Article
Cell-specific deletion of glucosylceramide synthase in brain leads to severe neural defects after birth.
Proceedings of the National Academy of Sciences of the United States of America - 30 Aug 2005
Jennemann Richard, Sandhoff Roger, Wang Shijun, Kiss Eva, Gretz Norbert, Zuliani Cecilia, Martin-Villalba Ana, Jäger Richard, Schorle Hubert, Kenzelmann Marc, Bonrouhi Mahnaz, Wiegandt Herbert, Gröne Hermann-Josef
Abstract excerpt
Sialic acid-containing glycosphingolipids, i.e., gangliosides, constitute a major component of neuronal cells and are thought to be essential for brain function. UDP-glucose:ceramide glucosyltransferase (Ugcg) catalyzes the initial step of glycosphingolipid (GSL) biosynthesis. To gain insight into the role of GSLs in brain development and function, a cell-specific disruption of Ugcg was performed as indicated by...
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