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Dolichol and phosphorylated dolichol content of tissues in ceroid‐lipofuscinosis
Author(s) -
Hall N. A.,
Patrick A. D.
Publication year - 1985
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf01805431
Subject(s) - dolichol , neuronal ceroid lipofuscinosis , phosphorylation , biochemistry , metabolism , chemistry , medicine , endocrinology , biology , biosynthesis , enzyme , gene
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.