (VFP) and leaf area index (LAI) are critical descriptors in terrestrial ecosystem modeling. Although light detection and ranging (lidar) observations have been proven to have potential for deriving the VFP and LAI, existi...
Forest canopy height is an important biophysical variable for quantifying carbon storage in terrestrial ecosystems. Active light detection and ranging (lidar) sensors with discrete-return or waveform lidar have produced reliable measures of forest ca...
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