Abstract
A method for the measurement of autofluorescence emission spectra of intraneuronal lipopigment in tissue sections has been applied to specimens from dogs and sheep with forms of neuronal ceroid-lipofuscinosis (NCL). The characteristics of an emission spectrum probably reflect the composition of the lipopigment, and the results are compared with those previously reported from human NCLs and lipofuscin in non-diseased elderly human brains. Lipopigment in the animal NCLs differed from lipofuscin in the non-diseased human brains, but no differences could be demonstrated between the spectra from animal and human NCLs. These findings support the use of the animal diseases as models for research into the pathogenesis and treatment of human NCLs. Both human and animal NCLs would be more accurately designated as the “ceroidoses”.
Published Version
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