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  • Title: Animal model: ceroidosis (ceroid-lipofuscinosis) in Australian cattle dogs.
    Author: Wood PA, Sisk DB, Styer E, Baker HJ.
    Journal: Am J Med Genet; 1987 Apr; 26(4):891-8. PubMed ID: 3591832.
    Abstract:
    Ceroid-lipofuscinosis is described in Australian 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.
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