Developmental changes in the expression of neuronal ceroid lipofuscinoses-linked proteins

Neuronal ceroid lipofuscinoses (NCL) form a distinct group of storage disea ses where the normal development of the central nervous system is interrupt ed and neurons of the neocortex begin to degenerate. Mutations in genes enc oding three lysosomal enzymes are the causes for three early-onset forms of NCLs: palmitoyl-protein thioesterase 1 (PPT1) is deficient in human infant ile NCL, tripeptidyl peptidase 1 (TTP1) in late-infantile NCL, and cathepsi n D in congenital ovine NCL. We wanted to compare the developmental express ion profiles of these enzymes in rat brain. In conclusion, the PPT1 express ion pattern differed from the two other lysosomal enzymes implicated in NCL diseases, thus suggesting a distinctive role for PPT1 in brain development . (C) 2000 Academic Press.