Abstract
Ceroid-lipofuscinosis (CL) (Batten’s disease) is an inherited neurodegenerative disorder characterized by the accumulation of tertiary lysosomes containing a storage material. The underlying primary biochemical abnormality has yet to be discovered. However, very marked increases in phosphorylated dolichol (P-dolichol) levels have been found in brain from all four forms of the human disease (Hall and Patrick, 1985; Pullarkat, 1987; Hall and Patrick, 1987; Wolfe et al., 1988), and in a canine form of CL (Keller et al., 1984). We have also found that P-dolichol is highly concentrated in purified storage bodies from an ovine form of CL (unpublished observations).
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