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We report on the biosynthetic principles leading to this large molecular diversity and focus on sialic acid\u2013containing glycolipids of the ganglio\u2013series. These glycolipids are particularly concentrated in the plasma membrane of neuronal cells. Their\n                    <jats:italic>de novo<\/jats:italic>\n                    synthesis starts with the formation of the membrane anchor, ceramide, at the endoplasmic reticulum (ER) and is continued by glycosyltransferases of the Golgi complex. Recent findings from genetically engineered mice are discussed. The constitutive degradation of glycosphingolipids (GSLs) occurs in the acidic compartments, the endosomes and the lysosomes. Here, water\u2013soluble glycosidases sequentially cleave off the terminal carbohydrate residues from glycolipids. For glycolipid substrates with short oligosaccharide chains, the additional presence of membrane\u2013active sphingolipid activator proteins (SAPs) is required. A considerable part of our current knowledge about glycolipid degradation is derived from a class of human diseases, the sphingolipidoses, which are caused by inherited defects within this pathway. A new post\u2013translational modification is the attachment of glycolipids to proteins of the human skin.\n                  <\/jats:p>","DOI":"10.1098\/rstb.2003.1265","type":"journal-article","created":{"date-parts":[[2003,9,9]],"date-time":"2003-09-09T11:27:32Z","timestamp":1063106852000},"page":"847-861","source":"Crossref","is-referenced-by-count":147,"title":["Biosynthesis and degradation of mammalian glycosphingolipids"],"prefix":"10.1098","volume":"358","author":[{"given":"Konrad","family":"Sandhoff","sequence":"first","affiliation":[{"name":"Kekul\u00e9-Institut f\u00fcr Organische Chemie und Biochemie der Universit\u00e4t, Gerhard-Domagk-Strasse 1, 53121 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Kolter","sequence":"additional","affiliation":[{"name":"Kekul\u00e9-Institut f\u00fcr Organische Chemie und Biochemie der Universit\u00e4t, Gerhard-Domagk-Strasse 1, 53121 Bonn, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"175","published-online":{"date-parts":[[2003,5,29]]},"reference":[{"key":"p_1","doi-asserted-by":"publisher","DOI":"10.1093\/glycob\/10.10.1025"},{"key":"p_2","doi-asserted-by":"publisher","DOI":"10.1021\/bi961164o"},{"key":"p_3","unstructured":"Becker E. 1999 Magnetic isolation of Iysosomes from human fibroblasts. 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