title: A fascination with sugars.
abstract: We now recognize that a large number of membrane and soluble proteins contain covalently linked oligosaccharides that exhibit a vast array of structures and participate in a wide variety of biological processes. Nowhere is this better illustrated than the mannose 6-phosphate (Man-6-P) recognition system that mediates the trafficking of newly synthesized acid hydrolases to lysosomes in higher eukaryotes. The Asn-linked high-mannose oligosaccharides of these hydrolases facilitate folding of the nascent proteins in the endoplasmic reticulum via interaction with lectin-type chaperones and after phosphorylation in the Golgi, function as ligands for binding to Man-6-P receptors, a critical step in their transport to lysosomes. Failure to synthesize the Man-6-P recognition marker results in a serious lysosomal storage disease, one of a growing number of genetic conditions, termed congenital disorders of glycosylation, that result from faulty glycan biosynthesis.
authors.affiliation: Washington University in St. Louis, Internal Medicine, Saint Louis, MO 63110, USA. skornfel@im.wustl.edu
authors.forename: Stuart
authors.initials: S
authors.lastname: Kornfeld
mesh_terms: D001325:Autobiographies as Topic; D001363:Awards and Prizes; D003585:Cell Biology; D054794:Glycomics / Q000266:history*; D049673:History, 20th Century; D049674:History, 21st Century; D006867:Hydrolases / Q000378:metabolism; D008247:Lysosomes / Q000378:metabolism; D008360:Mannosephosphates / Q000378:metabolism; D009844:Oligosaccharides / Q000266:history* / Q000378:metabolism; D017527:Receptor, IGF Type 2 / Q000378:metabolism; D012958:Societies, Scientific; D014481:United States
chemical_list: D008360:Mannosephosphates; D009844:Oligosaccharides; D017527:Receptor, IGF Type 2; C027693:mannose-6-phosphate; D006867:Hydrolases