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Relevance of Neutralizing Antibodies for the Pharmacokinetics of Pegunigalsidase Alfa in Patients with Fabry Disease.
BioDrugs
January 2025
Internal Medicine D (Nephrology, Hypertension and Rheumatology), and Interdisciplinary Fabry Center (IFAZ), University Hospital Muenster, Albert-Schweitzer-Campus 1, D-48149, Muenster, Germany.
Background: Pegunigalsidase alfa is a newly approved drug for the treatment of Fabry disease, designed to increase the plasma half-life and reduce immunogenicity of infused α-galactosidase A (AGAL). We provide the first comprehensive pharmacokinetic and immunogenic data apart from industry-initiated studies.
Methods: Pharmacokinetics of pegunigalsidase alfa, amino acid, and polyethylene glycol (PEG)-specific antibodies and immune complexes were measured in treated patients (11 switched, two naïve).
In-Depth Phytochemical Profile by LC-MS/MS, Mineral Content by ICP-MS, and In-Vitro Antioxidant, Antidiabetic, Antiepilepsy, Anticholinergic, and Antiglaucoma Properties of Bitlis Propolis.
Life (Basel)
October 2024
Faculty of Agriculture, Vocational School of Food, Agriculture and Livestock, Bingöl University, 12000 Bingöl, Türkiye.
Propolis is very significant in terms of its phytochemical content and biological activity among bee products. In this study, the antioxidant activities (total phenolic and flavonoid, Fe, Cu (CUPRAC), Fe-TPTZ (FRAP) reducing, and DPPH, ABTS scavenging assays) of propolis collected from the Bitlis province of Türkiye were determined. In addition, the carbonic anhydrase I and II isoenzymes (hCA I and hCA II), α-glycosidase, acetylcholinesterase (AChE), and butyrylcholinesterase (BChE) inhibition activity and phytochemical profile of propolis and mineral content were determined by LC-MS/MS and ICP-MS, respectively.
View Article and Find Full Text PDFStriatum-enriched protein, arginase 2 localizes to medium spiny neurons and controls striatal metabolic profile.
Neurochem Int
January 2025
Laboratory of Molecular Basis of Neurodegeneration, Mossakowski Medical Research Institute, Polish Academy of Sciences, Warsaw, Poland. Electronic address:
Arginase 2 (Arg2) is the predominant arginase isoenzyme in the brain, however its distribution appears to be limited to selected, region-specific subpopulations of cells. Although striatum is highly enriched with Arg2, precise localization and function of striatal Arg2 have never been studied. Here, we confirm that Arg2 is the only arginase isoenzyme in the striatum, and, using genetic model of total Arg2 loss, we show that Arg2 in this region is fully responsible for arginase catalytic activity, and its loss doesn't induce compensatory activation of Arg1.
View Article and Find Full Text PDFDiminished lactate utilization in LDHB-deficient neurons leads to impaired long-term memory retention.
Exp Neurol
February 2025
Department of Physiology, Ajou University School of Medicine, Suwon 16499, Republic of Korea. Electronic address:
Neurons' high energy demands for processing, transmitting, and storing information in the brain necessitate efficient energy metabolism to maintain normal neuronal function. The astrocyte-neuron lactate shuttle (ANLS) hypothesis suggests neurons preferentially use lactate from astrocytes over glucose for energy. This study investigated lactate dehydrogenase B (LDHB), which preferentially converts lactate to pyruvate, in neuronal energy metabolism and cognitive function.
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