title: Metabolic pathways and intracellular trafficking of gangliosides.
abstract: Gangliosides constitute a large and heterogeneous family of acidic glycosphingolipids that contain one or more sialic acid residues and are expressed in nearly all vertebrate cells. Their de novo synthesis starts at the endoplasmic reticulum and is continued by a combination of glycosyltransferase activities at the Golgi complex, followed by vesicular delivery to the plasma membrane. At the cell surface, gangliosides participate in a variety of physiological as well as pathological processes. The cloning of genes for most of the glycosyltransferases responsible for ganglioside biosynthesis has produced a better understanding of the cellular and molecular basis of the ganglioside metabolism. In addition, the ability to delete groups of glycosphingolipid structures in mice has been enormously important in determining their physiological roles. Recently, a number of enzymes for ganglioside anabolism and catabolism have been shown to be associated with the plasma membrane, which might contribute to modulate local glycolipid composition, and consequently, the cell function.
authors.affiliation: Departamento de Química Biológica, Facultad de Ciencias Químicas, Centro de Investigaciones en Química Biológica de Córdoba (CIQUIBIC, UNC-CONICET), Universidad Nacional de Córdoba, Córdoba, Argentina. daniotti@dqb.fcq.unc.edu.ar
authors.forename: Jose Luis
authors.initials: JL
authors.lastname: Daniotti
authors.forename: Ramiro
authors.initials: R
authors.lastname: Iglesias-Bartolomé
mesh_terms: D000818:Animals; D001692:Biological Transport; D002236:Carbohydrate Conformation; D002240:Carbohydrate Sequence; D002462:Cell Membrane / Q000378:metabolism; D004705:Endocytosis; D005089:Exocytosis; D005732:Gangliosides / Q000737:chemistry / Q000378:metabolism*; D006028:Glycosphingolipids / Q000737:chemistry / Q000378:metabolism; D006801:Humans; D053858:Metabolic Networks and Pathways / Q000502:physiology*; D008969:Molecular Sequence Data
chemical_list: D005732:Gangliosides; D006028:Glycosphingolipids