Quantification of glucosylceramide in plasma of Gaucher disease patients [PDF]
Gaucher disease is a sphingolipidosis that leads to an accumulation of glucosylceramide. The objective of this study was to develop a methodology, based on the extraction, purification and quantification of glucosylceramide from blood plasma, for use in ...
Maria Viviane Gomes Muller +7 more
doaj +4 more sources
Skin barrier function, prevent colon cancer, head and neck cancer, and decrease liver cholesterol. However, the mechanism of action has not yet been elucidated.
Huanghuang Dai +8 more
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CgGCS, Encoding a Glucosylceramide Synthase, Is Required for Growth, Conidiation and Pathogenicity in Colletotrichum gloeosporioides [PDF]
Fungal glucosylceramide plays important role in cell division, hyphal formation and growth, spore germination and the modulation of virulence and has recently been considered as target for small molecule inhibitors. In this study, we characterized CgGCS,
Yimei Huang +38 more
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Selection of Salt-tolerant Yeast Strains Accumulating Glucosylceramide [PDF]
application/pdfDebaryomyces hansenii NBRC 0855 accumulated high amounts of glucosylceramide relative to 25 strains of salt-tolerant yeasts in the genera Candida,Debaryomyces. Kodamaea,and Pichia.
Takakuwa, Naoya +4 more
core +3 more sources
Therapeutic potential of targeting ceramide/glucosylceramide pathway in cancer [PDF]
Sphingolipids including ceramides and its derivatives such as ceramide-1-phosphate, glucosylceramide (GlcCer), and sphingosine-1-phosphate are essential structural components of cell membranes.
Melis Kartal Yandım +5 more
core +2 more sources
Glucosylsphingosine Is a Highly Sensitive and Specific Biomarker for Primary Diagnostic and Follow-Up Monitoring in Gaucher Disease in a Non-Jewish, Caucasian Cohort of Gaucher Disease Patients [PDF]
Gaucher disease (GD) is the most common lysosomal storage disorder (LSD). Based on a deficient β-glucocerebrosidase it leads to an accumulation of glucosylceramide. Standard diagnostic procedures include measurement of enzyme activity, genetic testing as
Rolfs, A +59 more
core +2 more sources
The association of shiga-like toxin with detergent-resistant membranes is modulated by glucosylceramide and is an essential requirement in the endoplasmic reticulum for a cytotoxic effect [PDF]
Receptor-mediated internalization to the endoplasmic reticulum (ER) and subsequent retro-translocation to the cytosol are essential sequential processes required for the productive intoxication of susceptible mammalian cells by Shiga-like toxin-1 (SLTx).
Falguières, T +20 more
core +1 more source
Glucosylceramide (GC) metabolism in Gaucher diseases.
Ceramide, glucosylceramide shift between the Golgi apparatus and lysosomes. Ceramides, generated in the ER, are transported to the Golgi apparatus, where UDP-glucosylceramide synthase (UGCG synthase) converts Cer to GC on the cytosolic side of the Golgi [
Margarita M. Ivanova (11604141) +8 more
core +1 more source
Lysosomal membrane integrity in fibroblasts derived from patients with Gaucher disease
Gaucher disease (GD) is a recessively inherited lysosomal storage disorder characterized by a deficiency of lysosomal glucocerebrosidase (GBA1). This deficiency results in the accumulation of its substrate, glucosylceramide (GlcCer), within lysosomes ...
Asuka Hamamoto +7 more
doaj +1 more source
Cerebrosides (Crb; including glucosylceramide and galactosylceramide) and lactosylceramide (LacCer) are structurally complex lipids found in many eukaryotic cell membranes, where they play important roles in cell growth, apoptosis, cell recognition and ...
Lin Ma +3 more
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