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Glycosphingolipid
Glycosphingolipids are a subtype of glycolipids containing the amino alcohol sphingosine. They may be considered as sphingolipids with an attached carbohydrate. Glycosphingolipids are a group of lipids (more specifically, sphingolipids) and are a part of the cell membrane. They consist of a hydrophobic ceramide part and a glycosidically bound carbohydrate part. This oligosaccharide content remains on the outside of the cell membrane where it is important for biological processes such as cell adhesion or cell–cell interactions. Glycosphingolipids also play an important role in oncogenesis and ontogenesis. Classification In general, glycosphingolipids can be categorized into two groups: neutral glycosphingolipids (also called glycosphingolipids) and negatively charged glycosphingolipids. The latter can be distinguished again by means of the charge carrier. While in gangliosides sialic acids are found, sulfatides have a sulfate group. The structural similarity of most glycoli ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cerebroside
Cerebrosides (monoglycosylceramides) are a group of glycosphingolipids which are important components of animal muscle and nerve cell membranes. They consist of a ceramide with a single sugar residue at the 1-hydroxyl moiety. The sugar residue can be either glucose or galactose; the two major types are therefore called glucocerebrosides (a.k.a. glucosylceramides) and galactocerebrosides (a.k.a. galactosylceramides). Galactocerebrosides are typically found in neural tissue, while glucocerebrosides are found in other tissues. Structure The fundamental structure of a cerebroside is ceramide. Monoglycosyl and oligoglycosylceramides having a mono or polysaccharide bonded glycosidically to the terminal OH group of ceramide are defined as cerebrosides. Sphingosine is the main long-chain base present in ceramide. Galactosylceramide is the principal glycosphingolipid in brain tissue. Galactosylceramides are present in all nervous tissues, and can compose up to 2% dry weight of grey m ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Isoglobotriosylceramide
Isoglobotriosylceramide, Gal(α1→3)Gal(β1→4) Glcβ(1→1) Cer, abbreviated as iGb3, is an iso-globo-series of glycosphingolipid, which mysteriously disappeared in most mammals studied (pig, mouse, and human), except trace amount reported in the thymus. iGb3 was discovered in canine and rat intestines among iso-globo-series of glycosphingolipids. First NMR spectrums for standard iGb3 were published by Dr. Tomoya Ogawa. The physiological function of iGb3 is not clear. It has been identified as a CD1d- presented self-antigen for an innate type of immune cells termed as Natural Killer T (NKT) cells. Extensive biochemical studies by multiple methods including HPLC, mass spectrometry, and NMR Nuclear magnetic resonance (NMR) is a physical phenomenon in which atomic nucleus, nuclei in a strong constant magnetic field are disturbed by a weak oscillating magnetic field (in the near and far field, near field) and respond by producing ... did not lead to positive finding of iG ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Sphingolipid
Sphingolipids are a class of lipids containing a backbone of sphingoid bases, which are a set of aliphatic amino alcohols that includes sphingosine. They were discovered in brain extracts in the 1870s and were named after the mythological sphinx because of their enigmatic nature. These compounds play important roles in signal transduction and cell recognition. Sphingolipidoses, or disorders of sphingolipid metabolism, have particular impact on neural tissue. A sphingolipid with a terminal hydroxyl group is a ceramide. Other common groups bonded to the terminal oxygen atom include phosphocholine, yielding a sphingomyelin, and various sugar monomers or dimers, yielding cerebrosides and globosides, respectively. Cerebrosides and globosides are collectively known as glycosphingolipids. Structure The long-chain bases, sometimes simply known as sphingoid bases, are the first non-transient products of '' de novo'' sphingolipid synthesis in both yeast and mammals. These co ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Globoside
Globosides (also known as globo-series glycosphingolipids) are a sub-class of the lipid class glycosphingolipid with three to nine sugar molecules as the side chain (or R group) of ceramide. The sugars are usually a combination of ''N''-acetylgalactosamine, D-glucose or D-galactose. One characteristic of globosides is that the "core" sugars consists of Glucose-Galactose-Galactose (Ceramide-βGlc4-1βGal4-1αGal), like in the case of the most basic globoside, globotriaosylceramide (Gb3), also known as pk-antigen. Another important characteristic of globosides is that they are neutral at pH 7, because they usually do not contain neuraminic acid, a sugar with an acidic carboxy-group. However, some globosides with the core structure Cer-Glc-Gal-Gal do contain neuraminic acid, e.g. the globo-series glycosphingolipid "SSEA-4-antigen". The side chain can be cleaved by galactosidases and glucosidases. The deficiency of α-galactosidase A causes Fabry's disease, an inherited metabol ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lipid
Lipids are a broad group of organic compounds which include fats, waxes, sterols, fat-soluble vitamins (such as vitamins A, D, E and K), monoglycerides, diglycerides, phospholipids, and others. The functions of lipids include storing energy, signaling, and acting as structural components of cell membranes. Lipids have applications in the cosmetic and food industries, and in nanotechnology. Lipids are broadly defined as hydrophobic or amphiphilic small molecules; the amphiphilic nature of some lipids allows them to form structures such as vesicles, multilamellar/ unilamellar liposomes, or membranes in an aqueous environment. Biological lipids originate entirely or in part from two distinct types of biochemical subunits or "building-blocks": ketoacyl and isoprene groups. Using this approach, lipids may be divided into eight categories: fatty acyls, glycerolipids, glycerophospholipids, sphingolipids, saccharolipids, and polyketides (derived from condensatio ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ceramide
Ceramides are a family of waxy lipid molecules. A ceramide is composed of sphingosine and a fatty acid joined by an amide bond. Ceramides are found in high concentrations within the cell membrane of Eukaryote, eukaryotic cells, since they are component lipids that make up sphingomyelin, one of the major lipids in the lipid bilayer. Contrary to previous assumptions that ceramides and other sphingolipids found in cell membrane were purely supporting structural elements, ceramide can participate in a variety of cellular lipid signaling, signaling: examples include regulating cell differentiation, differentiation, cell proliferation, proliferation, and programmed cell death (PCD) of Cell (biology), cells. The word ''ceramide'' comes from the Latin ''cera'' (wax) and ''amide''. Ceramide is a component of vernix caseosa, the waxy or cheese-like white substance found coating the skin of newborn human infants. Pathways for ceramide synthesis There are three major pathways of ceramide ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Glycolipids
Glycolipids () are lipids with a carbohydrate attached by a glycosidic (covalent) bond. Their role is to maintain the stability of the cell membrane and to facilitate cellular recognition, which is crucial to the immune response and in the connections that allow cells to connect to one another to form tissues. Glycolipids are found on the surface of all eukaryotic cell membranes, where they extend from the phospholipid bilayer into the extracellular environment. Structure The essential feature of a glycolipid is the presence of a monosaccharide or oligosaccharide bound to a lipid moiety. The most common lipids in cellular membranes are glycerolipids and sphingolipids, which have glycerol or a sphingosine backbones, respectively. Fatty acids are connected to this backbone, so that the lipid as a whole has a polar head and a non-polar tail. The lipid bilayer of the cell membrane consists of two layers of lipids, with the inner and outer surfaces of the membrane made up of t ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Tay–Sachs Disease
Tay–Sachs disease is an Genetic disorder, inherited fatal lysosomal storage disease that results in the destruction of nerve cells in the brain and spinal cord. The most common form is infantile Tay–Sachs disease, which becomes apparent around the age of three to six months of age, with the infant losing the ability to turn over, sit, or crawl. This is then followed by seizures, hearing loss, and paralysis, inability to move, with death usually occurring by the age of three to five. Less commonly, the disease may occur later in childhood, adolescence, or adulthood (juvenile or late-onset). These forms tend to be less severe, but the juvenile form typically results in death by the age of 15. Tay–Sachs disease is caused by a genetic mutation in the ''HEXA'' gene on chromosome 15, which codes a Protein subunit, subunit of the hexosaminidase enzyme known as hexosaminidase A. It is inherited in an autosomal recessive manner. The mutation disrupts the activity of the enzyme, whi ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gaucher's Disease
Gaucher's disease or Gaucher disease () (GD) is a genetic disorder in which glucocerebroside (a sphingolipid, also known as glucosylceramide) accumulates in cells and certain organs. The disorder is characterized by bruising, fatigue, anemia, low blood platelet count and enlargement of the liver and spleen, and is caused by a hereditary deficiency of the enzyme glucocerebrosidase (also known as glucosylceramidase), which acts on glucocerebroside. When the enzyme is defective, glucocerebroside accumulates, particularly in white blood cells and especially in macrophages ( mononuclear leukocytes, which is often a target for intracellular parasites). Glucocerebroside can collect in the spleen, liver, kidneys, lungs, brain, and bone marrow. Manifestations may include enlarged spleen and liver, liver malfunction, skeletal disorders or bone lesions that may be painful, severe neurological complications, swelling of lymph nodes and (occasionally) adjacent joints, distended abdomen, a b ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Gangliosides
A ganglioside is a molecule composed of a glycosphingolipid (ceramide and oligosaccharide) with one or more sialic acids (e.g. ''N''-acetylneuraminic acid, NANA) linked on the sugar chain. NeuNAc, an acetylated derivative of the carbohydrate sialic acid, makes the head groups of gangliosides anionic at pH 7, which distinguishes them from globosides. The name ''ganglioside'' was first applied by the German scientist Ernst Klenk in 1942 to lipids newly isolated from ganglion cells of the brain. More than 60 gangliosides are known, which differ from each other mainly in the position and number of NANA residues. It is a component of the cell plasma membrane that modulates cell signal transduction events, and appears to concentrate in lipid rafts. Recently, gangliosides have been found to be highly important molecules in immunology. Natural and semisynthetic gangliosides are considered possible therapeutics for neurodegenerative disorders. Location Gangliosides are presen ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Krabbe's Disease
Krabbe disease (KD) (also known as globoid cell leukodystrophy or galactosylceramide lipidosis) is a rare and often fatal lysosomal storage disease that results in progressive damage to the nervous system. KD involves dysfunctional metabolism of sphingolipids and is inherited in an autosomal recessive pattern. The disease is named after the Danish neurologist Knud Krabbe (1885–1961). Signs and symptoms Symptoms in asymptomatic infantile-onset (<12 months after birth) and later-onset Krabbe disease present themselves differently. Of individuals with infantile-onset Krabbe disease, 85–90% display progressive neurologic deterioration in infancy and death before the age of two. Symptoms include , s, limb stiffne ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Sialyl-Lewis-x
Sialyl LewisX (sLeX), also known as cluster of differentiation 15s (CD15s) or stage-specific embryonic antigen 1 (SSEA-1), is a tetrasaccharide carbohydrate which is usually attached to O-glycans on the surface of cells. It is known to play a vital role in cell-to-cell recognition processes. It is also the means by which an egg attracts sperm; first, to stick to it, then bond with it and eventually form a zygote. Sialyl-LewisX is also one of the most important blood group antigens and is displayed on the terminus of glycolipids that are present on the cell surface. The sialyl-LewisX determinant, E-selectin ligand carbohydrate structure, is constitutively expressed on granulocytes and monocytes and mediates inflammatory extravasation of these cells. Resting T and B lymphocytes lack its expression and are induced to strongly express sialyl-LewisX upon activation. The sialyl-LewisX determinant is expressed preferentially on activated Th1 cells but not on Th2 cells. Structure Sial ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
