Dolichol and phosphorylated dolichol content of tissues in ceroid-lipofuscinosis.
Concentrations of free dolichol, total non-phosphorylated dolichol, and total phosphorylated dolichol were measured in autopsy specimens of brain and liver from ceroid-lipofuscinosis (CL) and control cases. Levels of non-phosphorylated dolichol, mainly as free dolichol, were increased approximately two-fold in late-infantile CL brain. No increase was observed for CL liver. In late-infantile and juvenile CL brain, the increase in phosphorylated dolichol was at least ten-fold, but no significant difference was found for CL liver. A substantial part of the increase in phosphorylated dolichol in CL brain appeared to be due to lipid-linked oligosaccharides. The results suggest that CL might involve a defect in the metabolism of dolichol-linked oligosaccharides.
D000293:Adolescent; D000328:Adult; D001923:Brain Chemistry; D002648:Child; D002675:Child, Preschool; D004224:Diterpenes / Q000032:analysis*; D004288:Dolichol Phosphates / Q000032:analysis*; D004286:Dolichols / Q000032:analysis*; D005260:Female; D006801:Humans; D008099:Liver / Q000032:analysis*; D008297:Male; D009472:Neuronal Ceroid-Lipofuscinoses / Q000378:metabolism*; D011106:Polyisoprenyl Phosphates / Q000032:analysis*
D004224:Diterpenes; D004288:Dolichol Phosphates; D004286:Dolichols; D011106:Polyisoprenyl Phosphates