Cellular effects of deoxynojirimycin analogues: uptake, retention and inhibition of glycosphingolipid biosynthesis. (ox.ac.uk)
These results establish that D-PDMP encapsulated in a biodegradable polymer provides a superior mode of delivery compared to unconjugated D-PDMP by way of increased gastrointestinal absorption and increased residence time thus providing this otherwise rapidly cleared compound with therapeutic relevance in interfering with atherosclerosis, cardiac hypertrophy, and probably other diseases associated with the deleterious effects of abnormally high glycosphingolipid biosynthesis or deficient catabolism. (johnshopkins.edu)
GalNAc-T is the enzyme involved in the biosynthesis of G(M2) and G(D2) glycosphingolipids. (nih.gov)
Induction of glycosphingolipid biosynthesis and neurite outgrowth of primary cultured neurons by L- threo -1-phenyl-2-decanoylamino-3-morpholino-1 propanol (L-PDMP). (or.jp)
Gangliosides5
Glycosphingolipids include: CerebrosidesGangliosidesGlobosidesGangliosides are mainly found in the cell membranes of the central nervous system, where their carbohydrate group is responsible for the interaction between individual cells and for signaling. (wikipedia.org)
GM2 and GD2 gangliosides are sialic acid-containing glycosphingolipids. (nih.gov)
Gangliosides, complex glycosphingolipids on the plasma membrane containing one or more sialic acid residues, are key components of microdomains. (jneurosci.org)
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)
Gangliosides 1 are acidic glycosphingolipids that form lipid rafts in the outer leaflet of the cell plasma membrane, especially in neuronal cells in the central nervous system. (matreya.com)
Glycosphingolipids (GSLs) are a subclass of glycolipids that are found in cell membranes of various organisms ranging from bacteria to humans. (springer.com)
These glycosphingolipids (GSLs) were expressed in all cell lines with concentrations ranging from 210 to 330 ng per 2 × 10 6 cells. (karger.com)
The glycosphingolipid (GSL) storage diseases are caused by genetic disruption in the lysosomal degradation pathway of GSLs, and include Tay-Sachs disease, Sandhoff's disease, Gaucher's disease, Fabry's disease, Krabbe's disease, and several others. (nih.gov)
A variety of glycoconjugates, including glycosphingolipids (GSLs), expressed in mammalian tissues and cells were isolated and characterized in early biochemical studies. (nih.gov)
Complex glycosphingolipids1
In this review, strategies to synthesize these complex glycosphingolipids are presented. (springer.com)
Synthesis3
While most studies use Gb 3 extracts, chemical synthesis provides a unique tool to access different tailor-made Gb 3 glycosphingolipids. (springer.com)
D-Threo-1-phenyl-2-decanoylamino-3-morpholino-1-propanol (D-PDMP), a glycosphingolipid synthesis inhibitor, holds promise for the treatment of atherosclerosis and cardiac hypertrophy but rapid invivo clearance has severely hindered translation to the clinic. (johnshopkins.edu)
Synthesis of Gb 3 glycosphingolipids with labeled head groups: Distribution in phase-separated giant unilamellar vesicles. (mpg.de)
Antigens1
Neutral glycosphingolipids are also important, for example as blood type antigens. (wikipedia.org)
Metabolism1
Fabry disease (FD) is an X-linked lysosomal storage disease, affecting glycosphingolipid metabolism. (medscape.com)
In general, glycosphingolipids can be categorized into two groups: neutral glycosphingolipids (also called glycosphingolipids) and negatively charged glycosphingolipids. (wikipedia.org)
Deficiency or absence of alpha-galactosidase A (α-GAL A) activity as a result of gene mutations in the GLA gene (Xq21.3-q22) leads to lysosomal accumulation of neutral glycosphingolipids, most notably globotriaosylceramide (G3b). (medscape.com)
Lipid rafts1
Integrin-regulated Rac binding sites are in lipid rafts (membrane domains enriched in cholesterol and glycosphingolipids). (europa.eu)
Carbohydrate1
Sulfated glucuronosyl glycosphingolipids (SGGLs) are cell surface molecules that are endowed with the Human Natural Killer-1 (HNK-1) carbohydrate epitope. (karger.com)
Ceramide1
The addition of a phosphocholine group to ceramide yields sphingomyelin, but the addition of glucose or galactose to ceramide yields glycosphingolipids and sulfatides. (frontiersin.org)
Enzyme1
Angiokeratoma corporis diffusum is the cutaneous hallmark of Fabry disease, an X-linked inherited disorder caused by a deficiency in the lysosomal enzyme alpha-galactosidase A. Decreased or absent enzyme activity causes uncleaved glycosphingolipids to accumulate in various cell types, particularly in the vascular endothelium, smooth muscle cells, and pericytes, causing ischemia and infarction of tissues. (medscape.com)
Antigen1
yet, the glycosphingolipid P antigen is considered its primary target. (medscape.com)
Cells4
We have synthesized a series of DNJ analogues to study the contribution of N-alk(en)yl side chains (C4, C9 or C18) to the behaviour of these analogues in cultured HL60 cells. (ox.ac.uk)
The present study further characterizes the properties of N-alk(en)ylated DNJs, and demonstrates that increasing the length of the side chain is a simple way of improving imino sugar retention and therefore inhibitory efficacy for CGT in cultured cells. (ox.ac.uk)
These glycosphingolipids accumulate in many different types of cells. (medscape.com)
Glycosphingolipid deposits in lysosomes of endothelial, perithelial, and smooth muscle cells of blood vessels cause swelling into the blood vessel lumen. (medscape.com)
Endogenous1
Deposition of glycosphingolipids can be attributed to both endogenous production and diffusion of material from the circulation. (medscape.com)
Disorders1
B4galntltm1Rlp for the study of glycosphingolipid storage disorders. (nih.gov)
Diseases2
Glycosphingolipids were once considered to play a purely structural role in cell membranes, but it has since become apparent that they play a key role in the nervous system and are associated with several diseases. (febs.org)
Guest editor Sandro Sonnino introduces the contents of the issue in a fascinating interview conducted by Ioannis Tsagakis, starting with eight Review articles that focus on the role of glycosphingolipids in diseases as diverse as Parkinson's disease, cystic fibrosis, infertility, and parasite infections. (febs.org)
Mass1
Persons with Fabry disease who have type AB or B blood also accumulate blood group B glycosphingolipids (those with alpha-galactosyl-terminated residues) and can have more severe Fabry disease (related to greater body substrate mass) than patients with blood group A. This is because these blood groups have two additional terminal alpha-galactosyl moieties. (medscape.com)
Role2
Glycosphingolipids play also important role in oncogenesis and ontogenesis. (wikipedia.org)
Human embryonic stem cell differentiation: role of glycosphingolipid structure. (sinica.edu.tw)
Cell1
Extended globo-series glycosphingolipids that mediate cell adhesion and signaling. (or.jp)
Links2
Here, we employed a Drosophila model to test links between glycosphingolipids , neurotransmission and neurodegeneration. (bvsalud.org)
These findings indicate causal links between glycosphingolipid -dependent neurotransmission and neurodegeneration in this NPC disease model. (bvsalud.org)
Human1
Degradation of human intestinal glycosphingolipids by extracellular glycosidases from mucin-degrading bacteria of the human fecal flora. (kegg.jp)
Disease1
The September issue of FEBS Open Bio puts the involvement of glycosphingolipids in disease in the limelight. (febs.org)
Model1
Glycosphingolipids are linked to elevated neurotransmission and neurodegeneration in a Drosophila model of Niemann Pick type C. (bvsalud.org)