Accumulation of phosphorylated dolichol in several tissues in ceroid-lipofuscinosis (Batten disease).

The concentrations of phosphorylated dolichol (P-dolichol) and of non-phosphorylated dolichol were estimated in a number of different tissues from ceroid-lipofuscinosis (CL) and control cases. The P-dolichol contents of four tissues, brain, pancreas, muscle and kidney were significantly greater than control values. Liver and spleen showed little or no increase in P-dolichol content. Though non-phosphorylated dolichol levels were higher in CL tissues than in control tissues, the differences were significant only for brain and pancreas. In control muscle, a linear age-related increase in non-phosphorylated dolichol content was observed. The relative amounts of the major homologs of P-dolichol and of non-phosphorylated dolichol were estimated. Each tissue has a characteristic homolog size-distribution, and there is close similarity between the size-distribution profiles of P-dolichol and of non-phosphorylated dolichol for each tissue.