Immunoreactivity of Ceroid Lipofuscin Storage Pigment in Batten Disease with Monoclonal Antibodies to the Amyloid Beta-Protein

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Abstract
To the Editor: Batten disease, or neuronal ceroid lipofuscinosis (NCL), is a term applied to a group of inherited storage disorders whose cause is unknown, occurring mainly in infancy and childhood and characterized by the rapid accumulation of an autofluorescent storage pigment, primarily in neural tissue.1 The storage material consists of peptides, polyisoprenoids (dolichols), lipid derivatives, and metal ions (ferrous, zinc). Similar compounds are found in lipofuscin, accumulating during the normal aging process, and some authors have suggested that NCL might serve as a model for aging.2 An additional feature of the aging process is the presence of amyloid deposits . . .