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)
The NOR phenotype-related antigens are unique neutral glycosphingolipids recognized by these antibodies and Griffonia simplicifolia IB4 isolectin (GSL-IB4). (slu.se)
Lipid8
The essential feature of a glycolipid is the presence of a monosaccharide or oligosaccharide bound to a lipid moiety. (wikipedia.org)
Enzymes called glycosyltransferases link the saccharide to the lipid molecule, and also play a role in assembling the correct oligosaccharide so that the right receptor can be activated on the cell which responds to the presence of the glycolipid on the surface of the cell. (wikipedia.org)
Glycoglycerolipids: a sub-group of glycolipids characterized by an acetylated or non-acetylated glycerol with at least one fatty acid as the lipid complex. (wikipedia.org)
Although lipids are a distinct class of biomolecules, we shall see that they often occur combined, either covalently or through weak bonds, with members of other classes of bio-molecules to yield hybrid molecules such as glycolipids , which contain both carbohydrate and lipid groups, "and lipoproteins , which contain both lipids and proteins. (medmuv.com)
Lipid modulation of glycolipid receptor function‐an approach to soluble mimics. (or.jp)
Glycoproteins are one class of glycoconjugate or complex carbohydrate -molecules containing one or more carbohydrate chains covalently linked to protein (to form glycoproteins or proteoglycans , see Chapter 50 ) or lipid (to form glycolipids , see Chapter 21 ). (mhmedical.com)
The glycolipid storage gives rise to the characteristic Gaucher cells, macrophages engorged with lipid with a crumpled-tissue-paper appearance and displaced nuclei. (medscape.com)
Carbohydrate4
Glycolipids are lipids with a carbohydrate attached by a glycosidic (covalent) bond. (wikipedia.org)
The saccharide of the glycolipid will bind to a specific complementary carbohydrate or to a lectin (carbohydrate-binding protein), of a neighboring cell. (wikipedia.org)
Matreya's monoclonal and polyclonal anti-ganglioside antibodies are directed against the carbohydrate chains of ganglioside and globoside glycolipids. (matreya.com)
Glycosphingolipids (GSLs) are glycolipids with ceramide and carbohydrate head groups that play an important role in numerous biological processes. (bvsalud.org)
GSLs1
Glycosphingolipids (GSLs) are a subclass of glycolipids that are found in cell membranes of various organisms ranging from bacteria to humans. (springer.com)
Glycosphingolipid deposits in lysosomes of endothelial, perithelial, and smooth muscle cells of blood vessels cause swelling into the blood vessel lumen. (medscape.com)
FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. (assaygenie.com)
Glycolipids are found on the surface of all eukaryotic cell membranes, where they extend from the phospholipid bilayer into the extracellular environment. (wikipedia.org)
Blood types are an example of how glycolipids on cell membranes mediate cell interactions with the surrounding environment. (wikipedia.org)
These sphingolipid transfer proteins were initially identified as glycolipid transfer proteins (GLTPs) due to their specificity for transferring glycosphingolipids between membranes. (umn.edu)
Glucosylceramide, the accumulated glycolipid, is primarily derived from the phagocytosis and degradation of senescent leukocytes and, to a lesser extent, from erythrocyte membranes. (medscape.com)
Antigen1
The four main human blood types (A, B, AB, O) are determined by the oligosaccharide attached to a specific glycolipid on the surface of red blood cells, which acts as an antigen. (wikipedia.org)
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)
Binding to a glycolipid, any compound containing one or more monosaccharide residues bound by a glycosidic linkage to a hydrophobic group such as an acylglycerol, a sphingoid, a ceramide (N-acylsphingoid) or a prenyl phosphate. (mcw.edu)
Carbohydrates1
Selectins, a class of lectins found on the surface of leukocytes and endothelial cells bind to the carbohydrates attached to glycolipids to initiate the immune response. (wikipedia.org)
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)
Galactosidase A are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease with glycolipid accumulates in many tissues. (assaygenie.com)
Biosynthesis4
N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis. (ox.ac.uk)
We have investigated the effects of deoxynojirimycin and its alkylated derivatives on the biosynthesis of glycolipids in HL-60 cells. (ox.ac.uk)
This results in the inhibition of biosynthesis of all glucosylceramide-based glycosphingolipids. (ox.ac.uk)
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)
Accumulation1
The factors that contribute to neurologic involvement in patients with types 2 and 3 disease are still unknown but may be related to the accumulation of a cytotoxic glycolipid, glucosylsphingosine, in the brain due to the severe deficiency of glucocerebrosidase activity or to neuroinflammation. (medscape.com)
Lysosomal2
As a result of the lack of this lysosomal enzyme that breaks down the glycolipid, persons with Fabry disease have 3-10 times the normal amount in their serum. (medscape.com)
This compound prevents lysosomal glycolipid storage and offers a novel therapeutic approach for the management of this and other glycolipid storage disorders. (ox.ac.uk)
Antibodies1
Antigens which are not present in an individual's blood will cause antibodies to be produced, which will bind to the foreign glycolipids. (wikipedia.org)
The oligosaccharide chains of NOR glycolipids are terminated by Galalpha1-4GalNAcbeta1-3Galalpha units. (slu.se)
Synthetic2
Matreya's in-house chemists perform the total synthesis of our catalog of glycerolipids and glycosphingolipids and have synthetic capabilities in bioconjugate and stable isotopic labeling chemistry. (labclinics.com)
New synthetic oligosaccharides, corresponding to the terminal di- and trisaccharide sequence of NOR glycolipids and the conjugate of the NOR-tri with HSA were included. (slu.se)
Endothelial1
An example of how glycolipids function within the body is the interaction between leukocytes and endothelial cells during inflammation. (wikipedia.org)
Impact of sugar stereochemistry on natural killer T cell stimulation by bacterial glycolipids. (uchicago.edu)
Notably1
Glycolipids are also responsible for other responses, notably the recognition of host cells by viruses. (wikipedia.org)
Soluble1
The saccharides that are attached to the polar head groups on the outside of the cell are the ligand components of glycolipids, and are likewise polar, allowing them to be soluble in the aqueous environment surrounding the cell. (wikipedia.org)
The vesicle merges with the cell membrane so that the glycolipid can be presented on the cell's outside surface. (wikipedia.org)
The main function of glycolipids in the body is to serve as recognition sites for cell-cell interactions. (wikipedia.org)
Consists1
The disease consists of an inborn error of glycosphingolipid catabolism. (assaygenie.com)
Found1
This product is a glucosylceramide synthase inhibitor, an enzyme that is essential for the synthesis of a very large number of different glycolipids that are found in many organisms.1 PDMP has four possible isomers due to its two chiral centers (D- threo , L- threo , D- erythro , and L- erythro ). (matreya.com)
Diffusion1
Deposition of glycosphingolipids can be attributed to both endogenous production and diffusion of material from the circulation. (medscape.com)