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Effects of Variants and Prenatal Exposition on the Glucosylsphingosine (Lyso-Gb1) Levels in Gaucher Disease Carriers.
Int J Mol Sci
November 2024
Department of Pediatrics, Nutrition and Metabolic Diseases, The Children's Memorial Health Institute, 04-730 Warsaw, Poland.
Gaucher disease (GD) is a lysosomal lipid storage disorder caused by β-glucocerebrosidase (encoded by gene) activity deficiency, resulting in the accumulation of glucosylceramide (Gb1) and its deacylated metabolite glucosylsphingosine (lyso-Gb1). Lyso-Gb1 has been studied previously and proved to be a sensitive biomarker, distinguishing patients with GD from carriers and healthy subjects. It was shown that its level corresponds with β-glucocerebrosidase activity, thus it remains unknown as to why carriers have slightly higher lyso-Gb1 level than healthy population.
View Article and Find Full Text PDFMilk Fat Globules: 2024 Updates.
Newborn (Clarksville)
March 2024
Global Newborn Society, Clarksville Maryland, United States of America.
Article Synopsis
- * MFGs feature a unique structure with a lipid core and a membrane rich in bioactive components that aid in energy release and support immune health in developing gastrointestinal tracts.
- * Research suggests MFGs can be enhanced to address specific nutritional deficiencies while also having potential therapeutic benefits for neurodevelopment and defense against infections.
The iNKT cell ligand α-GalCer prevents murine septic shock by inducing IL10-producing iNKT and B cells.
Front Immunol
October 2024
Department of Integrative Bioscience and Biotechnology, Institute of Anticancer Medicine Development, Sejong University, Seoul, Republic of Korea.
Introduction: α-galactosylceramide (α-GalCer), a prototypical agonist of invariant natural killer T (iNKT) cells, stimulates iNKT cells to produce various cytokines such as IFNγ and IL4. Moreover, repeated α-GalCer treatment can cause protective or pathogenic outcomes in various immune-mediated diseases. However, the precise role of α-GalCer-activated iNKT cells in sepsis development remains unclear.
View Article and Find Full Text PDFProbiotic treatment with viable α-galactosylceramide-producing Bacteroides fragilis reduces diabetes incidence in female nonobese diabetic mice.
J Diabetes
August 2024
Department of Pathology, The Bartholin Institute, Rigshospitalet, Copenhagen, Denmark.
Background: We aimed to investigate whether alpha-galactosylceramide (α-GalCer)-producing Bacteroides fragilis could induce natural killer T (NKT) cells in nonobese diabetic (NOD) mice and reduce their diabetes incidence.
Methods: Five-week-old female NOD mice were treated orally with B. fragilis, and islet pathology and diabetes onset were monitored.
Bioaccessibility of Glucosylceramide in Rice Based on the Cooking Condition and Cultivar.
J Oleo Sci
June 2024
Department of Life and Food Sciences, Obihiro University of Agriculture and Veterinary Medicine.
Glucosylceramide (GlcCer), a major sphingolipid in plants, possesses various food functions, including improvement of intestinal impairments. This study evaluated rice cooking conditions and cultivars based on GlcCer levels transferred into the digestive juice using an in vitro digestion model to investigate the factors related to GlcCer availability. GlcCer levels transferred into the digestive juice were higher in rice gruel than in boiled rice.
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