Dolichyl-pyrophosphoryloligosaccharide protein oligosaccharide transferase in neuronal ceroid-lipofuscinosis.
The kinetics of the oligosaccharide transfer from oligosaccharyl pyrophosphoryldolichol to endogenous protein acceptors in human fibroblasts were studied. No alterations in the transferase activity and enzyme characteristics could be observed in fibroblasts from neuronal ceroid-lipofuscinosis (NCL) patients. Analysis of urinary dolichol of two NCL patients also did not reveal substantial differences with respect to controls.
Van Dessel
G
G
Universitaire Instelling Antwerpen, Belgium.
Lagrou
A
A
Hilderson
H
H
Dierick
W
W
D000293:Adolescent; D000328:Adult; D002413:Cations, Divalent / Q000494:pharmacology; D002478:Cells, Cultured; D004286:Dolichols / Q000652:urine; D005260:Female; D005347:Fibroblasts / Q000201:enzymology; D006602:Hexosyltransferases; D006801:Humans; D008297:Male; D008565:Membrane Proteins; D008875:Middle Aged; D009472:Neuronal Ceroid-Lipofuscinoses / Q000201:enzymology* / Q000652:urine; D011104:Polyisoprenyl Phosphate Oligosaccharides / Q000378:metabolism; D014166:Transferases / Q000378:metabolism*
D002413:Cations, Divalent; D004286:Dolichols; D008565:Membrane Proteins; D011104:Polyisoprenyl Phosphate Oligosaccharides; C024962:dolichyl diphosphate oligosaccharides; D014166:Transferases; D006602:Hexosyltransferases; C025620:dolichyl-diphosphooligosaccharide - protein glycotransferase