• The compounds tested are established glucosylceramide synthase (GS) inhibitors based on the d-1-phenyl-2-decanoylamino-3-morpholino-1-propanol (PDMP) structure. (edu.au)
  • It is phosphorylated by ceramide kinase (CK) to form ceramide-1-phosphate or it can be glycosylated by glucosylceramide synthase to form glycosphingolipids (cerebrosides, globosides, gangliosides). (frontiersin.org)
  • The chemical structure of 90% of mammalian GSLs is based on glucosyl ceramide, whereas galactosyl ceramide serves as a precursor for the remainder. (springer.com)
  • which hydrolyzes glucosylceramide (GluCer) into glucose and ceramide and is deficient in Gaucher disease (GD). (ipa2014.org)
  • Previous studies suggest that membrane lipids may regulate proteolytic processing of the amyloid precursor protein (APP) to generate amyloid-beta peptide (Abeta). (edu.au)
  • This gene encodes a highly conserved glycoprotein which is a precursor for 4 cleavage products: saposins A, B, C, and D. Each domain of the precursor protein is approximately 80 amino acid residues long with nearly identical placement of cysteine residues and glycosylation sites. (affbiotech.cn)
  • The precursor protein exists both as a secretory protein and as an integral membrane protein and has neurotrophic activities. (affbiotech.cn)
  • A high-mannose oligosaccharide precursor is synthesized and transferred to proteins in the endoplasmic reticulum and then processed in the Golgi apparatus ( 4 ). (frontiersin.org)
  • In this study, we evaluated a cell therapy treatment using mouse iPSC-derived neural precursor cells (NPCs) engineered to overexpress GCase (termed hGBA1-NPCs). (bvsalud.org)
  • Myelophthisis is a form of bone marrow failure that results from the destruction of bone marrow precursor cells and their stroma, which nurture these cells to maturation and differentiation. (medscape.com)
  • [ 2 ] to the presence of a few teardrop-shaped red blood cells and early myeloid precursor cells in the peripheral blood smear. (medscape.com)
  • This blood film at 1000X magnification demonstrates a leukoerythroblastic blood picture with the presence of precursor cells of the myeloid and erythroid lineage. (medscape.com)
  • With regard to Gaucher disease, infiltration by cells with "onion-peel" cytoplasm, called Gaucher cells, is caused by a lipid storage disorder (ie, glucosylceramide lipidosis). (medscape.com)
  • Apart from immortalized cell lines and mouse models, human-induced pluripotent cell (iPSC) lines and derived neuronal cells (e.g., neuronal precursor cells) and other vertebrate models (e.g., zebrafish) are demonstrating their usefulness in preclinical studies. (mdpi.com)
  • Dive into the research topics of 'Structural determination of glucosylceramides isolated from marine sponge by fast atom bombardment collision-induced dissociation linked scan at constant B/E'. Together they form a unique fingerprint. (ewha.ac.kr)
  • Diacylglycerol is a precursor to triacylglycerol (triglyceride), which is formed in the addition of a third fatty acid to the diacylglycerol under the catalysis of diglyceride acyltransferase . (wikidoc.org)
  • Glycerol-3-phosphate (G3P) also acts as a precursor for TAG assembly at the ER. (biomedcentral.com)
  • L-Cysteine is a conditionally essential amino acid, which acts as a precursor for biologically active molecules such as hydrogen sulphide (H2S), glutathione and taurine. (medchemexpress.com)
  • Isolated LGs contain phosphoglycerides, sphingomyelin, and glucosylceramides. (nih.gov)
  • The results suggest that in the secretory pathway of Vero cells O-glycan initiation and sphingomyelin as well as glucosylceramide synthesis mainly occur beyond the ERGIC in the Golgi apparatus. (unibas.ch)
  • [ 22 ] There were, however, significant decreases in plasma sphingomyelin and glucosylceramide and ganglioside in the diabetic cynomolgus monkey group. (medscape.com)
  • He found that the NBD-glucosylceramide was enriched two to four times on the apical membrane, whereas the NBD-sphingomyelin was equally distributed between the apical and basolateral sides. (silverchair.com)
  • These agents inhibit the enzyme glucosylceramide synthase, the initial enzyme in a series of reactions that result in the synthesis of most glycosphingolipids, including glucocerebroside. (medscape.com)
  • The most unusual lipid component is a linoleate-containing glucosylceramide comprising 30- to 34-carbon ω-hydroxy-acids. (nih.gov)
  • Self-antigen(s) as well as the positive collection of iNKT cells Positive collection of iNKT cell precursors within the thymus needs reputation by their TCR of personal lipid(s)-Compact disc1d complexes [18]. (ole2011.org)
  • Antagonization Assays Different peptidoglycan precursors namely undecaprenyl phosphate (C55P) UDP-MurNAc-pp lipid II or UDP-GlcNAc were tested for antagonization of the oyster defensin antimicrobial activity. (morainetownshipdems.org)
  • Strictly antifungal defensins which include defensins from plants and from lepidopteran insects are not only membrane-disrupting agents but also interact with fungal glucosylceramides (19). (morainetownshipdems.org)
  • Mary C. Miozzo, 1992, Purification and characterization of membrane associated proteoglycogen precursor of glycogen in retina. (unc.edu.ar)
  • One refers to the increase in glucopolymerization degree of glycogeninin, beyond the 13 glucose units acquired by autoglucosylation, produced by glycogen synthase and required for branching enzyme to introduce the first branch into the proteoglycogen precursor. (unc.edu.ar)
  • Glucosylceramides were detected within lamellar bodies and in trans-Golgi network-like structures of the stratum granulosum. (anaspec.com)
  • The precursors of TAGs are mainly derived from glycolysis, and glycolysis is catalyzed via enzymes to generate acyl-CoAs [ 15 , 16 ]. (biomedcentral.com)
  • In this context, LGs serve as the precursor to the permeability barrier of the skin. (nih.gov)
  • It is likely that benzylcysteine sulfoxides serve as precursors to thiosulfinates as S-(2-hydroxyethyl)-phenylmethanethiosulfinate and sulfines as thiobenzaldehyde S-oxide. (biomedcentral.com)
  • Eliglustat is a specific inhibitor of glucosylceramide synthase, thereby reducing production of glucosylceramide. (medscape.com)
  • Reduces GSL production by inhibiting glucosylceramide synthase. (medscape.com)
  • This image shows a cross section of a day 28 human forebrain organoid, showing FOXG1-expressing neural precursors (Red), surrounding a ventricle-like structure outlined by N-Cadherin staining (Green). (sfn.org)
  • However, recent results founded that the previous reports of iNKT cell stimulatory activity by mammalian-purified -glucosylceramide [21] is due to minute traces of contaminating -linked forms [24]. (ole2011.org)