title: Analysis of dolichyl pyrophosphoryl oligosaccharides in purified storage cytosomes from ovine ceroid-lipofuscinosis.
abstract: Ovine ceroid-lipofuscinosis is an inherited neurodegenerative disorder characterised by the accumulation of storage cytosomes in brain and visceral organs. Phosphorylated dolichol-containing compounds, largely in the form of dolichyl pyrophosphoryl oligosaccharides, have been shown to constitute 1-2% of the dry weight of storage cytosomes isolated from brain and pancreas, and 0.5 and 0.1% respectively of storage cytosomes isolated from liver and kidney. The carbohydrate portion of these glyconjugates in storage cytosomes isolated from brain, pancreas and liver consisted of a series of oligosaccharides of composition Man2-9GlcNAc2, with Man5-8GlcNAc2 predominating. The concentrations of dolichyl pyrophosphoryl oligosaccharides in storage cytosomes from ovine ceroid-lipofuscinosis are much higher than has been reported for endoplasmic reticulum, their normal functional location.
authors.affiliation: Department of Clinical Biochemistry, University of London, U.K.
authors.forename: N A
authors.initials: NA
authors.lastname: Hall
authors.forename: R D
authors.initials: RD
authors.lastname: Jolly
authors.forename: D N
authors.initials: DN
authors.lastname: Palmer
authors.forename: B D
authors.initials: BD
authors.lastname: Lake
authors.forename: A D
authors.initials: AD
authors.lastname: Patrick
mesh_terms: D000818:Animals; D001921:Brain / Q000648:ultrastructure; D002851:Chromatography, High Pressure Liquid; D004286:Dolichols / Q000032:analysis; D007668:Kidney / Q000648:ultrastructure; D008099:Liver / Q000648:ultrastructure; D008247:Lysosomes / Q000032:analysis* / Q000648:ultrastructure; D008854:Microscopy, Electron; D009472:Neuronal Ceroid-Lipofuscinoses / Q000378:metabolism / Q000473:pathology / Q000662:veterinary*; D009844:Oligosaccharides / Q000032:analysis; D010179:Pancreas / Q000648:ultrastructure; D010766:Phosphorylation; D011104:Polyisoprenyl Phosphate Oligosaccharides / Q000032:analysis*; D011105:Polyisoprenyl Phosphate Sugars / Q000032:analysis*; D012756:Sheep
chemical_list: D004286:Dolichols; D009844:Oligosaccharides; D011104:Polyisoprenyl Phosphate Oligosaccharides; D011105:Polyisoprenyl Phosphate Sugars; C024962:dolichyl diphosphate oligosaccharides