glycosphingolipids


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glycosphingolipids

(glī″kō-sfĭng″ō-lĭp′ĭds)
A group of carbohydrate-containing fatty acid derivatives of ceramide. Three classes of these lipids are cerebrosides, gangliosides, and ceramide oligosaccharides. When the enzymes essential to the metabolism of these compounds are absent, the glycosphingolipids accumulate, particularly in the nervous system. Death is the usual outcome.
References in periodicals archive ?
Their product line includes a broad range of sphingolipids, glycolipids, glycosphingolipids, fatty acids and esters, sterols, and vitamin E isomers (tocopherols and tocotrienols).
These disorders are characterized by a disrupted lysosomal defect of glycosphingolipid, accumulating in the organelle with the respective glycoconjugates.
Kwiterovich, "Glycosphingolipids of human plasma lipoproteins," Lipids, vol.
Lysosomal storage diseases (LSDs) are uncommon metabolic disorders produced by an accumulation of glycoconjugates (glycosaminoglycans, glycoproteins, or glycosphingolipids) within lysosomes, which affects normal tissue architecture in diverse areas of the human body.
In this context, herein we investigated the role of gangliosides, which are sialic acid-containing glycosphingolipids (GSLs) ubiquitously distributed in cell membranes [5], in the osteogenic differentiation of hTSCs.
The psychosine receptor is a receptor for the glycosphingolipid psychosine (PSY) and several related glycosphingolipids.
In CerS2 knockout mice, migration of neutrophils is impaired that is possibly related to reduced production of very long chain glycosphingolipids and a reduced G-CSF expression as well as Lyn signaling in these mice [80].
They belong to the family of glycosphingolipids (GSL) and contain one or more sialic acids, N-acetyl derivatives of neuraminic acid, in their hydrophilic oligosaccharide chain.
Cell surface sialylation of glycoproteins and glycosphingolipids in cultured metastatic variant RNA-virus transformed non-producer BALB/c 3T3 cell lines.
(1990) Total synthesis of the tumor-associated Lex family of glycosphingolipids. J.
The overload of these glycosphingolipids disturbs the morphology of affected cells and leads to cell dysfunction [2-4].
Anderson-Fabry disease is a rare X-linked recessive lysosomal storage disorder, caused by mutations of the GLA gene that encodes [alpha]-galactosidase A which breaks down neutral glycosphingolipids [85].