Animal model: Ceroidosis (ceroid‐lipofuscinosis) in Australian Cattle dogs

Abstract

Ceroid‐lipofuscinosis is described in Austrialian Cattle dogs. Lesions included storage of ceroid‐lipofuscin in most tissues with characteristic ultrastructural inclusion body patterns in neurons and other cells. Dolichol concentration of the affected dog's brain was similar to those in age‐matched control dog brains. However, concentrations in brain, liver and kidneys were markedly higher than in a slightly younger dog. Biochemical data including lysosomal enzyme activities exclude other lysosomal storage diseases. The clinical and pathological features of this disorder resemble those of the juvenile Spielmeyer‐Vogt type of Batten disease in humans.