Dolichol and phosphorylated dolichol content of tissues in ceroid‐lipofuscinosis
- 20 February 1985
- journal article
- research article
- Published by Wiley in Journal of Inherited Metabolic Disease
- Vol. 8 (4), 178-183
- https://doi.org/10.1007/bf01805431
Abstract
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.Keywords
This publication has 23 references indexed in Scilit:
- Separation of some neutral lipids by normal-phase high-performance liquid chromatography on a cyanopropyl column: Ubiquinone, dolichol, and cholesterol levels in sheep liverAnalytical Biochemistry, 1984
- Dolichol and Dolichyl Phosphate Levels in Brain Tissue from English Setters with Ceroid LipofuscinosisJournal of Neurochemistry, 1984
- High Levels of Brain Dolichols in Neuronal Ceroid‐Lipofuscinosis and SenescenceJournal of Neurochemistry, 1983
- Dolichols in brain and urinary sediment in neuronad ceroid lipofuscinosisNeurology, 1983
- Different tissue concentrations and rates of synthesis of dolichol, dolichyl acyl esters, and dolichyl phosphate in mouse testes and preputial glandsBiochemical and Biophysical Research Communications, 1983
- A simple procedure for preparing dolichyl monophosphate by the use of POCl3FEBS Letters, 1981
- A general, fast, and sensitive micromethod for DNA determination: Application to rat and mouse liver, rat hepatoma, human leukocytes, chicken fibroblasts, and yeast cellsAnalytical Biochemistry, 1981
- Microdetermination of dolichol in tissuesBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1977
- The Role of Polyprenol-Linked Sugars in Glycoprotein SynthesisAnnual Review of Biochemistry, 1976
- The preparation of tritiated betulaprenols and dolicholsAnalytical Biochemistry, 1975