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Psychosine
Psychosine is a highly cytotoxic lipid that accumulates in the nervous system in the absence of galactosylceramidase. Chemically, it is a galactoside of sphingosine Sphingosine (2-amino-4-trans-octadecene-1,3-diol) is an 18-carbon amino alcohol with an unsaturated hydrocarbon chain, which forms a primary part of sphingolipids, a class of cell membrane lipids that include sphingomyelin, an important phospholip .... References {{organic-compound-stub Lipids Galactosides ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Galactosylceramidase
Galactosylceramidase (or galactocerebrosidase) is an enzyme that in humans is encoded by the ''GALC'' gene. Galactosylceramidase is an enzyme which removes galactose from ceramide derivatives (galactosylceramides). Galactosylceramidase is a lysosomal protein which hydrolyzes the galactose ester bonds of galactosylceramide, galactosylsphingosine, lactosylceramide, and monogalactosyldiglyceride. Mutations in this gene have been associated with Krabbe 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 ..., also known as galactosylceramide lipidosis. References Further reading * * * * * * * * * * * * * * * * * * * * External links GeneReviews/NCBI/NIH/UW entry on Krabbe disease OMIM entries on Krabbe disease* PDBe-KBprovides an overview of all the structure information avai ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Cytotoxic
Cytotoxicity is the quality of being toxic to cells. Examples of toxic agents are an immune cell or some types of venom, e.g. from the puff adder (''Bitis arietans'') or brown recluse spider (''Loxosceles reclusa''). Cell physiology Treating cells with the cytotoxic compound can result in a variety of cell fates. The cells may undergo necrosis, in which they lose membrane integrity and die rapidly as a result of cell lysis. The cells can stop actively growing and dividing (a decrease in cell viability), or the cells can activate a genetic program of controlled cell death (apoptosis). Cells undergoing necrosis typically exhibit rapid swelling, lose membrane integrity, shut down metabolism, and release their contents into the environment. Cells that undergo rapid necrosis in vitro do not have sufficient time or energy to activate apoptotic machinery and will not express apoptotic markers. Apoptosis is characterized by well defined cytological and molecular events including a change i ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lipid
Lipids are a broad group of naturally-occurring molecules which includes 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 may be 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 condensati ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
The Journal Of Lipid Research
The ''Journal of Lipid Research'' is a monthly peer-reviewed scientific journal that was established in 1959. Since 2000, it has been published by the American Society for Biochemistry and Molecular Biology. It covers research on lipids in health and disease, including lipid function and the biochemical and genetic regulation of lipid metabolism. The journal also covers patient-oriented and epidemiological research. In its aim and scope, the journal "''aims to be on the forefront of the emerging areas of genomics, proteomics, metabolomics, and lipidomics as they relate to lipid metabolism and function.''" The journal is published in print and online. As of February 1, 2019, its editors-in-chief are Kerry-Anne Rye and Nick Davidson. The journal is open access and all articles are available for free online immediately after acceptance. The journal was established in response to the lack of published methodologies in lipid research as perceived by Edward H. Ahrens, Jr., Donald Zilv ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Galactoside
A galactoside is a glycoside containing galactose. The H of the OH group on carbon-1 of galactose is replaced by an organic moiety. Depending on whether the glycosidic bond lies "above" or "below" the plane of the galactose molecule, galactosides are classified as α-galactosides or β-galactosides. A β-galactoside is a type of galactoside in which the glycosidic bond lies above the plane of the galactose residue. The most commonly recognized and used β-galactoside in biochemistry is lactose Lactose is a disaccharide sugar synthesized by galactose and glucose subunits and has the molecular formula C12H22O11. Lactose makes up around 2–8% of milk (by mass). The name comes from ' (gen. '), the Latin word for milk, plus the suffix '' - .... However, other chemicals, such as ONPG, are known, but these are typically synthesized for biochemical assays. Galactosides play significant roles in metabolic processes of many organisms and are hydrolyzed by a class of enzymes called ga ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Sphingosine
Sphingosine (2-amino-4-trans-octadecene-1,3-diol) is an 18-carbon amino alcohol with an unsaturated hydrocarbon chain, which forms a primary part of sphingolipids, a class of cell membrane lipids that include sphingomyelin, an important phospholipid. Functions Sphingosine can be phosphorylated in vivo via two kinases, sphingosine kinase type 1 and sphingosine kinase type 2. This leads to the formation of sphingosine-1-phosphate, a potent signaling lipid. Sphingolipid metabolites, such as ceramides, sphingosine and sphingosine-1-phosphate, are lipid signaling molecules involved in diverse cellular processes. Biosynthesis Sphingosine is synthesized from palmitoyl CoA and serine in a condensation required to yield dihydrosphingosine. Dehydrosphingosine is then reduced by NADPH to dihydrosphingosine (sphinganine), acylated to dihydroceramide finally oxidized by FAD to ceramide. Sphingosine is then solely formed via degradation of sphingolipid in the lysosome. See also * ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Lipids
Lipids are a broad group of naturally-occurring molecules which includes 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 may be 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 condensati ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
