Analysis of dolichyl pyrophosphoryl oligosaccharides in purified storage cytosomes from ovine ceroid-lipofuscinosis.
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.
Hall
N A
NA
Department of Clinical Biochemistry, University of London, U.K.
Jolly
R D
RD
Palmer
D N
DN
Lake
B D
BD
Patrick
A D
AD
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
D004286:Dolichols; D009844:Oligosaccharides; D011104:Polyisoprenyl Phosphate Oligosaccharides; D011105:Polyisoprenyl Phosphate Sugars; C024962:dolichyl diphosphate oligosaccharides