title: Structure of the glycopeptide storage material in GM 1 gangliosidosis. Sequence determination with specific endo- and exoglycosidases.
abstract: 1. An endo-beta-galactosidase from Escherichia freundii, specific for the hydrolysis of desulfated keratan sulfate, quantitatively liberated a trisaccharide (Gal-GlcNAc-Gal) from a glycopeptide (Mr 1800) isolated from the liver of a patient with GM 1 (generalized) gangliosidosis. 2. The remaining glycopeptide was susceptible to sequential digestion with purified beta-N-acetylhexosaminidase and exo-beta-galactosidase from Jack Bean meal. 3. These and other studies established the structure of the stored glycopeptide to be:(see article) which probably represents the desulfated linkage region of skeletal keratan sulfate.
mesh_terms: D000596:Amino Acids / Q000032:analysis; D055598:Chemical Phenomena; D002621:Chemistry; D004926:Escherichia coli / Q000201:enzymology; D005690:Galactose / Q000032:analysis; D005696:Galactosidases; D005732:Gangliosides / Q000378:metabolism*; D006020:Glycopeptides / Q000032:analysis*; D006595:Hexosamines / Q000032:analysis; D006801:Humans; D008052:Lipid Metabolism, Inborn Errors / Q000378:metabolism*; D008099:Liver / Q000032:analysis*; D010944:Plants / Q000201:enzymology; D014312:Trisaccharides / Q000032:analysis
chemical_list: D000596:Amino Acids; D005732:Gangliosides; D006020:Glycopeptides; D006595:Hexosamines; D014312:Trisaccharides; D005696:Galactosidases; D005690:Galactose